+1226 Seeking Second Opinion

Today I put in a request to get a second opinion from the Fred Hutchison research center in Seattle. It might take a while to get the records transferred, and permissions complete. I have seen other GVHD patients get good care from the Hutch, so I hope they can help David.

David is doing pretty good. His skin is stable so that's good news. We will not be able to do a taper in a while. I still cannot get his dry flaky hands under control. I'm waiting for the dermatologist at the NIH to respond to the photos I emailed him.

Our family is fighting a cough. Ariella is wearing a mask today. We have all the air filters on full blast. We are doing our best to prevent David from catching it. We had our flu and H1N1 vaccinations so I'm not sure what happened.

+1198 Vision and Learning

We are now entering a realm of David's care that I was hoping never to enter. It's becoming clear that the doctors at Stanford are only interested in medications and operations. They have very, very little interest in the whole child. Honestly, this was clear from David's first diagnosis when they wanted to rush into a bone marrow transplant without first understanding his symptoms. I just wish they took more of a wholistic approach.

Let me give an example. After David's doctors learned he had cataracts, they did nothing except shake their head in acknowledgement. They gave no advice to me, like no nutrition advice and no sunglasses advice. Do they not understand that the eyes need vitamin C and antioxidants?

So, by God's grace last night we had a vision therapist come to my homeschool group meeting. He spoke about vision and learning. I asked him about David's cataracts and previous brain hemorrhage. God opened a new door for me. David will eventually acquire blurry vision. This will effect his reading and learning. Also, he may have vision problems due to the brain hemorrhage. Things David can do so well today, will slowly deteriorate. The reading skills he acquires this year in Kindergarten will get harder, not easier like for most children. There is therapy that will help him to compensate for the everyday activities that will be more difficult to do. He could compensate on his own, like many people do when they have a disability, but that will lead to bad habits. It's best to develop good compensation skills.

This leads me on a new journey. My prayer is that God will hold our hands, continue to open doors, and strengthen David in every possible way. Thank you for your prayers.

-Aimee and especially David

+1198 Skin, Stools, Liver

Found an article for helping people with Stage 4 skin GVHD

http://www.o-wm.com/article/3479

The traditionally accepted clinical stages for skin findings are as follows:
Stage 1 — maculopapular rash <25%>

Stage 2 — maculopapular rash 25% to 50% of body surface

Stage 3 — rash on >50% of skin (per revised grading system)
Stage 4 — bullous formation
The “rule of nines” for burn patients is used to determine the extent of the rash.

David has Stage 3. His rash covers more than 50% of his body.

His hands are still not looking very good. I guess it's the GVHD. I'm going to get counsel from the GVHD doctor at NIH.

His poops have been a little too soft. He's also had a little rectal bleeding. I've been studying about stools.

His upper, right side occasionally hurts (that's where his liver resides). Does not surprise me. The medications are so toxic to his liver.

+1192 - Not sure steroid taper is working

I'm not feeling confident about the steroid taper. I see him itching again. He is bloated. His skin seems puffy. The rash is more visible. This is really hard. I like that book title Do Hard Things. This is very hard.

I had to buzz his hair. His scalp is so dry that I cannot put ointment on when he has a full head of hair. I'm thankful he still has hair. He was not happy about getting a buzz. The girls were so helpful by saying "Look, David, your hair is growing back so quickly!" He wholeheartedly agreed and told me several times tonight that his hair is already growing back. I just gave him a big smile and told him how happy I was about it. Now we'll see if the ointments will work on his dry scalp.

Some more news...he started his first gymnastics class. I am convinced that some good exercise will help strengthen his bones, and help lower his blood pressure. He enjoyed it. He was timid at first but it was not long before he warmed up to the teacher. The teacher is so gracious to take him as a student. It's a small gym. Only one other little girl was in the class. I'm so proud of him for sticking with it! I had to drag him. He has lost much of his enthusiasm for social interaction unless he knows people very well. I don't want to push him because it feels like I'm making him suffer more. He and I are coming out of our comfort zones. I will encourage him while he will do his best at trying to have fun. Praise God he is such a good little boy!

I haven't updated his meds in a while:

Prednisolone - 1.7 mls/twice/day

Prograf - 3.2 ml/twice/day

Isradipine - 2 ml/twice/day

Rapamune (Sirolimus) - .5 ml/once/day

Catapress patch - 1/2 patch/once/week

Septra - 4 ml/twice/day

Hydrocortisone 2.5% ointment - all over body/once/day

Protopic ointment on hands, face, and tough spots - once/day

Aquaphor ointment all over - once/day

IVIG and premeds - once/month

+1176 Steroid Taper, No More Zenopax

The McNally family had a nice time in Branson, Missouri for the Feast this year. We feel blessed to have the opportunity to still travel.

I took David to Lucille Packard on Thursday for his monthly infusions. They decided to discontinue one of his IV medications (Zenopax) because his skin is looking pretty good and has not gotten worse; I was so thankful! Out of faith, I asked the doctor if we could start a steroid taper and he said yes! This is a huge step forward. We have not tapered his steroids in two years. We ask that you please pray for his skin, that he not have a flare-up. I should know in about one week if the taper is successful.

You may have heard that Stanford Hospital will not allow any visitors or guests who are under the age of 16, not even nursing infants. They want to prevent the spread of H1N1. This could potentially put our homeschooling, breastfeeding family in a predicament. As of now, Michael can take one day off work to take David to his monthly appointment. If there are more visits required or if Michael gets sick, I will need someone to watch my girls and the baby. Even if David needs a small blood draw, I cannot take any of my children with me. Please pray that we will get help when needed. Also, please pray Jonathan starts taking a bottle. We are having a real hard time getting him to take it.

With deep thankfulness,

Aimee and Michael McNally
along with Adira, Ariella, Jonathan, and especially David

+1146 Skin so-so

Not much to report. His labs looked fine. His skin is doing ok but did not impress the doctors, so Dr. Argawal did not taper the steroids. She wants to see his hands and face look better. His face is definitely looking better as long as I keep applying the Aquaphor and Prograf.

Highlights in David's Life

• David got such a tickle from Jonathan joining him in the hospital bed!
• David built that star wars x-wing all by himself!
• We went to the Santa Cruz County fair. He had an OK time.
• We went lavender picking at Carol's but he refused to put on sunscreen so he had to stay in the shade.
• He built a space ship out of cardboard for himself and Jonathan.
• He made a "leaf man" from pine cones, etc.
• He enjoyed helping Jonathan play with his colorful blocks.
• He had a wonderful time swimming at Talitha's heated pool in the evening while there was no sun! This was a first!
• He enjoyed the Seymour Marine Lab field trip.

+1130 Cataracts and Skin Looking Good

David saw the eye doctor today. He must like the eye doctor because he was so cooperative! It was amazing. They do at least 10 different tests and he did great! The bad news is that the doctor found, in each eye, 1mm cataracts. It's not in his line of vision so that's good. They will not do anything about it, except see him in 6 months. The cataracts are from chronic steroid use, not GVHD.

His skin is looking pretty good actually! He is still on Sirolimus. If his skin is looking good for another 2 weeks then Dr. Agarwal will taper the steroids ever so slowly.

Thanks for your concern and constant prayers.

Aimee and Michael

3 years!

How could I have forgotten? I forgot to mention that it's been three years since David's transplant.

Also, my friend Sumathi created this website for WAS patients: http://sites.google.com/site/athreyi/ Thanks Sumathi and Ros.

Update

Actually, David's skin is looking better. His legs and torso have definitely improved. His forearms and face are still really red, inflamed, and hot to the touch. Please let me know if you know why his forearms are so hot and red, whereas, the rest of his body is a normal color and temperature. The doctors say it's the GVHD but I wonder if the high blood pressure has anything to do with it.

This scripture is so true when it comes to GVHD!

"Better is the end of a thing than its beginning, and the patient in spirit is better than the proud in spirit." (Ecclesiastes 7:8, ESV)

Thanks for your prayers!

+1084 Sirolomis Not Working

David had IVIG and Zenopax yesterday. His labs all look great. His rash is not improving though on the new medication called Sirolomis (Rapamune). His blood pressure is still very high because of the steroids. He's been feeling pretty good though. He is enjoying his new room in our new home. We just moved into a large home in a very peaceful area of the Santa Cruz mountains. He enjoyed his vacation at grandma's house this month too. I'll post some pictures soon.

We are going to use the Sirolomis for another 4 weeks. If there is no improvement, we will discontinue it and try something else.

Thank you for your prayers and emails. It's so nice to know there are so many people concerned and praying for David and our family.

+1049 Be a Bone Marrow Donor - FREE

Take the first step to become a bone marrow donor - join the registry.
It's FREE June 8 - 22.
You could be the one to save a life.
Click the following link to get a donor kit sent to your homeor to find a donor site near you.
http://www.marrow.org/JOIN/Join_Now_Special/Marrowthon09/join_now_mt09.html
Thank you on behalf of all the people who need a bone marrow transplant to help them fight for their lives.

*******************
David's Prograf level was 6.5, the Sirolimus level was 2.5. He is still on .4ml of Sirolimus and 3.4ml of Prograf.

+1044 Up on the Sirolimus

We had to go up on the Sirolimus. It was .2ml/once day. Now he's taking .4ml/once day. Not seeing any results yet.

One of the hardest things in life is to see a loved one's health deteriorate, especially for the caretaker! As his skin thickens and gets puffy, it's pretty hard to deal with emotionally. The thickening is from the GVHD, the puffiness is from his organs not working effectively to get rid of the toxins. His body does not feel like the same lovely body I used to cuddle with. My heart breaks pretty deeply.

We go onward, even though we take a few steps backwards because every step ahead requires more strength, so we grow stronger in the process too.

Thank you for reading this...

+1038 Sirolimus

Photos of Henry Cowell. David was only briefly in the sun.

David started a new medication today called Sirolimus. Same idea as before: we hope it will help his skin GVHD so we can taper the steroids.

+1025 Skin Looking Good

The skin on his hands and feet has pretty much peeled off. Boy was that alarming and pretty painful for him. He has never had hand or feet involvement. When he could not close his hands I got pretty upset because he uses his hands for building many Lego creations. Then when he told me his feet hurt to run, my heart sank because he loves running. Well, he is pretty much back to normal now. He's building, he's drawing, he's running.

Here is a sneak peak at our newest addition Jonathan Judah McNally, born May 16.

Last Day of French Class!

9 days past the baby's due date. David has his hands wrapped. He really wanted to go swimming. He and I relaxed in the shade while the girls swam. Then he took a snooze in the hammock.

+1015 - A Positive Blog Post (kind of)

David came home on Monday. He's been doing well. He is very happy to be home.

Please pray for his skin condition. He is loosing the top layer of skin on his hands and feet. His skin is also itchy all over his body. He likes to keep his hands and feet wrapped because the peeling hurts.

We were told the skin peeling might be the after-effects of a staph infection, but that was never officially determined. We were also told it could be the GVHD. He has never had hand or feet involvement, so we just ask you to please pray that this is not the beginning of GVHD because he could very well loose the use of his hands.

Thank you for hanging in there with us.

+1014 - A Negative Blog Post

I was told recently by someone who said she does not like reading my blog because it is so negative. I really don't have time to address this, but here are my thoughts:



1. You are right. I should be rejoicing in tribulation and giving thanks in all things. I'm sorry I am not perfect and I'm sorry my son is not perfect. I am also sorry God has not healed him.

2. Try having a chronically ill child.

3. At some point in this journey it became apparent that David's skin was not improving thus my emotions about his health have changed. Again, I'm sorry to so deeply disappoint you.

4. I wonder how taking trips to beautiful Yosemite, to the beach, to Florida, etc. are considered negative. If you only knew what a blessing these trips have been to our family.

5. Welcome to the world of sickness which will not change until God's kingdom is established on this earth. He will, in His time, take away every sorrow, every sickness, every disease.

6. For those who have similar thoughts, please read this blog as a way to pray for David and his needs.



Thank you to the moms and friends, especially those who know exactly what we are facing, for your encouragement, for your support, for your love (even though I have never met you face to face), and for your courage to keep reading David's blog even though I don't constantly sing sunshine.

+1009 Sepsis and Other

David was admitted to PICU on April 27 for an unknown infection that Dr. Weinberg thinks was sepsis (blood infection). His WBC was high, and his kidneys were not functioning well. He also had very low blood pressure. He was in PICU for 3 days then transferred to the BMT unit. He will be inpatient until May 11. He recovered quickly with fluids and antibiotics and is doing fine. The blood, stool, and urine cultures all came back negative. We are not sure what got to him, but my suspicion is from the Etanercept shots into the skin. Also, he received IVIG 4 days before getting ill. The doctors don't think any procedures David received would have been the culprit (of course not).

Dr. Weinberg wants us to start photopheresis. Maybe I'll talk about that more. In the meantime, I'm not happy about that.

Please pray for a better alternative.

+991 Things not going so well with Etanercept

The Etanercept (Enbrel) does not seem to be working for his skin. He's had 8 injections thus far (or 4 weeks worth) and I keep using the hydrocortisone creme. They would like to keep him on Etanercept injections for a total of 8 weeks.

He vomitted once last night but seems to be feeling ok this morning. He had a 99.2 temp and his blood pressure was ok (114/80 - which is low for him). He was looking very red faced and was slurring his words.

The whole family has had a very busy and stressful couple of weeks which contributes to our overall health and well-being. We will make an effort to slow down this week.

Thank you for checking in with us and for your continued prayers.

+967 - Bass Center Today for IVIG and Zenopax

WBC - 9.9
Hemoglobin - 14.3
Platelets - 305k
Weight - 39lbs
Height - He's only grown about 1cm in the past year

We are in the BASS Center (formally called the Day Hospital) which is a brand new wing on the first floor with fancy, clean, large rooms. He is here for IVIG, Zenopax, and blood counts/chemistries. His chemistries look pretty good.

David's been feeling well.

They had to poke him 4 times to get an IV. We had Judith the nurse today. We have had her before and...well...let me just say that I would prefer another nurse. He layed almost motionless, he is numb to almost everything. However, Dr. Amylon asked to look in his mouth. No way! I think he wants to have some control over something and he purses his lips so hard there is no way in except with a tongue depressor. Dr. Amylon was so patient and kind but had to see inside his mouth. His mouth looks good. I brush his teeth every day so I can see.

+965 - #2 Etanercept

He received his #2 Etanercept shot yesterday in the other leg. He said it hurt a little.

I looked at his neck last night and, if I'm not mistaken, I could actually see white skin! His skin has been so blotched and red in color from the GVHD that I have not seen real skin color in a long time! A sign of hope, hope, hope, hope!

Aimee

+962 First Etanercept and Trip to NIH

A lot has happened since I last wrote an update.

