I am writing this note as an urgent request to reach out to HLH families all over the globe. There are lots of newly diagnosed families with HLH that need our help in making the right decisions for their children and this is my plea to reach out to them because I know they read Hannah’s carepage. If you are an HLH family and you agree with this statement, then please copy and paste this to your caring bridge pages, carepages, blogs, etc. You have the ability to reach so many.
I may get shut out after this, but that’s okay, because I know that I will have been heard. If this message can reach just one family going through HLH, and transplant, and I can possibly save a life, then I will have done my job. I cannot just sit and wait idly by knowing that this is happening.
Remember .. . . “Knowledge is Power”
I am posting this as a general statement, because I am sick to my stomach hearing what doctors are doing in hospitals that don’t have enough experience with HLH. First, when searching for the hospital for transplant, ask the right questions. . .
How many HLH cases have you treated? What is your success rate? What are your isolation and precaution standards (gowns, gloves, masks, reverse isolation)? What factors do you use to you consider the transplant successful? What is the preparative regimen you use? Is it different for marrow, stem cells, or cord blood transplants? How often is engraftement checked (should be weekly until about 6 months post transplant at minimum, then switched to monthly checks). There are so many more.
Get informed, don’t believe everything your doctor says. Do your research, ask questions, talk to other families. HLH is a whole different beast and the BMT process is very unique, as well as the treatment afterwards. People are given misleading information from their doctors because these doctors deal mainly with other disesease (Leukemia, anemias, other cancers, etc.) and they end up treating them like any other BMT patient.
This is not the case with HLH!!!
Holding engraftment numbers in the 90s is absolutely incredible, so are the 70s and 80s. When engraftement numbers start trending downwards steadily and reach the 20s or 30s, that’s when a boost of a Donor Leukocyte Infusion (DLI) is considered! HLH CAN be kept at bay with numbers that low. We’ve seen it time and time again from patients that are holding in the 20s with no return of HLH. If a doctor says that anything below 100% donor cells is a failure in an HLH patient, he/she is a complete and total idiot, with OBVIOUSLY no experience with HLH. If this happens, get your child to another hospital with lots of HLH experience IMMEDIATELY! and I don’t mean wait and see . . . I mean RUN!
When you give DLIs to a patient with high engraftment numbers, the next thing to occur will be graft versus host disease (GVHD) and it will come down with a vengance. It will end up being an overload of cells and the body’s instinct will be to fight. Fighting mild GVH is one thing, but a full on attack can be deadly.
Also, DLIs should be given and then wait a period of a month at minimum to wait for engraftment changes. It can take a while for cells to make their way and mature enough to be seen on tests.
If there are HLH symptoms and these can be checked doing Soluble IL2 testing, this is one of the true markers for HLH, and not necessarily based on Ferritin numbers. Ferritin numbers can be skewed by medications given after transplant like anti-fungal medications. Be vigilant and get educated.
I’m not claiming to be the expert here, so please don’t misunderstand, HOWEVER, I have made sure to stay on top of what’s going on in the HLH world because of my own experiences with Hannah. I have attended the HLH conferences the last two years, and have posted these notes on the HLH Family group on Facebook and here in this forum. I try and stay educated on the process, and keep in touch with HLH families across the globe. I am also very intertwined in the HLH research as well, and yes I have a 3 year HLH survivor. So I guess that makes me a little more ‘in the know’.
I welcome everyone’s comments, both good and bad, and am posting this from my heart because too many children have lost their battle with HLH as it is, but to see them lose their life needlessly to the stupidity of doctors is just too much.