Reduced Intensity Bone Marrow Transplant (RIC)

Connor is going to be going through reduced intensity bone marrow transplant (RIC).  There are not too many pediatric hospitals that performs RICs at this time, I think there were 3 the last time I checked and we happen to live near one of them. Connor has been receiving IVIG treatments since he was 2 years old, first every 3 weeks for 6-8 hours and as he got older and gained some weight it is every 4 weeks and can be out of here in 5 hours (on a good day).  With IVIG therapy, he has managed to stay healthy, some would say that he is the healthiest kid on our block, but we know that he can't stay on this forever as the median age of survival for people with his disease is only 25 years.  So upon his diagnosis of X-linked hyper IGM at age 4, we have gone thru the process of finding a bone marrow match.  Our biggest dilemna in all of his was which hospital to have the BMT at.  He was diagnosed by Cincinnati Children's Hospital ( a wonderful hospital that I would recommend to anyone)  that has a wonderful track record of bone marrow transplants and does over 2000 a year. 

Cincinnati has a protocol that has been very successful for them. It is the same 10 days of chemotherapy but at more intense levels than Children's Hospital in Chicago.  They are VERY VERY strict with the entire process and it would require 100 days of inpatient isolation and followed by up to 6 months of relocating to Cincy. And then coupled with the all the harsh side effects of the intense chemotherapy was just too much to bear.  We had to make this decision that will affect the rest of his life and his ability to have children, his ability to learn and his overall future health.  The chemo is so intense that it could potential harm his liver and other functions permanently. How does a parent make this decision for an otherwise perfectly healthy and happy being? 

Children's is one of three hospitals (at least in 2008) that does Reduced Intensity Chemotherapy (RIC) in pediatrics.  We had so many doubts and questions that we met with the stem cell team at least 5 6 times over the course of 3 years to try to get a definitive answer on what we should do. Children's has a great success rate on RIC for children with immunodeficiency but unfortunately these results have not been published and due to privacy issues, it was difficult to really believe the stats due to the very small sample size of under 20 immuno patients being treated.  Ultimately, there were no definitive answer- it was all based on a cost-benefit analysis.  We pray each and every day that we made the right decision, that this decision was led by God. We pray that this BMT will take so that we don't have to go to Cincy for the full intense BMT procedure.

I will try my best to tell you our entire experience with RIC at Children's so that other parents' can make informed decisions for what is right for their child.  And on that note, Connor's disease mostly presented itself in ear infections, he has had one lung infection and all the rest were in the ears.  He has had neutropenia since he was 4 years old but has not been treated for it since he has been infectin free.  Also had some GI issues where he had soft stool and diarrhea often and had failure to thrive due to weight loss from age 3 to 4.  His GI issue was resolved thru a homeopathic remedy. His donor match is a 32 year old male 7/8 match with a mis-match at the A-loci. 

Please pray for peace and healing!