Patient discussions

Appropriate referral to a specialty sickle cell clinic for education, genetic counseling, and routine follow-up care is essential and should occur as soon as diagnosis is made.

Initial education for parents and caregivers should include the importance of:[151] 

  • attending regular healthcare appointments, and

  • having antibiotic prophylaxis and immunizations, as recommended.

Offer education to parents and caregivers about signs and symptoms of the disease's complications, prevention techniques, and treatment options.[37]

Give instructions on abdominal palpation and measurement to look for an enlarged spleen and the need for urgent medical attention if this is discovered.

Discuss management of dactylitis and other painful complications.[151]

Inform parents and caregivers that urgent medical attention is required if the child develops any of:

  • Fever

  • Pallor of the skin, lips, or nail beds

  • Respiratory symptoms

  • Signs of pain or inability to move extremities

  • Early signs of splenic sequestration, including pallor and listlessness

Advise parents and caregivers on maintaining adequate hydration and a healthy diet, preventing infections (including vaccinations), and avoiding factors that may trigger a crisis.

Discuss symptoms of the later stages, and complications affecting older children and adults with patients and parents, including stroke, enuresis, priapism, cholelithiasis, delayed puberty, proliferative retinopathy, avascular necrosis of the hip or shoulder, and leg ulcers.[151]

Give adolescent and adult patients information about issues related to contraception, carrier testing of partners, genetic counseling, and prenatal diagnosis. Contraception is recommended to prevent unwanted pregnancies.[151]

Advise patients on appropriate methods of contraception, such as progesterone-only contraceptive implants and pills, and levonorgestrel intrauterine devices. Combined hormonal contraceptives are not recommended because they may increase the risk of thromboembolic disease. Injectable progesterone-only contraceptives (depot medroxyprogesterone acetate) are associated with risk of venous thrombosis. Copper intrauterine devices are not usually recommended due to concern that risk of blood loss may be increased.[152][153]

Use of this content is subject to our disclaimer