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Easing the Pain of Sickle Cell Anemia
Tuesday May 30, 2000



A microscopic view of sickle cells.

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"At age three, Sola received an IV morphine infusion for severe pain in her legs. It was seven days before she had any substantial pain relief. [When we took her home from the hospital], her legs were so weak that it took a week for her to regain her strength. My husband and I carried her around the house, or she would crawl until she was able to walk again."
- Victoria Odesina
ACHILD IN PAIN - an image guaranteed to tug at a nurse's heart. We immediately start thinking, What can I do to help this child?
Victoria Odesina, RN, CNS, knows this feeling firsthand, not just as a nurse, but as a mother. Sola, whose hospitalization she describes above, is one of her two teenaged daughters with the most serious form of sickle cell disease (SCD) - sickle cell anemia.
Out of necessity, Odesina, a clinical nurse specialist, has become an expert in SCD pain management. She knows when to apply hot packs and analgesics. She knows when to consult with the pain management specialist. And she knows when to rush her daughters to the ED. Once in the ED, she knows how to be sure her daughters receive proper treatment.
But other patients aren't as lucky as Odesina's daughters. These patients too often fall victim to practitioners' lack of knowledge about how to manage SCD pain. In fact, the American Pain Society cites clinicians' limited knowledge as a leading barrier to adequate assessment and management of SCD pain.1
Nurses can help turn around this deplorable situation by enhancing their knowledge of SCD.
Pain Assessment
"Sola's pain usually begins in her legs and radiates to other parts of her body, such as her back, waist, thighs, and sometimes to her chest. She describes her pain as sharp, throbbing, and aching. The pain may last from three to nine days."
A logjam of cells causes the pain of SCD. As red blood cells "sickle" from a lack of oxygen, they clump and slow blood flow. This vasoocclusion slows oxygen delivery, which leads to tissue damage. These painful episodes, which are unpredictable and vary in severity, are commonly referred to as sickle cell crisis.
Odesina has a simple recommendation for assessing pain: ask the patient. Nurses should use an age-appropriate assessment tool. For example, children may color pictures of the parts of the body that hurt with certain colors to signify severity. Another tool, the Oucher Scale, rates pain from 0 to 100, with 100 being a face that illustrates the most pain.2 Nurses should closely observe infants for evidence of pain.
Other tips include -
· Ask the parents. "Just as patients know what their pain is, parents know their children and when they are in pain," says Odesina.
· Don't mistake the sedative effect of opioids for pain relief.
· Don't be mislead by patients' use of distractions, such as watching television. Remember that people with SCD have learned to live and function with some level of pain.
Early treatment is essential to prevent worsening of pain and major complications such as vasulopathy and progressive organ failure.
Managing SCD Pain
"We have managed several episodes of mild to moderate pain and [a] few severe pain episodes at home. Most of the severe pain episodes and all of the complicated moderate pain episodes have led to hospitalizations."
Most pain episodes are treated at home. Odesina gives her daughters nonsteroidal antiinflamatories, such as ibuprofen and acetaminophen with codeine, or morphine for severe pain. The round-the-clock regimen includes increased oral fluids, massage and a heating pad applied to the affected area, deep-breathing exercises, and distraction methods, such as music, videotapes, and prayer.
Nurses must help patients develop this type of care plan to manage pain at home. Such a plan can reduce the need for trips to the ED; however, severe pain should be considered a medical emergency.
A major factor in pain management is a lack of knowledge of opioid use, including patients', families', and clinicians' fear of addiction.3 Drug addiction differs from drug tolerance and dependence, expected pharmacological properties associated with long-term opioid use. Addiction, thought to be related to underlying psychological problems, occurs when medications are craved, obtained, and used for reasons other than pain relief.
According to the nation's first evidence-based clinical practice guideline, Guideline for the Management of Acute and Chronic Pain in Sickle Cell Disease, which Odesina coauthored in connection with the American Pain Society, people with SCD are no more likely to be addicted than other groups of patients. Withholding opioids from these patients is unwarranted and leads to inadequate treatment.
Guideline Recommends Comprehensive Management
"When pain requires hospitalization and opioids, I have the luxury that most parents do not - the ability to assess my children and speak the language of pain medication adjustment with other healthcare professionals."
The recognition that undertreatment or inappropriate treatment is common prompted the development of the SCD pain management guideline, published in August 1999. "The incredible lack of research regarding pain management for SCD and the negative experiences that patients frequently have in EDs demonstrate the guideline's need," says Ada Jacox, PhD, RN, professor, associate dean for research, college of nursing, Wayne State University, Detroit, and chair of the Sickle Cell Pain Guideline Panel.
Jacox describes the guideline as the effort of a multidisciplinary panel of experts to make recommendations that can help healthcare professionals manage SCD-related pain. The guideline makes clear that one of the most important steps toward improving pain control in SCD is education for patients, families, and healthcare providers. Healthcare professionals also need to frequently assess pain control and offer support to patients and families coping with the realities of SCD.
"If understood properly and if critical barriers to assessment and treatment are overcome, sickle cell pain can be managed effectively by qualified healthcare personnel," says Odesina. "Nurses must understand SCD and get the word out about the disease, its pain, and management."




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