As most of you know, we just returned from a week long visit to Seattle, Washington for consultations with some top physicians regarding Rowan's overall health, diagnosis, and possible future treatment options. It was a very informative trip. We met with several different specialists at Seattle Children's Hospital and a Bone Marrow Transplant Specialist at Seattle Cancer Care Alliance. Each doctor spent hours with us, going over Rowan's detailed medical history and family history. They will be getting in touch with our physicians at home and making recommendations as to some changes in his current medications and care, and also would like to see us back in Seattle in August to go over the results of the very detailed lab work and genetic testing that they did while we were there this time. There was a lot of information given, and some of it is complicated, but I wanted to try to give a summary to our friends and family. I hope this all makes sense. Feel free to call or message me if you have questions.
(I realize some may think this is TMI, but that is why I am doing the update here and not on Facebook. People are free to read this all, in part, or not at all. It is just here for the family and friends who do want the details.)
(On the plane, headed to Seattle)
The first specialist we met with was the gastro-enterologist. A large part of Rowan's health issues are gi related, so this was obviously a very important appointment. We loved the doctor and he really did his research on Rowan, and had some great information for us.
- This doctor agrees that most of Rowan's septic infections are due to his poor functioning gut, and that he is trans-locating the bacteria into his own blood stream.
- He is recommending that we start Rowan on a special probiotic, one that is not grown on lactose based media. Rowan has major reactions to all the probiotics that we have tried in the past because of his protein intolerance/allergy. This one is not protein free, but would be the least amount of protein, so it is definitely worth a try.
- He also recommended a special antibiotic that is not well absorbed by the gut. Rowan is often on antibiotics, in an attempt to sterilize his gut since he has bacterial overgrowth of the colon. As you can imagine that can lead to some not too nice side effects after long-term use. This one would just move straight this his system, cleaning out the gi-tract without being absorbed into his system. We are hopeful that this antibiotic will work, and prevent us from over-using, and potentially becoming immune to, all the other typical antibiotics.
- He is recommending that Rowan be re-tested annually to see if his pancreatic functions improves. He told us that some of the Shwachman-Diamond patients do seem to hit a point where they outgrow that issue to some extent. That would obviously be great news.
- He also wants Rowan tested for amore specific Cystic Fibrosis gene mutation, one that mainly affects the pancreas. Since Rowan was originally diagnosed with CF s a baby, and has such similar gi symptoms, he wants to be sure they didn't miss that specific version of the test.
- The doctor said that Rowan may eventually be a candidate for a gut-transplant, and that they have had some success doing those there. However, there is a large rejection risk after about 10 years because so much of the immune system is in the gut.
- He wants Rowan to be seen by their Intestinal Failure Clinic the next time we return to Seattle.
- He wants us to check the brand of IVIg that Rowan receives and if he does end up preparing for transplant, there is one brand that we need to steer clear of.
- Regarding bone marrow transplant, he obviously had to defer to the hematologists and marrow failure physicians but he did say he would want Rowan's overall gi health to be much much better before he went down that road.
The next two specialists we saw were the hematologist and immunologist, and they tag teamed together in one three hour long appointment. They both did a very thorough examination, and ordered lots and lots of blood work and further genetic testing. We were very impressed with their depth of knowledge, wonderful bedside manner and eagerness to solve Rowan's medical mysteries.
- Dr. S. the main HEM/ONC and Marrow failure specialist went through Rowan's medical history and our family history with a fine tooth comb. She has entered the entire family into an extremely expensive, very selective, genetic research study and hopes to be able to provide us with some answers through this study. This will obviously take time. She already has one patient in particular that she says is very similar to Rowan (including the Chiari malformation), and she wants to compare their genetic test results.
- She wants to do further genetic sequencing on Rowan to determine if he does in fact have Shwachman-Diamond, or a Shwachman-Diamond like disease, or something different altogether.
- She is doing genetic testing to explore the possibility of Rowan having a Mitochondrial Disorder. This would open up a whole new can of worms, so we won't go there unless he does receive this diagnosis.
- She recommended that Rowan receive a smaller but daily dose of his neupogen (bone marrow stimulant) instead of his current larger dose that is given twice a week. She thinks that this will keep his counts more stable and will also eliminate some of the pain that he experiences with the larger doses. This made perfect sense to us.
