Maverick was diagnosed with CGD (Chronic Granulomatous Disease,
http://www.cgdsociety.org/whatiscgd ) in July 2017. He has the X-linked gene that is passed from a mother to their children. It primarily affects boys but girls are the carriers with a few side effects (
http://www.livingwithcgd.org/x-linked-cgd-carriers). CGD affects Mavericks ability to fight certain bacterial and fungul infections. He has been hospitalized for his condition 3 times so far. Once when he was about 8 months old for an enlarged lymph node which needed to be drained. We had mentioned to several doctors that this disease runs in my (Niki's) family (my aunt has 5 boys with CGD) yet no one thought he had it as it is so rare. Finally, after many doctor visits for consistent fevers (ranging from 100-104 most days) on and off over the last year, someone listened to me when I said something isn't right with Mav and I once again discussed my family history of CGD. Maverick was tested and we had a confirmation that he does have the X-linked CGD. At the time of confirmation, he was also diagnosed with pneumonia which lead to his second hospital stay for a lung lavage (rinsing of the lungs to find exactly what was growing in his lungs) and for IV antibiotics. We have been treating Maverick daily with preventative medication (2 daily oral antibiotics, an oral antifungal and actimmune shots 3 times a week) and nebulizer treatments for the pnemonia(which ended in November 2016) since his diagnosis in July. These medications have helped him fight off some illnesses that may have come his way. During work up week for the BMT they found 2 different spot on his lungs that required surgery. He ended up with a chest tube and then developed a chyle leak (leaking of lymphatic fluid) and he ended up with a 2 week hospital stay.
This is a life long illness with many expected hospitalizations and for him the only cure is a bone marrow transplant(BMT,
http://www.cgdsociety.org/whatiscgd/treatment#bonemarrowtransplant ). After much thought and lots of consultations with several physicians, we have decided to go ahead with the BMT. He has a lot of things in his favor for this procedure to be successful right now. These include, being so young, being a male, having several excellent cord blood matches, and is currently as healthy as he has been in his life. We tested his brother Hunter and he was not a match, we then went to the national registry and did not find a viable live donor. We did find several excellent cord blood matches!!
He will need a Gastro-tube(for feeding and meds) and a Hickman catheter(for IV meds, etc..) put in prior to the BMT. The surgery for these is scheduled for Oct 26th. Maverick and I will be staying overnight. He then needs 3 weeks to recover, meanwhile several tests and imagining will be done in that 3 week recovery period, and then the BMT process can begin. The day he gets admitted for the BMT he will start an 11 day course of chemotherapy daily to make room for the new cells. Once he is done with chemo it will be transplant day, they call this day zero. Then all we can do is wait for his body to accept the cells and this will take several weeks. They told us it will most likely be a 4-6 week hospital stay. While the hospital stay is weeks, the first 100 days after transplant is a critical period for recovery. If this is successful, he will be cured of CGD and the everyday risks will be gone and he can live life with a normal immune system and no lifelong medications needed.
We ask that there be no visitors during the 4-6 week hospital stay. He will be completely immune compromised during most of the stay and anything we can do to avoid infection will be best for his success. We will see how things progress during the BMT process and how the doctors feel about visitors for him and for myself after that time.
Also, if anyone is thinking of visiting our house prior to the BMT and isn't feeling well or has been around someone close to them that is ill, we ask that you kindly stay away. There are several things that are out of our control but keeping him isolated from outside germs is our goal.
All we can do now is pray that he stays as comfortable and as happy as possible hoping that the BMT is 100% successful!! He is the toughest kid we know (if you've ever tried to make him do something he did not want to do you would know as well!!). He is also the sweetest and absolutely most adorable son anyone could ever ask for.
Thank you all for your thoughts and prayers as we go along this journey with our tough little dude!!!
Niki & Chad
