Allan Platt, PA-C is a Senior Associate in the Department of Family and Preventive Medicine, Physician Assistant Program, Emory University School of Medicine. From 1984 to 2004 he was Program Coordinator and Physician Assistant at the Georgia Comprehensive Sickle Cell Center at Grady Health System. He is also the web designer of their site which has an international audience. www.SCInfo.org

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Questions

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Evelyn Corsini:

What is the prevalence of sickle cell diseases in the U.S. and are they only found in African-Americans?

Allan Platt, PA-C: Sickle cell diseases fall along a spectrum of different blood disorders. Sickle cell anemia is by far the most common variant. We estimate that 80,000 people in the U.S. have sickle cell anemia, and 3 million more people have sickle cell trait. Both groups should be aware of their diagnosis and what treatment might be needed, as even those with only sickle cell trait are at risk for an episode of sickling if their body is under an extreme physical stress.

It is a myth that only African-Americans have a sickle cell disease, although it is most prevalent in this group, as it is a genetic trait that has been diagnosed in Arabs, Greeks, Italians, those from India, and Latin Americans. Definitive diagnosis is made with a full hemoglobin electrophoresis. Most states now do newborn screenings for sickle cell anemia so more early diagnoses are being made. We have seen a number of adults who were diagnosed in their 30’s or 40’s, only after a sickling episode brought on by an infection or other bodily stressor.

EC: What kinds of medical problems can people with sickle cell disease develop and what is the treatment?

AP: The clinical manifestations of sickle cell anemia result from increased blood viscosity and vascular obstruction by deformed sickled red cells. The most common complication is a pain crisis, but many other organs can be involved and many patients will develop multiple disabilities, such as severe chronic bone pain, neurologic problems, pulmonary problems, and renal failure.

The daily use of oral hydroxyurea is a very effective treatment and research has shown that it can cut the number of crises in half, and can cut the need for blood transfusions in half. Studies show that this daily medication increases fetal hemoglobin production and prolongs life. The medication needs to be carefully monitored as it can cause bone marrow suppression, skin ulceration, and hair loss. One is always "walking the line" between toxicity and a good effect.

It was thought in the past that children with sickle cell anemia would not survive to adulthood, but now with better treatment the average child will reach their 40’s to 50’s, and we are even seeing patients who are in their 60’s and 70’s

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EC: What are some of the social issues associated with sickle cell anemia?

AP: Information about sickle cell anemia has not been taught well in medical schools, so many health care providers do not start with a good basic understanding of the disease. When you add to this the fact that the majority of the patients are from a minority group, have poor health insurance, and when experiencing a crisis need to go to an emergency room for pain medication, it is easy to understand why they face many barriers.

Ideally, both the child and the adult with sickle cell anemia should be managed by a multi-disciplinary team that can respond to all of their needs, including pain management and rehabilitation. While there are programs for children, like most childhood diseases that formerly were considered fatal in childhood, there are inadequate resources for adults with sickle cell anemia.

EC: What is unique about the Georgia Comprehensive Sickle Cell Center at Grady Health System?

AP: Our center was founded using funds from a state grant. The state has continued with funding, and other sources of funds have been developed, because it was found to be very cost-saving to the state of Georgia by reducing hospital admissions, to provide this comprehensive program. We provide the only 24 hour emergency department in the country that is dedicated to the treatment of patients with sickle cell anemia, staffed by members of our team. People with sickle cell anemia may come from all over the country for a one day consultation, and then return to their providers at home with a plan. Some patients have moved to Atlanta just to be near our facility.

EC: What should community health care providers keep in mind when treating patients with sickle cell anemia?

AP: There are many things that can be done to improve the care of sickle cell patients in their home community. Unfortunately, the worst place for a patient to go when in a pain crisis is to an Emergency Department, but that is where they have to go. I have found that most emergency department staff members are jaded. When I speak with them they frequently tell me that they believe 100% of the sickle cell patients they see are addicted to opiates. Research reports and my own personal knowledge show that true addiction is only a problem with 5% of the population.

I tell staff in Emergency Departments that they have to get over their opioid phobia and believe the patient in pain. They may get "snowed" once or twice but they should always presume real pain and have the patient put a number on it. If the patient is taught to use a pain scale and is believed, this can work. I suggest that the patient’s primary care provider create a sort of "medical passport" to give ER staff, that describes the patient’s problems and current pain treatment plan.

Another problem is that people who appear to be in a sickle cell crisis may have an underlying problem that is life threatening. What hurts is not always sickle pain but can be an ectopic pregnancy for example, in a female with abdominal pain. The patient themselves can say whether this is unusual or typical pain. Remember to listen to the patient and accept that they may know more about their disease than you do.

There are few hematologists who are interested in sickle cell anemia. I think these patients are best followed by one primary care provider who knows how to get consultation when needed. We post emergency guidelines on our website and do many telephone consultations.

Know that there is sickle cell expertise available and that the health care community that specializes in this difficult problem is usually generous with its time and help.

EC: Is there current research being done, and are there advances in the care of sickle cell anemia?

AP: Our program has a research consortium as well as providing clinical care. While hydroxyurea is the current standard of care, there are new treatments in the pipeline. We have gathered good information about the use of nitric oxide. There are also studies of high dose omega -3 fish oil, red cell dehydration prevention, and l-argenine. Stem cell transplantation using cord blood presents the potential of a cure. We have seen cures with successful bone marrow transplants with well-matched sibling donors.