Previous changes

July 2021 — reviewed. A literature search was conducted in July 2021 to identify evidence-based guidelines, UK policy, systematic reviews, and key randomized controlled trials published since the last revision of this topic.

November 2016 — reviewed. A literature search was conducted in November 2016 to identify evidence-based guidelines, UK policy, systematic reviews, and key randomized controlled trials published since the last revision of this topic. No major changes to recommendations have been made, but the topic has been restructured.

July to November 2010 — this is a new CKS topic. The evidence base has been reviewed in detail, and recommendations are clearly justified and transparently linked to the supporting evidence.

New evidence

Evidence-based guidelines

• NICE (2024) Voxelotor for treating haemolytic anaemia caused by sickle cell disease. National Institute for Health and Care Excellence. https://www.nice.org.uk [Free Full-text]

HTAs (Health Technology Assessments)

• NICE (2025) Exagamglogene autotemcel for treating severe sickle cell disease in people 12 years and over. National Institute for Health and Care Excellence. https://www.nice.org.uk [Free Full-text]

Economic appraisals

No new economic appraisals relevant to England since 1 July 2021.

Systematic reviews and meta-analyses

• Kang, H.A., Wang, B., Barner, J.C., et al. (2024). Opioid Prescribing and Outcomes in Patients With Sickle Cell Disease Post–2016 CDC Guideline. JAMA Internal Medicine. [Free Full-text]
• Bolarinwa, A.B., Oduwole, O., Okebe, J., Ogbenna, A. A., Otokiti, O. E., & Olatinwo, A. T. (2024). Antioxidant supplementation for sickle cell disease. Cochrane Database of Systematic Reviews, (5). [Abstract]

Primary evidence

No new randomized controlled trials published in the major journals published since 1 July 2021.

New policies

No new national policies or guidelines since 1 July 2021.

New safety alerts

No new safety alerts since 1 July 2021.

Changes in product availability

No changes in product availability since 1 July 2021.

What are the acute complications of sickle cell disease?

Acute complications of sickle cell disease include:

