NEWARK–Forty years ago, children born with sickle cell disease were not expected to live past their teenage years. Today, medical advances are allowing these patients to live well into adulthood. Patients with sickle cell anemia, the severest form of the disease, now have a survival rate of 85 percent at 18 years old, and patients with milder forms of sickle cell disease have a survival rate of 97 percent at 18.
Charles Feggans, 71, of Elizabeth remembers that when he was diagnosed with sickle cell anemia as a child, the physicians where not sure how to treat the condition. His ongoing pain led to many hospitalizations and medical crises.
“The doctors didn’t really know what to do; it was like a forest to them,” says Feggans, who is a patient at The Frederick B. Cohen, M.D., Cancer and Blood Disorders Center at Newark Beth Israel Medical Center. “My parents took me to the hospital when I got sick and, by the grace of God, I came through. No one lived past age 20 with sickle cell back then.”
Despite a life of illness, Feggans has married and raised three children. He says that “doctors are much more knowledgeable about the disease today and about how to relive the pain,” and he urges sickle cell patient to seek out ongoing medical treatment.
Today, Newark Beth Israel Medical Center treats more sickle cell anemia patients than any other hospital in New Jersey, from the very young to patients in their seventies. One of the hallmarks of the program’s success is the comprehensive transition of patients from pediatric care to adult care at the same facility.
“As the largest adult sickle cell program in the state, we understand that sickle cell disease is not just a condition for pediatric patients,” says Alice Cohen, M.D., director of the Division of Hematology and Oncology at Newark Beth Israel Medical Center. “As patients live longer, our treatment has changed to reflect their need for primary care as well as care for the condition itself.”
Newark Beth Israel Medical Center was recognized by the U.S. Department of Health and Human Services with a grant of $1.6 million to provide continuity of care to sickle cell anemia patients as they transition to adulthood, one of only seven sites nationally to receive funding. Children’s Hospital of New Jersey treats the most pediatric sickle cell anemia cases in the state at its Valerie Fund Children’s Center for Cancer and Blood Disorders, and adult patients are cared for at The Frederick B. Cohen, M.D., Cancer and Blood Disorders Center.
“If we can get sickle cell patients into our comprehensive, ongoing care, we can make a significant difference in their lives and reduce their rate of hospitalization,” reports Dr. Cohen.
Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal crescent shape. The red blood cells of a sickle cell patient are hard and sickle-shaped, causing blockages in the blood vessels.
Approximately 70,000 Americans suffer from the disease, and it is most common among African-Americans. In the United States, it is estimated that 9 percent of African-Americans have the sickle cell trait, and 1 in 600 has sickle cell anemia.
Dr. Cohen reports all of the following as advances in the treatment of sickle cell anemia including:
- The use of prophylactic antibiotics to prevent fatal infections.
- Screening to assess risk of stroke using Transcranial doppler (TCD) technology, and using transfusions for stroke prevention.
- The use of Hydroxyurea, a treatment shown to reduce mortality in sickle cell patients by allowing for increases in hemoglobin production. Hydroxyurea is the first approved drug that can decrease the number and severity of attacks.
- Widespread newborn screening, which allows physicians to identify the disease early and begin treatment sooner.
- Vaccinations against pneumococcus, haemophilus influenza type B, and meningococcus and hepatitis B and influenza. Infection causes life-threatening complications in those with sickle cell disease.
- The increased use of bone marrow transplants, long-term blood transfusions.
While patients are living longer with sickle cell anemia, the hospitalization rate for patients is still high. A study published in the April 2010 Journal of the American Medical Association (JAMA) reported that 41 percent of patients ages 18 to 30 who are hospitalized in acute care end up re-hospitalized within 30 days.
To address these hospital visits by sickle cell patients, The Emergency Department at Newark Beth has enhanced and updated their “Sickle Cell Disease Management Guidelines” to more rapidly triage and treat the many sickle cell patients who are admitted through the ED and relieve their symptoms more quickly.
“We worked with the Emergency Department to improve the experience for sickle cell patients, with staff intervening quickly to rapidly triage and treat the patient during a pain crisis,” says Dr. Cohen. “Most importantly, patients are reassesses within 30 minutes of first treatment and retreated or admitted immediately.”
“Sickle cell patients have a medical home here, and with advance care they can see a dramatic difference in quality of life,” adds Dr. Cohen.
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