Rowan's Second Visit to Seattle Children's Hospital...the medical side of our trip.

 As you all know, we just returned from our second trip to Seattle Children's Hospital. 

 

 

 

I apologize that it has taken me almost a week to post the medical summary, but it was a lot for us to digest personally first, and then it took me a couple of days to get all my notes summarized.  It was a very fruitful trip, in that we met with three of the specialists we saw in April, gained more insight into some of Rowan's issues, had another bone marrow biopsy done, and ultimately...a short-term plan was devised.  I realize that this is a lot of medical jargon, and some of it may be more than some people want to know, but that is why I am posting it here instead of on Facebook.  People are free to read this post, all or in part, at their discretion.  If you just want to know the short summary, I would suggest jumping down to the final section "Summary".  Either way, we hope that this helps you all pray for Rowan, and our family, more specifically and we thank you all for your concern and support.

 

First we had our consult with the Immunologist:

The main new piece of information she gave us was from some of the blood-work that had been sent off the last time we were there.  This very specific test, involving Rowan's lymphocytes, proved that Rowan definitely has a significant primary B cell problem , not just hypo-gammaglobulemia.    He has significantly decreased CD 27 memory cells.  This abnormal defect is causing decreased antibody production and making it difficult for him to maintain normal antibody responses.  In short, he can't make enough or maintain enough immunoglobulins to fight off infections.    

                *Primary immunodeficiencies are disorders in which part of the body's immune system is missing or does not             function normally. To be considered a primary immunodeficiency, the cause of the immune deficiency must               not be secondary in nature (i.e., caused by other disease, drug treatment, or toxic environmental exposure).

Rowan also has a mildly abnormal T cell problem, but she is sending further studies to look into that to see if it has any clinical significance.  He does have normal CD40L protein expression, which essentially rules out a diagnosis of x-linked Hyper IgM Syndrome.

Recommendations:

Instead of our current schedule of receiving IvIG infusions every 5 weeks, the immunologist wants  Rowan to receive his IViG infusions every week. The doses are what she called “immune-modulating doses” not just the booster or maintenance doses, that we are used to. She also wants his IgG levels to be significantly higher than we have ever been able to get Rowan to.  These doses can have major effects on the kidneys though, so his labs will have to be followed closely to make sure that his kidneys hold up ok.  She explained to me that in her experience, patients with chronic problems, who can maintain higher levels of immunoglobulins have a better chance of going through something as serious as a lung transplant without infections, but when their levels drop lower (like Rowan's usually are), they can get bacteremia from just a colonoscopy or a visit to the dentist (like Rowan has).  The immunologist said that she hopes this more aggressive approach will help Rowan significantly, and it would at the very least be necessary to prepare him for bone marrow transplant, as no one would touch him with his current immune system.

 

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.

Recommendations:

Instead of our current schedule of receiving neupogen (GCSF), which is the bone marrow stimulant that Rowan has been taking for years twice a week, on Mondays and Thursdays, Dr. Shimamura would like him to now take daily doses of GCSF in an effort to eliminate so many peaks and valleys in his ANC.  Instead of trying to get an ANC around 1500, they now want increased counts between 2000-6000 for Rowan.  Again, dosing like this can have adverse effects as well, so Rowan's labs will have to be closely monitored.   The hematologist said that she is getting "extremely nervous” with all of Rowan's dangerous bacterial infections and that bone marrow transplant may be imminent. Rowan is at increased risk for transplant though, because of his compromised gut function on top of his poor immune system.  Complications such as graft vs. host can be serious and even fatal, especially for patients like Rowan.  Though she wants to use bone marrow transplant as a last resort, she was presenting Rowan’s case with the Transplant Team at Seattle Cancer Care Alliance the day after we left.  Once his marrow results are in, from the bone marrow biopsy he had done there in Seattle, she said that the timing may be more obvious.

 

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. 

Recommendations:

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. 

Labs/bone marrow procedures done while Rowan was in Seattle:

Rowan went under anesthesia at Seattle Children’s Hospital one of the days he was there, and had a unilateral bone marrow aspirate and biopsy.  He did well during the procedure.  But the anesthesiologist did inform me that it took “a considerably large amount of propofol” during the procedure to keep him under (I guess that happens when you have had 69 surgeries and probably been under anesthesia over 100 times:). The bone marrow results are pending, and they will let us know when they come back.  Rowan also had approximately 30 mls of blood drawn via his broviac central line two days in a row and various labs were either done in house or sent off.  Those results were pending when we left as well.

 

 

 

 

Final notes from Seattle:

The hematologist, called me back personally, as we were leaving the hospital, and went back over the plan again.  She reiterated how they would like us to start the trial of daily GCSF, weekly IviG, the new enteral formula, new oral antibiotic and new enema, as soon as possible.  They want us to try these changes for 3 months.  If Rowan is able to stay out of the hospital, bacteremia free then we will continue with this plan for now.  If his hospitalizations and infections persist, we will have to expedite his bone marrow transplant.  If Dr. B the transplant team offer a different perspective, or if the BMB results indicate significant marrow changes, that plan could change.  All of the doctors will be in close contact with us and would like to know immediately about any and all changes to Rowan’s health, infections and/or hospital admissions that he has.  They also contacted our physicians back home, to give them their recommendations and to make sure they were all on board.  This plan can only work if they all agree to follow the new protocol.

Summary:

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:)

 

 

Farewell until next time Seattle...