• Acute painful crisis 
  • Also known as vasculo-occlusive crisis, it is the most common complication of sickle cell disease in all age groups and can affect any part of the body, but most commonly involves the limbs and back.  
  • It may present as skeletal pain due to bone infarction or avascular necrosis, most commonly affecting the femoral head or head of the humerus. 
  • Pain is thought to be caused by vaso-occlusion by sickled erythrocytes, leading to ischaemic tissue damage and subsequent inflammation and pain. 
  • It may be precipitated by cold, dehydration, prolonged exertion, infection, or ischaemia.
• Abdominal complications
  • Abdominal pain is common and may be due to complications — the differential diagnosis includes sequestration syndromes, mesenteric syndrome, constipation, gallstone complications, infective aetiologies (such as pyelonephritis, intra-abdominal abscesses, and diverticulitis), and dysmenorrhoea. 
  • Gallstones are common and often cause intermittent abdominal pain. 
• Anaemia
  • A rapid, significant fall in haemoglobin, usually of at least 20 g/L, may result in the individual becoming symptomatic and major reductions may lead to cardiovascular compromise. 
  • Anaemia may be due to haemolysis (for example, due to a transfusion reaction, infection or glucose-6-phosphate dehydrogenase [G6PD] deficiency), reduction in erythropoiesis, blood loss, and sequestration in the spleen and less commonly in the liver.
  • Transient red cell aplasia (most commonly due to parvovirus B19 infection) causes an arrest in erythropoietic maturation — symptoms may include fever, headache, myalgia, arthralgia, and respiratory and gastrointestinal symptoms. 
  • Acute splenic sequestration occurs when large numbers of erythrocytes are trapped in the spleen, resulting in splenomegaly and profound anaemia. 
  • It can develop rapidly and is more common in children than in adults, although it is occasionally seen in people with milder forms of sickle cell disease. 
  • The typical clinical features of acute hepatic sequestration are pain in the right hypochondrium, a tender and enlarged liver, pallor, and circulatory collapse (infrequent). 
• Acute chest syndrome (ACS)   
  • This is a form of acute lung injury that can be severe and life-threatening. It is particularly common in early childhood and can be clinically indistinguishable from pneumonia. 
  • Around 30% of people with sickle cell disease will have one episode of acute chest syndrome in their lifetime, and 50% of these people will have recurrent episodes.  
  • ACS is the third leading cause of death in adults with sickle cell disease and may result in longer term morbidity such as chronic lung disease and pulmonary vascular disease.
  • It is characterized by respiratory symptoms, such as chest pain, tachypnoea, cough, shortness of breath, and/or respiratory distress, generally with fever, in the presence of a new infiltrate on chest X-ray.
  • Possible causes include infection, infarction (due to fat emboli from bone infarcts) or a combination of both. 
• Infections 
  • People with sickle cell disease are susceptible to a range of bacterial and other infections including invasive pneumococcal disease, which is the most significant cause of infection-related morbidity and mortality. Increased susceptibility is related to splenic dysfunction.
  • Acute infection is one of the most common causes of admission to hospital in children aged under 10 years — children younger than 5 years of age are at increased risk of pneumococcal infection. 
  • Sepsis is an important cause of mortality in both adults and children with sickle cell disease — the most common infections are pneumococcal sepsis, Gram-negative sepsis, lower respiratory tract infections, urinary tract infections, and osteomyelitis. 
• Osteomyelitis 
  • This is one of the most common infectious complications in people with sickle cell disease, and it occurs in people of all ages and genotypes.  
  • The commonest sites are the femur, tibia, and humerus and it is most commonly caused by Salmonella species, Gram-negative enteric bacteria, and Staphylococcus aureus. 
  • There may be pain, localized warmth, swelling, tenderness, and fever, so it can be difficult to distinguish from vasculo-occlusion. 
• Priapism 
  • Priapism is very common and has a lifetime incidence of 35–90% in males with sickle cell disease with the majority of first episodes occurring before the age of 20 years.
  • It is caused by obstruction to the venous drainage of the penis due to vaso-occlusion.
  • Males with a sustained and painful erection lasting for more than 1 hour should be encouraged to attend hospital. A priapic episode lasting more than 3 hours is classed as a surgical emergency.
• Acute kidney injury 
  • Some people with sickle cell disease develop chronic kidney disease and are at increased risk of acute kidney injury. 
  • It occurs in around 2–10% of people admitted with sickle cell anaemia and is more common in those with acute chest syndrome. 
  • Acute kidney injury can be caused by dehydration, sepsis, or drugs, or it may occur during general multi-organ failure. 
• Neurological complications  
  • Central nervous system complications cause significant morbidity and mortality.
  • Acute presentations can include headache, seizures, focal neurological signs, visual impairment, altered consciousness, and acute deterioration in cognition — causes include infection and stroke. 
  • Studies have reported high rates of ischaemic stroke in adults with sickle cell disease of 11% by the age of 20 years and 24% by the age of 45 years, and sickle cell anaemia is the most common cause of stroke in children [Brousse, 2014]. Contributory risk factors are relative and acute anaemia, and multi-organ dysfunction, as well as the presence of comorbidities which are cardiovascular risk factors. 
  • Haemorrhagic stroke also occurs at increased rates in adults with sickle cell disease. Hypertension and multi-organ failure are risk factors for cerebral haemorrhage, and aneurysms (reported in over 10% of adults with sickle cell disease) can rupture leading to subarachnoid haemorrhage. Low steady-state haemoglobin concentration, high steady-state white cell count and transfusion within the previous 14 days are risk factors for haemorrhagic stroke. 
• Multiple multisystem organ failure  
  • This rare life-threatening complication involves the acute development of severe dysfunction in at least two of three major organs (kidney, liver, or lung) and is usually associated with vasculo-occlusive crisis and/or sepsis. 

[Claster, 2003; Mehta, 2006; Brousse, 2014; Sickle Cell Society, 2018; Martí-Carvajal, 2019; Sickle Cell Society and PHE, 2019; BMJ, 2021]

What are the chronic complications of sickle cell disease?