1. On Thursday, March 19, David had his first shot of Etanercept (a TNF blocker). We hope this helps control the skin GVHD so we can begin tapering the steroids. The great news is that today is Saturday, March 21, and it appears to be working already! He'll have two shots/week. The next one is on Monday. He did well with the shot even though he thought is was going to hurt. I used the EMLA cream.
2. The whole family took a trip to Maryland to visit the team of doctors at the NIH. The nutritionist said we need to cut back on his dairy because the has high cholesterol (due to the medications). He donated a lot of blood for research. We spoke with the WAS team too. It was nice to see them. We saw the dermatologist and decided to do a skin biopsy next year, if David still has skin GVHD.
3. While we at the NIH we met a wonderful mom and her WAS son who flew in from Australia. The NIH is willing to see anyone from other countries. This mom's son was just diagnosed, he is 14 years old!
4. I am amazed at another WAS boy, Declan, who was only at the hospital for a short time after transplant and already went home! We were all on the edge of our seats as he battled breathing issues and was in ICU on a ventilator. He is doing really well and is engrafted!
5. The next boy we are praying for is Ayden. He is undergoing preconditioning right now.

+949 That was Short-lived

That was the shortest episode ever! He ate and drank last night after taking a long rest. He also took his medications. He slept through the night. This morning Ariella read hampster books to him until I got out of bed. I took him for a walk in the double stroller with Ariella in the second seat reading the little new testament bible David loves to cart around. It's another gorgeous day!

+948 Suddenly ill again

We had a good time at church, then drove to the beach for some fun. It was almost time to leave the beach then David threw up. We got home ok, gave him a bath, and put him to bed. It's coming out both ends. He is talking, wants to eat, and drink. I thought we just went through this about 1 month ago. I could tell he was not feeling well for the past week. I had a strong sense he was going to throw up. He is resting. I will probably be up most of the night tending to his needs unless he recovers quickly.

I cried a lot today (many of us moms with ill children cry a lot), even before this all happened. I just want my boy back. He does not look like the David we used to know. He is obviously not growing and it makes me sad to see him so small.

The beach was beautiful today. I enjoyed watching the girls play in the water (freezing cold) and dig in the sand. The sailboats were going in and out of the harbor. We had fun flying our kites. The Ely Family joined us. I had fun visiting with them and watch their son act silly.

+942 Pictures!

+939 Getting Closer to Etanercept

We were in the new Day Hospital today. Stanford added a brand new wing and it's beautiful and clean. David had IVIG and Zenopax. I did not get a copy of the labs.

Dr. Weinberg saw how the Protopic ointment is working. I told him it's hard to apply. They only give me a small tube at a time and it does not go on the skin smoothly (I have to wear gloves so some of it sticks to the gloves). We pay $35 per 2 ounces tube! It only lasts about 1 week. Protopic is really good for specific spots, not for the entire body, which is how we use it for David. So, it's not working well.

I announced that we would like to start the Etanercept which is a subcutaneous shot in the thigh twice/week. After they train me, I can do the shot at home which will save me a lot of time. If there are improvements, we will taper the steroids to every other day. They will not taper any other meds at this time. The Etanercept is a strong immunosuppressor. It has a risk of making a person susceptible to blood bacterial infections (sounds like a WAS boy problem without the spleen). This sounds bad, but if you go back and read what our alternatives were, Etanercept looks to be the best option for us at this time. So, starting on March 19, he will get his first shot. That's one more med to the following list:

hydrocortisone (steroid) ointment - 1/day
Protopic ointment - 1/day
Prograf (immunosupp.) - 2/day 5.2 mls
Prednisolone (immunosupp) - 2/day 2mls
Isradapine (blood pressure) - 2/day 2.5mls
Catapress Patch (blood pressure) - 1/week 1/2 patch
Septra (antibiotic) - 2/day 4mls
Prevacid Meltaway (antacid) - 1/day
Zenopax Infusion (immunosupp) - 2/month
IVIG Infusion (immune boost) - 1/month
Etanercept (immuneosupp) - 2/week/subcutaneous

He was amazing today. He gets 4 premeds before the IVIG and it does not knock the boy out. He just loves life too much to let a minute slip by! He also loves his sisters. They give him so much love an attention.

Today was Ariella's 8th birthday! We spent most of it at the Day Hospital but went home for a home made dinner and cupcakes. It was a good day!

+934 - Article on Pancreatitis due to Medications

An article from the Bone Marrow Transplantation Journal called "Acute pancreatitis due to tacrolimus in a case of allogeneic bone marrow transplantation." This is a serious problem that has a high mortality rate. David is on Tacrolimus, has been for 936 days. He does not have this problem, but his great-grandmother died of pancreatic problems, so I hope there is no genetic disposition towards this problem. It's an interesting article that all GVHD patients should be aware of.

+926 - Met more WAS Moms

David helped me make 42 bean (rice) bags today for Sports Day. He had so much fun filling the bags!

My dear friend and fellow WAS mom, Rosalind and her horse Boo and dog Ruffles.

My girls LOVED riding Boo!




I loved Ruffles but he is camera shy.

David loved Rosalind's white horse.

It took David a while to warm up.

He was a good helper too!

David came up with this idea to make a pinata for his papa. Inside was a hand-made book mark. As you can tell on David's face, the children loved doing this for Michael!

Michael is playing the game!

Through Facebook, I met two more families whose sons have WAS.

I was finally able to get the Protopic on Monday. It appears though that these topical ointments are just not working well. I just cannot seem to get a handle on the skin rash. We'll try the ointments for another three weeks and see what happens.

+918 - Protopic issues

We are out of Protopic ointment for David's skin. Protopic is Prograf in ointment form. I have called the doctor since last Friday to see if the new prescription is ready. No one is returning my calls. The hydrocortisone (a steroid) is making him puff up. Dr. Weinburg says his body must be absorbing the hydrocortisone so it's just like upping the oral dose of steroids.

If the ointments do not work in 2-4 weeks, Dr. Weinberg does not want to get rid of any medications, instead, he wants to add a stronger one, then start to taper the steroids. The current dose of steroids are not good for David so he feels that is the first thing he wants to taper. This was in conflict with what Dr. Argawal said b/c she said we could add another medication (the Etanercept injections) and taper the Zenopax. Dr. Weinberg says GVHD is systemic, so David has it throughout his system, it's only the skin GVHD that is the only symptom. So, treating the skin is not going to help, they have to treat it systemically by using oral, injections, or infusions so these drugs will target the cells in his system, not just the skin.

Current meds:

hydrocortisone (steroid) ointment - 1/day
Protopic ointment - 1/day
Prograf (immunosupp.) - 2/day 5.2 mls
Prednisolone (immunosupp) - 2/day 2mls
Isradapine (blood pressure) - 2/day 2.5mls
Catapress Patch (blood pressure) - 1/week 1/2 patch
Septra (antibiotic) - 2/day 4mls
Prevacid Meltaway (antacid) - 1/day
Zenopax Infusion (immunosupp) - 2/month
IVIG Infusion (immune boost) - 1/month

We would appreciate your prayers. Medical decisions are so challenging. Please pray we make the right choices and trust God to heal our son.

Thank you,

Aimee

+916 - Watch out for Dog Licks

No wonder God gave me a very strong dislike for dogs licking me! I read this article to my children. We were all shocked that a dog lick could be so dangerous, but if you think about what the dogs are licking - totally grosses me out!

http://www.news.com.au/heraldsun/story/0,21985,24075396-661,00.html

We hope to get a dog one day. Can someone engineer a dog without a tounge please?

And to think the pet therapy dog who came to visit David last week, licked my daughters hand. I told my daughter not to let the dog do that, and the owner said, "Why?" I just told her I do not like dogs licking my children...is this too much to ask?

Dog owners...please take inventory about what your dogs are licking and tell me if you would like to loose your face?

+907

Can you believe that we are almost on day +1000! Pretty amazing! I was hoping we would never be here...but here we are ;-) I told one family that the doctors should update the WAS survival statistics because WAS boys are living many more years than what the statistics say...even boys who do not undergo treatment (spleenectomy nor BMT). Also, boys are living longer with post BMT complications because of medical advancements (I'm not sure this is a blessing or not, so I'll let you decide).

In cyberspace, I have met some of the most beautiful people and parents who are dealing with WAS.

Recently, I had the pleasure of meeting:

Declan's mom, Lanta - her son is starting preconditioning on Feb 2. http://web.me.com/rockmaplemama/Rock_Maple_Farm/Declans_Journey/Declans_Journey.html

Viggo - The Larsson family from Sweden - http://allez.blogg.se/ You can use Google Translate to translate their Swedish language into English. This 1-1/2 yr old boy is having preconditioning right now and his transplant on Wednesday.

I also want to thank Sumathi who started the new WAS Forum on the Primmary Immuno Deficiency Forum: http://www.primaryimmune.org/forum/start.htm Sumathi's son is 10 years old and has not had a spleenectomy or BMT. WAS parents, please introduce yourself.

I have been privileged to meet many families around the USA and in other parts of the world! Thank you all for creating an encouraging community!!!!

May you all have peace and joy while dealing with a very complicated disorder.

-Aimee

Yeah! He's back on his bike

He rode his bike today and ran around outside with his sisters today. It was good to see him back.

I listened to one of John O'Leary's motivational talks tonight. Because of listening to him, I have to praise my two daughters for the way they have learned to love David. The bond they share (between each other and with David) would not be as close were it not for David's health problems. They have become more sensitive and aware of people with health problems. They have seen a lot of sick children at Stanford. I know they have a deeper appreciation and understanding for people with disabilities. Aside from my daughters, Michael and I have become better parents because of David. We have learned to love our children with a deeper love and understanding that not every parent experiences. There are always positive ways to look at circumstances. I don't want to create my own muddy pit, decorate it, feel comfortable, and pull others in with me. I want to rise above it and stand on solid ground. There are a lot of good outcomes to our situation, depending on how you look at it.

Thank you, Rosalind for your encouragement. I will "breath in" every bit of sweetness this trial has to offer.

+881 Feeling Much Better

David is feeling much better. He was down in bed for two days. His legs were so weak he could not walk without me holding his hand. We thought he was recovering, then he ate and threw everything up again (I felt like we took a step back). I reported his condition to the doctor and he thought since David was eating and drinking, that it was not urgent. I was fine with that. He recovered but now has his sister's cold. He is still not feeling 100%. He does not want to play at the park, ride his bike, or play with friends. However, he had fun at the neighbor's house this week. He is also active with his sisters while they are in the house. I'm not sure what made him ill in the first place. It's really a mystery.

He went for Zenopax on Thursday.

The Protopic was approved by our insurance, but it is a less concentrated dose (.03% instead of the .1%). A 2ounce tube cost me $35. David has skin GVHD all over his body so the tube will not last long. Karen told me to apply a thin layer. I'm supposed to apply the hydrocortisone 1/day and the Protopic 1/day - at different times.

It's difficult for my heart to know how to feel. At times I feel David is not living a full life, is experiencing damage to his major organs from all the medications, and for the most part not happy. My heart aches because he is becoming more sensitive to his height (or lack thereof), and the many comments made by strangers every day (not just on his height, but his red face or him looking tired). I feel that if I am not hopeful of his recovery, that he will sense my feelings, and give up. But then I think, what could possibly be good about living a life in his bad state of health? He is in a bad state of health...there is no denying that! The doctors can try all they can to make it appear that he looks good from the outside by giving him more than ten medications, but the reality, the truth, is he is not doing fine and the medications are killing him! I really don't know how to feel. I know the reality of living with chronic skin GVHD. However, I receive mixed messages from the doctors that the skin GVHD could "burn itself out." Again, I do not know how to feel. We continue to pray and look forward to the time when there will be no more sickness, or death, or suffering.

We try to live a normal life. Here are some pictures of our snow trip to Yosemite, Angles Camp, and Calaveras Big Trees - December/January 2009.

+873 Not feeling well

I'm not sure what is wrong. He's been laughing and happy all day. Even someone at church commented how happy he looked today. When we got home he got sick. His back is also hurting (low back, right side) so maybe his kidney is having trouble. He is going to the bathroom fine. He is wiped out so I put a diaper on him. It's been about 2-3 months since he last got sick. He was not potty trained then. He is 100% trained now but when he gets sick it's so hard for him to muster up the energy to get out of bed. Michael is buying some cranberry juice. I'll write more later.

+867 Trip to Yosemite, Angles Camp, and Big Trees

David's BP today was 110/91. So, the patch must be working. However, he found the patch on the kitchen floor...hmmm...he said it must have fallen off when he got dressed. I applied a new one.

We had an awesome trip to Yosemite! There was snow everywhere. The trees and branches were covered in beautiful white snow. The river rocks looked like puffy marshmallows. The children found a hill for the snow saucer. David went down the hill so fast we could not stop him from bumping his head on a tree. It was not too hard and he got up and went down the hill again, this time on his sister's lap. I can imagine if he still had WAS, that the bruise would look like a huge knot. We are thankful he healed quickly and got right back on his feet.

Then we drove to Angles Camp, famous for the frog jumping festival. We stayed at the Angles Camp RV and Camping Resort, which I highly recommend, especially on the off-season. The campsite had a covered picnic table, a flat surface for our tent, an outdoor light, and an electrical outlet for our heater! The resort offered warm bathrooms, warm clean free showers, a microwave, games for the children, a playground, and was peaceful. We had rain for one day, but our tent stayed dry and warm! We drove to town and found a letterbox too. The children had so much fun.

The following day we drove up to Big Trees. We went to the Visitor's Center which told of the history of the great sequoia trees. The children quickly found a hill for the snow saucer. Then we signed up for a snow shoe walk (1-1/2 miles) on the North Grove loop trail. It was so much fun and invigorating.

It's so important to take retreats like this. We bonded and had fun together. It also helped me get my mind off some heavy issues happening in my life. I'm so thankful we have enough flexibility to get away.

I'll post pictures in about 1 week!

+863 High Blood Pressure Patch (Clonidine) and Protopic

We are at the Day Hospital today for regular infusions (IVIG and Zenopax). Dr. Argawal looked at his skin and says he has rash all over. To me, his skin looks much better and you cannot feel the rash all over, only in some patches.

She said she will prescribe the Protopic ointment,which is a topical ointment. (update: my insurance will not cover it, so we have to ask for approval which will take about 1 week) We will be back in 2 weeks and see how his skin is doing. If there is no improvement, we will need to talk about another option. I was surprised that he only has two weeks to resolve the rash.

His blood pressure with the small cuff was 153/98. With the medium size cuff it was 125/85. She is concerned about his high blood pressure (which is caused from the Prograff and Steroids) so she is prescribing a patch called Clonidine (catapres). The patch is put on the arm and good fo 7 days. The medication is absorbed into the skin. He is starting with a 1/2 of a patch. Since it was so high today at the Day Hospital, they are giving him an extra dose of Nifedipine (Procardia) (it was just 132/98) and will probably not let us go home until it goes down.

Clonidine works directly on the brain. It pretends to be an adrenaline-like chemical causing reduced levels of true adrenaline-like chemicals (noradrenaline, and serotonin). This is a simple explanation - its true effects are more complex.

So, this is what went into his body between 8am-12noon today:

Isradipine (blood pressure)
Prednisolone (steroid for skin GVHD)
Prevacid (antacid)
Benydryl (premed)
Hydrocortisone (premed)
Ranitidine (premed)
IVIG - Gamunex (boost his immune system)
Zenopax (suppress his immune system)
Clonidine patch (blood pressure)
Nifedipine (blood pressure)
There are two more oral meds but I just do not have the heart to give them. Some social worker will probably say I'm medically neglecting him and send me to jail.

He's doing great considering all that medication. He is feeling tired but he never goes to sleep at the hospital. I know he will take a nap on the way home. Right now he is sitting in bed drawing circles all over his note pad while watching Little Bear on tv.

