We have numbers. Lots of numbers. I recently printed Aria's CBCs from the last year and laid them all out next to each other. A word to the wise: if your kid is living with progressive bone marrow failure, you don't want to do that.
Bone Marrow Failure is described in severity as mild, moderate, or severe. At this point Aria has anemia, thrombocytopenia, and neutropenia in addition to a number of other hematological things going on (poorly oxygenated blood/high MCH, low hematocrit, low WBCs and RBCs, low hemoglobin, etc). Without talking about Aria's specific numbers, at this point we can say that she is in severe failure in the top two categories shown below and moderate to severe in the last.
We can talk about our options and how we will handle things when the time comes but we don't really have any idea what those answers will be until we are there, actually forced to make the hard decisions. Will we do androgen therapy? No. Will we move to transfusion dependency? We don't know. We do know that the complications of long-term transfusion dependency aren't always pretty and its likely that at some point an allergic reaction to transfusion will occur and we will be left with no options. I don't know what iron overload looks like. I don't know what a reaction to transfusion looks like. I don't know what choosing not to transfuse looks like. We don't even know what the quality-of-life we may or may not try to prolong with transfusion will look like at that point. I don't know what will be the best option for our child at that point and I'm not interested in investigating what the experience of prolonging life with transfusions vs not would be for our child.
Not today. It's not time for that today.
Some might wonder why we aren't desperately searching for a bone marrow match for Aria. That's another discussion for another day but just know a few things about Fanconi Anemia and bone marrow transplantation.
- Not every child with Fanconi Anemia is a bone marrow transplant candidate.
- Bone marrow transplant requires other organs to be healthy.
- Bone marrow transplant is not a life-saving treatment for Fanconi Anemia. It is life prolonging. There is no treatment for Fanconi Anemia.
- A risky bone marrow transplant would help with hematological issues but will not fix the problems present in nearly every other system of Aria's body. Nothing that Aria has been through in her four years on this earth is directly related to her hematological issues, including the 20 surgeries she has had and years spent inpatient. These issues would continue on and would continue to get worse post-transplant.
- Bone marrow transplantation is a grueling process through which communication with the recipient is extraordinarily important.
- People who survive transplantation are still at extremely high risk for multiple forms of cancers, particularly in Aria's complementation group. These cancers require treatment, during which communication with the affected individual is extremely important.
- Bone marrow transplantation is an excellent choice for many children living with Fanconi Anemia. For our child, it simply is not.
Now that I've vaguely laid out all the information we have about Aria's current hematological status I'm sure you're all wondering what our families next move is. How will we deal with the uncertainty with which we are living?
That answer is simple. We've bought season passes to Disney World for all four of us and we are going to go as often as we can. We are going to love our girls and create memories.
|We really like Disney World!|