When should I consider referring a patient with sickle cell disease to a specialist for further management?

Consider referring a patient with sickle cell disease to a specialist for further management in the following situations:

• Immediate referral or admission if the patient presents with new symptoms of cerebrovascular disease, such as acute stroke or transient ischaemic attack, or cognitive abnormalities suggestive of silent infarcts or intracranial haemorrhage (including decline in academic performance in children) ¹.
• Urgent referral for eye problems related to sickle cell disease ¹.
• Referral for consideration of cholecystectomy if symptomatic gallstones develop ¹.
• Referral for management of pulmonary hypertension symptoms (fatigue, limited exercise tolerance, chest pain, dyspnoea on exertion, syncope) to a specialist pulmonary hypertension centre with expertise in sickle cell disease ¹.
• Referral for surgery such as splenectomy after two or more episodes of acute splenic sequestration or for avascular necrosis pain relief ¹.
• Referral for stem cell transplantation evaluation in children and some adults with severe sickle cell disease, as this is the only curative treatment ¹.
• Urgent referral for suspected renal medullary cancer if symptoms such as haematuria, weight loss, loin or abdominal pain, and fever occur, using the suspected cancer pathway (appointment within 2 weeks) ¹.
• Referral for management of acute renal failure or declining renal function jointly with a renal physician ¹.
• Referral of children with hypertension for further investigation ¹.
• Referral for psychological support, including cognitive behavioural therapy, for children experiencing frequent pain episodes and emotional difficulties ¹.
• Referral for hydroxycarbamide treatment in patients with recurrent hospital admissions for acute chest syndrome or painful crises ¹.
• Referral for elective blood transfusion in cases of pulmonary hypertension, acute priapism, or severe anaemia ¹.
• Urgent referral for haematuria in children and adults to exclude malignancy ¹.
• Referral for sleep apnoea management ¹.

Primary care clinicians should also admit or refer patients immediately if new serious symptoms arise and manage minor complications in the community where possible, with regular secondary care follow-up ¹.