Patient discussions
Appropriate referral to a specialty sickle cell clinic for education, genetic counselling, and routine follow-up care is essential and should occur as soon as diagnosis is made.
Parents and carers are educated about signs and symptoms of the disease's complications, prevention techniques, and treatment options.[36] These include being instructed on abdominal palpation and measurement to look for an enlarged spleen and the need for urgent medical attention if this is discovered. Parents and carers should be informed that urgent medical attention is required if the child develops fever or has pallor of the skin, lips, or nail beds, any respiratory symptoms, signs of pain or inability to move extremities, and early signs of splenic sequestration, including pallor and listlessness. Information should be provided on the child's earliest symptoms (i.e., painful swelling of the hands and/or feet [hand-foot syndrome]). Symptoms of the later stages, and complications affecting older children and adults should be discussed with patients and parents, including stroke, enuresis, priapism, cholelithiasis, delayed puberty, proliferative retinopathy, avascular necrosis of the hip or shoulder, and leg ulcers.
Patients should receive information about issues related to contraception, carrier testing of partners, genetic counselling, and antenatal diagnosis. Contraception is recommended to prevent unwanted pregnancies. The World Health Organization (WHO) recommendations for the use of alternative methods of contraception stem from concerns that combined hormonal contraceptives may increase the risk of thromboembolic disease and therefore are not recommended first line. Progesterone-only contraceptives have no restrictions as they are not associated with an increased risk of thrombosis. In addition, copper and levonorgestrel intrauterine devices (IUDs) have no restriction for use in sickle cell disease.[152]
Patients with sickle cell disease have increased requirements for folic acid, especially pregnant patients. Large doses of folic acid may mask haematological effects of vitamin B12 deficiency while allowing neurological complications to progress, thereby obscuring diagnosis of pernicious anaemia.
Increased cardiac workload and additional haematopoiesis increase nutritional growth requirements.[153]
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