- The immunologist will be breaking down all of Rowan's immunology panels to see if there is further treatment that could boost his immune system, in addition to his neupogen and IVIg infusions or a better diagnosis for his immuno-compromised system.
- After our appointment and labs, we took a great tour of the BMT facility there at Seattle Children's Hospital, and they had very impressive patient rooms, parent common areas and amenities.
The final appointment was the one I took the most notes in, the meeting with the Bone Marrow Transplant Specialist at Seattle Cancer Care Alliance. We discussed Rowan's past and present blood and bone marrow issues. And then he gave us his thoughts and recommendations, and finally explained the bone marrow transplant process in great detail. It was a very informative, somewhat scary appointment. They are doing some cutting edge things there at SCCA though, and we left feeling like that was the right place to be.
- He let us know that Rowan's dependency on routine neupogen and his recurrent septic infections alone could be enough for him to justify bone marrow transplant. However, he said that Rowan's case is not straight forward and comes with extra complications/risks.
- He told us that Rowan's long-term tpn dependency is problem. He hasn't transplanted patients who have been on tpn for as long as Rowan has, and is concerned with the effects it may have had on him already.
- Since Graft vs Host Disease (common transplant complication) typically attacks the gut/intestines, Rowan's gut health would need to be much better before a bone marrow transplant was attempted. It is hard enough on the gut of gi healthy patients, but for Rowan that GVHD risk would be exponentially higher.
- He said it would be good to know if Rowan had a perfect sibling match now, because that may make the decision to proceed easier. There is only a 25% chance that they will be a 10 out of 10 match, but if they are GVHD risk is reduced. We will keep fighting insurance to get that testing done, or hope that the genetic testing that they are currently doing on Rowan will give enough cause to prove the need to get Zoe and Ian tested now as well.
- His preference is obviously a sibling match, and then after that a 10 out of 10 match from the national registry. He said once you go outside the siblings, it does not matter, no one else in the extended family is more likely to be a better match than a stranger. So, he would go to the national registry and search for a couple of matches and prioritize them. Please register at bethematch.com if you haven't. Even if you aren't Rowan's match, you could save someone else's life.
- SCCA has one of the only clinical licenses in the United States to use treosulfan, a drug that is being tested in the treatment of cancer, for non-cancer bone marrow transplants. It is widely used in Europe, but not approved in the US. The benefit of using treosulfan is that it has less toxicity. That means that the intensity of the chemotherapy is reduced so it is easier on organs. This is the type he would want to do on Rowan. There is some decrease in engraftment, but it is safer for patients like Rowan. Here is where it gets real: They have done 50 of these non-malignant transplants (40 with bone marrow, 10 with cord blood) Of the 50, there were three deaths (1 from GVHD, 1 surgical complication, 1 brain complication).
- If Rowan does proceed with this type of bone marrow transplant and has it done at Seattle Children's Hospital, he would most likely spend six weeks in the hospital. After that most patients need to stay in the area (can't go further than 30 minutes from hospital) for 100 days. He said it would be more realistic for Rowan, to plan on staying close to the hospital for 4-5 months after he was released. During that time he would have to be seen 2-3 times a week at SCCA for different infusions, treatments and follow-ups. This would obviously require me to move to Seattle with Rowan for about 6 months or more.
- The doctor is going to review Rowan's labs and marrow slides in great depth, and would like to have Rowan get another bone marrow biopsy in August when he goes back to Seattle.
- If Rowan has myelodysplastic changes in his bone marrow, or starts requiring more transfusions/platelets/etc. then that would be a no-brainer and it would be time to push for transplant immediately for sure. In the meantime, he wants us to focus on improving his gi health while these decisions are being made, to give him the best chance if it comes to transplant soon.
I think this about sums up the information we received from the doctors while we were there in Seattle. I know it is a lot to take in. Trust me, there was even more I could've shared, but until we get to one of those points, I don't see the need to go into all those details. What we are asking from you all now is just prayer and support as our doctors confer with the doctors there, as results are received and dissected, and eventually as decisions are made. We are trying to remain optimistic, and are definitely feeling like we made the right choice to travel to Seattle. August's visit should bring even more clarity and help with the decision making process.
Thanks for your continued prayers and support!
(On the plane, headed back home to Texas)
