Aggressive Pain Therapy Helps Sickle Cell Patients Cope

Experiencing a crisis can mean different things to different people. However, experiencing sickle cell crises means learning to live with recurring episodes of excruciating pain.

Sickle cell is an inherited disease, found primarily in African Americans, which can lead to stroke, heart attack and a shortened life span. The disorder derives its name from fragile red blood cells that resemble crescents or sickles. Rigid and sticky, the cells are unable to transport oxygen and essential nutrients to tissues and organs.

Dr. Nancy Glass, director of the pain service at Texas Children's Hospital, says the biggest barrier to effective sickle cell pain management is making judgements about whether or not patients are hurting.

"A child says, `I have sickle cell disease and I'm in pain,"' Dr. Glass says. "Because of prejudicial attitudes, medical staff not trained to treat children might say, `You don't look like you're hurting that badly.' We then get into a cycle where the child has to put on an act to get the pain relief he or she needs. It's very frustrating for the child and family."

Dr. Glass explained there might not be any obvious signs during a pain crisis.

"We have to depend on the patient's self-reporting," she says. "Vital signs and physical exams are not as effective as asking the patient."

School-age children are asked to rate their pain on a scale of one to 10. If they say their pain is an eight, Dr. Glass administers medicine to reduce the pain to what the patient says is a tolerable level. Most patients identify a pain score of two to three as something they can manage.

"By the time patients are admitted to the hospital, they have usually started some type of pain therapy at home," Dr. Glass says. "Commonly, oral narcotics are recommended, and when that is no longer effective they are admitted through the emergency room. We start them on intravenous narcotics and order a patient-controlled analgesia (PCA) pump.

"If the pain is in the lower extremities or the lower back, we can use an epidermal analgesia. We insert a catheter and use low doses of local anesthetic and narcotics: the epidermal opens the vascular vessels so more blood can get through, which has been very effective in some patients."

Other therapies include around-the-clock ibuprofen or acetaminophen; applying warm, moist heat or heating pads to painful areas; and physical therapy. Morphine and other agents have replaced Demerol, which accumulates in the body and places the patient at risk for seizures.

Dr. Glass notes that sickle cell patients do not use or ask for narcotics when they are pain-free and are not at a greater risk for developing opiate addiction than the general population.

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©2006 Texas Medical Center

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