Obviously I talk a lot about Emily's journey with HLH. You read the stories on here and what she has had to deal with and what is to come. You have heard the side of HLH that has been ONLY about the infectious side of things. The side that is NOT genetic. You have heard me say how blessed we are because Emily has been able to celebrate another birthday with us. We have been able to see how far she has come and how much she has overcome since 2007.
There's another side to this disease. A side that is not a comfortable side. A side that you don't hear to much about. Let's face it ~ frankly, you dont hear too much about HLH since its so rare (one in one million ~ 1.2 million to be almost exact). The side that I am speaking of is the genetic side of things. The side of things where the outcome is an almost guarantee of death.
I just finished reading a book called "Love Trumps Grief" by Kristen Akin. This is the FIRST book I have read that is a personal story dealing with HLH. This lady and her husband are incredible...not to mention their kids, Matthew and Andrew. You see, Kristen is a carrier and her kids both carried the mutation for HLH. Andrew, the youngest, first showed signs at 10 weeks old. Her struggle to cope with things and living basically in the hospital for the entire time (from 10 weeks until his passing) is just unbelievable. Her older son, Matthew, carried it but hadnt shown signs yet. After four bone marrow transplants (3 for Andrew and 1 for Matthew) both of her boys died from this horrible disease.
Can you imagine? Thinking your kids are perfectly healthy one minute and the next fighting to keep them alive. Even though Emily never had to have a bone marrow transplant, I could relate to so many things she mentions in her book. All the medications, CAT scans, MRI's, biopsies, x-rays, surgeries, being in ICU, seeing your child on a vent, therapy, living in the hospital, all the medications and the list goes on. I had a terrible time reading this book and found myself having to set it aside numerous times because of the overwhelming emotional reaction I had to it.
Reading this, I have learned even more about the disease ~ a side of the disease that feel so blessed to have never had to encounter. A side of the disease seems too unbearable to think about but also to unbelievable how horrible HLH can be. This side of HLH, the genetic side of things, makes me feel so blessed with the infectious side that Emily has.
This has made me realize how blessed I am to be able to kiss my kids good night each night. How blessed I am that this isnt genetic and that Benjamin doesnt have it or hasnt shown any type of signs of having it. How blessed we are that Emily is still here. Yes, she is behind in school; yes, she has issues with memory; yes, she still takes chemo and will forever and there will probably be other issues we encounter later due to the medications that she has had to be on. But she is here.
Be thankful for what you have for you know not what tomorrow brings.
If you would like to read more about Matthew and Andrew Akin's story, you can find the website at http://www.matthewandandrew.org
