Now that Zoe has stabilized and begun Chemotherapy, Michelle and I have started to think more critically about the Bone Marrow Transplant (BMT) that has been recommended. There are a number of problems to be tackled, and it really is a matter of life and death so it’s incumbent upon us as Zoe’s parents and advocates to do our best to make a good decision.
Here are the complicating factors, as best we know so far:
- While Zoe is expected to survive chemo and see no long term problems from it, the BMT has a very high mortality rate should she be subjected to it: generally accepted to be 30%, as in, 30% of patients die from complications
- BMT is the only cure for the Familial type of HLH, however it is not necessarily required to survive and thrive in the wake of acquired HLH
- Determining if Zoe has FHLH is difficult and not 100%; we’ll do genetic testing and if she has the markers, we will know, however it is also possible for the marker to be “hidden” as I understand it, meaning doctors are likely to recommend a BMT no matter what just to be sure
- There have been cases of children under 1 year of age who were indeterminant as to which type they had and who opted not to get the BMT and who still survive many years later with no symptoms of the disease remaining
- In addition to the base mortality rate of the procedure, the side affects of complication to BMT can include both neurological and physical defects, meaning Zoe has a rather high chance of either dying or being left with lifelong mental or physical disabilities to contend with; the chemotherapy introduces none of these risks by itself.
- If we are unable to determine if she has FHLH and opt not to do a BMT, there is a very high chance that if she turns out to have FHLH the disease could reemerge rapidly and a simple infection could in fact kill her before the disease can be put into remission again
At this point we are focused on getting Zoe through the first stages of Chemo and discussing these issues with Dr. M. We need to know a great deal more about the genetic testing and how sure we can be about the results — is it ever possible to know that a child does not have FHLH and instead has the acquired type? The answer to this will help us move toward a decision. We are very wary of the idea of putting Zoe back in harm’s way without being sure that there is a necessity, given that the BMT can be so very deadly.
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Hello Kaelin. I am so glad you have a few days break to relax and recover. It sondus like you as brave as always this week and I am very proud of you. I was thinking today how when you were about four years old you and you friend William were playing together. He had a playhouse there and it sat above a fenced twelve foot drop off. I turned my back for a few seconds and the two of you climbed on the roof and were preparing to jump off and down into the gully. You were born fearless and always have been. It is such an admiral trait. To this day I am amazed at how you face any challenge that comes your way with your chin up, full of courage. You have such a positive attitude, it comes from deep within you. Often when I get into a scary situation at work I think if you were there with me you would be telling me with a smile on your face and a positive attitude not to be afraid. I know that no matter what comes at you these next few months you will face it as you have faced all the other challenges in your life. You have so many people that look up to you and find inspiration for their own lives by the example that you have set with yours. Know that your family, your friends and your Heavenly Father all stand by your side during this time. Not a minute goes by that I don’t think how proud I am of you, and how honored I am to call you my son. I love you and I will see you soon!LoveDad