+856 Probiotics and BMT

Article: Probiotic effects on experimental graft-versus-host disease: let them eat yogurt

David has had yogurt before, during and after BMT. We have had no problems. He is currently taking Culterelle probiotics too.

+853 No Decision Yet...Toilet Trained

Good news! David is toilet trained. He also went to night-time training pretty quickly. Whew!

Just wanted to say that we have not made any decisions about what treatments to try next. I would rather just hide under a rock. In a sense, I am hiding under a rock - that rock is my Lord. He will guide and direct the decision in His timing.

+849 Skin still flarring up

David's skin is still irritated. I am having to use the hydrocortisone creme about twice/day. I don't always get around to putting it on him twice/day. He is becoming more independent. He gets up in the morning and dresses himself before I have a chance to apply the freezing cold creme and emu oil.

We have the consultation on Thursday (tomorrow).

+839 Getting Good Feedback

I just wanted to say that I'm thankful for the individuals who have given me a piece of advice for the difficult decision we have to make about David's treatment changes. You all have responded so quickly and kindly. Thank you so much!

God's word has spoken clearly to me: Do not fear. "Where, O death, is your victory? Where, O death, is your sting?" I Cor 15:55. Whatever happens to David, he is in God's hands. God created him and God owns his life. All I can do is pray, ask, seek, and knock for David. Everything that happens to David is for a reason. God has a purpose for him and for us (his parents). I will not fear. This does not mean I will let any doctor just do what he pleases to "heal" David, or to prevent the disease from progressing. I'm very protective over him and will do my best to make the best decision possible for his medical care.

Some Adice From a Mom Whose Son is Battling Extensive GVHD

A. PUVA - was not helpful for us as the joints had already begun to restrict. Have David's joints been measured by PT to get a baseline? This can be an extremely helpful tool from which possible future joint involvement from gvhd can be identified. PUVA was emotionally hard. Standing naked with a homemade thong covering his private area, totally lubricated with mineral oil, eyes covered with cotton ball stuffed goggles,standing spread eagle in a "tanning booth" lead to sobbing every time. Regardless, we tried it because it would be worth it if it worked. Unfortunately it was too little too late for the gvhd had already begun to go into deeper soft tissue. PUVA doesn't go deep into skin tissue.

B. ECP - Due to David's small size he would probably have to do a style of treatment similar to ours, lots of blood products over time, massive lifestyle change and commitment,big infection risk from pheresis catheter, least toxic to organs after PUVA but this is a LIFESTYLE treatment. I would almost think of it like kidney dialysis in time commitment. Most likely would help but very open ended.

C. Sirolimus - Just tried it, noticed improvements in the skin. Unfortunately he had an adverse reaction (rare though) so he will not continue. Our dermatology doctor said Sirolimus, when tolerated well (as is usual) is much better for skin gvhd because of the T-cell impact from Sirolimus that Cyclosporin and Tacro do not have. Sirolimus must be monitored so your doctors should have some history with using this med (as with all meds) or they should be in close contact with a center and doctors who are comfortable with its use and side effects.

D. Etanercept - we are currently trying this once per week injection now. Relatively few side-effects, should be effective for skin and perhaps lung issues. Seems worth trying for the very little effort and side effects associated. I would consider this as the first option before the other choices presented.

E. I have no first knowledge about Thalidomide - sorry

Prochymal - ASK FOR THIS to be tried. Ask your doctors to contact Osiris and get a compassionate use approval if for some reason David doesn't fit the criteria. I think all our children should fit into this category but sadly our son is still not cleared for its use..... we are hanging on though. One 7yr old patient of our doctor has used prochymal. It helped initially but was not effective in stopping her gvhd. Definitely worth trying if you can light a fire under your doctors to push for its use.

+835 Our Multiple Choice Treatment Test

Karen gave me the list of recommended treatments.

This is a multiple choice test. There is only one right answer. Choose wisely.

A. PUVA - Photochemotherapy is a combination of psoralen (p) and ultraviolet A (UVA)

B. ECP - Extracorporeal photochemotherapy (be careful, this is not the same as Extreme Corporal Punishment)

C. Sirolimus

D. Etanercept

E. Thalidomide

F. None of the above

G. God please help us!

Some other treatments I'm looking into on my own:

Pentostatin

Prochymal

Soriatane (Acitretin)

Lamprene (Clofazimine)

Article I found: How I treat chronic graft-vs-host disease by Georgina Vogelsang, 2001

Blood Pressure, Probiotic, and Colostrum

His labs were ok, I guess. Karen did not mention anything out of the ordinary.

IVIG and Zenopax infusions today. The appt. was 5 hours long (it should have been about 4 hours). We were there 2 hours before any medication started to infuse! They had to poke David 3 times before they found a vein in his arm. The Senara numbing patch worked for one poke and he was brave to withstand the other pokes without numbing. They put us in the "treatment" room which is a double door room for extra sensitive children. The only problem is that they always forget about us, the service is super slow, like I have tons of time to be there! Last time we were there for 3 hours for a 1/2 hour infusion; we missed an important library event and everyone was sad.

I bought a blood pressure cuff but do not know how to use it yet.

I finally bought the probiotic and colostrum that the doctors said was ok to give him.

+828 Pictures of our Trip to Jekyll Island Georgia, and Disney World (in October 2008)

+826 The End

First question:

David: "Mama, when is the end?"
Mama: "The end of what?"
David: "The end of the world?"
Mama surprised: "The end of the world!?!"
David: giggle! giggle! giggle! (such a teaser)

Next question:

David: "What's 100 pluss 100?"
Mama: "Two hundred."

Next question:

David: "Mama, do you know how to make glass windows?"
Mama: "No, I don't"
David: "God make glass windows. He make everything in the whole world. He a very tough man." (that's MY BOY!)

End of conversation.

+844 Zenopax and Consultation

We were in for Zenopax on Thurs. It was slowwwww service that day! A 1 hour appt. turned into 3 hours.

David's labs looked pretty good. They are doing CBC only once/month. The chemistry's looked pretty good. His liver and kidney numbers were really good too. Yeah!

His phosphorus was 2.9 which is still low but looks good. I started to give him the Whole Foods brand of liquid vanilla flavor Calcium, Magnesium and Phosphorus. It has 600mg/2 tablespoons. He was only getting 250mg with the small packet of phosphorus prescribed by the doctors. We'll see how the supplement works. The NP did not have an issue with me giving him the supplement.

His blood pressure is still high. I finally ordered a cuff for him so I can monitor him at home. I also finally ordered the Colostrum and probiotic which the doctors o.k.ed for him. It has taken me a long time to order these items because I am so busy and don't have a lot of time to research which cuff is best, etc. I just ordered one that looked fun and hope it works well. Now I'll have to learn how to take the pressure using the cuff and stethoscope. Fun! Fun!

The doctors want to have a "pow-wow" about what options we have for treating David's skin GVHD. They are going to recommend some other treatments because David's current treatment is not working well. They do not want to increase the steroids. They would rather try a different treatment and if that works, wean him off steroids.

+838 How Heparin is Made

Heparin is a anticoagulant solution that is used to keep IV or Hickman lines open so they will not clot with blood. I used to flush David's Hickman line twice/day. He doesn't have a line any more.

There was a brand of Heparin recalled (produced in China) about 6 months ago due to a dangerous contaminant. It does not surprise me since Heparin is commonly made from bovine or pig tissue. Heparin has also been extracted from the following species:

Turkey
Whale
Camel
Mouse
Humans
Lobster
Mussel
Clam
Shrimp
Crab
Sand Dollar

I almost got ill! This becomes an issue with me because I follow dietary rules. The only animal acceptable to me is the Turkey. Would you want to eat a whale, a camel, a mouse, a sand dollar? Why would I want to put something in my son's body that is made from these animals? Gross!

David does not get Heparin any more, but if he does, there is an alternative called: Argatroban which is a synthetic thrombin inhibitor derived from L-arginine (amino acid).

+828 IVIG and Toilet Training for Chronicly ILL Children

David's labs are OK. His CBC (complete blood count) is normal. His chemistry's are not all normal. His kidneys were high. His Phosphorus was low. And his Prograf level was 20 (it s/b between 7-10). We skipped two doses of Prograf and went back today for a blood draw. It's 6.9 now. So we went down to 5.0ml/twice/day.

There really are no improvements with David. I wish I had good news, but I don't.

Dr. Argawal said they keep him on so many oral medications because they want to prevent scleroderma. She said if he gets scleroderma, no improvements can be made. So they will not try more topical treatments. This means they are not going to taper the steroids.

Good article on how to toilet train a chronically ill child.

+820 Trip to Georgia and Day Hospital

WBC
RBC
Platelets
Height - 36-1/2 inch
Weight - 35lbs

We are in the day hospital for Zenopax today.

His ear wax removal was successful. I had to put over-the-counter drops into his ear to soften the wax. The doctor was able to easily suction it out. We repeated the hearing test and he got good scores.

We took a 14-day trip to Jekyll Island, Georgia and Disney World, Florida from Oct 12-26. David did well. His skin started to flare up right before we left, so I've been using the steroid (hydrocortisone) creme. The creme helps but the improvement does not convince the doctors enough to taper his oral steroids. Thus, he continues to look and act like an intelligent 2-year old. That bothers me.

He contracted a cough and runny nose when we returned which could be why his skin has been more itchy. The thought being is that his immune system goes to fight the cold, not the GVHD so the GVHD flares up.

His hair has been growing into the sparse spaces and his scalp is not flaking either. I stopped using the scalp creme (trimethisolone) about 2 months ago. Hopefully, his hair will keep improving.

+792 Ear Wax Removal

The removal of his ear wax went smoother than I thought. The Otolaryngologist used a thin long vacuum. She removed it from the right ear, but the left ear wax is hard so she told me to use over-the-counter Debrox drops for 4-5 days/twice/day. He'll go back for a follow-up on Oct 10, and he'll see the Audiologist to repeat the hearing test.

+787 Dentist, Eye Doctor, Ear Doctor

WBC - good
Hemoglobin 15.0
BUN - 19 (really good kidney function)
Weight - 33 lbs
Height - 89cm or something like that...each nurse measures him differently

He had Zenopax this week and did fine.

He was so excited to see Dr. Natalie, the children's dentist. He was so cooperative and I have to owe it all to Dr. Natalie and the beautiful way she loves her patients. His teeth and mouth look wonderful! No GVHD in the mouth. His x-ray looked good too.

His eyes were checked by Dr. Winton. He has no cataracts. Cataracts are common with long-term steroid use. He was cooperative with the eye drops but did not like the blurriness from the dilation drops. His eyes look good too.

His ears were tested and the results were flat, not good hearing at all. There is a lot of wax covering the ear drum so we have to go back for a wax removal, then we will repeat the test.

His Prograf was 13.5 which is too high, so it went down to 6mls.

+771 - Day Hospital

WBC 11.6
Hemoglobin 15.2
Platelets 281k
Weight 33lbs
Height
BUN 30H (we came to DH first thing in the AM he had not had a drink)

He is getting IVIG and Zenopax. About 4 weeks ago we went to the beach on a Friday then again on a Sunday. I had him completely covered head to toe but the sun did a bad job on his skin. It totally flared up. Dr. Argawal decided to do Zenopax every two weeks. So we come to the DH every other week.

His blood pressure today, before his meds, was 152/109! I gave him the meds and it went down to 123/80.

He is still not growing much ;-(

We are preparing for a trip to Jekyll Island, Georgia and Orlando, Florida. We will have two more DH visits then we will be set to go.

Meds:

Predisone 2mls/twice/day
Meltaway antacid - once/day
Septra 4mls/twice/day
Prograf 7mls/twice/day
Isradapine (blood press) 2mls/twice/day
Multi-vit w/out iron - once/day
Liquid Calcium w/Magnesium - once/day
Nordic Naturals Omega Oil - once/day
Zenopax - every 2 weeks
IVIG - every 4 weeks

+743 Day Hospital, Headaches/High Blood Pressure, Skin Looking Great, Trip to Eugene OR, Stanford Research Studies

WBC - 11.0
Platelets - 311K
Hemoglobin - 14.5
Blood pressure 129/84 (high) (got up to 138/94 while we were at the Day Hospital)
Weight 14.9 kilos
Blood Urine Nitrogen (BUN) - 27 (high - but Dr. Argawal reminded me to give him a lot of water. The Prograf (immuno. suppress) is toxic to the liver so they watch this number carefully)



We're in the Day Hospital today for IVIG. David's skin has been looking great even though I have not been applying the steroid creme. He has had headaches though, and as you can see his blood pressure has been up, which I think was causing the headaches. The medications like Prograf and Predisone cause high blood pressure, but I see another coralation between high cholesterol and blood pressure. David's cholesterol has been high ever since transplant. When you have high cholesterol, the fatty tissues get deposited into the arteries, when that happens the arteries cannot do a good job pumping your blood, thus high blood pressure. He was given an extra dose of Isradapine today to help bring it down.

Dr. Argawal decided to do Zenopax (immuno. suppress) every 4 weeks to see how he does. So, we'll be coming every two weeks for IVIG and every two weeks for Zenopax.

Current Meds:

Predisone 2mls/twice/day
Meltaway antacid - once/day
Septra 4mls/twice/day
Prograf 7mls/twice/day
Isradapine (blood press) 2mls/twice/day
Multi-vit w/out iron - once/day
Liquid Calcium w/Magnesium - once/day
Nordic Naturals Omega Oil - once/day
Zenopax - every 4 weeks
IVIG - every 4 weeks



Last week we took a trip to Eugene, OR. We are trying to sell our home there. It's vacant right now. I bought a few Lego kits for him and he loved them! He even built his own ATV motorcycle by following the directions. It was amazing to me to see a 4 yr old concentrate so intently on the instructions, find the pieces and build something useful to play with.

Stanford has a new group of people who are working on developing a study on the Long Term Health Effects on Recipients of Hematopoietic Stem Cell Transplantation. David will partake in the study so that we can further research and hopefully improve the outcomes of post BMT patients. There is another study they are asking us to join that is sponsored by The National Marrow Donor Program. This study only requires 2mls of blood which will be tested against different pathogens and banked for other purposes.

As for the rest of us, we are doing very well. Michael just released his product Knol. The girls went to my church pre-teen camp and had a blast! I've been very busy getting ready for the upcoming school year and enjoying marriage and motherhood immensely!

Day +725 Please Pray for these WAS Children!

Please pray for these children who have Wiskott-Aldrich Syndrome and are going through bone marrow transplants:

1. Zac William Rugless - NSW, Australia - This 10-month old child is very, very sick. His sweet mother and Zac have been in the hospital for 5 months. He is in ICU with major fluid retention problems (around major organs) and heart problems. They want to do a biopsy of his liver to see if he has liver GVHD. He is very weak and susceptible to infections. His older brother had WAS and went through a BMT but not without major setbacks. His older brother is doing well after fighting GVHD for 5 years. Susan, his mother, has been encouraging and helpful to me by providing advice for David's ailments. Please pray for this family and send them a note of encouragement, please!
2. Sebastian Lazarte - lives in Peru having transplant in Chile, donor from Gainsville Florida - This family is very sweet. The mother is very well educated. Sebastian has a 5/6 MUD (matched unrelated donor) from Florida. He is having chemo right now to make room for the new cells. Please pray their transplant is successful. Please pray that Ivonne, the mother, has peace during this difficult time.

