September 24, 2001

Sickle Cell Awareness Month

BINGHAMTON, NY - There are thousands of men, women and children living normal lives until the pain of sickle cell disease pays an unpredictable visit. Sickle cell disease is an inherited blood disorder that causes red blood cells to distort, from a rounded disk to a crescent or "sickle" shape, and clog capillaries. Sickle cell disease affects people of all racial and ethnic groups. People of African descent are highest risk, with approximately 1 in 400 affected. Hispanics and those from countries around the Mediterranean Sea, the Middle East, India and Southeast Asia are also at risk.

"Most of the problems associated with sickle cell disease - including pain in bones, muscles and the abdomen, as well as ulcers, stroke and internal organ damage - stem from the blockage of blood flow," stated Mr. Charles Wolford, Public Health Director, of the Broome County Health Department.

Persons with sickle cell disease can have many complications. There are often problems with normal growth and development. Some individuals may experience pain in their joints and chest pain that is worse when they take a deep breath. "Sickle cell causes repeated bouts of severe pain," noted Mr. Wolford. "These attacks of pain are sudden, severe and affect many areas of the body. The most common areas are the arms, legs, abdomen, chest and back, and can last hours, days or even weeks," acknowledged Mr. Wolford. "Sometimes, a high fever can further complicate an attack."

"There is no specific cure for these episodes," stated Mr. Wolford. "However, treatment is more effective when complications are identified soon after they occur. Ideally, patients with sickle cell disease should be under the regular care of their health care provider."

The complications of sickle cell disease can include:

• Joint swelling
• Enlargement of the spleen (early in the disease)
• A heart murmur and/or enlargement of the heart
• Decreased vision
• Leg ulcers

"During September, Sickle Cell Disease Awareness Month, we want to remind people how early diagnosis, patient education and proper treatment can significantly improve both the quality and length of life of all patients," he said.

The New York State Newborn Screening Program has been testing every newborn in the state for sickle cell disease since 1975. Every year, more than 250 infants are diagnosed with the disease and referred to comprehensive sickle cell centers for care.

Sickle cell is a lifelong, chronic disease with no cure. "Establishing a good relationship with a health care provider is very important for the patient with sickle cell disease," Mr. Wolford stressed. "Because sickle cell causes so many health problems, treating the symptoms is crucial in helping to prevent more serious complications."

Special precautions that people with sickle cell disease should consider include:

• The pneumococcal vaccine to prevent infections of the respiratory system
• Preventive antibiotics before a dental procedure
• A daily folic acid supplement to prevent a deficiency of this nutrient
• An annual eye examination to detect eye problems early
• Prompt visits to a health care provider at any sign of infection, including fever and chills
• Drinking plenty of fluids to maintain urine flow and to prevent blood clots

"General supportive care for sickle cell disease, such as pain control and fluids, are very important for a good quality of life," noted Mr. Wolford. "Each patient needs to take responsibility for his or her own treatment. The use of antibiotics to treat infections and narcotics to relieve pain should be closely monitored by a health care provider, as well as the patient."

For more information on sickle cell disease during Sickle Cell Awareness Month, contact the Broome County Health Department at 607.778.3944.

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