The good news:
1. We found out that Zoe is not just a 9 out of 10 match, she is a 10 out of 10 match. This is wonderful news! You just can't get any better than a 10 out of 10 sibling match. We no longer need to find 2 donors from the registry. If we ran into any problems, or if Rowan didn't engraft, they could go back to Zoe again to retrieve more bone marrow cells. This was the best news of the trip. Please keep Zoe in your prayers. She is more than willing to help her brother, but has some anxiety about the process.
2. The genetic doctor did find a few mitochondrial deletions in Rowan's genome sequencing. One in particular can be disease causing, but there was so little data on it, that they were unsure if it was a root cause of Rowan's issues or not. You may remember that they tested my blood as well, prior to the trip. Well, the geneticist hounded the lab every day, sometimes multiple times a day, and he actually got the information he was looking for, just as I arrived for my appointment with him last week. I too carry that same mitochondrial deletion. Since I do not have any of the same health issues that Rowan does, the doctor was able to pretty confidently rule out that specific gene deletion as being a problem for Rowan. Zoe also carries this gene, but since he feels that he can rule it out as disease causing, this does not take her out of the running to be his marrow donor (which was a concern that some of the doctors were having).
3. During the endoscopy and colonoscopy they took pictures and biopsies. Because of Rowan's multiple health issues, the biggest point of contention has been, "What do we fix first, the gut or the bone marrow?" Well, the GI doctor said that Rowan's gut does not look bad enough right now to be the only cause of Rowan's repeat septic episodes. He definitely has GI issues still, but he feels that the immune system is the primary defect, not the gut. This also points us in the direction of bone marrow transplant first.
The other news:
1. Rowan's bone marrow biopsy shows that his cellularity has dropped 20% since his bone marrow biopsy last year. However, there is no evidence of Leukemia in his working marrow.
2. Rowan's blood work indicates a significant primary B cell defect, and a mild T cell defect. His immune system does not work properly. He has trouble making and maintaining cells that help him fight infections. This is inherent, meaning that it is not caused by one of his other health issues. If left untreated, he will continue to fight life threatening infections the rest of his life.
3. The geneticist does still feel that we are dealing with a genetic disorder, just one that is currently unknown. He says that Rowan is so rare, so unique, that he has something that hasn't been discovered or named yet. Is anyone surprised? He hasn't stopped looking, but he warns me that we may not find out what Rowan has for years to come. We joked that it will be called "Rowan syndrome", which is something all of our doctors have said for years:) The geneticist wants to take Rowan's case before the genetic board and get approval for an even more detailed test, a whole axom study, which will break down his cells even further. Science has come a very long way, but he still warned me that they can only compare what they already know, and right now they maybe have data on about 30% of the genes. As they get more children who present like Rowan does, and they can test their genetics, they will hopefully discover mutual findings, which will lead them to know and understand more genetic disorders. Because of this ongoing scientific research and discovery, the doctors want to bank Rowan's DNA, so that even after his bone marrow is transplanted, they would be able to continue to test his original marrow against new found diseases. Fascinating, isn't it?
The transplant news:
1. All of the doctors that we met with seem to agree that now is the time for Rowan to have a bone marrow transplant. They fear for his life if he continues to have these repeat, life threatening septic infections every few months. They said that even their sickest kids don't end up in ICU several times a year on pressors, fighting for their life. Rowan does not mount the appropriate response to infections. When he goes into the ER with 105+ fevers, fighting bacteria in his blood, his marrow tanks, it does not mount a strong defense. Despite daily neupogen infusions, and IVIG infusions every 2 weeks, he still continues to battle serious bacterial and yeast infections multiple times a year.
2. Even the specialists who were against transplant last year, now feel that it is warranted. They know that they can't fix everything. But, with Zoe as a 10/10 matched donor, and with all of the in depth research and testing that they have done for the past 2 years since we started traveling there, they feel that they have come to a point where this is the way that we can at least fix something. Their goal is to "give him more good days". The hope is that this transplant will fix the blood and bone marrow issues, which will make him healthier overall, which will keep him out of the hospital more, which will hopefully give him a chance to improve in other areas of his health as well.