Day +723 Day Hospital Zenopax and update

He went to the Day Hospital for routine infusion of Zenopax. The doctors were not happy with how his skin looked so they put him back on steroids twice/day. We tried for about 4 weeks to do steroids once/day to see if his body could go without steroids for 24hours. This would enable his body to rest and grow. However, his body cannot handle that so he is back on steroids twice/day.

His daily aches and pains seem to have gotten better. I was not giving him the regular dose of liquid calcium, so I started that daily. I think it helped.

Current meds:

steroids (immunosuppressant) - 2ml/twice/day
Prograf (immunosuppressant) - 7ml/twice/day
Septra (prevent bacterial infections)
Isradipine (blood pressure) - 2ml/twice/day
Melt-away (can't remember the name but it's to control acid in the stomach)
Zenopax (immunosuppressant)
IVIG (Gamunex - immune booster)
Multi-vitamin
Liquid Calcium

I still haven't ordered the Culturella (probiotic) or the Colostrum. I can't seem to find the time!

Day +721 Knuckles Hurt

We took a trip to southern California this past weekend. We camped and attended a reunion for Michael's church. David was complaining on/off about his abdomen hurting. He looked tired but had a good time. We went indoor rock climbing. The children had a super time! He was active but did not have a good appetite.

He's been complaining about his knuckles hurting, his elbow, and the bottom of his feet. The pain comes and goes. It's not hindering any of his activities.

Current meds:

Prograf - 7ml/twice/day (GVHD)
Steroids - 4ml/once/day (GVHD) (I'm not sure this is working, he might need to go back on 2ml/twice/day)
Septra - 4 ml/twice/day (infections)
Emu Oil and Topical steroids - once day/sometimes twice (GVHD)
Isradapine - 2ml/twice/day (blood pressure)
Multi-Vit
Calcium

I would like to add the following:

Culturelle - probiotic
Omega Oils - Nordic Naturals (for inflammation and auto immune disorders)
Sedona Colostrum

Neurology Called:

The neurology dept called to set up a post BMT neurological assessment. This will happen mid August. Since David had chemotherapy (oh, I dislike that word!) there are risks associated with things like short-term memory. It's expensive $350/hr. The assessment will take 4 hours. The insurance should cover it.

Day +708

WBC - 10.5
Hemoglobin - 13.3
Platelets - 442
Height - 93cm

David's been doing pretty well. Today he had Gammunex IVIG without any problems.

The NP said we could give him one of the following probiotics: Culturelle or Lactinex.

Doctor Weinberg looked me in the eye and announced "He has Chronic Graft-vs-Host Disease." David will be on medications for a long time unless God provides a miracle.

The stool samples did not grow rotovirus or c-diff. We are waiting for the other cultures, if any.

Day +701 Probiotics

David is feeling pretty good today. He rode his bike. His appetite is back too.

I took him to the Day Hospital for Zenopax (immunosuppressant) infusion today. We were not even scheduled to go today; they messed up the appointment. Since we were laying low today, I offered to come in.

His white blood count was high. The nurse practitioner (NP) said that he might have a recurring bacterial infection like c-diff, rotovirus, or a number of other infections. She sent us home with sample containers, so when he did his dirty deed it went into the container. I'll take it to the lab tomorrow. The NP said in the future I should call and let them know if David vomits or has diarrhea. The feta cheese may not have caused it after all, but we shall see what the lab results reveal, if anything.

If he goes on antibiotics, the NP said we can give him probiotics to recolonise his GI track. I asked his doctors back in February if we could give him probiotics and they said only if they are pasteurized. I could not find anything on the Internet like that, who would pasteurize their probiotics killing the good bacteria? I spent days researching. All of the research pointed to the dangers of giving probiotics to the immunosuppressed. I also consulted with other GVHD patients and they said to steer away from probiotics.

I would not normally announce this in a public setting, but I was fasting today. This week, I was reminded about Jesus Christ's instruction to keep asking, knocking, and seeking. I did not have a particular reason to fast except to loosen the burdens. I feel some burden was lifted when I was told he could take probiotics.

The NP said we have to be careful to choose the right probiotic. I will soon find out and let you know.

Day +701

I left him with Michael last night and he took his meds and went to bed. He slept through the night and woke up with a smile on his face. He is back! He is eating little portions and taking his meds. Although we had a day full of fun things to do, we are staying home to make sure he is feeling ok. We might pop over to the library but that's it.

I'll post an update tomorrow after he sees the doctor for his regular appointment.

Thank you for stopping by to read the blog. If you have a moment to leave us a message, that would be wonderful!

Day +700 Club - Anyone Want to Join?

Thank you all for your prayers and emails. You have encouraged me one hundred fold.

David is doing a little better. He slept through the night except to wake me up at 2am to tell me he was hungry. He's been eating very small portions and drinking.

Unfortunately, I think I brought this upon him. I realized it might not have been the feta cheese afterall. Yesterday I smelt the Vit D milk I gave him and it was bad. We usually do not drink cow milk but he requested it so I bought some. I was not as diligent to make sure it was good. It had not expired yet, so I did not think about smelling it.

The flip side is I think he will be ok for now. Thank you from the bottom, top, inside, outside, and underneath of my heart!

David is on Day +700! I thought this was a good number, so I made a music video for him. The video should have been posted on his birthday, but I did not have time.

Day +699 - Things Change So Quickly

I'm glad we got out of the chiropractor's office (I'm having a foot problem) before David started throwing up. God blesses me with the foresight to have what we need in the car to help him when things don't go well. I won't go into detail, but he provided the extra help I needed today. Thank you all for your prayers and encouragement. I'm feeling a lot better compared to when I sent out the following email today, but David has a 101 temp and cannot still keep anything down.

Would you please pray for David? 1-1/2 weeks ago he had a GI problem and got over it without a hospital visit. However, today he is showing the same GI problem. He is loosing everything from both ends. I'm not sure if it's coincidence but the problem occurred both times after he ate feta cheese, which is supposed to be ok for him to eat. He'll see the doctor on Thursday for a regular visit, if not sooner if he does not improve. He is thirsty but cannot keep any water down. He insists I give him water, so I do. It keeps him hydrated. I'm fairly confident he will recover quickly this time, however, I'm finding it emotionally hard today to watch him suffer for this long (all his life) and endure everything. He is such a good boy, hardly complains, and just puts up with everything so gracefully. I'm so afraid his overall health will never be the same and things will not end positively, as I'm sure you can understand.

The girls are doing well today and keeping themselves busy while I help David. Unfortunately, when he is not well their lives are disrupted, for example, Ariella cannot go to her regular gymnastics class today.

In tears,

Aimee and Michael McNally

What is IVIG?

So what is IVIG? IVIG is a mixture of immunoglobulins taken from 10,000 sample donors and costs $18,000 per bag/treatment, and the insurance companies don't blink an eye.

David receives this infusion every 4 weeks to boost his immune system.

Day +698 and Doing Great!

David has been feeling great. The fires in this area have created a lot of particulate matter that is dangerous for the lungs and can seep into the blood, so he has been wearing a mask. He did not put up much of a fuss. He will tell you exactly why he needs to wear it and pronounces "particles" perfectly! He knows the particles will get in his lungs and could hurt his blood. He is really smart!

Someone Named Jeff Who Has WAS Needs Help

Day +692 - Not Feeling 100%

Today is Day +692 or....

+59,788,800 seconds
+996,480 minutes
+16,608 hours
+98 weeks

On Wednesday, June 18, David came down with vomiting and loose stools. He continued to take his meds, drink, and eat but it would not stay down. On Thursday (Happy Birthday, Teri!), he slept a lot. On Friday, he was doing better but vomited right before bed. He slept all night and on Saturday was feeling ok so we went to church. Sunday he did well all day. Monday (today) he woke up not looking that great. I'm worried yet not worried because he is eating, drinking, and taking his meds. He looks tired but is active and annoying his sisters. I must count my blessings.

Prayers are appreciated.

IVIG and Zenopax Today

WBC 12.3
Hemoglobin 15.0
Platelets 334K
Weight 14.7 k
Height 88.7 cm = 34 inches

David is doing pretty good. His skin is still problematic, requiring topical and oral steroids. Dr. Weinberg said today that I should go to 2mls/twice/day (right now we are at 1.5, 1.5, and 1.2mls throughout the day). If David can tolerate that schedule and we see no skin flareups, then we will go to 4mls/once/day. Dr. Weinberg feels that if David's body can have somewhat of a "rest" from the steroids then he might have a chance to grow. Eventually, we would like to see him on steroids every other day (the on day being a large dose 13-15mg) so his body can rest on the "off" day and start growing. I have no complaints with this plan.

His current medications:

steroids (immunosuppressant)
Prograf (immunosuppressant)
Septra (prevent bacterial infections)
Isradipine (blood pressure)
Melt-away (can't remember the name but it's to control acid in the stomach)
Zenopax (immunosuppressant)
IVIG (Gamunex - immune booster)
Multi-vitamin
Liquid Calcium

Dr. Weinberg said he appears to have recurrent acute GVHD. I'm not so sure about that. It's only recurrent because sometimes I use the topical steroids and sometimes I don't (rarely). Today I did not and his skin looked more serious. I think he is calling acute because he does not have the joint immobility issues or deep skin problems that bleed and itch.

YouTube Video of Daniel Santos - 2 Months Old

This family is urgently looking for a donor http://www.danieldosantos.blogspot.com/. If you have not become a bone marrow donor, please consider attending a donor drive. You can find locations at: http://www.marrow.org/HELP/Events/index.html Just enter in your zip code to find a local donor drive near you.

Here is an adorable video they put together for him:

http://www.youtube.com/watch?v=lm8-PtCHYkI

New Pics

Making Irish Bread

Hiding out under driftwood at Mendicino beach

Mother's Day in Mendicino - Huge artichoke plant

Michael's Mom - Mother's Day in Mendicino

Climbing the Walls in new 70 SPF Swim Suits

Ariella and David looking for Lizards

David's reddish face - from high blood pressure and skin GVHD

More reddish face and puffy cheeks from steroids (called cushnoid)

Zenopax Today

Labs look fine.

He had Zenopax today. It took 1 hour before they admitted him to the Day Hospital. His skin was looking great from about 5/14 to 5/20. I mean really great, like I thought we were on the path to taper the steroids. Then, on 5/21 I gave him cheddar puffs for a snack. His skin started to get really rashy almost immediately after eating some. There was no other apparent reason for the skin to flare up. Urg! I was so disappointed. No more cheddar puffs for him.

He's been doing well and enjoying life. At least I can be comforted that his quality of life is pretty good. I'm just not sure how much his little body and organs can withstand the long-term use of medications.

Sad face...

IVIG Day

WBC - 12.3
Hemoglobin - 14.2
Platelets - 329
Blood Pressure - 122/77

He is getting IVIG today. No problems. His skin looks really good lately. I've been working very hard keeping him covered, using hats, sunscreen, and shade. The temp is supposed to be 100 degrees so it's not easy staying cool. I bought him a mister fan which he thinks is fun to play with and squirt his friends. We are not making any changes to his medications.

Growth - He is growing very slowly. Doctor Argawal said his body's energy is going into fighting the chronic GVHD not growing. She encouraged me to keep feeding him a lot of calories and cater to his requests within reason. August 1st will be his second BMT re-birthday. He will see an endocrinologist to test his growth hormone. Dr. Argawal is not concerned about his growth at this time. She says he has not been on steroids "for that long." I disagree since being on steroids for any period of time...is too long in my opinion. She said at around 8 yrs old, they will seriously consider giving growth hormones if he still has a problem.

Blood Pressure - His blood pressure was better today. It's still on the high end of normal. He has a red face most of the time, but Dr. Argawal said that is due to the blood pressure medication, not the blood pressure.

Socially - We receive so many comments from strangers that I am beginning to get irritated. His growth is slow and the red face makes him stand out wherever we go. He often looks tired. Strangers make innocent comments that make him uncomfortable. On the flip side, many people say he is so cute, which is nice to hear.

Other than that, we are doing fantastic. We had a wonderful Mother's Day camping in Mendocino and meeting Michael's parents for a vegetarian brunch. The girls showered me with many cards and hand-made gifts. I love all of my children so much! Happy Mother's Day to everyone in cyberspace who reads this.

Zenopax Every Three Weeks

WBC - 16.3
Hemoglobin - 14.0
Platelet - 355k
Weight - 30.1 lbs
Height - no change :-(

On April 11 (where he had Zenopax and IVIG), David went to every 3 weeks (was 4) for Zenopax infusions. On April 30 he had Zenopax and a physical exam. He is not scheduled again until May 14.

Overall he is doing well but his skin GVHD is not 100% controlled. The NIH recommended that we use more topical steroids, while Karen at Stanford insisted we use more oral steroids. She said the people at NIH are not as well versed with dealing with unrelated bone marrow transplants.

Incredible List of Meds

I saw this post and my eyes almost popped out of my head. The man who has to take all these meds is very sick. It breaks my heart to see this but I wanted to share this as I try to keep things in perspective.

In the mornings: Advair81 mg.
AsprinBactrim DS (twice daily Thurs. and Fridays)
Cellceot 50mg
cyclosporin 50mg
glutamine 15 grm pkt twice daiy
Hytyrin 5 mg
Keppra 500 mg. twice daily
Micalin nasal SprayMycelex (4 x daily)
Prednisone 20 mg
Protonix 40 mg
Spriiva Inhalersynthroid 112 mcg
Voriconazole 200 mg twice daily
Nature eye tears, every 3 hours
Azithromycin 250 mg
Decadron Elixir 4 x daily
Altace 2.5 mg
Cholecalciferol 400 units
Clonidine TTS 0.1 patch every sunday
Hydrochlorothiazide 25 mg. daily
Xeponex 1.25 MG 3ML (4 x daily)
Oscal 3x daily12

Noon:

MycelexDecadron ElixirAndrogel 1%Xopenex 1.25 MG/3ML
OscalNature tears

8:00 PM
GlutamineMycelexDecadron ElixirXopenex 1.25
OscaloNature Tears

9:00 PM
Advair 500/50Bactrim DS (twice on Th/Fri)
Cellcept 500
Keppra 500
MycelexVoriconazole 200 mg
Decadron ElixirXopenex 1.25
Nature Tearscyclosporin 50 mg

AS NEEDED
VicodinFour Times Daily a special compound for his eyes to reduce the "crud" in it . It is a form of mycomistCMPD-Acetylcystine 10% drop four times dailyTwo times daily Eye scrub (which seems to be helping a lot)

High Blood Pressure

Blood Pressure 130/85 - way too high for a child

Michael took David to Stanford for me today so I could attend a Homeschool Leadership Conference.

David received IVIG (Gammanex) and Zenopax. I put EMLA creme on both hands and in the crease of both elbows. They were able to find a vein in the crease of this elbow. All went well thankfully. He is going to Zenopax every three weeks.

However, his blood pressure was high again. It's been running high for about 1 month. Dr. Argawal said it is due to the Prograf and Prednisolone. It is my understanding that these drugs have a cytotoxic effect on the vascular endothelium, and damage the cells.

He is on one more medication for high blood pressure. The pharmacists says he may feel tired because the drug causes relaxation, but this has not stopped David. Again, I mix it with Spicy Apple Cider from Trader Joes and he cannot tell the difference.

I've been using the topical steroids and I think they are working. He is having a little flare up so I will have to buckle down and use the topical cremes twice/day. It's not easy applying topical creme to a squirmy 3 yr old and I don't have a lot of extra time to do this. It's really a hassle.

Current Meds

Prograf (immunosupp) - 7ml twice/day
Prednisolone (immunosupp.) - 1.5ml, 1.5ml, 1.2ml - 4.2ml/day
Septra (antibiotic) - 4ml/twice day
Prevacid (antacid) - 1/day
Zenapax (immunosupp) - 2/month
IVIG (gammunex)- 1/month

Thursday at NIH and Friday Fly Home

He saw the dentist. The dentist saw him. He never opened his mouth. Case closed.

Our flight home was great. It's nice to be home safe and sound.

Wednesday at NIH

Today he saw the eye doctor. Have you ever taken a 3 yr old to the eye doctor? Reading the images was fun and he has 20/20 eye sight. The hard part was the yellow eye drops, then shinning a blue light to see if there is any stain. More good news, there was no stain, so there is no eye GVHD. However, I don't think the doctor put enough of the yellow drops in his eyes. I don't think he has any problems with dry eyes, so I was satisfied. It was a difficult test for David.

We had a consultation with Dr. Candotti, the WAS specialist. He handed me a graph showing that David now produces 100% Wiskott Aldrich cells, so he no longer has WAS.

Next, we went to Rehabilitation. I filled out a survey asking questions about his mobility and emotions (like depression and happiness). Dr. Li measured his elbow, wrist, ankle, and knee flexibility. He has 100% mobility. She told me David is the youngest they have in their GVHD study. The study is about 3-4 years old. She also asked me questions about his endurance. He does not have any problems in this area of life. More good news.

Tuesday at NIH

David's labs looked pretty good. His cholesterol is still really high which they say is from the immunosuppressants, this is nothing new, it's been high since after transplant.

We saw Dr. Cohen, a dermatologist. He is fantastic. He and his colleagues agreed that we need to keep up the topical steroids (hydrocortisone) because that will prevent him from any increase systemic steroids. He also mentioned a couple of instances from other patients who had skin irritation from Zenopax (immunosupp.). David receives this drug twice monthly by IV. Also, there are some people he knows who take Septra (prophylactic) and are very sensitive to light, which causes an eczema like reaction. This was very interesting information.

He had pictures taken, then we saw a nutritionist. We're going to see if his antacid can be taken at a different time of the day so it does not interfere with his nutrition absorption. From what we understand, the steroids cause an over-production of stomach acids. The extra acid can cause stomach lining problems. The antacid helps decrease the extra acid. However, the antacid DOES prevent the absorption of calcium and iron. If we can time the antacid just right, perhaps we can avoid the nutrition issues I'm worried about. We do not need another bad cycle, like many medications cause.

The nutritionist says he is not on the standard growth chart for his height (88cm or 35 in) and is in the 10 percentile for his weight (30lbs or 13.8 kg). However, those two numbers put together put him in the upper 90 percentile for BMI (body mass index). This is good news but not accurate since he is on steroids.

We had a brief meeting with the WAS doctor, Dr. Sucolic. He said we made the right decision about David's BMT. He said David is doing well, overall. He agreed that topical steroids should be used regularly so to prevent the use of Rapamune or more oral steroids.

David and I took a taxi downtown D.C. to see the cherry blossoms. They were really beautiful. This was the first time I had seen the monuments and the White House!

Monday - Children's Inn at NIH

Weight: 13.8 kg - I need this boy to GROW!
Height: 88cm - GROW BABY GROW!
Prograf level: 10 - good
Blood Pressure: 112/84 - good!

I feel so blessed by the generosity of strangers! Last night a local woman's group made a delicious buffet meal for the entire facility. Today, when we got back from NIH, there was a huge creative gift basket (it was not a weaved basket, rather a cake storage container) for every family which contained different Israeli foods for Purim. A volunteer made a trip to the store for us to buy some basic food for us. If you ever have the opportunity to volunteer for a children's hospital or their temporary home (like Ronald McDonald House), please do it. We are so thankful for people like you!

The staff at NIH were really nice to David. He had a lot of blood drawn for the WAS and GVHD studies. This was the hardest part of the day. He had an Echocardiogram and was so exhausted he slept during the procedure. His VSD (heart murmur) is 3mm. It has not changed since transplant. We spoke to a few experts. Bones are often brittle due to steroid use, so he had a bone density scan that lasted 3 minutes. He layed on a bed while a laser scanned over his spine, femur, and wrist. We will receive results of all the tests in about 2 weeks.

It does not sound like much happened, but we were there from 7:30am to 2:30pm. I'm going to relax now. It's been a long day.

Oh, here is one more Christian family that puts me in awe. They have a genetic disorder which was passed to all four of their boys! All of the boys required a BMT. Can you imagine what a burden that must have been?

Relactation Update and Blood Pressure

I just wanted to give a quick update about relactation. David and I had a precious relactation conversation the other night. In short, he said one had milk and the other did not. I keep trying but he's just not interested, except at night.

His blood pressure for the past 3 weeks has been 120/84, which is like an adult blood pressure. We will have it checked again at the NIH on Monday. Will update later.

Aimee

On a Tail Wind to the NIH

We flew from San Jose to Chicago with a tail wind. Then, from Chicago to Washington D.C. We hired a cab in Washington D.C. to the NIH (National Institute of Health) at Bethesda, MD. David loved flying. He really is a light to those around him. The people we shared an isle with were very pleased with his behavior and personality.

We are staying on the NIH campus at the Children's Inn. We've already met a few inspiring families with very ill children. I met a man who married a woman and both of them carry the gene for Chronic Granulomatous Disease. Both of their daughters (age 11 and 15) are very ill, especially the 15 yr old. She had shunts put in her brain, but the drainage was not working well so her brain began to swell. On top of this, she now has a lung infection. They have a 20 yr old daughter with Multiple Sclerosis, so she cannot be a bone marrow donor to her sisters, even though she is a match! Their visit was supposed to be for 3 days and now has turned into 45 days. They are from Puerto Rico and have been coming to the NIH since 1996. This man had the most beautiful, infectious smile and attitude. I was inspired.

David is participating in two studies at NIH. One is for his chronic skin GVHD. The other is for the on-going Wiskott Aldrich Syndrome study (it's a life long study as long as we want to participate).

Here is his schedule:

Monday: blood tests, clinic appt with GVHD team, echo cardiogram, bone density scan
Tuesday: clinic appt with WAS team, dermatology, photography, nutrition
Wednesday: ophthalmologist, meet with WAS team again, rehabilitation
Thursday: pain, palliative care and symptom management, dental

I'll let you all know how things go.

Relactation

I'm trying to relactate. David stopped nursing about two months ago. I am worried about his GI track. I don't have visual evidence to be worried; his GI track looks beautiful. I just want to provide everything I can to keep him healthy.

He's been very interested in soups! This is music to my ears. He used to reject soups. His preference is to eat soup by dipping bread, which is ok as long as the bread is high quality multi-grain.

I've pretty much eliminated tortillas and cheese from our household which has been a smart move. He's been eating so much better.

Worry Doesn't Help

I read an encouraging chapter in To Fly Again: Surviving the Tailspins of Life by Gracia Burnham. She is the woman who wrote In the Presence of My Enemies. She and her husband endured a life changing trial in the jungle with Islamic extremists. It's a tragic story that will make you cry. To Fly Again is her second book. In this book there is a chapter called: Worry Doesn't Help. She had a lot to worry about while in captivity but she rises above it again and again. She is an incredible woman! I would like to share portions of the chapter:

"Worry is Atheism" this was a devotional by Methodist missionary E. Stanley Jones. He was away from his wife and family on a trip, and a well-meaning hostess said to him one evening, "You have had a quiet day; you've had time to worry." I felt inwardly startled. "Time to worry" - as if a Christian ever has "time to worry"! The Christian has expunged worry from his vocabulary... A person who worries says, "I cannot trust God; I'll take things into my own hands." Result? Worry, frustration, incapacity to meet the dreaded things when it does come. With God, you can meet it, overcome it, assimilate it into the purpose of your life. Alone, you fuss and fume and are frustrated. Worry says, "God doesn't care, and so He won't do anything - I'll have to worry it through." Faith says, "God does care, and He and I will work it out together. I'll supply the willingness, and He will supply the power - with that combination we can do anything."

Gracia goes on to say...One dictionary definition of worry is "to torment oneself with disturbing thoughts." The active very from is interesting, isn't it? Worry is something we do to ourselves. It is not an involuntary twitch, and allergic reaction to some mysterious chemical, a spell that is cast upon us from the outside. We initiate the worrying. We torment ourselves. The word's roots trace back to the Old English wyrgan, "to strangle." That is precisely what worry does to us; it cuts off our air. It prevents us from inhaling the Divine Breath, the Holy Spirit of God. Instead, it slowly asphyxiates us. In my dictionary, the word worry comes just after wormwood and worn-out. Right behind it comes worse and worst. What an awful page! What an awful wast of mental energy.

The antidote to worry, as everyone who has ever read the familiar Philippians 4:6-7 knows, is to turn to the Lord. "Don't worry about anything; instead, pray about everything. Tell God what you need, and thank him for all he has done. If you do this, you will experience God's peace...." It sounds commonplace, I know. But it is still true.

There are many instances in the Bible where the modus operandi of the greatest monarch Israel every knew, was to repeatedly, consistently, even incessantly, "inquire of the Lord." The response to that inquiry brought calmness, clarity and wisdom. God does not mean for us to sit and fret, stew, agonize, wring our hands, or be troubled. Instead, he invites us to interact with him, to gain his perspective, and to rest in his good and perfect will for our lives.

In the jungle I quickly realized that in order to keep pace with the others on the trail, I was going to need a considerable supply of drinking water. When I didn't have it, my face would begin to flush, my mouth would go dry, and fatigue would set in rapidly. I would plead with the captors, "Please, I need water. Can you get me some water?" I would also beg God. "Lord, I need water. It's really bad. God, help me! I need water." I was imploring him over and over, almost frantically. I was hammering on heaven's door. My anxiety would rise another notch when I watched the Abu Syyaf using up precious water for purely ceremonial purposes, such as washing their feet or their face before evening prayers. Not being Muslim, I found this to be wholly unnecessary. What a waste of the resource I craved so badly. Then one day on the trail, as I was harassing God once again, it began to dawn upon me that he knew very well about my need for hydration. He wasn't oblivious to that fact. My prayers gradually changed from, "God, water-now!" to "Lord, you know what I need. You understand my body's need for water. Help me be patient until you take care of my need." And he did. Not long after, a captor gave me my very own water bottle! It was made of translucent plastic, with a handle and a red top; it held about a liter. I could fill it up myself every time we came to a stream, and no one would take it away from me or ask to use it. I felt God had restored my ability to control the situation. God is big enough to make his own decisions and manage his own actions. He doesn't have to run everything by us before he acts. he is in charge, and we are not. If he doesn't need to worry about the current state of affairs (and obviously he doesn't), then neither do we.

Living Without Limbs

This video is of Nick Vujicic, the man living without limbs. His message is encouraging. It cuts off at the end but I hope this will encourage you if you are having trials and difficulties.

Antacids = Nutrition Mal-absorption

Antacids can prevent the absorption of Calcium, Iron, and Vitamin A. I asked Dr. Argawal if antacids prevent the absorption of certain nutrients. I'm concerned that he will not get all the adequate nutrition if the antacid is preventing it (by reducing the amount of stomach acid). She denied it would do that, but when I look on the internet, it's pretty clear that this happens. Medical doctors are in a different world than I am. They see many things so differently. I'll have to do some more study about the absorption problem.


I also learned that antacids are commonly manufactured with aluminum in them. The Prevacid David is taking does not have aluminum in it.

Colitis = "col" colon "itis" inflammation

Good news! All of the blood cultures are negative. His GI track shows mild inflammation and colitis which was the main cause for the bloody stools. No graft-vs-host disease in his gut. The mild acute colitis with cryptitis. They say its from long-term use of steroids or the Cellcept medication he was on. I read that the effects of colitis can be reduced by changing the diet, by avoiding carbohydrates and lactose products. David is a huge fan of these! They put him on an antacid (Prevacid solutab, not the Zantac) to help counter the effects of the steroids/colitis. They stopped the Cellcept until further notice. They increased the Prograf to 6mls/twice day.

It feels like a never-ending cycle. His is doing very well, back to his active self. His skin graft-vs-host disease is still flaring up, so they are trying hard to control it. The CT scan of his lungs is 100% clear. He had a very hard-to-treat micobacteria in his lungs last spring, was on antibiotics for 6 months, now it is healed.

There are many things to be thankful for but he is not living a normal healthy life which bothers me a lot. We continue to ask for your prayers that God's will be done in David's life. We hope that His will is to heal him, grant him great health, therefore, we press on with faith (even though it wavers) and prayers. This is all stressful for Adira and Ariella as well. Please pray for them. They had a hard week too. I think the kids and I will go to the beach this week and re-group. The ocean has tremendous healing powers, which I mean in a Godly way, for it was He who created it for us to enjoy.

In Christian Love,

Aimee, Michael, Adira, Ariella, and David

Lost Third IV

His third IV became painful for him when the CT scan nurse flushed it. When they started the IV fluids and meds again he was crying in pain. The doctor on duty tonight decided to put him on oral Prograf. So, no IVs and we are going oral. Unfortunately, the Prograf level was still low (2.2). David and I are so much happier tonight without the IVs.

We should have CT scan and GI track biopsy reports tomorrow (Tuesday).

Albumin Transfusion

I forgot to mention that before his scope procedure last Friday, his Albumin was low (2.6) so they gave him an Albumin transfusion which is a combination of proteins taken from many human donors.

Zantac 150 - Antacid

To those that take steroids, does anyone take Zantac (Ranitidine) while on steroids? David's doctor said he should start taking it because he is on steroids. They said with any steroid use, the steroids irritate the stomach and they want him to start taking it. His stomach lining looked great, pink and very healthy. They sounded surprised that he has never had any type of antacid after 1-1/2 years of being on steroids. Please, have any of you run into this?

Now I have to figure out how to give it. It's a liquid, tastes a little minty and awful. Update: I tried his Spicey Cider from Trader Joes, which he takes with all his other clear medications, and he drank it.

I am appalled by the side effects by those who have taken Zantac. Here are personal comments from those people who have first hand experience with it: Click here

Stool Cultures - All Negative

The bloody stools which brought us here in the first place are all negative. They tested for Rotovirus, C-Diff, and a general panel of viruses. They all came back negative.

We are waiting for three things to happen before we are out of here:

1. The intestinal biopsy results
2. The CT scan of his chest on Monday to confirm he does not have a lingering mycobacteria infection from last year (April 06). Note: mycobacteria is very hard to treat because of it's waxy cell wall.
3. His IV meds to convert to oral meds.

He is dying to go home. I think we are shooting for Monday afternoon as long as he continues to do well.

He is eating and eliminating perfectly.

Thank you so much for your phone calls (Rosalind, it was great to catch up with you!), visits, emails, and prayers.

GI Track Looks Beautiful!

Good news! David's GI track looks beautiful. I am a happy mommy! That's not to say he does not have gut GVHD, but just seeing his beautiful insides was encouraging. They took 12 biopsies, some of them are for intestinal viral PCRs. They will test the others for GVHD. We should get preliminary news on Monday or Tuesday. His cultures came back negative - more good news. The plan is, if he shows no GVHD and no positive intestinal cultures, the next culprit is the Cellcept (immunosuppressant). Then they will have to regroup and come up with an alternative to Cellcept. I'm glad we did the endoscopy. The whole proceudre (cleansing) was not that bad. Easy for me to say...I'm not the one undergoing the proceudre. Kids are so amazing!

We'll still be here until Sunday or Monday.

Scripture Dedicated to David's Endoscopy

This scripture is dedicated to David's GI scope procedure: "For we have great joy and consolation in thy love, because the bowels of the saints are refreshed by thee, brother." (Philemon 1:7 King James Version). Amen, brother. His bowels are refreshed!

Let us not live in ignorance, the literal translation of "bowels" means "heart."

Doing Much Better - Still at Stanford

WBC - 12.1 - normal
Hemoglobin - 12.0 - normal
Platelets - 225k - normal
His labs/chemistries look really good!

He is doing much better today/yesterday. He stayed awake all day and was more playful. The craft lady did spin art with the girls. Unfortunately, David has IVs in both arms so he could not hold the paint but enjoyed watching the girls have fun. Child Life brought crafts, stickers, sizzors, and tape so the girls had double fun. It was a good day.

Unfortunately, they want to proceed with the upper and lower GI scope, which is planned for Friday. Two reasons: (1) infections can reside in the intestines which a routine blood test will not detect (2) since he has skin cGVHD which is systemic (meaning, not localized only to the skin but can be a problem throughout his body) his GI track may be affected with GVHD as well. The GI team said they will be able to tell the difference between ulceritis and GVHD because ulcers reside on the surface, while GVHD goes deeper. They will take 8-10 biopsies which are very small (watch the videos, it shows the little arm that take biopsies, it's neat) They are preping him (cleansing his intestines) with a magnesium solution and clear diet. I won't go into how I'm feeling about everything. I'm asking God to be with David and trusting they will not find anything. The next couple of days will be difficult because he will be NPO (nothing by mouth) and they will put him to sleep for the GI scoping procedure. The procedure will happen at 7:30am Friday morning.

For educational purposes, here are two short videos which explain Upper and Lower GI scoping.

Admitted to Stanford

WBC - 19.4
Hemoglobin - 16.0
Platelets - 275k
Weight - 12.9 kilos
Height 36.7 inches

On Monday night he started vomitting. It lasted all night. On Tuesday morning he had two diapers full of blood, which brought me to tears. They admitted him to Stanford. They are giving him fluids and his meds by IV. The labs are pointing to an intestinal bacterial infection. He drank some milk Tuesday afternoon and held down some crackers. After some more milk, it did not sit well with him. Today, Wednesday, he said he is not hungry. He is urinating fine. No bloody diapers as of 8:30am. The nurse waited all night for him to break a fever but his temp resided around 100 degrees all night. They told me we will be here through the weekend. His skin was looking really bad since he could not keep the meds down. After being on IV meds for 12 hours his skin looks much better.

The plan is to:

Hydrate, give meds - done
Take cultures of the bloody diaper and peripheral blood - done
Wait for cultures to grow
Get him to eat and drink
If he has more bloody diapers, then they will do an endoscopy
Keep him comfortable and happy

The girls will be here with me all day. They have homeschool work to do. I hope Child Life will chip in and provide art and craft supplies. Yesterday, the Warriors (NBA Basketball) mascot, Lightening, came to visit David. He had a cheerleader with him. I was totally offended and embarassed by her lack of clothing! Totally unprofessional. What is this world coming to? The girls could not bring their eyes to look at her, they were equally embarassed. I wonder what they have in store for us today...the BMT Playboy Bunny?

Zenopax and Potty Training

WBC - 13.4
Hemoglobin -13.4
Platelets - 320k
Prograf level - 4.5 low but may have been a mistake

His labs are all normal except the liver function numbers looked high, but they told me to give him more liquids and they will look at it again in two weeks.

We went to the Day Hospital. The nursepractioner had ordered IVIG but I reminded them that he had IVIG two weeks ago. He receives IVIG every 4 weeks. The nurse had his IV premedications all ready to administer but I caught it. Please stay on your toes! David's care has become so automatic, I sometimes don't question procedures. It's so very important to stay on guard.

I bit the bullet and started toilet training. He's doing pretty good. Lots of accidents but for the most part he is sensitive and prefers not to make messes in his pants. What a blessing! He is still young enough to have fun with sticker charts which are a huge motivation. He insisted his sisters have a potty sticker chart too. The girls are still at the age to have fun with it, so they agreed. They are so encouraging and helpful to him. He filled up his sticker chart first and declared all day, "I won!" The girls abliged.

Pictures of Trip to Utah - January 2008

MMF Added and Trip to Utah

WBC - 15.2
Hemoglobin - 14.0
Platelets - 307
Weight - 30 lbs
Height - 35 inches
Lym, ABS - 1.55
His labs look great.

He had IVIG and Zenopax today without incidence. But it's not a good day. His skin GVHD has been flarring up so Dr. Argawal wants to add MMF (mycophenolate mofetil a.k.a. Cellcept) which is another t-cell immunosuppressant. I'm going to need God through this. MMF was removed in March 2006 because Dr. Dvorak did not think it was working. The idea is for the MMF to suppress the t-cells (which are attacking his skin) so that the GVHD will get under control then they will start to reduce the steroids. We really need him off the steroids. The GVHD support group has been very helpful and supportive.

On the way home from Stanford David took a long nap. He woke up at 8pm, then stayed awake until midnight. I could not keep my eyes open one more minute. I dosed off then woke up to find that he cut open eight bean bags and dumped the rice on the floor. Yawn! Clean it in the morning. Then this morning while in the bath he dumped cups of water on the floor! Of course, just as I'm rushing around to accomplish other things.

Then he made me giggle. The MMF needs to be mixed with milk but still has an aftertaste. He asked for a regular "milk chaser!"

We drove to Utah for a church event and went skiing in Park City. It was our intention to leave David in childcare. We arrived to find the childcare closed! We had to take him with us. It was such a huge blessing. He cried to go skiing. Once we got him on the skis it was fun all around! He loved the "rides" a.k.a. ski lifts. I figured out a way to ski with him near my side while he held onto my poles. It was the first time skiing for the girls and they had a blast! The excitement did not stop there. On our way home we hit a snow storm and did a 360 and recovered nicely. It was a memorable trip and we are happy to be home in California.

Day Hospital, Zenopax, IVIG and Age of Discovery

WBC 13.4 - normal
Hemoglobin 12.4 - normal
Platelets 407 - high! Those with WAS will understand the exclamation mark!
Lymphocytes, Absolute - 3.23 - this is the first time it's been normal!

We were at the Day Hospital today. The IVIG (Gamanex) went much better. No reactions. His skin looks better but still has residual pink splotches to remind us he is not free of skin GVHD. His scalp still gets really dry but the topical steroid is helping his hair grow back. I keep it very short so the creme will go on better. I think the treatments are helping but we really need him off of the steroids. He is feeling great. No more cough.

He is beginning to read. He can read EXIT and is very excited about that. He is learning his letters and Michael taught him how to write an "A" and "D" in one night. The next day he woke up and started writing. Yesterday he was narcissistic about writing a list. He would not let go of his paper list and pen. He even takes it to bed. Today he is peeling all the paper off his crayons and making a humongous mess on the floor. His sister, Ariella, is teaching him how to use tape to connect objects, so we have objects stuck to tape hanging all over the place. She also taught him how to tie things together, so there are a maze of fabric webs all over the house. It's a great age of discovery!

Poem of Thanksgiving

This is a photo of David when he was one day old.

I included the following poem on David's online birth announcement. I've been reflecting today how much I want my boy back! I just want him to be normal and healthy. After I read this poem I was encouraged by how many of the adjectives truthfully describe David! He has a strong mind and body. He already shows passion, courage, humility, humor and patience. Lastly, God has given me the strength to nurture him!

Poem of Thanksgiving

With all my heart, with all my soul, with all my might I thank You, God, for the gift of this wonderful child. I thank You for a healthy pregnancy, a safe delivery and a speedy recovery.

With all my heart, with all my soul, with all my might I pray for the continued health of this child. I pray for him to be strong in mind and body,to grow steadily and sturdily in a home filled with joy. I pray for him to become a person who greets the world. With passion, courage, humility, humor and patience.

With all my heart, with all my soul, with all my might I pray for God to watch over me and my family. I pray for the ability to love and nurture this child. To provide for him and educate him, To understand him and allow him the freedom to grow.

--Judith Shanks

Bad Cough

David has a bad cough and nose cold. Coughs always scare me because his lungs are so delicate and susceptible to infection while being immunosuppressed. Some serious downward spirals can occur. He's had a lung infection in the past (mycobacteria) so that makes me think his lungs are easy targets.

So he recovered from the IVIG allergic reaction - now he has a bad cough to battle.

He's not breastfeeding any more. I used a pump the other day and got a couple drops of milk out. Sometimes he sucks at night but that is the worse time since milk production is usually greater in the morning. Now that he is not on breastmilk I worry because of GI infections and other immune problems which I believe the breastmilk can prevent. IVIG infusions work great to boost the immune system too...when they don't give him the wrong brand...or when his body does not mount up a defense.

Good night all!

Aimee

Doing Much Better

Thank you for your prayers. We are happy he did not have to go to the hospital. He is eating and taking his meds. His skin GVHD is flarring up though. It might take a while before his skin settles down.

Quote - Simplicities

"Out of intense complexities, intense simplicities emerge" - Winston Churchill

Wiskott Aldrich Syndrome is an intensely complex disorder. What David and our family have withstood is intensely complex. We are thankful for the small things in life. We are thankful when he awakes every morning, runs, talks, and yes...causes us grief.

We are thankful for the intense trials associated with Wiskott Aldrich Syndrome and the bone marrow transplant, for what has emerged is a thankfulness and a gratefulness for the simplicities that so many take for granted.

For those who have children, be thankful for when they walk and talk. Hug and tell your children how much you deeply love them, everyday!

Bad Reaction to IVIG - Again

His labs look good. His kidney function creatine was a little high. The doctor said his Prograf level (10.5) is a little high which could contribute to the high creatine.

Today is Thursday. David went into the Day Hospital on Tuesday for IVIG and Zenopax infusions. They gave him the other brand of IVIG (GammaGuard) which he is allergic to. His lips started puffing up and they went into emergency mode, which is hospital procedure. If you ever have a child whose lips suddenly puff up, do something quick. It means his airways are closing off. They stopped the infusion, gave Zenopax then we went home. They made a note that he is allergic to GammaGuard, so next time he will get GammaNex.

After we got home things got worse. He could not keep anything down. I called the doctor today since he hasn't had any meds either. The Dr. said to give him Benydryl. That knocked him out for another 4 hours. When he awoke he ate and drank some stuff but could not keep that down either. He is up right now (Thursday night about 10:30pm) and had milk, 4 bites of yogurt, and leftover macarroni. He seems more perkey but definetly not himself.

Great Looking Labs and Psychological Assessment

His labs look excellent. I'm not sure what the ABS Lymphocyte was but will check on that. Dr. Dvorak transferred to another hospital so we saw a retiree who is filling in for him. He made the comment that David's skin GVHD looks like acute GVHD, but is considered chronic because of the length of time he's had it. David was not cooperative with the doctors. He let the nurse poke him for the IV though.

Stanford called today to say they want to do a psychological assessment on David. I'm not sure if it is optional. They want to assess his development too. I'm not crazy about this because I'm sure he is developing fine, physically and emotionally. What's messing him up is the steroids. I'm surprised they want to do an assessment while he is on steroids. It doesn't make sense.

For those in similar circumstances, here is an article named "Quality of life and behavioral adjustment after pediatric bone marrow transplantation."

Happy Tofurkey Day! (I'm Vegetarian)

I went to the Day Hospital today...wrong day...the appt was changed to next week. My fault. I had to pick up Septra (antibiotic) at the pharmacy so the trip was not wasted. On the way home we went shopping at Whole Foods. David is more emotionally unstable on steroids, so we got some interesting looks from people when he screamed at his highest pitch and shook the cart with all his might. We were Thanksgiving shopping, so it ended up being a long trip at WF.

David is doing fine but his rash has only improved about 10%. He does not tollerate me rubbing steroid creme all over his body. When I get to his cheeks and nose, being very careful not to get it in his eyes or mouth, he spits and screams. It's been difficult.

He is almost finished breastfeeding. Caput! I'm worried because his tounge developed large white pigment/splotches. I'm worried about mouth GVHD. The doctors tried to look in his mouth but David was totally uncooperative. They had to hold him down and use the tounge depressor which only made him more upset. Breast milk contains a lot of good things, so good that I believe my breast milk played a huge role in the health of David's GI track. I'm not worried about the emotional separation of breastfeeding. It's all about his health. He was only breastfeeding at night, which was really a pacifier to get him to sleep. Lately, I've been encouraging him to breastfeed in the morning when the supply is best. He's been receptive. I'm not sure if there is any milk. I'll pull out my breast pump and see.

We are keeping Thanksgiving at my aunt's house. My grandmother will be there too. In November 2006, we had just came home from Ronald McDonald House. It's all a blur. My only memories are from the photos we took.

Thanks for checking in and for your continued prayers. I hope you all have a scrumptious, marvelous, and superb Thanksgiving!

Would you mind praying for our little toddler friend CJ? He is post BMT and cannot keep liquids and food down. He is in the hospital right now. I would love to see him home for Thanksgiving; eating, drinking and having a merri time with his family.

David's current meds:

Prograf: 5.2ml twice/day

Steroids 3.2ml once/day

Septra 4.0ml twice/day

Folic Acid

Multi Vitamin

Calcium

Zenopax infusion every two weeks

IVIG once/month

Day Hospital, Zenopax, IVIG

WBC - 14.3
RBC - 12.2
Platelets - 321
Prograf - 6.5
IgG level - 678 (low normal)
ABS Lymphocytes 0.9

David's steroids were increased a few weeks ago. The steroids suppress the immune system so they want to keep him on IVIG. They used a different brand of IVIG and gave him hydrocortisone and Benadryl. These measures prevented any allergic reaction. He did fine. He also got a Zenopax infusion.

He is doing pretty good. He's been in good health and active. His skin still feels sand-papery but it does not bother him. His scalp peels a lot so I continue to use the steroid creme on his scalp. I don't use the creme on the rest of his body since the dryness does not bother him.

We continue to expand our horizons. We take him almost everywhere, but always using caution. I'm waiting to hear from the doctors if I can put him in a gymnastics class.

Day Hospital Today - Zenopax only and Labs

no labs yet. will post numbers later.

They pulled a sample for the IgG levels. If his levels look ok, they will think about discontinuing IVIG. We hope and pray.

Zenopax was today. Dr. Argawal came to look at his skin. It is better but feels sand-papery. She said to give the steroids 2-3 months before we see his skin really improve. I have not been using the topical steroids much this past week, to see if his skin would improve with the steroids. It just takes time.

He's been feeling good and very active. He is a very strong little guy and we feel so blessed that he is that way!!!! His vocabulary and understanding of this world is increasing quickly. He asked me last night if he could play a computer game. I told him no. He said, trying to persuade me, "But God says it's good." Perhaps God told him is was good? Who knows? He also hugged me and said, "You're the grapest." We're working on prounounciation.

I got my flu shot

WBC: 10.6
Hemoglobin: 12.2
Platelets: 346k
LYM, ABS: 1.67
Weight: 13.2 k

For the sake of my son's health, I got a flu shot. The rest of the family will do the same.

Thank you for checking on us and praying! Things are not over, David is not 100% recovered, so your prayers are still appreciated. Things could be much worse, so we count our blessings and praise God for the healing He has given thus far.

We're back from San Diego

Our family celebrated the Feast of Tabernacles in Escondido, CA this year. We drove 8 hours south and rented a beautiful clean home in Carlsbad near the ocean. We took a few days before and after the Feast to go to the Wildlife Park, Sea World, Lego Land, and Disneyland. We ate at restaurants too. We washed our hands a lot and were very careful what David ate. He is doing great and had a great time! We ran out of one of his meds so Stanford called in a prescription to a local pharmacy and we had no troubles picking it up!

Expanding Our Horizons

The sunrise looks brighter today. We got cautionary okay to do all kinds of stuff. We can go to Sea World. Any outdoor event where there are people, is okay. We just have to use a lot of hand sanitizer and avoid close contact with other people.

Allergic Reaction to IVIG

WBC 14.8 normal
Hemoglobin 11.3 low
Platelet 350K normal
Weight 28.6 lbs
Height 34.6 inches

Day Hospital today. David is doing well. He received Zenapax (immunosuppressant) and IVIG (to boost his immune system). They gave him Benydryl b/c he has an allergic reaction to the IVIG. This time, he had another reaction (vomiting, lips swelling, coughing, redish rash) which means the reactions are getting worse. They doubled the Benydryl dose, gave him hydrocortisone, and hooked him up to monitors. They assume since his lips swell, the other body parts are swelling, like his trachea, which could cause breathing problems. The hydrocortisone relieves the swelling. The doctors said to continue the IVIG. Bummer. I thought they would discontinue it and let us go home. Maybe after this dose they will discontinue it. I'm finally getting tired of all of this. The pokes, the doctor visits, the emergencies, blood tests, x-rays, infusions, everything.

Iron Overdose

WBC 14.0
Hemoglobin 12.1 - normal! All by itself! Doc says it's not due to iron overdose.
Lymphocytes Absolute - 1.94 (3.0-9.5 normal range) I'm keeping my eyes on this.
Weight 27 lbs.
Prograf level 3.5 - low because he lost a dose last night.

David is in emergency right now (Sunday afternoon). His 6 year old sister knows how to open child proof caps and opened a bottle of pediatric iron tablets on Friday afternoon, which taste like candy. I caught David locked in the girls' room with tablets spewed all over the floor. He was fine, smiling, looking at me like, "I didn't do nothin'." Poison Control takes worse case scenario. There were 20 tabs missing at 7mg each. Not enough to die from, but he vomited 4 hours straight last night and really bad diarrhea. Not good for a post BMT patient! He is doing really good now. He is keeping food down and looks great. He recovered all on his own without hydration or extra meds. They are keeping him here for blood tests, then we should be able to go home. There was also a lot of blood in the diaper (update: I think it might have been iron, which looks red like blood!).

We are working really hard with my 6 year old daughter who has a bad sneaking habit, which is a sin, also called stealing! It could have killed him! It hit home to both her and my older daughter. They were bawling their eyes out to think David was in danger. My older daughter is such a blessing! She informed me of the two other kids taking the tablets, then after a hard night for me last night, she got up and told David stories while I got breakfast and coffee ready. It took a lot of stress off my shoulders. She is such a sweetheart, 100 times over!

We've been here from 10am-7pm. A day wasted for me, but David is fine!

Mitogen Test Numbers

Here are David's T-cell Mitogen (proliferation) numbers after transplant (at 3 years old):

PHA: 118324 (range: >93,000) - normal
ConA: 56168 (range: >76,000) - low but not too bad
Poke Weed: 84355 (range: >85,000) - slightly low, considered normal

Before Transplant (15 months old. I think the range is different b/c of his age):

PHA: 86964 (range: >35,000) - normal
ConA: 11097 ( range: >18,000) - decreased response
Poke Weed: 49509 (range: >17,000) - normal

Blood Drive - August 25, 2007

Dear donor,

In honor of my son, David McNally, who still requires transfusions after his bone marrow transplant, the Stanford Blood Center is holding a community blood drive on Saturday, August 25, 2007 from 01:00 PM to 05:00 PM at South Bay Christian Church, 4250 Kirk Road, San Jose.

For your convenience we are offering the ability to schedule your appointment online by clicking here http://www.membersforlife.org/stanford/mobilesch/Search by city "San Jose" "South Bay Christian Church" then click on August 25. The rest is self-explanatory. You may also contact me for an appointment.

60 percent of the U.S. population is eligible to donate, however only 5 percent do on a yearly basis. Please show your support and schedule your donation today.

Thank You,

Aimee McNally
408-340-0323
Give blood for life! Donate once a quarter.

IVIG, Topical Prograf, T-cell Function Tests (Antigens and Mitogens)

WBC 12.0
Hemoglobin 11.3 - stable! Yeah!
Platelets 330K
Weight 13.2 kilo - gained some weight too


From the labs, he is doing pretty good. Dr. Dvorak is happy about his stable hemoglobin. He cannot say or not if the steroids are affecting the hemoglobin. His steroid does is so low, he thinks it is not affecting the hemoglobin.


He received IVIG and Zenopax today. Dr. Weinberg wants to keep him on IVIG, while Dr. Dvorak wants to take him off. They agreed to keep him on for a few more months.


His skin is still dry and bumpy, not smooth like a child's skin should be. The only smooth parts of his body are the bottoms of his feet and hands. Dr. Dvorak would like to put him on a topical Prograf (he takes oral Prograf). David's skin GVHD does not appear to be systemic, rather, targeting the skin. If we can do a topical Prograf, he would be less immuosuppressed. No definite decision was made so we will continue with oral Prograf.

Unfortunately, he did not have enough lymphocytes to complete the Lymphocyte Antigen Tests (Candida, PPD, and Tetanus). They could not tell if his immune system had the correct response to the antigens. He is too immunosuppressed.

However, two out of three Lymphocyte Mitogen test (phytohemagglutinin (PHA), concanavalin A (Con A), and pokeweed) came back normal. The ConA and Pokeweek came back normal. The PHA came back low.

We have the OK to go back to church!

We won't have much else to say until Aug 22. That's his next clinic visit.

Immunoglobulins - IgM, IgG, IgA

I forgot to post this last week. His immunoglobulins look to be in the normal range, except IgA is a little elevated. Here are the numbers:

IgM .97 (range for 3 year olds: 0.47 - 2.00)
IgG 8.77 (range: 4.41 - 11.35)
IgA 1.79 (0.22 - 1.59)

This was great news! The doctors will discuss taking him off of the monthly Zenopax infusions.

We are still waiting for the T-cell function test to come back. He has a Day Hospital appt. on August 8 for IVIG and Zenopax. Maybe the T-cell results will be ready by then.

+365 One Year Post BMT!!!!!!!

Chem 23 test looks good.

BUN is up to 27 (he's been running about 24 which is on the high end. Should not go over 25) The doctor told me he might not have had enough water the day before. So, my goal is to give him plenty of water the morning before the test.

Prograf Level 6.7 - should be between 7-11. We went up to 4.25ml/twice/day. I think this will do the trick and we might see more skin improvements.

His skin GVHD is still noticeable but it's not bothering him. His skin is dry so I ordered a larger bottle of Emu Oil. Emu Oil has been known to help with inflammation.

We are getting a little better at dealing with the roid rage. The high pitched screaming is hard to deal with. I really need to bring this all to God b/c everything feels so much out of my control.

Forgive my math. There is no way we went from +346 to +365 in seven days. Amy, would you please ask your husband to write a macro for me so I can accurately keep track of what +day we are really on!

I met a wonderful family from England whose son, William, is having a BMT in September 2007. He produces no WAS protein, therefore, has a severe case of WAS. Thankfully, his symptoms have not been severe!Please visit their blog and send words of encouragement: http://www.williamandthebmt.blogspot.com/ Many of us know the difficulties associated with BMTs. Please send them a note of encouragement.

This is David's one-year post-BMT anniversary! As Belinda reminded me, many children do not live past this point. We are thankful, AS EVER, to have a living son who is thriving!

+346

WBC 9.1
Platelets 352k
Hemoglobin 11.4 - is up on its own but I think it's due to steroids...read more...
Weight 12.9 kilo

His steroids were increased to 1.5ml/once/day. The all-over body rash has almost disappeared. There are lingering patches on his legs. The Emu Oil has helped with the dry skin. It's a wonderful oil that soaks in and leaves the skin smooth.

With steroids comes roid rage (Thanks, Amy for that term! I love it! The term, that is, not the rage!). He is bouncing off the walls, throwing things, spitting, screaming, and his tantrums are more intense. Roid rage is one of the most difficult things to deal with. It's hard to find patience and grace. Disciplining him with a pleasant attitude is the most difficult mental and emotional task I have ever had to muster. Many times I just walk away from him because of frustration. His ears and heart are closed off to my instruction. I tell the girls to just stay away from him. That lasts for 30 seconds then they are back playing again. I guess they like torture. Just kidding. Looking at the bright side, we are blessed he is walking, talking, running, and enjoying life.

There is no telling when the steroids will be reduced. I think we agreed that the other immunosuppressants need to be reduced first before steroids.

Life is stressful right now. Our nanny moved on (end of her contract) to better things. The girls are going to miss her A LOT! I'll miss her too, but she doesn't believe me. Ha! She was such a blessing to our family for the past year. David will get his room back and we will have to adjust to "life how it was" before the transplant. I am busy planning our homeschool goals for Fall. Michael and I are working hard to accomplish some personal and family goals before the winter. There is a lot of transition going on which makes life stressful at times.

Would you do something for me today? Today, would you please enjoy your children and tell them how special they are to you. Would you please point out their good character traits and tell them how happy you are with their progress. Praise them! Praise them! Then, would you teach them how to thank God, for it is He who has blessed them with those special abilities and character traits.

+334 Steroids and Prograf increase

The steroids are up to 1.5 ml/once/day

Prograf level was 3.9 (which is low) so the new dose is 3ml/twice/day.

Hopefully, this will take care of the skin GVHD. Once it's under control, we will taper the Prograf first. Give us about 3 weeks.

My current concern is that many years using steroids damage the adrenal gland. Some people are on steroids for the rest of their lives because the adrenal gland does not produce cortisol anymore.

He is doing great otherwise. Eating and drinking well. Since the steroids were increased, I expect we will start to have some extreme attitude problems in the days to come. His vocabulary and critical thinking skills have greatly increased in the past 2 weeks. I'm still breastfeeding which I enjoy some days and other days feel drained.

Thanks for checking in on us!

David in the Local Paper

We traditionally attend a small town 4th of July celebration in Ben Lomond. The local press was there taking pictures. Here is one they published of David playing with a puppy, arms-length away (plus stick).

http://www.pressbanner.com/paper_files/2007/07/11/05.pdf

+332 Day Hospital Today

WBC 13.5 - a little high b/c he has a nose cold
Hemoglobin - 11.0
Hematocrit - 31.6 - low
Platelets - 352K
Weight - 12.5 kilos

He had IVIG and Zenopax today. The skin GVHD has gotten worse, dry, and itchy so we are going up to 4.5mg steroids/once/day. After we get a stable Prograf level and the rash under control with steroids, we will then taper the Prograf. The plan is to taper the other immunosuppressants before tapering steroids. They always like to taper steroids first b/c there are more side effects caused by steroids. In David's case, it's proving to be different, so they will taper the other immunosuppress. before the steroids.

Systemic GVHD

A friend of mine with skin GVHD emailed me a good explanation of what "systemic" GVHD means:

"Systemic just means that his GVHD is effecting or can effect his whole body or "system". GVHD is capable of effecting your entire body and all your systems within it. Just because a person only manifests the disease in say their skin or mouth doesn't mean it isn't present or potentially having effects on other parts of his body. An example of this is that I have had little or no symptoms of gut GVHD, however when they recently did a biopsy of my stomach lining during a endoscopy, there were signs of GVHD present. This is a clear indication that the GVHD is "systemic" or system wide, though only problematic in my skin and connective tissues mostly. Essentially what I think the doctor is saying is that GVHD though only severe in his skin, is quite capable of having effects other ways and in other areas of his body. When you have GVHD you have it "system wide," even though it only perhaps is severe enough to show up in your skin or other more common places."

+329

The 4th of July was a lot of fun. Behind the fun is a lot of work planning and cleaning up afterwards so I was exhausted. The kids enjoyed the fireworks, parade, and home-town celebration. I'm happy the family was together to celebrate. It's been great not having tubies to flush or worry about.

Yesterday, David got sick in All Ears Audio. I had no inclination he was ill, otherwise I would have never left the house. He was okay by dinner time. Perhaps the GVHD has inflamed his GI track.

His rash comes and goes. He looked tired today. Sometimes I look at him and wonder if he is going to survive. The next minute he is laughing and playing. It certainly is a trial and we are not sure if it will end with a healthy, strong boy. We keep praying for healthy blessings because we know God desires to bless us with a healthy family.

+327 Chimerism 95% engrafted all cell lines

The chimerism test came back. He is 95% engrafted in all cell lines. This was great news but it does not exactly mean all cell lines, because his RBC have not converted yet. That's a different topic.

The bad news, his skin GVH flared-up so he is back on 1ml/steroid/every day. The Prograf level was 4.5, so the amount I give him was increased to 2.2m/twice/day. The rash is doing much better today.

Dr. Argawal is calling his sking GVH "systemic." She thinks the lack of his blood type conversion is due to the "systemic" skin GVH. I tend to disagree. We've read reports that talk about delayed blood type conversion when the receipient has a non-myleoablative pre-conditioning. That's what David had. It was less toxic pre-conditioning and with it comes delayed engraftment. Also, I have known people who've had delayed conversion - up to 19 months! I told Dr. Argawal I'm happy with just waiting for the conversion. She agreed...I think. She is a hard person to communicate with. She says one thing but means another. She does not communicate clearly or use enough technical jargon for me. Getting technical information from her is like pulling teeth. It's often too difficult for me, so when the other BMT doctors take over his care (they rotate), that's when I get my questions answered.

+322 - No NG Tube! Horray!

Here's an Emu...before the oil.



It turns out that David agreed to take his meds by mouth. He wanted the tube but when the nurses got it ready to insert he put up a fit. We talked to him and he decided to throw the tube in the garbage and take his meds. When we got home, it was a different story. He would not take them. One of the nurses told me to hold him down and administer it that way. I only had to do that once before he decided it's much easier to take the meds by himself rather than me forcing him down. ;-)

We are still dealing with skin GVHD. I think he will be on Prograf and Steroids for a long time. He gets patches of pinkish dry skin here and there. Sometimes they only last for hours and are gone. The oils work nicely. I've been using Emu Oil and Grapeseed Oil.

+319

On June 22, they took blood for another chimerism test (to test donor cells). Results will come back around July 20.

He is having a red blood transfusion today. His hemoglobin was 8.0.

They will remove the PICC line from his arm today.

He is refusing to take his meds. We've tried to mix them with various liquids but he is not taking them. Dr. Argawal said he will stay on Clarithomyocin (antibiotic for lung bacteria) for at least 6 months. It tastes terrible! I'm so proud he went two months taking it without much complaint. He is at his limit. I tell him they will put a tube in his nose. That threat worked a couple of times. He said he would rather have a tube in his nose. It feels like we are going backwards. He's having his PICC line removed, just to have an NG tube put in.

Here are his current meds:

Prograf (daily)
Prednisolone (every other day)
Folic Acid (daily)
Clarithomyocin (daily)
Septra (daily)
Zenopax (monthly)
IVIG (monthly)

+315 CT Scan Results

The opacities on his lungs have resolved. The radiologist does not see any.

We will stop the IV antibiotics.

His PICC line will be removed from his arm next week!

Please continue to pray for his hemoglobin and blood type conversion. His hemoglobin is down to 8.5. Thankfully he is not showing symptoms but it's concerning me.

Thank you so much for your prayers!!!

+315 CT Scan

I am so proud of my son! He did not need anesthesia! He did not need Ativan or anything to keep him calm! He was peaceful and calm during the entire scan. It was incredible. He said it was fun and wants to do it again. He was fascinated by the machines. Well, I did one thing to calm him...breastfed for very short intervals. He was NPO (nothing by mouth) just in case he could not stay still and needed anesthesia. I was ecstatic that he showed so much self-control. When the scan was over the staff burst into rejoicing. They poured out toys, stickers, and drinks for him. I think they were impressed.

I'll post the results later today as soon as I hear.

+315 - IgG, IgA, and IgM Immune Test Results

Here are the results from his immunoglobulin test. Dr. Argawal would like to see him sustain these numbers. They will discuss moving his IVIG infusions to every 6 weeks instead of every 4 weeks. The numbers are in normal range. Please pray he can sustain them.

IgA - 1.67 (range for 3 year olds: 0.22 - 1.59)
IgG - 6.36 (range: 4.41 - 11.35)
IgM - 0.92 (range: 0.47 - 2.00)

Before BMT:

IgA 0.64
IgG 4.51
IgM 0.20

His immune system before transplant was not too bad. This is why we were not going to do the BMT. It was the brain hemorrhage that made us move quickly towards BMT.

The doctors pay more attention to the IgG because they are the most abundant class of antibodies found in blood serum and lymphs and activate against bacteria, fungi, viruses, and foreign particles.

+313

Big sigh! BMTs have a long recovery.

I don't have much to say today. I'm happy with the rash on his face. It is not worse. I think the Emu Oil is helping.

Tomorrow, Thursday, is CT scan day. No solids after midnight. Clear liquids up until 10am, breastmilk until 11am. We need to be there at 12:30pm. Scan is at 2:00pm. They want to put him under anesthesia. This upset me because during the last CT scan he did fine with Ativan (just made him sleepy but he was awake) and did not move during the scan. They also need to put an IV in his hand because the PICC line in his arm is not adequate for the contrast they need to inject for the scan. One more upset is that I cannot be with him. I was with him last time. The anesthesiologist said they need to concentrate. His last scan was done while inpatient. That's the big difference. The outpatient staff do things a lot differently. Hopefully, I'm upset for nothing. Hopefully, the scan will come out clear and all of this will not matter.

Prayerfully, we move onward...

We celebrated our 10th anniversary in Bodega Bay and Napa. We had a great time. Took a hot air balloon ride over the valley, ate good food, relaxed, and drank grape juice.

+311

He's been feeling under the weather. He's cranky and more tired. He's restless during the night too, but still sleeping through the night. I might take him in today to have his hemoglobin tested.

He has a rash on his face which I think is skin GVHD. Someone on the GVHD board recommended Emu Oil, so I'm using that and it's helping. It has not gotten worse. At first I thought it was a rash from the many strawberries he was eating. I reduced the number to one strawberry per day and the rash is still there. I don't think it is heat related either. The fact it is not getting worse is positive. The doctor is not concerned.

+306

WBC 10.0
Hemoglobin 9.1 - going down slowly
Platelet 321K
Weight 12.8 kilos - very good!

We're at the Day Hospital today for IVIG (immune booster) and Zenopax (immuno suppressor) infusions. I know...boosters? suppressors? Each one does its own work. He had an allergic reaction to the IVIG today so they gave him Benadryl. He's had IVIG many times before and is just now mounting a reaction.

He did not need a blood transfusion. His hemoglobin is low but he's not showing strong symptoms. Dr. Dvorak spoke with Dr. Glader (hemotologist) about David's blood type conversion and why his hemoglobin is consistantly low. It may be the IVIG that is causing the hemoglobin problems. Today, before the IVIG they took blood to check his immunoglobulins and the following:

• CBCD (complete blood test)
• Chem 23 (sodium, potassium, magnesium, etc.)
• Prograf level (immuno suppressant level)
• Direct Antiglobulin (broad spectrum, C3, IgG) - this is the Coombs test again
• Quantitative IgG, IgA, IgM - this is the first time they have tested his immunoglobulins since transplant. This test will give us information about how well his immune system is recovering! This is an exciting test! Although, it may be skewed because of all the immunosuppressor medications he is on. If it looks like his immune system is recovering they will discuss discontinuing the IVIG.
• Amikacin peak (this is the level for the mycobacteria antibiotic)
• Retic Count
• CMV PCR
• EBV PCR

His prednisolone (steroid) was reduced today! yeah! He has a small rash around his mouth but it's not significant enough to prevent a steroid taper. He'll be on the same amount (1 ml or 3.5mg) but will take it every other day. I'll have to make a weekly checklist to help me remember when I gave it last.

+300 DAYS! HAPPY 3RD BIRTHDAY, DAVID!!!

+299 Mycobacteria Chelonae

The bacteria was identified on Saturday, June 2. It's called Mycobacteria Chelonae. There is plenty of info on the Internet. Dr. Argawal said the type of bacteria is a good one to have considering the other species of rapid growth mycobacteria. I think she means it is easy to treat. Take that with a grain of salt. Dr. Dvorak told me it is likely he will be on antibiotics for 6-9 months!

The CT scan of his lungs will happen on June 21. If the results show the nodules on his lungs have resolved, they will pull his PICC line and start oral antibiotics - those oral antibiotics will go for 6-9 months. The infectious disease doctors (the bacteria is not infectious by the way) want to see a reduction in his immunosuppressors before they decrease the amount of time he will be on antibiotics.

He's been doing fine on 1ml of Prednisolone (steroid/immunosuppressor). No rash or GVHD.

Thank you for your prayers! We are so happy David is still with us to celebrate his third birthday! By the grace of God, He helped me create a special birthday video for him. I did not have a manual for the software and I had no clue what I was doing. I put it together in one day. I hope you enjoy it.

Camping May 2007

Michael took the girls camping. They had a blast! I met them for a potluck. I'm not ready to take David camping overnight with all the dirt and fungi. For those not in California, the trees in the background are really red. They are called Redwoods. Giggle!

Steroid taper update: So far so good. No rash on his skin.

This is WEEK #4 on antibiotics. Two more weeks to go!

+290

WBC - 6000

Platelets - 316k

Hemoglobin - 10.0

His labs this week were fine. The Prograf level was 4.8 but nothing was changed. He's been tolerating the Amacaycin antibiotic just fine without kidney or liver problems. We give him a lot of water. The lung bacteria has still not been identified! I called Dr. Lewis the infectious disease doctor and he cannot explain why it's taking so long except it takes a long time for bacteria to grow.

+278

WBC - 9300
Hemoglobin - 12.2
Platelets - 309K
Prograf level - 7.8 - perfect
Amakacin level - within levels
Weight - 12.1

David's been doing pretty good. His blood test last Monday was all great. His magnesium levels have been good too. They are monitoring the Amakacin levels and blood tests because Amakacin is hard on the kidneys.

Dr. Argawal saw him today in the Day Hospital while he was getting his monthly IVIG (immune boost) and Zenapax (immuno suppres.). She said the Acid Fast Bacilli has not been identified yet. It's considered a RAPID growing bacteria, they sent it out for RAPID identification, but there is nothing rapid about its identification.

She said to reduce the steroids to 1ml/once/day! Yeah! Michael will be happy about this news. We'll do another taper in 2-3 weeks if all looks good.

The plan is to go to the clinic for the next three Mondays for blood draws (checking Amakacin levels). Then on June 11, he will have IVIG and Zenapax infusions in the Day Hospital.

Image of Bone Marrow

Article on Mycobacteria Post Transplant

This is an interesting article. It appears that mycobacteria post transplant is rare. Out of 2000+ patients, 7 of them had rapidly growing atypical mycobacteria in their central lines. This article was written 10 years ago. I'm not sure if the incidence has increased. There is not much more info.

http://www.nature.com/bmt/journal/v19/n5/pdf/1700686a.pdf

Today Was a Good Day

It was a very good day. We wrote up a schedule for David's meds. I got his IV antibiotic going.

Many people contributed to an outstanding birthday for me! From champagne breakfast, to flowers, to gifts, to cards, to strawberry crepes for dessert. Thank you all for making my day incredibly special!

David is doing well. He gets the IV antibiotic 3 times/day. He says, "Medicine again?!?" He's such a good boy. He takes everything we give him without much fuss. I mix the white colored meds with soy milk and the clear looking meds with Trader Joes Spicey Cider. He says the Septra tastes good. My daughter wanted to taste a little so I put a drop on her finger. She said it tasted good too. I disagree.

My kids are really getting into Mother's Days. I can't wait to see what they have prepared for Sunday!

We're Going Home

They are releasing us. The bacteria has not been I.D. yet. He got a blood transfusion b/c his hemoglobin was 8.5.

+272

The bacteria I.D. has still not been done. I am begging to go home. Tomorrow is my birthday and I really would love to be home for the weekend.

+281 - Coombs Tests

The Indirect Coombs test was negative. The Direct Coombs test was positive. This does not mean he has Coombs b/c both the Indirect and Direct have to be positive to be Coombs. He does not have a super bad case of anemia. Those with hemolytic anemia have sharp drops in hemoglobin and are really sick. Dr. Glader, David's hematologist, is reviewing the information. Dr. Dvorak explained that his red cells have antibodies on them. They should be able to wash those antibodies off, but they can't. They are not sure if it's because the antibodies are so small or if there are not enough to wash off. They said this is very unusual, not to be able to wash the antibodies off. This is why the Indirect Coombs test is negative. I'm not sure I have all this information correct, just for the record.

+280

David and I have been through a lot the past four days. The bacteria species has not been identified yet. The general family of bacteria was identified but they had to send out the specimen for rapid identification. It's not rapid by any means. We're all working according "bacterial" time. They will not release David until it's been identified.

Then comes the placement of a PICC line in his arm. This infuriated me because they do not even know the bacteria, yet they "think" he will need IV antibiotics when he goes home. Fine. But why put the PICC line in BEFORE you know!?! I was so upset. Normally, I'm very easy to get along with, but the past two days has been difficult to keep a smile on my face. Everyone who knows us is taken by surprise and takes it personally. It's hard to remain in good spirits when your child is poked unnecessarily. So now I have to do more line care at home, administer IV antibiotics, clean it, and if he gets a fever it's back to emergency. Big sigh!

The good news is that David is doing very well. He is not sick. He did well with the PICC line and anesthesia (again!). He is such a joy to everyone who comes in contact with him. The little phrases he says to the nurses are so cute. He makes us all laugh so much. Right before going under anesthesia he told the nurse he wanted a sandwich. He did so well fasting for 14 hours.

Red Blood Cells and Coombs Test

I forgot to mention the red blood cell situation.

The test they did to see if he is launching antibodies against his rbc is called the Coombs Test.

Dr. Weinberg said they are going to repeat the test b/c the results that came back showed that he is launching antibodies to IVIG. He said this did not sound right so he is requesting it be retested.

+277

I'm doing a little better today after talking to the doctors. Thank you for your prayers.

The doctors need a couple of days to consult with each other to come up with a treatment plan. I'm sure we will go home this week, maybe on Wednesday. Please pray the Environmental Mycobacteria is sensitive to the current antibiotics he is on. If it is resistant then he will need a PICC line in his arm so we can administer IV antibiotics at home. Please pray!

They do not want do a lung biopsy! Whew!

They are very happy that he has been fever free for two days! Funny thing is that he was anointed on Friday night and did not get a fever that night or Saturday night.

The hardest things we deal with is giving the meds and having his arms poked for blood draws.

Current meds. This is awful. Brace yourself.

Ciprofloxacin (antibio. for lungs)
Amikacin (antibio. for lungs)
Vancoycin (antibio. for staff infec.)
Prednisolone (for skin GVHD)
Prograf (immunosuppres.)
Clarithroycin (antibio. for lungs)
Septra (antifungal for PCP)

Not to mention the monthly stuff:

Zenopax
IVIG

+276 Lung Nodules and Red Blood Cell Destruction

WBC 7,800 - normal
Hemoglobin - 9.1 - not bad for all the blood draws
Platelets 239K
Weight 11.8 - stable

Happy National Scrapping Booking Day! I love passing the time by looking at the layouts at www.scrapbook.com. Most of the layouts are family focused and positive. They perk me up and make me laugh.

Were still here at Stanford. Been here all week. Not sure when we are going home. I'm beside myself right now. Please pray for David. His CT scan came back with three nodules on his lungs. The doctor just showed them to Michael and I. They don't know exactly what they are. The Atypical Mycobacteria that he has does target the lungs. It could be this. It could be fungal, which means a whole different set of antibiotics. They want to do a lung biopsy. The biopsy is done by inserting a tube down the throat which is better than opening him up. I'm in tears right now. I'm not sure how to handle this. What part of his body are they NOT going to touch? I'm not sure I can hold myself together tomorrow when the doctors do their rounds.

After a good cry, I'm ready to continue with the other piece of bad news. His body is in fact destroying his red blood cells, so that's why he's had anemia. Why the doctors waited so long to figure this out is beyond me. I told them months ago about another boy we know who was in the same circumstance. They kept repeating the reticulicite test and it would come back normal. Dr. Weinberg ordered another type of test and it came back positive. David is launching antibodies to destroy the red blood cells. They will put him on another medication for about 6 months to stop the anemia. Yes, it will take care of the problem, however, we will not know for sure until the next generation of cells grow, which takes another 6 months. He will be very susceptible to infection, like a wild fire in the dry woods.

Now it's all fitting together why his doctor said it would be another year before this is all over. Thankfully, David is doing very well. He does not have any symptoms. No fever, no coughing, no nothing. We can rejoice in that! It's hard to imagine so many things are going wrong week. I'm happy to report that he is active, eating, talking, silly, laughing, and so sweet. Please pray God gives us peace and gives me strength to face the doctors tomorrow without crying my eye balls out!

Thank you for reading!