New guidelines cover preventive, chronic, acute care for sickle cell disease

New evidence-based guidelines on the management of sickle cell disease were released last week by a group of experts convened by the National Heart, Lung, and Blood Institute.

The guidelines, published in the Sept. 10 Journal of the American Medical Association, provide recommendations for sickle cell care, including health maintenance, management of acute complications, management of chronic complications, use of and treatment protocols for hydroxyurea therapy, and use of transfusion therapy.

Strong recommendations include the following:

• Rapidly initiate opioids for treatment of severe pain associated with a vasoocclusive crisis.
• Use incentive spirometry in patients hospitalized for a vasoocclusive crisis.
• Use analgesics and physical therapy for treatment of avascular necrosis.
• Use angiotensin-converting enzyme inhibitor therapy for microalbuminuria.
• Refer patients with proliferative sickle cell retinopathy to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension.
• Use hydroxyurea therapy for adults who have 3 or more severe vasoocclusive crises in a year, pain or chronic anemia interfering with daily activities, or severe or recurrent episodes of acute chest syndrome.
• Provide preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL (with a moderate recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy).
• Assess iron overload (with a moderate recommendation to begin iron chelation therapy when indicated).

There is a shortage of high-quality evidence regarding sickle cell treatment, so some of the strong recommendations are supported by low- or very low-quality evidence, the authors noted, expressing hope that despite these shortcomings the guidelines would begin to “facilitate improved and more accessible care for all affected individuals.” The guideline was endorsed by the American Society of Hematology, the American Academy of Pediatrics, and the American Society of Pediatric Hematology/Oncology.

An accompanying editorial praised the guidelines for many strengths but also criticized them for not including patients in the development process and not providing guidance in certain areas.