Chronic complications of sickle cell disease increase with age and include:

• Hepatobiliary complications 
  • Hepatobiliary complications in sickle cell disease are common and have a multifactorial aetiology — they may be caused by the sickling process itself, by hypoxic injury due to vaso-occlusion, or by iron overload secondary to multiple blood transfusions. 
  • Mild abnormalities in liver function tests are common and may be seen in uncomplicated vaso-occlusive crises, but some patients develop progressive liver disease and end-stage liver disease. 
• Pain 
  • Pain may be due to leg ulcers, tissue damage, avascular necrosis (particularly of the femoral head), or chronic osteomyelitis. 
  • Osteopenia may occur due to hyperplasia of the bone marrow.
  • In some people, there is no obvious cause for the pain. Such pain has some features of neuropathic pain and may be related to increased central sensitization to painful stimuli. 
• Anaemia 
  • Sickle cells have a short lifespan of approximately 20–30 days, compared with 120 days in normal erythrocytes. 
  • In people with sickle cell anaemia (Hb SS) and Hb S/β⁰ thalassaemia haemoglobin levels are typically between 60–90 g/L, and those with Hb SC and Hb S/β⁺ thalassaemia usually have a higher Hb level. 
  • Some people with sickle cell disease can tolerate anaemia for many years, but with increasing age and comorbidities may develop symptomatic anaemia despite a stable Hb level.
  • Chronic haemolysis causes bilirubin to be slightly increased, and the products of haemolysis may cause gallstones. 
• Respiratory complications 
  
  • Pulmonary hypertension 
    • Pulmonary hypertension is common, occurring in 30–40% of adults with sickle cell disease and is associated with increased morbidity and mortality [Klings, 2013].
    • Sudden death is common — in a review of the results of right-sided cardiac catheterization in people with sickle cell disease (n = 34) the estimated median survival for people with pulmonary hypertension was 25.6 months from the date of cardiac catheterization [Castro, 2003].
    • Symptoms may be nonspecific and include fatigue, limited exercise tolerance, progressive evidence of dyspnoea on exertion (particularly worsening on walking up an incline or climbing stairs), chest pain, light-headedness, and syncope. Sudden death is common.
    • It is associated with increasing age, poor functional capacity, prior history of leg ulceration, renal insufficiency, and markers of haemolysis.
    • Multiple mechanisms have been postulated to contribute to the development of pulmonary hypertension, including chronic intravascular haemolysis leading to reduced nitric oxide scavenging, thrombosis, hypoxaemia, oxidant stress, and asplenia. 
  • Chronic sickle lung 
    • This is an interstitial abnormality of lung parenchyma characterized by progressively worsening restrictive lung disease, pulmonary hypertension, hypoxaemia, and chest pain and with fibrotic changes on CT scan. 
    • It affects around 5% of people with sickle cell disease with an average age of onset of 25–33 years, and may develop as a result of recurrent episodes of acute chest syndrome (ACS), but it may also occur in people without a history of ACS. 
  • Sleep-disordered breathing 
    • This is common and includes a group of conditions characterized by complete or partial cessation of normal respiration during sleep.
    • It results in nocturnal hypoxia or obstructive sleep apnoea (OSA) — these are correlated with morbidity including frequent painful vaso-occlusive crisis, risk of future central nervous system (CNS) event, and priapism.
    • Up to 40% of children and adolescents have nocturnal hypoxia, and OSA has been reported in up to 60% of adults with sickle cell disease. Nocturnal hypoxia and OSA may co-exist.
• Cognitive impairment  
  • Around 20% of children with sickle cell disease have silent cerebral infarcts visible on magnetic resonance imaging (MRI). These are due to very small white-matter lesions and are a risk factor for an overt stroke. The silent infarcts are associated with mild cognitive impairment that may be detected by using neurocognitive tests.
  • Cognitive impairment due to silent infarcts may also occur in adults. 
• Eye complications — sickle retinopathy
  • This is the most important complication as it can lead to significant visual impairment.
  • It is classified into non-proliferative (NPSR) and proliferative (PSR) forms of retinopathy, although NPSR does not normally lead to visual loss.
  • Mechanical obstruction of retinal capillaries by sickled erythrocytes and direct endothelial damage may also occur.
  • Ischaemic changes result in proliferative retinopathy which is characterized by the development of peripheral retinal neovascularization. 
  • The incidence and severity of PSR is greater in people with Hb SC and Hb S/β thalassaemia than those with Hb SS, with a peak prevalence in people aged around 15–30 years. 
• Gallstones 
  • Gallstones are common in sickle cell disease, but are more common in sickle cell anaemia.
  • They occur in 50% of children aged over 10 years in the UK and up to 70% of adults with sickle cell disease. 
  • They are usually asymptomatic and are often detected incidentally on routine imaging, but complications may develop due to infection and inflammation of the gall bladder and biliary duct (acute cholecystitis, gallbladder empyema, ascending cholangitis) or due to obstruction of the biliary ducts and acute pancreatitis.
  • Symptomatic biliary tract disease occurs in around 20% of people with sickle cell disease.
• Impaired nutrition and growth in children 
  • Impaired growth is often apparent after the age of 6 months and may be due to decreased absorption of nutrients, an increased metabolic rate, and/or poor appetite caused by recurrent febrile and painful episodes. 
  • Puberty is often delayed by about 2–3 years in children with sickle cell anaemia and 6 months in those with Hb SC. 
  • Children on long-term transfusion programmes may have significant iron overload and develop pituitary and/or primary gonadal deficiencies.
• Leg ulcers 
  • Leg ulcers are a frequent and disabling complication and they may persist for months or years.
  • They are more common in people with higher levels of haemolysis. Mechanical obstruction, high blood viscosity, venous incompetence, hypercoagulability, and thrombosis may also be involved. 
  • Leg ulceration is less common in people aged under 20 years and is more frequent in males and in people with sickle cell anaemia.
  • They typically appear just above the ankle and are often bilateral, and can be very painful.
     
