what disease do I have?

This link is a informational post for those wondering what the disease I have been diagnosed with. I will link back to this post when speaking about the disease.

I have Langerhans cell histiocytosis Or also known as Histiocytosis X . Ah that is much easier to remember and say  right? No……. but hey we can act like it is!

what is Langerhans cell histiocytosis?

Histiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes.


There are three major classes of histiocytoses:

Langerhans cell histiocytosis, which is also called histiocytosis X ( the one I have , don’t it sound fancy?)

Malignant histiocytosis syndrome (now known as T-cell lymphoma)

Non-Langerhans cell histiocytosis (also known as hemophagocytic syndrome)

Histiocytosis X has typically been thought of as a cancer-like condition. More recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which immune cells mistakenly attack the body, rather than fight infections. Extra immune cells may form tumors, which can affect various parts of the body including the bones, skull, and other areas.


Some forms of the disorder are genetic.

Histiocytosis X is thought to affect roughly 1 in 200,000 people each year. It is most often seen in children ages 1 to 15. The rate peaks among children ages 5 to10.
( we always knew I was a rare Gem!)

Pulmonary histiocytosis X is a specific type of this disorder that involves swelling of the small airways (bronchioles) and small blood vessels in the lungs. It is most common in adults. The inflammation leads to lung stiffening and damage. The cause is unknown. It most often affects those ages 30 to 40.
Histiocytosis X often affects the whole body. A disease that affects the whole body is called a systemic disorder.


Symptoms can vary between children and adults, although there can be some overlap. Tumors in weight-bearing bones, such as the legs or spine, may cause the bones to fracture without apparent reason.

Symptoms in adults may include: (* by those that I have)


Bone pain *
Chest pain *

Cough *
Fever
General discomfort, uneasiness, or ill feeling (malaise) *
Increased amount of urine

Rash * (face)

Shortness of breath * ( now on Oxygen)

Thirst and increased drinking of fluids

Weight loss

I also have Vomiting ,Abdominal pain and Irritability which is seen in children with LCH and can be seen in adults.


Signs and tests ( * by the ones I’ve been given)

Complete blood count (CBC) *
X-rays of all the bones in the body (skeletal survey) to find out how many bones are affected * ( something was seen on my shoulders)

Bronchoscopy with biopsy * ( This is how they found out I had LCH)
Chest x-ray *

Pulmonary function tests *

I have also had a bone scan, MRI , cat scan and a tube put to my heart for photos.

Histiocytosis X is sometimes associated with cancer.

There is debate if LCH is cancer or just cancer like. 

Support Groups


Histiocytosis Association of America — www.histio.org

Expectations (prognosis)


Histiocytosis X affects many organs and can lead to death.
About half of those with pulmonary histiocytosis see improvement, while others eventually have permanent loss of lung function.



2 comments:

dddiva said…

You will beat this honey- for yourself and for your family. *Hugs* love you.

Lucid said…

I will pray for you.

Sorry you are having such a hard time! I read back a few posts. Just want you to know that I enjoy your "real" posts far more than the giveaway ones. I just do not log in very often and usually catch up on blogs once a month or so, rather than daily – and sometimes I feel pointless commenting on something a month or two old…