Health maintenance and management of chronic complications of sickle cell disease | oneSCDvoice
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guides & guidelines

Health maintenance and management of chronic complications of sickle cell disease

key information

source: American Society of Hematology

year: 2014

authors: Suzie A. Noronha, Sophie Lanzkron


Avascular necrosis (AVN): The hip joint is the most common site of AVN. About 40–80% of cases of AVN of the hips are bilateral. AVN can develop in children as young as 5 years of age. In individuals with intermittent or chronic hip pain, evaluate for AVN by history, physical exam, radiography, and MRI as needed (Strong recommendation, low-quality evidence). Treat with analgesics and consult physical therapy and orthopedics for assessment and follow-up (Strong recommendation, high-quality evidence). For advanced AVN, refer to an orthopedic surgeon and SCD specialist for evaluation and possible hip arthroplasty (Consensus–Expert Panel).

Leg ulcers: Inspect the lower extremities during physical examination for active or healed ulcers, record their number, and measure their depth (Weak recommendation, low-quality evidence). Trauma, infection, and severe anemia are associated with ulcer formation. Leg ulcers may be seen starting at age 10.
Treat with initial standard therapy (ie, debridement, wet to dry dressings, and topical agents) (Moderate recommendation, low-quality evidence). In individuals with chronic recalcitrant deep leg ulcers, evaluate for osteomyelitis (Moderate recommendation, low-quality evidence). Consult or refer to a wound care specialist or multidisciplinary wound team for persistent or recalcitrant leg ulcers (Consensus–Expert Panel)

Ophthalmologic complications: Beginning at age 10, refer to an ophthalmologist for a dilated eye examination to evaluate for retinopathy (Strong recommendation, low-quality evidence).
If examination is normal, re-screen at 1–2 year intervals (Consensus–Expert Panel). If retinopathy is suspected, refer to a retinal specialist (Consensus–Expert Panel).

Stroke: In children with sickle cell anemia, screen annually with transcranial doppler (TCD) according to methods employed in the STOP studies, beginning at age 2 and continuing until at least age 16 (Strong recommendation, moderate-quality evidence).
In children with conditional (170–199 cm/sec) or elevated (>200 cm/sec) TCD results, refer to a specialist with expertise in chronic transfusion therapy aimed at preventing stroke (Strong recommendation, highquality evidence). In children and adults who have had a stroke, initiate a program of monthly simple or exchange transfusions (Moderate recommendation, lowquality evidence). If a transfusion program is not possible to implement, initiate hydroxyurea therapy (Moderate recommendation, low-quality evidence).

organization: University of Rochester Medical Center; John Hopkins University School of Medicine

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