"Life is not measured by the number of breaths we

take, but by the moments that take our breath

away.” Author Unknown


Friday, September 12, 2008

All about HLH

Histio..WHAT?

Hemophagocytic Lymphohistiocytosis. Are you kidding??
When we first heard the term we wondered...could this possibly really be the name of a real disease? The Doctors just call it HLH. When they actually call it by its’ full name (and most Doctors have never even heard of it, no kidding), everyone looks a bit dazed as they always catch the “histio” but inevitably miss the part before and after…
Hemophagocytic LymphoHISTIOcytosis
It would be easy to do, wouldn’t it? To catch just the HISTIO part? Histio is easy to remember, the rest is just really unbelievable. It would be nice if you could do this with the actual disease too. Cut out the parts that are hard to deal with and just keep the parts that are easier. It would be great, really. Unfortunately the disease is actually very much like it sounds. It truly is horrendous.
In typical “Connor fashion” he managed to get this disease. A disease that strikes…drumroll please…

1.2 in every 1 million children under the age of 15

Yes, he got this on top of his other boatload of conditions, issues, and accessories. He must have figured he needed to be even more interesting to the medical profession, and get even more types of specialists involved in his care. This he accomplished!
Connor was struck with HLH at the age of 7 ½. It came out of nowhere and struck him hard. Here is a little bit of information about HLH…

"Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease of normal but overactive histiocytes (macrophages) and lymphocytes (white blood cells).
It is characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations."
“Typical symptoms of HLH besides persistent fever are pallor (paleness), jaundice, liver and spleen enlargement, and neurological symptoms, such as irritability or even seizures. The involvement of the bone marrow, the site of blood cell production, can lead to severe decline of the blood cell counts (red and white blood cells and platelets). On bone marrow examination, histiocytes that are “eating” other blood cells (also known as phagocytosis) can be detected.”
(source - http://www.histio.org/)

HLH is a very bad disease to have for any child, but especially for children who already have significant medical issues. For Connor, HLH was, as we’ve always called it, his lightning strike. HLH was the completely bizarre, out-of-nowhere disease that really made things significantly worse for him.
While we know that a severe fungal infection that led to sepsis caused Connor’s death, we also know that HLH and Mito disease contributed significantly, because they led to his body’s complete and total inability to fight the fungus. Due to this, we feel very strongly about helping to educate others about these diseases, and fundraising to find cures for them.
Connor’s buddy Joel also fought Histiocytic diseases and is now in heaven with him. Connor’s other friends: Jacob, Austin, Patrick, and Gavin are battling different forms of Histiocytosis today. Please pray for each of their families.

Please take the time to read more about HLH and other Histio diseases by checking out the Histiocytosis Association of America’s website at:
http://www.histio.org/

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