"HLH stands for Hemophagocytic Lymphohistiocytosis and is a rare disorder mainly affecting children at an early age. The illness consists of the malfunctioning of the hystiocyte, one of the primary cells to fight infections. Bone marrow produces these cells that then travel throughout the body to fight infections and destroy foreign material.
FLH/HLH patients have overactive histiocytes and lymphocytes, which are white blood cells that can cause swelling, redness, heat, pain and malfunction/damage of organs when they attack and accumulate on healthy lymph tissue (eg: Liver, Spleen, lymph nodes). There are two forms of HLH: 'Familial' and 'Secondary', both require Chemotherapy treatment but:
1) Familial FLH/genetic HLH is the worst of the two. Often young age onset is an indicator of FLH, however exceptions exist. If FLH, there is a 25% chance of other siblings having the same illness. The danger of FLH resides in the necessity of a bone marrow transplant in order for the patient to be cured.
2) Secondary HLH is none genetic and presents itself when the body's immune system goes into overdrive following a major infection. Secondary HLH is treated with more relative ease than FLH as although Chemo is required, generally following treatment the underlying infection is cured and no bone marrow transplant is needed.
Because the disease is relatively new from a research prespective (1985) and causes as well as symptoms are still being discovered, many cases go undiscovered (or too late) and lead to fatality for the patient. It is important to catch the illness early! The illness affects officially 1 in 1.2 million children, unofficially it is said that it affects 1 in 50,000 children. this means that the illness is as common as childrens' cancer and this implies that for every child that is diagnosed for HLH/FLH 24 die because they are misdiagnosed.
We prayed for Secondary HLH, so Miabelle could recover soon and Noelie is spared the illness, but we were served with an aggressive form of FLH. Destiny spared us Noelie, who was cleared of the desease, yet Miabelle was delivered a major blow from which we will not know for a long time whether she will survive.