• Priapism 
• • Priapism is very common and has a lifetime incidence of 35–90% in males with sickle cell disease with the majority of first episodes before the age of 20 years. The persistent erection is due to vaso-occlusion obstructing the drainage of the penis [Claster, 2003].
  • Events can be classified as stuttering (occurring for less than 3 hours but several times a week), minor (isolated or infrequent episodes of less than 3 hours) or fulminant (events lasting over 3 hours). 
  • Both prolonged episodes and shorter briefer episodes of priapism ('stuttering') may lead to erectile dysfunction in the long term. 
• Renal complications  
  • Renal complications occur in around 60% of people with the more severe forms of sickle cell disease at some point in their lives. 
  • Sickle nephropathy presents as a spectrum from painless haematuria, proteinuria, and progressive loss of function to end-stage renal disease. 
  • A reduction in medullary blood flow due to ischaemia, an increase in glomerular blood flow, and papillary necrosis all contribute to kidney damage. 
  • Sickle cell obstruction of small vessels leads to medullary insufficiency, which causes large quantities of dilute urine to be produced. This makes the person susceptible to dehydration. Nocturnal enuresis is common in all children, but there is an increased rate in children with sickle cell disease, particularly boys.  
  • Recurrent haematuria is due to medullary infarction, which leads to renal papillary necrosis. Sometimes, sloughing of the papilla may lead to ureteric obstruction and renal failure.
  • Renal medullary carcinoma occurs almost exclusively in people with the sickle cell trait, sickle cell disease, and occasionally sickle cell anaemia and may present with haematuria. It is aggressive and has usually metastasized by the time the symptoms (weight loss, haematuria, abdominal pain or back pain) have occurred.  

[Claster, 2003; Mehta, 2006; RCOG, 2011; Brousse, 2014; PHE, 2018d; Sickle Cell Society, 2018; Sickle Cell Society and PHE, 2019; BMJ, 2021]

What are the complications of sickle cell disease during pregnancy?

• Sickle cell disease is associated with maternal and fetal complications, including: 
  • Perinatal mortality. 
  • Premature labour. 
  • Fetal growth restriction. 
  • Acute painful crises. 
• Some studies have described increased risks of: 
  • Spontaneous miscarriage. 
  • Stillbirth. 
  • Antenatal hospitilization. 
  • Maternal mortality.
  • Delivery by Caesarean section. 
  • Infection — particularly urinary tract infection.
  • Thromboembolic events. 
  • Antepartum haemorrhage. 
  • Pre-eclampsia and pregnancy-induced hypertension. 

[RCOG, 2011; Sickle Cell Society, 2018]

Basis for recommendation

These recommendations are based on the Sickle Cell Society Standards for the clinical care of adults with sickle cell disease in the UK [Sickle Cell Society, 2018], the Sickle Cell Society and Public Health England (PHE) guidance Sickle cell disease in childhood: standards and recommendations for clinical care [Sickle Cell Society and PHE, 2019], the British Haematology Society guideline Significant haemoglobinopathies: guidelines for screening and diagnosis [Ryan, 2010], the British Medical Journal (BMJ) best practice guide Sickle cell anaemia [BMJ, 2021], and expert opinion in narrative reviews Managing sickle cell disease [Claster, 2003] and Management of sickle cell disease in the community [Brousse, 2014].

