Page 259 Guide to Pain Management in Low-Resource Settings

Pain in Sickle Cell Disease 247

• Gallbladder, liver, and lung infarction • Individuals with SCD often experience both acute
• Iron overload (if the patient has received numer- and chronic pain.
ous blood transfusions) • Pain episodes begin in childhood and continue
• Depression throughout the lifespan.
• Acetaminophen and NSAIDs are helpful in man-
What is the pathophysiological aging mild and moderate pain.
mechanism of sickle cell disease? • Opioids are often required to manage acute pain-
ful crises.
Pain crises are triggered by deoxygenation and by the • Some patients will require chronic use of opioids
resulting polymerization of the hemoglobin. A triad of on a daily basis to manage pain and improve daily
ischemia, infarction, and infl ammation contribute to the function.
pathophysiology of pain. Mechanisms include damage • Complementary strategies such as the use of
to the vascular endothelium and chemical mediators of heat, suffi cient sleep, hydration, massage, and ex-
infl ammation, microinfarctions caused by local capillary cellent nutrition are reported as being helpful.
sickling, ischemia, somatic symptoms (muscles, ten- • Again, opioids are very eff ective and should not
dons, ligaments, bone, and joints), and visceral symp- be withhold from a patient suff ering from sickle
toms (spleen, liver, and lungs), often described by the cell disease.
patient as being vague, diff use and/or dull pain.
References
Tips from a complementary
[1] National Institutes of Health. National Heart, Lung, and Blood Insti-
medicine specialist tute. Th e management of sickle cell disease, 4th ed. NIH publication no.
02–2117. Washington, DC: National Institutes of Health; 2002.
[2] Smith WR, Penberthy LT, Bonbjerg VE, McClish DK, Roberts JD, Dah-
Many complementary alternative medicine strategies man B, Aisiku IP, Levenson JL, Roseff SD. Daily assessment of pain in
adults with sickle cell disease. Ann Intern Med 2008;15:94–101.
have been found to both limit the frequency of pain
crises and improve patients’ quality of life. Careful at-
tention to nutrition, obtaining adequate sleep, the use
of heat, and massage have all been reported by persons Websites
with SCD who function at a very high level. Use of com-
http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html
plementary strategies should therefore be encouraged.
http://www.nhlbi.nih.gov/health/prof/blood/sickle/
http://consensus.nih.gov/2008/2008SickleCellDRAFTstatementhtml.htm
Pearls of wisdom

• Many persons with SCD experience pain on a
daily basis.