Page 257 Guide to Pain Management in Low-Resource Settings

Chapter 32

Pain in Sickle Cell Disease

Paula Tanabe and Knox H. Todds

Case report Th e severity and frequency of pain crises var-
ies with the specifi c genotype. Patients with SS and SB0
Ruben is a 25-year-old male with sickle cell disease who typically have more severe pain episodes when com-
presents for evaluation of moderate, constant right hip pared with patients with SC and SB+. Th is is not to say
pain (rated as 6/10) and intermittent episodes of severe that patients with SC and SB+ cannot experience pain-
pain, reported as “crisis pain.” Ruben describes these cri- ful episodes—the episodes are just more uncommon
ses as severe, occurring monthly, and feeling “as if all my and infrequent.
bones are breaking.” Th e pain is most often experienced Both physiological and psychological factors
in his legs. can trigger a painful crisis. Common triggers of painful
crises include infection, temperature changes, and any
How often do individuals with type of physical or emotional stress. Common causes of
sickle cell disease have pain? acute pain include:
• Hand-foot syndrome in children (dactylitis)
Th is case depicts a typical scenario faced by therapists • Painful crises: vasoocclusion
around the globe. Often, the pain associated with sickle • Splenic sequestration
cell disease (SCD) is poorly understood. Persons with • Acute chest syndrome
SCD often experience both acute and chronic pain. It • Cholelithiasis
is now clear that more than half of patients with SCD • Priapism
report some type of pain on a daily basis. “Crisis pain,” In addition to experiencing acute painful crisis,
the most severe pain experienced by persons with SCD, persons with SCD also often experience chronic pain.
has been reported on up to 13% of all days. Crisis pain Specifi c causes of chronic pain include:
(acute pain) has been described as “if all my bones are • Arthritis
breaking” or “being hit with a board.” • Arthropathy
Th ese lifelong episodes have an abrupt onset, • Avascular necrosis (often in the hips and shoul-
are episodic and unpredictable, and are associated with ders and more common in persons with SC geno-
very severe pain. Individuals are usually not able to con- type)
duct normal activities during a painful crisis, which may • Leg ulcers
last for several hours and up to a week or more. • Vertebral body collapse

Guide to Pain Management in Low-Resource Settings, edited by Andreas Kopf and Nilesh B. Patel. IASP, Seattle, © 2010. No responsibility is assumed by IASP 245
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