Basis for recommendation

This information is based on the Sickle Cell Society Standards for the clinical care of adults with sickle cell disease in the UK [Sickle Cell Society, 2018], the British Haematology Society guideline Significant haemoglobinopathies: guidelines for screening and diagnosis [Ryan, 2010], the Public Health England (PHE) guidance NHS sickle cell and thalassaemia screening programme handbook for newborn laboratories [PHE, 2017], and the British Medical Journal (BMJ) best practice guide Sickle cell anaemia [BMJ, 2021].

Basis for recommendation

This recommendation is based on the Sickle Cell Society Standards for the clinical care of adults with sickle cell disease in the UK [Sickle Cell Society, 2018].

Basis for recommendation

These recommendations are based on the Sickle Cell Society Standards for the clinical care of adults with sickle cell disease in the UK [Sickle Cell Society, 2018], the Sickle Cell Society and Public Health England (PHE) guidance Sickle cell disease in childhood: standards and recommendations for clinical care [Sickle Cell Society and PHE, 2019], and the British Medical Journal (BMJ) best practice guide Sickle cell anaemia [BMJ, 2021].

Priapism as a medical emergency

• The time that is required to elapse before priapism is considered to be a medical emergency differs slightly between guideline groups.
• The Sickle Cell Society and PHE recommend that [Sickle Cell Society and PHE, 2019]:
  • Children and their carers should be advised to seek treatment early and should attend hospital as an emergency if priapism persists for over 2 hours.
  • A prolonged attack, lasting over 3 hours should be treated as a surgical emergency as, if untreated, cavernosal fibrosis and impotence may ensue. 
• The Sickle Cell Society standards for adults recommend that patients with a painful, rigid, priapic episode of more than 1 hour should be encouraged to attend hospital [Sickle Cell Society, 2018].
• The BMJ best practice guide advises that episodes lasting 4 hours or more are considered medical emergencies, and urological evaluation is indicated [BMJ, 2021].
• Due to the risk of cavernosal fibrosis and impotence (which are serious and potentially permanent consequences of a prolonged, untreated attack of priapism), CKS recommends that the shorter time of 2 hours should be considered a medical emergency.

What is the national screening programme for sickle cell disorders?

• The Sickle Cell and Thalassaemia (SCT) Screening Programme screens for:
  • Genetic carriers for sickle cell, thalassaemia, and other haemoglobin disorders.
  • Sickle cell disease.
  • Thalassaemia.
  • Haemoglobin disorders.
• Sickle cell disease screening is offered to:
  • Infants
    • All newborn babies as part of the Newborn Blood Spot Screening Programme, usually when they are 5 days old. 
    • All infants aged younger than 1 year who have newly arrived in the UK or who are yet to have a blood spot test. 
  • Pregnant women in high-prevalence areas (where 2% or more of the booking bloods received by the laboratory are screen positive), usually before they are 10 weeks pregnant.
    • The Family Origin Questionnaire (FOQ) is also used to help interpret results, particularly in the interpretation of results indicating possible alpha or beta thalassaemia. 
  • Biological fathers if the mother is a genetic carrier for sickle cell disease or thalassaemia.   
• For pregnant women in low-prevalence areas (where less than 1% of the booking bloods received by the laboratory are screen positive) the Family Origin Questionnaire (FOQ) is used as an initial screening tool to determine if the woman or the biological father is at high risk of being a carrier of sickle cell disease or other haemoglobin disorder. 
  • Where either parent falls into a high-risk group a screening blood test for haemoglobin variants must be offered to the woman.
  • Women and biological fathers from low-risk family origins can opt in for screening if they request it.
• A list of high and low prevalence trusts is available on the GOV.UK website (www.gov.uk).
• All pregnant women are offered screening for thalassaemia.
• The British Society for Haematology advises that: 
  • The following people should also be screened:
    • People from high-risk groups who are about to undergo an operation or receive anaesthesia.
    • Women who are having assisted conception.
    • Women who are being investigated for infertility.
    • Women in high-risk groups for haemoglobinopathies before conception — if an abnormality is found, then partners should be offered testing.
  • Pre-marriage screening for haemoglobinopathies is not usual in the UK, but for some religious or ethnic groups, pre-marital screening for beta-thalassaemia heterozygosity may be more acceptable than pre-conceptual or antenatal screening.