3. Seattle Children's Hospital has a clinical trial license to use Treosulfan, a lower toxicity chemo drug, during bone marrow transplants. Our hospital here at home does not have the ability to use this, which is why we are traveling so far. Rowan is obviously still at great risk during the transplant process, but this type of chemo will hopefully be one that he can tolerate better. Seattle Children's Hospital is one of the most experienced hospitals in the country at non-malignant bone marrow transplants. They do not take this lightly though, and they have not rushed into a decision with Rowan.
4. The transplant doctor discussed whether or not to do a myeloablative transplant. Myeloablative means "complete deletion of bone marrow cells", or taking his marrow down to zero. The risk of not being that aggressive (doing a non-myeloablative transplant) is that Zoe's marrow may not take over, and Rowan's may come back. There is a better chance of engraftment if we take Rowan's marrow all the way to zero. However, this also puts him at a much higher risk of fatal infection during the period when he is completely compromised though. It is a lot to weigh, but the transplant doctor feels that we really don't want to have to do this twice. He feels like we probably need to be aggressive the first time. He will be meeting with his colleagues to confer on this, but that was his gut feeling.
5. The doctors were all very real with us, explaining that Rowan is at increased risk during transplant. The GI tract is the primary organ that Graft vs Host affects. He is also at increased risk for fatal infections because of his history of sepsis. They explained that the bottom line is, "How much risk are we willing to take?" and ask "What other course of action do we have left?" They feel that we have given it a good try, but now the reward may be worth the risk. They explained that we may end up losing him during one of these life threatening ICU septic infections if we just continue the way things are right now .
6. Should Rowan undergo bone marrow transplant there in Seattle, we would need to first take Zoe there for 3 days, for her own medical work up. Then Rowan would return for an evaluation of his systems, and then he would start his conditioning regimen and chemo. Zoe would return 2 weeks later to donate her marrow, and it would be transferred to Rowan via infusion. At that point, day zero, Rowan would be most critical for about 100 days, while Zoe's marrow hopefully begins to fill his body up with healthy marrow. The doctors did warn us that after Rowan's 6-8 weeks in patient, he would not be allowed to travel further than 30 minutes from the hospital for the next 100 to 120 days. He would need to be seen in SCCA's clinic several times a week too, and would receive multiple transfusions of blood, platelets, etc, as needed. After 4-6 months total, if Rowan was doing well, we would be allowed to fly back home to Texas. I know...it's a long time.
Where we left things:
1. Based on hematology and immunology, they decided that transplant was indicated.
2. Based on the GI and genetics findings, they did not find a reason to not transplant, so they also agreed that it was indicated.
3. When I asked the question (Brian's favorite question to ask doctors:), "Would you do it if it was your child?" They basically said, "With Zoe as a 10/10 match and treosulfan as the chemo, yes."
4. They will present Rowan's case to the complete Transplant Board at SCCA in December. This is when the final decision will be made. If approved, this is when they will begin planning out his specific regimen. With all 7 physicians being in such agreement this time, I do not for-see the transplant team disagreeing with them at this point, but we will know for sure next month.
5. The transplant doctor explained that they like to wait until after the "sick season" for obvious reasons, so he mentioned April or May as a start date. Rowan however had his own request...June:) He would like to "play one more soccer season with Coach Conder and the Gruene Dragons" and "do one more play with Mr. Chris at Woodlawn" before moving to Seattle for the hardest half year of his life. He was sad that he would miss Summer, and especially sad that he wouldn't be celebrating his 10th birthday at home, but he said he would rather do soccer and another play, even more. I promised him that if this happens, and we move to Seattle in June, we would throw him the biggest, best "9 3/4 birthday/going away/bone marrow transplant" party ever, this May. And he smiled from ear to ear, thinking of how many people would come help him celebrate that monumental event:)