Our second consult was with the Hematologist:
One test revealed that Rowan's Pancreatic Isoamylase is still low, signifying continued pancreatic insufficiency, which the hematologist said would be consistent with his Shwachman-Diamond Syndrome diagnosis. The B cell defect could also could be consistent with SDS she said. In general, she agrees with the clinical diagnosis of SDS right now. However, if Rowan is SDS, then it is the worst case she has ever seen. She said that she is casting a larger net now, to hopefully fully/better diagnose him, and re-sending the SDS gene test to lab in Canada to ensure it was done properly when he was an infant as well as sending telomere length testing, and the bone marrow aspirate and biopsy.
Our final consult was with the Gastro-enterologist:
The gastro-enterologist said that he was sorry to tell us that Rowan really just is “the perfect storm” with his poor functioning gut and motility issues on top of the B cell immunology defect. He said that Rowan physically acts exactly like a short-gut patient (someone missing a large portion of their colon/bowels), because his do not work properly, and that when you add the immune compromised component of Rowan’s health issues, he is just unfortunately going to repeatedly re-infect himself, seeding bacteria into his own bloodstream. This is why Rowan gets septic so often. He told us that while Rowan's motility issue is a big part of the problem, there just isn't a fix for that. He also did not feel that we would be able to eliminate Rowan’s need for nightly iv tpn based on his pancreatic function and current medical picture.
He recommended a new course of preventative antibiotics to help with Rowan's gut flora. It is one that doesn't cross into the blood stream, it just moves through the gi tract. He agreed with our gi doctor back home regarding the new g-tube formula, and also put him on a new medicine/regimine to keep him cleaned out as best we can. He said that he would like a 2-3 month trial, with us making these changes to Rowan’s gi care, and he had already been in contact with our gi doctor back home, and they are on the same page.
We definitely feel that we made the right decision to travel all the way to Seattle Children's for the expert advice of these three physicians. They have done, and are doing, every thing in their power to fully diagnose and treat Rowan's unique and complicated health scenario. We are confident that Rowan is in good hands, as we try to make some major life changing decisions regarding his care.
The long and short of the trip, medically speaking, is that we now know that Rowan has a primary B cell defect that is not allowing him to make or maintain the proper immunities that his body needs to protect itself. We also got confirmation that his pancreas is still not functioning properly, and that his colon is not fully functioning either. All of this makes Rowan his own worst enemy. He repeatedly infects himself because he trans-locates bacteria from his own gut into his blood stream, which of course causes sepsis and can end very very badly any one of these times.
All of the doctors there feel that Rowan could benefit from a bone marrow transplant because of his compromised immune system, repeat infections, and dependency on neupogen, IViG and TPN (total parenteral nutrition- or his nightly IV nutrition). However, because Rowan is at a higher risk for transplant complications, they prefer it be a last resort.
If we do go to bone marrow transplant with Rowan, it would be done there in Seattle. They hold the only license in the United States for the lower toxicity chemo drug that Rowan would need, given his more complicated issues. He would need to move there for 4 - 6 months, and after the initial 6 weeks in the hospital, would not be allowed to go more than 30 minutes away from the hospital. This of course means that I would do the same, and Brian for as much of it as possible, especially in the beginning. That obviously affects Zoe and Ian back here at home, so you can imagine how difficult a situation it is.
The final plan, is a 3 month trial of some major changes to Rowan's treatments. He will now need neupogen, his bone marrow stimulant, infusions every single day. He will now get his IViG (immunoglobulin) infusions every single week instead of every 5 weeks. These doses are larger, more aggressive, and more frequent, and therefor can cause complications of their own. His kidney function and blood work will have to be closely followed during this trial. We will also be making several changes to his gi care during this 3 month period.
Plan A: If Rowan is able to get to the desired levels and stay out of the hospital, we can stay in a holding pattern and continue this regimine for the time being. If Rowan continues to get infections and/or be hospitalized for bacteremia, then we move to Plan B: bone marrow transplant.
Either way, we need your prayers and support. We need our local doctors to be on board and help facilitate these protocols as ordered by the specialists in Seattle. We need Rowan's little body, specifically his kidneys and bone marrow to hold up and not be adversely affected. We need him to STAY OUT OF THE HOSPITAL. We need the best 90-100 days of his life.
Thank you from the bottom of our hearts for caring about our son, and our entire family. Thank you for thinking about him, for praying for him, and for being there for us all. It's going to be a long 3 months, but we are hopeful and confident that these changes will have a positive effect on Rowan's health. If not, then we are just asking God to make it blatantly obvious if transplant is the way we need to go. We will keep you all updated on how it is going and how Rowan is handling these changes. But you know as well as we do, that how Rowan will handle it is the least of our concerns:)