How should I manage a person having a sickle cell crisis?

• Conduct a clinical assessment in people with sickle cell disease who present with signs or symptoms of acute sickle cell crisis.  
• Arrange immediate admission for people with any of the following signs or symptoms:  
  • Severe pain not controlled by simple analgesia or low dose opioids.
    • All people presenting with an acute painful sickle cell episode should be offered analgesia within 30 minutes of presentation in secondary care.  
  • Dehydration caused by severe vomiting or diarrhoea.
  • Severe sepsis.
    • Temperature over 38.5°C, or over 38°C in children aged under 2 years, temperature less than 36°C, or hypotension.
  • Symptoms or signs of acute chest syndrome including tachypnoea, oxygen saturation more than 5% below steady state, signs of lung consolidation.
  • New neurological symptoms or signs. 
  • Symptoms or signs of acute fall in haemoglobin.
  • Acute enlargement of spleen or liver over 24 hours, particularly in young children.
  • Marked increase in jaundice.
  • Any change in vision.
  • Haematuria.
  • Fulminant priapism lasting more than 2 hours or worsening of recurrent episodes.
• For all people with fever (temperature of 38°C or higher) who do not require referral:
  • Offer empirical broad-spectrum antibiotic treatment with choice guided by local microbiological guidelines or haemoglobinopathy teams. 
• Consider empirical broad-spectrum antibiotic treatment in people who do not have a fever, but have signs and symptoms of infection.
• For people with mild to moderate pain that can be managed at home.
  
  • The person and/or their family/carers will often have received an individual care plan from secondary care with clear guidance on how to manage their pain, and when to seek medical advice. 
  • If they do not have a care plan, prescribe paracetamol and/or ibuprofen (avoid ibuprofen if the person has renal impairment or significant proteinuria). Weak opioids can be used for more severe pain (dihydrocodeine in children aged under 13 years and codeine phosphate in people aged over 13 years. See the CKS topics on Analgesia - mild-to-moderate pain and NSAIDs - prescribing issues for prescribing information.
    • Arrange an urgent assessment in hospital if the pain is not controlled by these measures. 
  • Offer advice about:
    • Coping strategies including distraction techniques like games, computers, and television. 
    • Increasing fluid intake as dehydration may prolong the painful episode. 
    • Avoiding triggers, such as exposure to cold or windy weather, excessive physical activity and dehydration. 
• Advise parents/carers to seek urgent medical advice if a child has any of the following signs or symptoms:
  
  • Fever. 
  • Respiratory symptoms or other signs of infection.
  • Priapism.
  • Unusual pallor.
  • Weakness (without pain), tingling, loss of speech, or any neurological complications.

Overview of management

The management of chronic complications of sickle cell disease needs a multi-disciplinary approach. Children and adults are seen regularly in secondary care. However, it is recommended that as much of the management as possible should take place in the community.

• Primary care clinicians should:
  • Admit or refer people immediately if they have new symptoms of cerebrovascular disease, as acute strokes are devastating in both children and adults.
  • Manage chronic pain with care.
  • Treat chest infections early, especially in people with chronic sickle lung.
  • Refer people with eye problems urgently. 
  • Refer people for consideration for cholecystectomy if they have symptomatic gallstones.
  • Address concerns about impaired growth or delayed puberty.
  • Manage leg ulcers appropriately.
  • Refer people with suspected pulmonary hypertension, or worsening symptoms.
  • Manage minor episodes of priapism. It is important to enquire about this issue as the person may be too embarrassed to report it.
  • Manage nocturnal enuresis, which is common because of the production of large quantities of dilute urine.
  • Identify renal problems early, and treat hypertension aggressively.
  • Refer people with sleep apnoea.
  • Advise on measures to prevent (or reduce the risk of) complications.

What are the recommended immunizations for people with sickle cell disease?

• All people with sickle cell disease should be fully vaccinated according to the national immunization schedule. 
• For children with sickle cell disease, ensure that they receive:
  • Immunizations in accordance with the Childhood Immunization Programme.
    • See the CKS topic on Immunizations - childhood for more information.
  • