David McNally - Wiskott Aldrich Syndrome, Bone Marrow Transplant, and Graft-vs-Host Disease

A Season of Thanks!

2011-11-10T12:29:00.001-08:00

Stationery card

View the entire collection of cards.

2011-10-30T19:30:00.000-07:00

Our first meal after arriving in Germany. Very spicy Thai food.

An apfel a day keeps the doctor away!

Heidelberg, Germany

The indoor pool in Schluchsee where we spent most of our time (Black Forest)

Lake Schluchsee in the background.

David loved jumping into this ball pit.

Years ago he would not be allowed to do such a thing for fear of bleeding.

This wild park playground was so much fun! He was brave to get on this zip line.

Being silly.

The boys

More positive changes for David

2011-10-28T11:37:00.000-07:00

We returned from a 3 week trip to Germany & Switzerland. David did fantastic! No sicknesses, no throwing up, lots of energy, eating well, having lots of fun, and enjoying the chocolate. Our trip experience was a blessing beyond understanding.

During our trip we were able to stop the Diamox for the swelling around this eyes because the swelling is gone.

He is down to two medications:

Levothyroxine for the hypothyroidism
Septra antibiotic for preventive medicine

Thank you all for checking in on us and for praying for David.

Aimee

He Grew!

2011-10-28T10:41:00.001-07:00

Whooo! Hooo!

David has grown 2-1/2 inches since he was taken off steroids in May 2011!

+1878 Lost His First Tooth

2011-10-07T22:48:00.000-07:00

Oh, happy, happy, joy, joy! David lost his first tooth while on a 12-mile hike from Big Basin to the Sea. We did not expect this to happen so soon because his growth has been stunted by 5 years of steroid use. He also has a molar coming in.

Exciting times around here!

Eyes Looking Great!

2011-10-07T08:29:00.000-07:00

I can't believe it's been 4 months since I last posted an update! Where does the time go?

David saw the eye doctor 4 weeks ago. The swelling around his optic nerve is almost resolved! He is on a Diamox taper and should be finished by October 17th. He is still taking Septra (antibiotic) and Levothyroxine (thyroid). His cortisol level is still on the low side so we will repeat it in about 4 weeks.

He is growing about 1cm/month. He's eating well and feeling good.

I have him in a homeschool choir and sports day. He's having a hard time integrating into social life but I have hope it will only be a matter of time before he is fully participating and making new friends.

I can sense this is going to be a great year for him. I'm so grateful, beyond words can express, for his healing and his restored life. Life is so much better for him and the rest of our family. I know it's only by God's healing hands, the prayers of many faithful brethren, and fasting, that he is still with us today.

We are off to Germany!

+1745 - Last Dose of Oral Steroids

2011-05-27T13:02:00.000-07:00

We are so happy to announce that May 25, 2011 was David's last dose of oral steroids.

Current meds:

Diamox (for eye pressure reduction) - 2 tabs/twice/day
Levothyroxine (Hashimotos Thyroditis) - 1 tab/once day
Septra (preventive antibiotic) - Mon/Weds/Fri twice/day

He still need topical ointments for his skin still flares up if we don't stay on top of it.

David saw the eye doctor (Dr. Frederick) last week. He thinks he sees drusen. Drusen is interesting.

We are heading to the NIH.

Until next time....

Tie Dye at our homeschool End-of-Year Picnic

After church

David, Adira & Jonathan at LegoLand in April

Adira, David, Ariella in front of the Star Wars sand sculpture.

Michael and David

Adira, David and I on the Coastersaurus

Almost as tall as R2-D2

The boys watching the quail eggs hatch

Aimee

+1716 - No more steroids (prednisolone) now on hydrocortisone

2011-04-28T12:52:00.000-07:00

We stopped the prednisolone steroids and are giving him the oral hydrocortisone called Cortef, which is similar to the natural hormone produced by the adrenal glands. They want to know if he feels like vomiting because that could mean a steroid crisis. Dr. Partap is handling the taper because of his papplidema.

The next six weeks on Cortef:

1-1/2 ml, 3 times/day - for two weeks
1ml, 3 times/day - for two weeks
.5ml, 3 times/day - for two weeks

Current meds:

4ml Septra - twice/day/mon/weds/fri
Cortef (see above)
Diamox - two tabs/twice/day
Levothyroxine - one tab daily

He saw Dr. Agarwal and Dr. Partap today. Dr. Partap looked at his eyes and did not see any significant changes. Dr. Agarwal saw his skin. His hands and face are a little flared up because we went to Lego Land (April 20-24). He was in the sun a lot. I'll post some photos.

Fertility

2011-03-08T13:12:00.000-08:00

I posted some fertility-related links. There are some trials and experiments going on to preserve a child's sperm or egg stem cells and replant them after chemo. Thanks for the article Sumathi.

Article - March 7, 2011 that talks about preserving fertility:
http://hosted2.ap.org/txdam/838a15821ce1419a9a5dd3877fd2951e/Article_2011-03-07-HealthBeat%20Cancer%20Fertility/id-f744320051f349eab4d8e19f5173f164

Fertility Organizations:

Fertile Hope
Fertility Preservation Program of Pittsburgh
My Onco Fertility

+1680 - "Is this all I take?"

2011-03-05T21:21:00.000-08:00

"Is this all I take?" These are the words I have been waiting to hear from David for a long time! Tonight he only had to take one diuretic, and steroids. That's it! Could it be? Help me with my disbelief!

He came home from the hospital on Friday. The past two weeks:

1. Spinal tap to check the spinal fluid pressure because of the Psuedo Tumor Cerebri. He was poked three times. The first no reading. The second had an opening pressure of 15 which is normal but the doctor did not believe it. The third poke also showed an opening pressure of 15. Good news is that his spinal pressure is normal! Yeah! So the eye doctor will just be checking the swelling to see if it's going down.

2. Spinal headache

3. Strong pain meds for headache (Tylenol with Codine did not help much so we went to HydroCodone which is three times as strong.)

4. In-patient for headache for 2 days, goes home

5. Feels good so we go to church and San Francisco to celebrate Ariella's birthday

6. Next night, he throws up, very bad diarrhoea, and headache again

7. I give more pain meds and hydrate

8. BMT docs want to admit him.

9. He stays three nights at Stanford. They diagnose him with a GI bug that is not serious and rebound headache. The doctors stopped all pain meds, we used ice packs, sucked on ice chips, and hydration.

10. Back home and doing pretty good.

Meds:

.45ml - prednisolone twice/day

.2ml - prograf once/day

2 Diamox pills twice/day

4ml Septra - Mon, Weds, Fri (this is a new change. He used to take this antibiotic every day)

Hydrocortisone ointment - all over body once/day

Desonide ointment - on face once/day

Memetosone ointment - hands and feet once/day

He will see the opthamologist on March 17 to check the status of his Psuedo Tumor Cerebri . We are hoping it is resolved or looking much better. The swelling could lead to blindness.

Things are looking better even though he has occasional set backs. We were told if he ever gets off immunosuppressants, it will be another two years before his immune system is fully functioning.

Thanks for your prayers and encouragment!

-Aimee

+1669 - Day 5 of Spinal Headache

2011-02-22T12:14:00.000-08:00

David had the lumbar puncture on Thurs, February 17 to check the spinal fluid pressure. The pressure is normal (15) yeah!

Afterward, he was admitted for 8 hours for observation. He went home on Friday. Today is Monday, Feb 22 and he has had a spinal headache for 5 days. All he can do is lay flat. Sitting up hurts too much. He was prescribed Tylenol with Codine but that did not help the pain. He's been taking Hydrocodone (substitute for Lortab), usually 4-8mls every 6 hours and it's helped the pain. He is playing games on the iPad and my cell phone. Angry Birds is his favorite game. He is eating, drinking, and eliminating ok. They Hydrocodone makes him constipated so I bought an OTC tummy helper. Still, sitting up to go to the bathroom is pretty painful. The neurologist recommended caffeine, so I bought him a coffee frappaccino at Starbucks. I had to add about 2 cups of vanilla ice cream to disguise the coffee flavor. I'm sure he is not getting much caffeine. Hopefully, he will be up and about soon!

+1660 Eye Pressure

2011-02-13T10:37:00.000-08:00

First, David has a couple of doctor appointments this week. On Tuesday, he is seeing the ophthalmologist to check the pressure around his optic nerve (please pray he cooperates with the eye drops). His last appointment, 4 weeks ago, showed very little improvement, so they increased the diuretic. On Wednesday, he is seeing the neurologist. If his eyes have not shown improvement, she wants to do another spinal tap to check the spinal fluid pressure. All of his doctors are worried because he could loose his vision. Some of you may remember the last time he had a spinal tap, he had a severe spinal headache requiring morphine. As you can imagine, I am nervous and worried. The last spinal tap was done by a male neurologist, so his doctors are reassuring me that the female neurologist is much better. My daughters are also nervous for him because the last episode was very traumatic (we honestly thought he was dying). Please ask God to give David, my daughters, and Michael and I peace of mind.

Second, David's skin has improved but not enough in the last two months to taper the steroids. We really need him off of the oral steroids, which is one cause of the optic nerve pressure. He has a new doctor that wants to try a new medication, which I am not happy about. He is looking really good but we have determined that he cannot have ANY sun. Sun sets him back. They want me to apply the skin ointments twice/day which has been difficult for me to keep up with. I know this regimen would help, so please pray for God to give me the time and energy to keep up with what David needs.

Third, David was tested for Celiac Disease (gluten intolerance). When a person has one autoimmune disease (he has Hashimoto's Thyroiditis) often they will have another. He has a secondary marker for Celiac, not the primary marker. He is considered "borderline" Celiac through a blood test but will be tested for genetic markers soon. He does not have any symptoms so his gastroentronologist said not to make any changes to his diet. That was good news. They will re-test his blood in six months to see if anything has changed.

Lastly, David is in first grade this year. I am working on his reading and math skills. He loves science, building with Legos, and wrestling with his baby brother. He is a bright boy. God has done mighty things in his life, which we are very thankful for.

Thank you for your prayers, cards, and concern.

Add caption

Bike ride in Santa Cruz

Ice skating at Winter Lodge

Winter Lodge fake bubble snow

Aimee and Michael, and especially David

+1629 - Hashimoto's Thyroiditis

2011-01-12T10:07:00.000-08:00

THYROID: In December, David's Thyroid Stimulating Hormone (TSH) was 33 (normal range is .34-4.82). Dr. Suchet increased the Levothoroxine dose to a full tablet/once/day. Good news: there are no nodules on the thyroid. On Jan 3, Dr. Suchet, said his TSH was 4.0. She said it is coming down, so no changes to Levothoroxine is needed. She will retest the TSH in February. She would like the TSH to be 3.0 or less. His thyroid antibody number was 1,000. This is very high but there is nothing one can do. His new diagnosis is Hashimotos Thyroiditis.

CELIAC DISEASE: Dr. Suchet said he has a secondary marker for Celiac Disease. I do not have any more info on this. He does not have the primary marker, so no worries at this time.

OPTIC NERVE PRESSURE: He's been taking Diamox pill twice/day. It's a diuretic. It should help relieve the pressure around his optic nerve. He will see Dr. Winton on January 18.

BMT DOCTORS: His doctors at Stanford have been very pleased with his skin condition. They did not make any major changes to his steroids yet. We have been keeping him out of the sun so I think that has helped a lot.

HIS CURRENT MEDS:

.45ml prednisolone - twice/day (steroid)
.02ml prograf - once/day (immunosuppressant. This dose is so low, levels are not taken)
1 tablet Levothoroxine - once/day (thyroid hormone)
1 tablet Diamox - twice/day (diuretic)
4ml Septra - twice day (antibiotic)
Topical ointments (I try to apply these daily, but it works out to be every other day) - Memetosone on hands, Desonide on face, hydrocortisone on body, and Triamsinolone on head if needed. His doctors would like me to apply these twice day/everyday. I'm sorry, it's just not happening.

SCHOOL AND LEARNING: David is in first grade. He is making progress with reading. He can do addition and subtraction equations (he likes to set up the beads himself and solve them). He loves science! He has lost some interest in Legos (he does not build for hours like he used to) because he wants to learn other things. Adira started to give him basic piano lessons. He likes to help me cook (more science lessons). His best friends at church are no longer attending but he does not realize it yet. I feel so bad for him. It's hard to make friends when I cannot set up play dates that involve outdoor activities. I will do my best to set something up for him indoors. I hope to enroll him in a gymnastics or karate class soon.

TRIPS: In December, we took a car trip to the Pacific Northwest and went skiing at Mt. Hood. David took his first ski lesson with Mogul Busters. He had a great trip!

OTHER WAS FRIENDS: There are more families going through BMTs, or soon to be. There are friends whose son's have really bad GVHD. All I can do is pray for them. Some boys seem to "fly" through the BMT process while some come out with GVHD or long-term health problems. We hold up hope for all of these boys and keep them in our prayers for complete healing.

Thank you for checking in on David and for keeping him in your thoughts and prayers.

-Aimee

Michael and David at the Oregon Science Museum, Portland. A fun vacuum and ball center.

The Grahams and us at the De Young Museum, San Francisco, where we saw the Post-Impressionists.

Near the Legion of Honor Museum, San Francisco. We went letterboxing right here. The Golden Gate Bridge.

Michael holding David and Jonathan on his shoulders with his brother Steve at Bob's Red Mill, Portland, OR.

David playing in the snow after we arrived at Mt. Hood, Government Camp, The Lodges, Washington.

David with his Mogul Busters bib on.

David's first ski lesson. December 2010.

He found this awesome icicle.

+1586 Italy, Eyes, Thyroid, Tapers, and Anti-fungals

2010-11-30T16:41:00.000-08:00

A lot of things have happened in David's life during the last 2 months 19 days since I last posted.

Sept 18 to Oct 3 - Destination: Italy - Oh, how we loved thee! David did great during the whole trip except on the last night we thought he was going to start vomiting. We prayed and watched him carefully. He slept and woke up ready to board the plane back to the USA, without throwing up once! Whew! No headaches during the entire trip. He ate like a champ! Traveled like a champ (Venice, Florence, Rome - twice, Saubaudia, and Sorrento)!
Oct 7 - Destination: Dr. Winton, Ophthalmologist - She dialated his eyes and took photos. They look great. Looks like the swelling is going down.
Oct 7 - Destination: Dr. Karabana, BMT - She saw David, was pleased to hear about the eye report, checked his skin, and tapered his steroids to .45ml/twice day.
Oct 13 - Destination: Dr. Van der Kam, Dentist - Teeth cleaned. She says he has Geographic Tongue, not GVHD of the tongue. Geographic Tongue looks like a map, and comes and goes. It could be caused by his immune suppression and antibiotics, which are killing off the good bacteria that would normally fight the growth on his tongue. Seems harmless but bothers him occasionally with acidic foods, but he still likes orange juice and tomato juice.
Nov 9 - Destination: Dr. Winton 4-week checkup - UTOH! His eyes look worse than before his inpatient episode right before Italy. We'll see her again around Dec 9.
Nov 9 - Destination: Dr. Agarwal, BMT - Back for a check-up and discuss his eyes. The pressure in his eyes looks worse, but he is not experiencing headaches and vomiting. She tapers his PROGRAF to .4ml/twice day. It's such a low dose that they do not monitor his Prograf levels any more. She says to call if his skin flares up. It's been 3 weeks - no flareups!!!! She wants him on another anti-fungal, but I requested we wait until the pressure in his eyes resolve.
Nov 22 - Destination: Dr. Suchet, Endocrinologist - She talks quickly and with an accent. I gather that his brain (pituitary gland) is functioning properly because his Thyroid Stimulating Hormone is functioning properly. She wants to look at the T4. She orders a thyroid panel. We'll have it drawn on December 2nd and post results. She mentions Hashimoto's Thyroiditis.
Nov 30 - Destination: Ultrasound in Santa Cruz - Hmmm. The technician asks if anyone has mentioned Hashimoto's Thyroiditis to me? Yes, Dr. Suchet did. So, he may have inflammation of the thyroid. Once the thyroid panel and ultrasound results are interpreted by Dr. Suchet, we will have a follow-up appointment. We talked about growth hormone. Growth hormone can cause increased eye pressure so she does not want him on growth hormone until the pressure in his eyes resolve.

Thanks for checking on us!

-Aimee

David's Geographic Tongue

Super Bad Headaches from Lumbar Puncture

2010-09-14T23:58:00.000-07:00

I rushed David to emergency on Monday because he obviously was having a lot of head pain. We were actually going to LCPH to get admitted to the BMT unit. But 10 minutes from the hospital he started to throw up blood! I freaked out. I forgot about all the WAS mom's out there whose son's often get nose bleeds and end up vomiting blood. We drove to emergency instead. He perked up, had a CT scan, and got admitted to the BMT unit.

Nothing again on the CT scan. Just limited CSF (cerebral spinal fluid) and two constricted vessels. They think his spinal fluid has not equalized yet after the Lumbar Puncture. OR...which we hope not...he has a spinal fluid leak and needs it patched.

We are supposed to go to Italy. We are hanging on the edge of our seats and getting on our knees wondering what God has in store.

He is doing better with a small, constant dose of morphine. He's never had morphine so now I cannot say that anymore :-(

Voriconozole (anti-fungal) lead to....the increased spinal fluid which lead to....the lumbar puncture which lead to....the very painful spinal headache. Urg! It's days like this where I dislike medicine, tests, and interventions.

Thanks for checking on us!

+1506 - Idiopathic Inter-cranial Hypertension (IIH)

2010-09-11T09:33:00.000-07:00

David's new diagnosis is Idiopathic Inter-cranial Hypertension (IIH).

Breaking it down, this means:

Idiopathic = they do not know the cause

Inter-cranial = inside David's brain

Hypertension = pressure inside the brain

This is also called Pseudotumor Cerebri but there is no tumor, thus the name "pseudo."

On Tuesday, Sept 17 he had an appointment with the ophthalmologist. She saw Papilledema or swelling of the optic disc and reported it to Dr. Agarwal in LPCH. Dr. Agarwal wanted to admit David immediately.

At LPCH they did a CT scan and a MRI. The results only showed excess cerebral spinal fluid. Next they did a lumbar puncture. He did well during these procedures. He did not need sedation for the CT scan. For the MRI he was put to sleep and told me the drugs made him feel weird; he did not like the feeling. For the lumbar puncture he was sedated, but the pain in his back and headaches afterward were hard on him. The results of the lumbar puncture showed he had a spinal fluid pressure of 47 (normal for a child is 15).

He was taken off Vfend (the anti-fungal) and put on a diuretic called acetazolamide (Diamox).

The doctors do not know exactly why he is producing excess spinal fluid. They said prolonged steroid use, or the Prograf. They don't know exactly.

Current meds:

Prednisolone - .45 ml/twice/day

Prograf - .7ml/twice/day

Septra - 4ml/twice/day

Diamox - 7ml/twice/day

Isradipine - 1ml/twice/day

Catapress patch - 1 patch per week

Unfortunately, his skin GVHD started to flare-up after the MRI. We don't know if he is having a reaction to the anesthesia meds or if it's a real GVHD flare-up. The doctors will keep a close eye on his Prograf levels and we hope the flare-up will subside.

One more week until we leave for Italy!

Thanks for checking up on us.

+1495 Possible Fungal Infection and CT Scan?

2010-08-31T23:47:00.000-07:00

David has been getting headaches on/off for about 6 months according to his nurse (I've lost track). He's also been vomiting usually associated with the headache. His Prograf level was 9, so it was normal, not toxic. His nurse said the doctors are concerned about a possible fungal infection in his brain, so most likely he will have a CT scan soon. They will also do blood work. I told David about the scan and how he would have to stay still. He thought that was better than taking medicine to go to sleep.

In August we took a trip to Oregon and Washington. David did great! There were times his tummy did not feel well, but he had a nice time. We threw rocks in the Columbia River and he got three new Lego creations from the Atlantis series.

Many people have told me how good David looks. He's not as puffy. He's eating pretty good esp. after Michael bought us a Vitamix. David keeps requesting orange drinks (fresh orange, ice, yogurt, and I sneak in some protein powder).

No other changes or updates. Thanks for checking on us!

Wiskott-Aldrich syndrome protein research

2010-07-26T17:12:00.000-07:00

+1455 Doing Pretty Good

2010-07-22T12:17:00.000-07:00

Time flies. We took a road trip to Yellowstone (May 28-June 12) and we all got a fast GI stomach bug. David ended up in the ER in Bozeman, Montana for about 12 hours for observation. We all got through OK but David was nauseous most of the car trip. He took an anti-nausea pill that helped a lot.

Some BIG news...He turned 6 on June 7th!

We've been home enjoying the summer without many activities planned.

David's skin looks good as long as I keep applying the topical ointments. His hands and face are the most involved and even the ointments don't work 100%, but he does look better. The rest of his body looks good. His blood pressure has been fantastic! I cannot see any signs of growth, except he said his shoes (the pair he has had for 3 yrs) are tight! He has lost weight, now he's 35 lbs. He's not eating as much as I would like. He is so picky; some days it drives the whole family crazy.

We had some insurance issues so he has not seen the endocrinologist yet. His nurse coordinator at Stanford is trying to get him in earlier than October.

Current meds:

Levothoroxane - 1/2 tab daily

Prednisolone - .6ml/twice/day

Prograf - 1.0ml/twice/day

Vfend - 2ml/twice/day

Septra - 4ml/twice/day

Isradipine 2ml/twice/day

Catapress patch - whole patch/once/week

Multi vitamin

Omega Oils

Calcium/Magnesium/Zinc supplement

Thank you for checking on us and for your prayers. Things are looking better and we hope it stays that way when school starts in September.

His Lego table where he spends hours of time.

I don't know the name of this. It's a Star Wars Lego kit he received for his 6th birthday.

He wanted me to sew some camo pants for him.

Random Lego creations that he loves to photograph.

+1400 Hypothyroidism and Skin Update

2010-05-27T13:52:00.000-07:00

On Weds, Stanford tested David's thyroid hormones again. Unfortunately, the TSH came back high again at 26 (normal is 6). The T3 was borderline low. The T4 is normal at 1.4.

Dr. Karabana speculates that this is an autoimmune problem that is not uncommon for WAS patients after transplant. See article.

Other tests that were done to confirm autoimmunity were: Antithyroglobulin antibody (TgAb), and the Thyroid peroxidase antibody (TPOAb). The TgAb tested positive, which indicates autoimmunity. The TPO was normal.

Since he tested positive for TgAb, he will need to take 1/2 tablet of Thyroxine each day for about 1 year. The positive aspect of this is that he might start to grow! Hypothyroidism can effect growth and development, so maybe this is an answer to his growth problems.

He has been on .6ml prednisolone for 30 days. His skin is showing some signs of flareup. His Prograf level was 4.5 which is a little low. We are increasing his Prograf to 2mls/twice day, and I will be apply more topical creams to his skin to help control the GVHD. I will do this for one week and see what happens.

Thanks for checking on us!

+1390 Steroid update

2010-05-17T08:42:00.000-07:00

David has been on .6mls or 2mg of prednisolone for 19 days. I have not seen any flareups. His red face and peeling hands are not improving but are not getting worse. His overall body composition is less swollen and less cushinoid. He's been feeling ok, but his appetite and diet are not that great. He seems to enjoy one large meal per day. His next appointment will be on May 26th. Thank you for checking on us and for your prayers.

+1372 Thyroid, Hemoglobin, Steroid Taper

2010-04-29T13:21:00.000-07:00

Thank you for praying for David and our family! I received fantastic news today and I'm sure you will rejoice with me!

Miracle #1: He saw the cardiologist and does not have excessively high hemoglobin, or polycythemia! His hemoglobin his on the high side of normal but it's fine.

Hemoglobin - 15

Miracle #2: His thyroid hormones were retested at Stanford and they were all within normal range!

T3 - 83

T4 - 1.2

TSH - 4.51

He is scheduled to see the endocrinologist, Dr. Bachrach, in July, 2010.

Miracle #3: He received another steroid taper! He is on 2mg/twice/day or .6mls. He will go back to Stanford in one month. If his skin does not flare up, they will take him off the more powerful steroid (prednisolone) and convert him over to a more natural steroid called hydrocortisone. I ask that you please continue to pray for him. The steroid tapers are really hard for him because he looses his appetite and energy. Although, God has blessed him with amazing energy even when he is not feeling well!

In four weeks when he has his next appointment, they will test his immunoglobulins again. If they are within normal range, they will remove the IVIG.

Thank you for checking on us!

Aimee and Michael McNally and especially David!

Current Medications:

Isradapine - 2.5mls/twice/day for blood pressure (111/70 - not bad!)

Catapress patch - 1 whole patch/once/week

Prograf - 1.6mls/twice/day (level was 8.6)

Prednisoline - .6ml/twice/day (2 mg)

Septra - 4ml/twice/day - prophylactic anti-biotic

VFEND - 2ml/twice/day - prophylactic anti-fungal

IVIG - last infusion was March 26

Lab Tests from NIH

2010-04-24T22:07:00.000-07:00

IgG - 914

IgA - 521

IgM - 209

These are in normal range. Stanford is spacing his IVIG to every 6 weeks or maybe longer. It appears that his IgG has been high. They will monitor it. If it stays stable, we will be able to stop the IVIG. Not bad news, huh?

Thyroxine, Free - 1.1

TSH - 19.00 (elevated)

Insulin-like Growth Factor (IGF1) - 186 (IGF1 is measured to help diagnose the cause of growth abnormalities and to evaluate pituitary function.)

Bone Age x-ray: Daivd is 5 yrs 10 months. This x-ray showed his phalangeal bone age about 4 yrs 6 months, and his carpal bone age about 3 yrs. The doctors said this was good....that he will have room to catch up.

His skin looks better than the previous picture!

2010-04-23T00:00:00.000-07:00

This was his idea for Earth Day.

+1365 Trip to NIH

2010-04-22T00:13:00.000-07:00

Our trip was good. Here are a few highlights:

Cardiology: It looks like he has developed polycythemia. The doctors say it's caused by his VSD, however, in reading more about it, a secondary reason can be caused by steroids. He has many of the symptoms of polycythemia. The dermatologist at the NIH suggested to look back at his hemoglobin and red face history. Around the time his blood changed from O+ to B+, his hemoglobin slowly increased and his face grew redder. I've kept photos of him and his hemoglobin numbers on my blog so it was fairly easy to go back and see the differences.
Endocrinology: The NIH doctors would like David to be seen by an endocrinologist. The Endo doctor will monitor his hormone levels (growth and thyroid) and give recommendations.
Growth: David only grew 1cm in 1 yr. I'm so sad. He had an x-ray of his left wrist that shows the size of a 3-4 yr old. The doctor was happy that there is room for him to grow. They sounded optimistic that he will catch up but it's first important for him to wean off the steroids.
Nutrition:
Dermatology: Dr. Cowen took a skin biopsy. We will learn if his irritated skin is due to light sensitivity or GVHD. More info to come...
Other labs:

That's all for tonight. I'll write more later as test results come in.

Thanks for checking on us.

+1355 New anti-fungal

2010-04-12T14:17:00.000-07:00

Sorry to post this photo of David. It's not a good one. Just an example of what his skin looks like when it peels. Not fun. I've been using Desonide and Aquaphor on his face.

David started his new oral anti-fungal, called VFEND for short, or Voriconazole. It's a preventative medication.

+1344 - Update

2010-04-01T09:49:00.001-07:00

David's doctor said he does not have conjunctivitis or GVHD of the eyes. His lower lids are still red and watery but Dr. Amylon thinks it will disappear and is not worried. I was worried he had GVHD of the eyes which is why I posted the previous eye images. GVHD of the eyes produces dry eyes, not watery.

He will be put on an anti-fungal - called voriconazole. This medication is used to prevent things like Candida or invasive aspergillosis in immunosuppressed people. We have a friend at Stanford who spent over 1 yr inpatient due to aspergillosis. Aspergillosis, if left untreated, can kill you. People living in the California coastal region are also at risk. I am unhappy about adding an antifungal to his regimine, however, I think this new medication will be helpful.

His face and hands are still dry and peeling. The new steroid tropicals we've been using do not seem to help, and are making his skin very thin, which causes cuts and scratches. Not good :-( He'll see the dermatologist in a couple weeks.

Thanks for checking on us!

Eyes

2010-03-25T00:22:00.000-07:00

Sorry, don't look unless it does not bother you. These are eyes with

cicatricial conjunctivitis associated with chronic graft-versus-host disease

+1335 Update

2010-03-23T07:41:00.000-07:00

Overall: Feeling well but he still has mucous in his nose and a productive cough - going on two months with this gunk.

Eyes: The lower lid of his eyes have drooped and his eyes are watering like crazy. The moisture is causing goop to build up too. Not sure if he has an eye infection or the drooping is caused by the steroid taper.

Skin on face: Peeling, tight when not moist, some patches of clear skin, but for the most part looks red.

Skin on hands: Improving, feels almost like a normal hand, but still looks dry and must keep moisturized. Mothers, enjoy holding your children's beautiful hands!

Skin on the rest of body: looks great! Just two patches on his neck that we treat.

Steroid taper: He is down to 1ml/twice/day and holding.

School: He and the girls are taking a 12-week art class. He loves it and looks forward to going. They started him at the age-5 level but when they saw how well he draws they bumped him up to the 7-8 yr old level. He is still working through www.headsprout.com. He loves learning and building things. He took nails and hammer outside, found two large logs, and hammered them together. He was so proud. He also found a millipede. He makes me nervous because he picks up bugs, newts, and salamanders. It makes him happy but I hope he does not get bit. Actually, he brought a newt into his room and placed it in his drawer. The next day it was gone. Oh, help. We still cannot find it.

Other activities: We went to Disneyland in March. They gave David a special pass so our group did not have to wait in line, in the sun. The pass made his day so much easier and we are very thankful for that.

Current meds:

1ml Prednisoline 2/day

4ml Septra 2/day

3.4 ml Prograf 2/day

2.5ml Isradapine 2/day

1 Prevacid tablet 1/day

1 Catapress patch 1/week

1 tsp Calcium/Mag/Zinc 1/day

1 tsp Multivit 1/day

1 tsp Omega Oil 1/day

IVIG 1/month

Thank you for checking on him.

