A bump in the road to recovery / Turning 12

August 24th - 31st

The "not so fast" caution sign has been flashing recently on Camry's speedy road to recovery. She has developed a rash, that "comes and goes". The suspected origin of the rash is "Graft versus Host"  disease, which in Camryn's case isn't so much of a "disease" as it is a "reaction". In last week's blog, I mentioned they Camyn was to begin being weaned from the medication "Prograf", the anti rejection medication. Smaller doses had already been initiated a few weeks ago, but this week's blood levels showed a significant drop in the Prograf levels which may have spawned the reactionary rash. So, Camryn had her Prograf doses adjusted upwards to counter the reaction. The docs assured us that this is a fairly common reaction and that while the rash and itching are a nuisance, they are not representative of serious complications or reactions to the bone marrow transplant (BMT). In other words, Camryn remains on track for a full recovery with a target date to be off Prograf at day 100, post transplant. To put everything in perspective, Camryn is at day 65, as of 8/31/14, post transplant and so far, has yet to have any "serious" reactions to the BMT. I suppose that watching her scratch herself like a dog infested with fleas, is a far better graft-versus-host reaction than what potentially could be happening. The reality is, the itching and rashes are manageable; a small price to pay for a new, healthy, and functioning immune system.

Well, she is 12 now. A magical age; no longer a "child" but not quite yet a "teenager", which Camryn's argues she is. Managing this birthday for Camy has been somewhat easy since many things we potentially could have done, had no chance of occurring because her situation. Also, the lifestyle restrictions with which Camryn has been living since the first week of June, has given her a very unique perspective regarding what matters most to her. As you can imagine, she has rearranged some of her "priorities". For example, family time is huge (GASP!) for Camy, because we are essentially her "social life", despite being as "lame" as we are, until January. Playing games (Uno, Pit, Apples to Apples, etc.) is what she cherishes now. As I mentioned last week, just riding in the car somewhere,
anywhere is a treat. For us, the birthday was simple, it had to be and Camryn understood that. What
Lee Ann and I really appreciated about this 12 year-old's reaction to her simplistic birthday party, was her positive reaction to all of it. She completely understood the situation, embraced it, and loved it. Camryn was grateful for just being able to celebrate her birthday, and many more to come. As I lay in bed later that night, having a few precious moments to myself, I pondered the experience and found significant meaning beyond "the birthday party". I humbly recognized the eternal lesson that was taught to me and our family by this precious 12 year old girl. The kid was going to be thankful for the birthday and for her birthday party, regardless of the restrictive circumstances. Not one single complaint. In fact, the "it's not fair" attitude has not once crossed her lips from the day she was diagnosed with GATA 2. Thank you Camryn, for demonstrating to us the meaning of the word "gratitude". May we be wise to implement the meaning of the word "gratitude" in our own lives.

