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reference materials

Pain Management

key information

source: Indiana Hemophilia & Thrombosis Center Inc.

summary/abstract:

Many patients with chronic diseases suffer pain as a component of the disease process. This is especially true for patients with sickle cell disease, with 90% of hospitalizations for this population being due to sickle pain. Like other populations of patients with chronic illness, there are both physical and psychosocial factors involved in the management approach to pain control.

The Nature of Sickle Cell Pain:

Sickle cell painful episodes are thought to be caused by ischemic tissue injury from obstruction of blood flow by sickled red blood cells. Lack of blood flow results in regional hypoxia and acidosis, which perpetuate the sickling process. Considerable evidence also supports the hypothesis that interactions between sickle cells and vascular endothelium contribute to the clinical manifestations of sickle cell disease. Adherence of sickle cells to endothelial cells may augment circulatory flow and promote deoxygenation of red blood cells resulting in sickling. Up-regulation of adhesive and hemostatic properties of endothelial cells by certain viruses may explain how viral infections often precipitate vaso-occlusive episodes.
Painful episodes usually last 4-7 days but can persist for weeks. Hypoxia, infection, dehydration, acidosis, menstruation, sleep apnea, and exposure to cold temperatures can precipitate vaso-occlusive pain episodes. Commonly, no precipitating factors can be identified. Understandably, anxiety, depression, and physical exhaustion are also identified as contributing factors.
A painful crisis can occur in the absence of known precipitating events or any objective clinical findings. The frequency and severity of pain episodes is extremely variable among patients with sickle cell disease. Indeed, one of the great puzzles regarding understanding sickle cell disease is how a single DNA deviation which causes a change in a single amino acid in a single protein in a single type of cell (RBC) can cause such variability in the clinical course.
Sickle cell related pain usually involves the low back, legs, arms, chest, or abdomen. The pain may migrate or be very localized. It may be sharp or dull; stabbing or throbbing. Patients with sickle cell disease can have chronic pain syndromes and additionally experience acute painful episodes.
Dactylitis (painful swelling of the hands or feet) is often the first manifestation of sickle cell disease seen in affected infants. In older children and adults, musculoskeletal pain is the most common complaint. It may be difficult to distinguish a vaso-occlusive pain episode from osteomyelitis, septic arthritis, toxic synovitis, rheumatic fever, or gout. For patients with abdominal complaints, pancreatitis, cholecystitis, urinary tract infection, pelvic inflammatory disease, pneumonia, or malignancy must be ruled out.

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