-Aimee

1st WAS Family Conference, July 30-Aug 1, 2010

2010-02-23T09:00:00.000-08:00

From Sumathi:

http://crm.primaryimmune.org/index.php?q=civicrm/event/info&reset=1&id=53

I am really excited that the WAS Conf registration is now open. You can register on line. Many of you have already registered!! Thanks for doing this so quickly. It helps with the planning. As it is all coming together I am in awe at the thought that in a few months we will be in the presence of these amazing physicians and that we will get to meet so many families!! I hope that all of you will be able to come to the conference. Please feel free to e mail or call me at 919-641-7134 if you have any questions.

With all sponsorship that we received we were able to reduce the conference rates significantly. It will cost $150 for a family of 4 for two nights and that includes conference registration, food and child care! This is an unbelievable deal and I hope that many families will be able to make it there.

Many of you may have received the IDF mailer. I have attached the forms with updated information. Please have your children participate in the T shirt contest. You do not have to attend for them to participate in the contest! Please send it in to Buffy at the NIH before April the 15th! If you know other WAS families, please forward this to them.

Thanks!

Best,

Sumathi

+1296 No Second Opinion from Seattle

2010-02-12T07:32:00.000-08:00

Our insurance denied a second opinion from the children's hospital in Seattle. I have a couple more options but I don't have the energy right now to pursue them.

Well...woke up feeling good

2010-02-12T07:25:00.001-08:00

He threw up all of the warm mint tea last night, but after using the bathroom, we gave him some warm milk and his juice with prednisolone (you have to give the steroid with milk or food) he slept through the night. The first thing out of his mouth this morning is that he did not throw up. Great! Now we'll see how the rest of the day goes.

Not feeling well tonight

2010-02-11T23:08:00.000-08:00

He's throwing up again tonight - yellow bile (actually looks like the Sirolimus (Rapamune) he's been taking for 6 months. Abdominal pain too but not enough for emergency. He could be having liver pain. Sirolimus (Rapamune) does cause liver pain. Bad medicine! I feel like throwing you to outer darkness and watch you burn! He was active all day and it just hit him tonight before dinner. Children are amazing! He was upset because he wanted to eat dinner. Please pray for his healing and for God to sustain my strength tonight as I deal with a sick boy, and a clingy baby.

+1286 - No Skin Biopsy

2010-02-02T20:52:00.000-08:00

Much to my surprise, they did not do a biopsy and do not intend to. I have asked them on/off for one year. I'm not going to ask again. The Dr. said, with a strong wink in the eye, the previous biopsy (which was inconclusive) had an addendum saying it looked like GVHD. Nice of them to tell me three years later. However, they will treat it like Eczema. The only problem is that it is not eczema. I was planning to have the biopsy sent to the Hutch in Seattle and to the NIH in Maryland, for second opinions. The Dr. today prescribed stronger topical ointments. I'm so confused. I'll have to pray about it more and see where God leads this.

-Aimee

+1284 days = 3.51547549411179 years post BMT

2010-01-31T09:45:00.000-08:00

Last Thursday Michael took David to LPCH for his two-hour infusion of IVIG. A two-hour infusion turned into a whole day wasted. Michael was there from 9:30am to 5:00pm. There was nothing out of the ordinary that happened; they are just slow. I complained.

Yesterday, Saturday, David woke up with a splitting headache. He was almost in tears which is unusual for him. It turns out that I forgot to change his blood pressure patch. So, I gave him Tylenol, changed his patch and gave him the BP medicine, Isradapine. I gave him a back rub too. He threw up a little too. His blood pressure was 129/96 which is high for a child. Another symptom he has is pain on his right abdomen, where the liver resides.

Putting it all together, he either had toxicity of the liver or a reaction to the high blood pressure.

He really needs to ingest more liquids! He gives me so many excuses for not drinking: I'm full, it's cold, it will make me have to go to the bathroom, or I already drank.

Please, if you ever see David, can you encourage him to drink his water! He always has it with him.

It's chore day today and we have a busy day planned.

Thanks for all of your prayers and love for us.

-Aimee

Two Thumbs Up Today! song by Johnny Bregar

2010-01-22T22:28:00.000-08:00

WAS Presentation Picturing David and His Blog

2010-01-21T08:43:00.000-08:00

This Power Point presentation was done by a few ladies working on their Master's Degrees. Briana found David's blog and asked if she could use some of the info and pictures. I was happy to help and wish them all the best! They got an A+!Wiskott Aldrich Syndrome Final Powerpoint

View more presentations from Jefferson School of Education.

Dealing with GVHD Webcasts

2010-01-21T08:02:00.000-08:00

These Webcasts are for parents who are having to deal with their children's chronic GVHD.

http://www.nbmtlink.org/webcasts/

Finally, some cyber support that is helpful!

+1273 - Doing pretty good!

2010-01-20T17:08:00.000-08:00

Feeding the Fainting Goats at the Old Faithful Geyser, Calistoga CA on New Year's Day

Old Faithful Geyser, Calistoga CA

Handmade Glow in the Dark bouncy balls

A boy who loves his slime!

