What the doctors are thinking

It is believed that John has Histiocytosis X as well as cancer. This progressive diagnosis is still inconclusive, so nothing you read until Tuesday is confirmed fact. I will explain Histiocitosis X below, let me clarify the cancer issue first, just because it's easier! The doctors are trying to determine between three different forms of cancer. They have concluded that lymphoma is NOT a factor any more. We do not know what other cancers they suspect.

Histio-what?!

Histiocytosis X, (Hsto X) is a condition of the immune system. It is characterized by an abnormal increase in the number of certain immune cells, called histiocyte cells. Histiocyte cells include monocytes, macrophages, and dendritic cells. (T-cell lymphoma is a related condition... so the doctors were definitely on the right track)

Hsto X has typically been thought of as a cancer-like condition, but more recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which rogue immune cells attack the body, rather than fight infections. Some forms of the disorder are genetic. Hsto X occurs in 1/200,000 per year.

Another type/name of this disorder, pulmonary histiocytosis X, is characterized by inflammation of the small airways (bronchioles) and the small blood vessels in the lungs. It is most common in adults. The inflammation leads to stiffening and destruction of the walls of the alveoli (parts of the lung). The cause is unknown. People 30 - 40 years old are affected most often. Cigarette smokers make up 90% of patients. Yes, John did smoke for some years. 50% with pulmonary Hsto X improve, while the other 50% loose lung function.

Symptoms:

• Cough
• Shortness of breath
• Chest pain
• Fever
• Weight Loss
  
• General discomfort, uneasiness, or ill feeling (malaise)
• Increased amount of urine output
• Thirst and increased drinking of fluids
• Bone pain
• Rash
  

The tumors produce a punched-out appearance on bone x-ray. Tumors in weight-bearing bones, such as the legs or spine, may fracture spontaneously. There is often systemic (whole body) involvement as well, causing rashes, lung problems, gum infiltration, lymph gland swelling, hormonal problems, enlargement of the spleen and liver, and anemia. Not surprisingly, with a disease that affects so many systems and organs, Hsto X may be deadly.
This disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Radiation therapy or limited surgery may also be used to treat bone lesions. Patients are also treated with supportive care to reduce symptoms and treat infections that may occur when the immune system is compromised. Treatment may include antibiotics, breathing assistance with a respirator, physical therapy, selenium-based shampoo for scalp problems, and hormone replacement to deal with hormonal dysfunction.

So, how is the family?

It's a balancing act. How to temper emotions against new medical conclusions every day is a struggle, obviously. The regular routine can be a wonderful thing. Tonight the decision was made to enjoy a stir-fry dinner with basmatti rice (a new found favorite for Matt & me) . After Matt & Priscilla bought the rice, veggies & bottle of sauce, Matt called me on my cell phone in VA. They had bought the rice from a bulk container... no cooking directions! So we all laughed as I tried to recall the ratio. I'm sure it was good.