Dr. Roach: Prognosis for rare disease depends on its extent
DEAR DR. ROACH: I have a friend who is the primary caregiver to her son, diagnosed with Langerhans cell histiocytosis. I am unfamiliar with this condition and would like to better understand the challenges and prognosis. Can you help? – O.C.
ANSWER: Langerhans cell histiocytosis is a rare disease that is difficult to understand. Expert opinion is mixed on whether it is, strictly speaking, a cancer. It acts like cancer in many ways and probably is caused by immune system dysfunction. The predominant issue is that a type of cell, called a histiocyte, is usually present in bone lesions and can infiltrate other organs, especially the skin, lymph nodes and lungs. The histiocyte seems to be a cell that is derived from bone marrow, and not the Langerhans skin cell it resembles.
LCH is a rare disease, but one that probably is underreported due to misdiagnosis. It most commonly occurs in children ages 1-3.
Treatment depends on how extensive the disease is, so a careful and thorough evaluation is mandatory. In disease limited to the bone or a single lymph node, surgical treatment may be possible. However, in people with disease in multiple organs or in places not amenable to surgery, treatment is usually with combination chemotherapy. Those with a particular gene mutation may benefit from a medicine used for treatment of melanoma.
Prognosis depends greatly on the extent of the disease. For those with limited disease, the prognosis is very good, with greater than 95 percent survival at five years. For those with higher-risk disease, especially with disease in the liver, spleen, or bone marrow, the five-year survival rate was 84 percent in a recent trial. Your friend is taking care of a child with a complex, difficult-to-treat disease as dangerous as many childhood cancers, which few have heard of and fewer still understand.
Those with multisystem disease should consider being in a clinical trial (information at www.histiocytesociety.org). More information is available at www.histio.org.
DEAR DR. ROACH: I am a 60-year-old woman in excellent health, no diabetes. At a recent annual physical, my urine test indicated some mild inflammation (positive urine nitrite and esterace, WBC 6-10,), for which I am asymptomatic. I was given a prescription for Cipro, as I am allergic to other antibiotics.
I do not want to take Cipro, so I have been drinking lots of water and tea, and eating lots of fresh (frozen) cranberries. Since I am asymptomatic, I don’t know if this is working. My doctor is a very kind man and we have a good relationship, but I am afraid to tell him that I don’t want to take Cipro. I think I’ll risk being labeled an uncooperative patient. I feel fine. Should I request a retest? – L.B.
ANSWER: First off, I don’t think you should worry about being labeled “uncooperative.” It’s your body, and you get to make the choices. Wanting to avoid antibiotics is commendable. So it’s OK to ask for alternatives, including self-care and no treatment.
In your particular case, I wouldn’t have recommended treatment. What you have is termed “asymptomatic bacteriuria,” and most experts recommend against treating it, except in pregnant women, people about to have urologic surgery and kidney-transplant recipients. Treatment in healthy people doesn’t prevent future infections, and if they do occur, they are more likely to be resistant.
There’s nothing wrong with increasing fluid intake, and cranberry juice does have some activity in preventing urine infections; however, it’s not necessary for you to do so. I wouldn’t get a retest unless you develop symptoms.
Dr. Roach regrets that he is unable to answer individual letters, but will incorporate them in the column whenever possible. Readers may email questions to ToYourGoodHealth@med.cornell.edu or request an order form of available health newsletters at 628 Virginia Dr., Orlando, FL 32803. Health newsletters may be ordered from www.rbmamall.com.