David's been doing pretty good. He did get the mucus cough that the rest of the family had. He seems to be doing fine. His diet has been so-so. He is so picky. Sometimes he will go without 2 meals a day because he is determined that he does not like what I serve him. What child does not like potato cheese soup? At least he loves broccoli and spinach.

His skin is looking pretty good too. We are still on 1.4mls/twice/day. I think it's a blessing that he is fighting a cold, yet he does not have a GVHD skin flare-up. It's common for the skin to flare when the body's immune system is busy fighting something else.

I wanted to post a photo of him participating in our science class. We made glow-in-the-dark slime. We used common ingredients so I am happy I did not have to pay a fortune for a slime kit. We also made hard bouncy balls. The kids wanted to add highlighter juice to make them glow. You can see his skin is looking pretty good in the photo. His hands are not doing too bad either. They are chapping but not peeling as much. I gave him a haircut too. I think he looks handsome.

Thank you for your prayers and love for us!

+1253 - Not feeling well tonight

2010-01-04T21:07:00.000-08:00

David threw up tonight about 6pm. He had some front lower right abdominal pain about 5pm. He took a bath and felt better but his stomach cannot hold anything down right now at 9pm. I have to give him his steroids. I'll give him a little rest and see if he can hold it down about 10pm.

His steroids were reduced to 1.4ml/twice/day (was 2ml, then 1.7ml). I don't see any major skin problems. His skin still looks pretty good.

Our whole family was ill with a cough for about 6 weeks. The girls got over it quickly. Jonathan still has phlegm. David had some phlegm this morning. I'm hoping it does not turn into anything more series for his lungs.

Current meds:

Catapress patch (blood pressure)

Isradapine

Prograf

Septra

Prevacid

Prednisolone

IVIG

Hydrocortisone ointment

+1226 Seeking Second Opinion

2009-12-08T12:59:00.000-08:00

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

2009-11-10T09:18:00.000-08:00

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

2009-11-08T00:13:00.000-08:00

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

2009-11-04T13:50:00.000-08:00

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

2009-10-19T09:06:00.000-07:00

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

2009-09-20T23:26:00.000-07:00

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

2009-09-04T00:15:00.000-07:00

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!

2009-08-10T15:23:00.000-07:00

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

2009-08-01T08:31:00.000-07:00

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

2009-07-21T11:25:00.000-07:00

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

2009-06-16T14:32:00.000-07:00

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

2009-06-11T19:14:00.000-07:00

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

2009-06-05T21:07:00.000-07:00

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

2009-05-23T16:36:00.000-07:00

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!

2009-05-14T21:59:00.000-07:00

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)

2009-05-13T10:06:00.001-07:00

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

2009-05-12T18:57:00.000-07:00

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

2009-05-07T00:40:00.000-07:00

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

2009-04-19T08:25:00.000-07:00

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

2009-03-26T14:16:00.000-07:00

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

2009-03-24T07:45:00.000-07:00

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

2009-03-21T20:40:00.000-07:00

A lot has happened since I last wrote an update.

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.
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.
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!
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!
The next boy we are praying for is Ayden. He is undergoing preconditioning right now.

+949 That was Short-lived

2009-03-08T12:27:00.000-07:00

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

2009-03-07T20:54:00.001-08:00

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!

2009-03-01T20:14:00.001-08:00

+939 Getting Closer to Etanercept

2009-02-26T21:50:00.000-08:00

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

2009-02-21T12:07:00.000-08:00

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

2009-02-11T21:52:00.000-08:00

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

2009-02-04T08:27:00.000-08:00

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

2009-02-02T16:04:00.000-08:00

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

2009-01-24T12:42:00.000-08:00

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

2009-01-19T23:18:00.000-08:00

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

2009-01-18T10:31:00.000-08:00

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

2009-01-10T20:01:00.000-08:00

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

2009-01-04T13:41:00.000-08:00

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

2008-12-31T10:38:00.000-08:00

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

2008-12-24T22:42:00.000-08:00

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

2008-12-21T22:55:00.000-08:00

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

2008-12-17T08:33:00.000-08:00

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

2008-12-07T19:10:00.000-08:00

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

2008-12-04T10:02:00.000-08:00

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

2008-12-03T20:35:00.000-08:00

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

2008-12-03T20:07:00.000-08:00

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)

2008-11-26T22:27:00.000-08:00

+826 The End

2008-11-24T20:15:00.000-08:00

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

2008-11-22T23:37:00.001-08:00

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

2008-11-16T06:10:00.000-08:00

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

2008-11-06T20:55:00.000-08:00

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

2008-10-29T11:35:00.000-07:00

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

2008-10-01T19:11:00.000-07:00

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

2008-09-26T23:34:00.001-07:00

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

2008-09-10T11:19:00.000-07:00

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

2008-08-13T10:51:00.000-07:00

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!

2008-07-26T08:46:00.000-07:00

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

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!
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

2008-07-24T09:19:00.000-07:00

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

2008-07-22T01:40:00.000-07:00

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

2008-07-09T21:54:00.000-07:00

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

2008-07-02T19:48:00.000-07:00

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

2008-07-02T09:57:00.000-07:00

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?

2008-07-01T09:43:00.000-07:00

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.