A Day In The Life of Camryn: Managing the Do's and Don'ts

August 16th - 23rd

Camryn has officially started school again, not THAT school, Woodland Middle will have to wait until January. She has begun her Fall Semester of school at home. Williamson County School District has assigned a "home bound" teacher to Camryn. She will come to the house every Monday and Thursday for about two hours and tutor Camy on her homework assignments and reading. This particular teacher has also been assigned to another student in the school district who had a heart transplant.  Camryn seems ready to ease her way into "normal" aspects of life for a girl her age. The transition to school work should be fairly smooth, we hope, as Camryn begins to live a little like her friends, just not WITH them. She misses them a lot, but does utilize social media to stay in touch, but the interaction just is not quite the same as in person. It's the personal interaction with friends that Camryn misses the most. Speaking of transitioning, Camryn turns 12 on Thursday, a birthday that for us, will be a special one in so many ways, albeit without friends, as she sadly reminds Lee Ann and me. Something occurred on Saturday (yesterday) that is worth mentioning in the blog. One of Camryn's very dear friends had a birthday party on Saturday, a party that Camryn normally would have attended, but because of obvious circumstances could not. The mother of Camryn's friend called Lee Ann to see if she could bring the three girls by the house to see Camryn. Lee Ann and I have been quite vigilant in protecting Camryn from potential "exposure" to infections, but felt this would be a good thing to boost her spirits since her medical check ups have gone very, very well this week. Well, the decision was a good one. Seeing Camryn light up in smiles and excitement to not only see her school friends, but to also interact with them on a personal basis was precious. For 30 minutes, Camryn was a normal kid again, not the bone marrow transplant kid, just normal. After they left, we reminded Camryn that this was a "special" occasion and not something to expect on a regular basis, if at all until she is cleared in December. Of course, that statement prompted the proverbial frowny face from you-know-who. But, for now, this is Camryn's reality for her own protection. During the day, Camryn begs to get out of the house, not just to go into the backyard, but rather to go "somewhere". Lee Ann has been taking Camryn with her on a few of her"errands". She cannot go into the stores but remains in the car (with the car on AND the air-conditioning running just in case you were wondering). Something so simple as a ride to the grocery store is huge to Camryn in reducing her feelings of being trapped. And so it goes, every day, until she is cleared to resume living a somewhat "normal" like.
The medical appointments went very well last week. This week, Camryn begins being weaned from her anti-rejection medication, of which I wrote in last weeks blog. This action is significant as it is a reflection of Camryn's continued progress of her immune system strengthening. Also, Camryn is beginning to get "hair stubble" on the top of her head. Now THAT is very significant progress!

McKay, Camryn's only brother, left this week to return to school at BYU, and Casey, Camryn's oldest sister, left to return to school at Utah State. A special thank you to the two of them for helping in so many ways while they were here. We really needed them while Cammy was in the hospital. Safe travels to both of you and we will see you during the Christmas break, right here in Tennessee.

A Week of Surprises and of Blessings

August 9th - August 15th

So I think that I have officially given up attempting to write two "Camryn" blogs per week. For some reason (six needy children might be a good place to start) I just can't seem to get it done. For now, one per week will just have to do and they will be posted on the weekend, like this one. Now, for Camryn's update...

Camryn continues to progress in so many ways, except in one area; being compliant with her medication regimen. Since I would prefer to not dwell on the negative, let's just move on to the more interesting items. Camryn had her two medical appointments at Vanderbilt this week. Both appointments went very well. Medically, Camryn is doing as well as any bone marrow transplant patient could be doing. In fact, one of her main medications, Prograf, the host versus graft anti-rejection med, is going to be slowly weaned off of Camryn. This is a significant change in Camryn's post transplant treatment. Since the transplant, Camryn has shown no signs of her body rejecting Morgan's donated bone marrow and the disappearing warts are a clear sign of a functioning immune system. Now here is the interesting part in all of this; The Prograf suppresses the body's natural immune response to attack "foreign bodies", or the transplanted marrow. Essentially, the Prograf has been "protecting" Camryn by suppressing her newly installed immune system. This is all my own "lay man's" terms for trying to understand something that I really don't!  The point is, today Dr. Frangoul said, that once Camryn is off the Prograft, her immune system will "really" kick in and that those remaining warts "won't have a chance". Camryn's formerly GATA2 condition will be completely reversed. Dr. Frangoul then shared with Lee Ann a newly published article from a medical journey discussing recent clinical data regarding GATA2. The article discusses the clinical outcomes of all GATA2 patients followed in this particular study. All of the children eventually developed some form of cancer, particularly leukemia, and / or had massive infestations of infections.  The harsh reality of the future of patients with GATA2 who go untreated. Once again, we count our blessings for those nasty, nuisance, pesky, life-saving warts. They have served their purpose, but must now face their demise. So, there you have it, our week of unexpected surprises and an appropriate reminder of blessings.      

