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Chronic pain in sickle cell disease: Here are the management recommendations.

In the previous article, we have summarized the chronic complications of sickle cell disease. We look at each of them as well as the recommendations of their management. Any pain reported by a patient with sickle cell disease that has lasted for more than three months is termed chronic pain.

Much as cute pain is nociceptive in nature, there’s a strong association of neuropathy with chronic pain in both people living with sickle cell disease and the general population.

There are four categories of chronic pain in sickle cell disease that you ought to know before you get well acquainted with the recommendations, that pertain to their management. These include;

Chronic pain of unclear aetiology, which in most cases is an extension of acute painful episodes that have been recurrent. The pain that arises from a specific tissue or organ such as in the hip due to avascular necrosis or leg ulcers; chronic neuropathic pain due to a damaged nerve following occlusion of blood vessels, for example, mental nerve neuropathy and spinal cord infarction; lastly, breakthrough pain. More details on chronic pain in sickle cell disease can be accessed here.

Let’s look at the recommendations that govern the management of chronic pain in sickle cell disease as set forth by the expert panel on evidence-based management of sickle cell disease.

  1. Determine the cause and type of sickle cell disease-related chronic pain. It includes chronic pain with objective signs such as avascular necrosis and leg ulcers. And chronic pain without objective features due to neuroplasticity of the peripheral or central nervous system.
  2. Use a combination of the patient’s response to treatment – including pain relief, side effects, and functional outcomes- to guide the long-term use of opioids.
  3. Encourage people to use deep tissue/deep pressure massage therapy, muscle relaxation therapy, and self-hypnosis as indicated.
  4. Use long-acting and short-acting opioids to manage chronic pain that is not relieved by nonopioids.
  5. Assess all people with sickle cell disease for chronic pain annually or more often as needed. This assessment should include pain descriptors; its severity on a numerical scale; its location; factors that precipitate or relieve it, including biopsychosocial factors, and its effect on the patient’s mood, activity, employment, quality of life, and vital signs.
  6. Use a partnership agreement leading to a written, individualized treatment plan (to include risks, benefits, and side effects) with the patient if they require long-term opioids. The partnership agreement should list the patient’s rights and responsibilities, and the treatment plan should the type, amount, and route of administration of the opioid in question, including random drug urine testing.
  7. Appoint one physician or another clinician to write the biweekly to monthly prescriptions for long-term opioids. Keep refills without seeing the patient to a minimum, and people on chronic opioid therapy must be evaluated in person every 2-3 months.
  8. Document all encounters with a patient, including medical history, physical exam, diagnosis, plan of management, type and amount of opioids prescribed and their side effects, if any, and lab data as needed.
  9. Encourage people receiving opioids to increase their fluid intake, maintain dietary fibre intake per the current dietary fibre recommendations and use stool softeners and bowel laxatives.
  10. Believe the patient’s report of pain and optimize therapeutic outcomes to achieve adequate pain relief and improve the patient’s quality of life.
  11. Refer patients for evaluation by a mental health professional such as a psychiatrist, social worker, or addiction specialist as needed.
  12. Assess all people for other types of non-sickle cell disease-related chronic pain, including postoperative pain, pain due to trauma, pain due to therapy, iatrogenic pain, and pain due to comorbid conditions.

It is crucial to understand how the pain in sickle cell disease develops such that you manage it well; while minimizing the side effects of the analgesics used.

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Dr A. M. Ssekandi is a medical officer, researcher, content creator, author, and founder of He does private practice with a public touch. He is a certified digital marketer. He has earned certificates in Understanding Clinical Research and Writing in Sciences from the University of Cape Town and Stanford University respectively. He also has a certificate of Good Clinical Practice from

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