Many EMS personnel have heard of sickle cell disease and the pain it causes. But it is quite possible that a number of EMS providers have treated patients with this disease without recognizing it as such. This may have prevented them from doing as much as they could to alleviate what is often acute suffering for the patient.

Sickle cell disease is relatively common in the United States ; just one of its many forms affects more than 70,000 people, and more than 1,500 babies are born each year with the disease. Sickle cell disease in black Americans occurs in 3 out of every 1,000 (or about 1 in 400) live births. It's most common in people who are descendents of residents of malaria-endemic areas of the world. Sickle hemoglobin is found in Africans, Arabs, Turks, Greeks, Italians, Iranians and Asiatic Indians. 1 Inheriting one sickle gene or sickle cell trait provides some protection from the red cell parasite malaria. The carrier state is usually asymptomatic, but hematuria and sickle complications can occur under severe dehydration, temperature or pressure change or other bodily stress. 2

Sickle cell disease results from mutation, or change, of certain types of hemoglobin chains in red blood cells called the beta hemoglobin chains. We know that red blood cells deliver oxygen to working or active tissues. This process called oxygenation happens in the lungs where hemoglobin (the molecule in the red blood cell) takes on oxygen and, at the same time, releases carbon dioxide. This process is reversed at the tissue level. As late as the 1980s, however, the actual population of those affected by sickle cell was small, as the median age of death was 14, with over 30% of all sickle cell patients dying before the age of 5. 3 Today, thanks to improved health care and better management of the disease, many victims are surviving into their 40s and 50s, increasing the prevalence of the disease and the likelihood of its victims entering the EMS system.

While sickle cell disease can cause a wide range of complications, some of which are life-threatening, many emergency caregivers lack sufficient understanding of the disease to comprehend the significance of theses complications. Also, possibly because of their apparent need for frequent and large doses of pain medication, there is a tendency to characterize these patients as problem patients ? or, worse, as drug seekers. Because of this bias, sickle cell patients often receive substandard care both in the field and in the emergency department.

With a better understanding of this common but complicated disease, its complications, the current methods of managing its symptoms and the need for compassionate care, providers can help alleviate the patient's suffering and prevent life-threatening complications from arising.

Case Presentation

Paramedics are called to a Cleveland residence in the middle of winter for a report of a sickle cell patient in crisis. The crew meets a police officer at the scene, who tells them that a female patient, now seated in his squad car, had been invited to the house by a male occupant to cook dinner for him and his girlfriend. When the girlfriend arrived, the man, believing that an unpleasant confrontation might occur if the woman was still present, asked the woman to leave. He escorted her out the back door and locked her out without her coat, boots, hat or purse. She remained outside until neighbors saw her looking cold and in pain, so they called 9-1-1.

The paramedics find the patient ? a 26-year-old female ? sitting in the police car, alert and oriented. The primary survey appears unremarkable except that her skin is cold and dry. She has a blood pressure of 130/90, a regular pulse of 86 and her respirations are 24 per minute. Concerned about further exposure to the cold, the paramedics move the patient to the ambulance to complete the secondary survey in a warmer environment.