98%: The Magical Number or Camryn Is No Longer Who She Was

August 2nd - August 8th

Yes, I know, so much for blogging on Tuesday. By the time I had time to blog, (I am traveling again), it was Thursday. So I decided to just wait until today (Friday) since Camryn had a regularly schedule appointment at Vandy. As you may have guessed from the title of this week's blog, the bone marrow biopsy results are in and Camryn's bone marrow is 98% Morgan's, exactly the number the transplant team was looking for. That number was met with relief and celebration on our part, smiles and elation from the good folks at Vanderbilt. We knew the results would be good because of all the changes in Camryn's warts, but that seed of doubt just sort of hung around until we had "proof" from the biopsy results. Also, another number to share with you; Camryn's white blood cell count has now risen to the "normal" range. Earlier today, members of Camryn's transplant team were comparing photos of the warts on Camryn's hands and feet taken from her first visit with the immunologist, Dr. Hummel. That visit was a bout a year and a half ago. What Lee Ann told them that they did not know was that the warts had actually gotten worse prior to the transplant. It was Dr. Hummel who connected who suspected a possible case of Gata 2 with Camryn because of her work with the doctor at the National Institute of Health, who "discovered"Gata 2 about 5 years ago. Our understanding is that Camryn is Vanderbilt's first case of Gata 2, which is one reason why all the interest in her diagnosis, treatment, and progress. her case has previously been presented at a "Grand Rounds" earlier in the year. I would expect a follow up grand rounds will be in the works in the months to come, especially if her progress continues its same course. Camryn's next bone marrow biopsy will be in about three months. At that time, or day 100 post transplant, the transplant team expects to see 100% of Camryn's marrow to be Morgan's. This is what I mean when I say, despite Camryn's amazing progress, she has a long way to go before we can officially pronounce Camryn's Gata2 diagnosis as being reversed; which ultimately is the desired outcome. So, there is the update for this week. I will blog again after Camryn's Tuesday appointment at Vandy.

A Better Week Than Last Week

July 28th - Aug 1st

I know that I mentioned that I would update Camryn's blog every Tuesday and Friday, well obviously that didn't happen. I traveled for work this week which just messed up my "free time". No worries though, I will get you caught up. Last week, Camryn really struggled taking all of her medications. The frustration level was high, for Camryn and for her ever loving, extremely patient parents. This week, progress was made; not giant steps, but steps. We were happy to have progress of any sort after last weeks debacle. The last three days have been good as we have made some minor adjustments to her medication schedule. Also, her appetite appears to have returned, but with a craving for pickles, lots of pickles. Not sure if the pickle craving is a sodium thing like Lee Ann's (way to long of a story to explain that one), but pickles are food.

So, today was the big day for the first post-transplant bone marrow biopsy. The results of the biopsy will give us an indication of what percentage of the marrow is Morgan's, hopefully around 98%-99%. The high percentage indicates that the transplant was successful. Lee Ann was with Camryn at Vanderbilt from 8:30 am until 2:30 pm. Camryn had her blood draws prior to the surgery, and then had her biopsy. The procedure went very well, no surprises or complications. Camryn slept most of this afternoon, due to the anesthesia, and her hip will be very sore the next 2-3 days.We will not have the bone marrow biopsy results for a few days, however, Dr. Frangul told Lee Ann that Camryn is definitely producing a significant amount of bone marrow and the consistency (jelly like) is exactly what they wanted to see. Also, from the blood draw, Camryn had the highest level yet for her white blood cell count. What is so encouraging about this particular level of white blood cells, is that her level is getting close to what is considered to be within the "normal" range. Also,  the wart situation continues with the miraculous disappearing act. Lee Ann and I are hoping to post some pictures for you of Camry's hands prior to her hospital stay for the transplant, and now. Even the docs who had not seen her for a couple of weeks were amazed at the difference. Once we find those darn "before" pictures, we will post them. You just have to see it to believe it. Next blog on Tuesday.

The pills, taken multiple times per day
with Camryn's "geriatric" like pill box.
 Camryn now knows exactly
how Grandpa Ras feels.

Hospital supplies for IV fluids,
cleansing and changing the venous
catheter, which Lee Ann does at home.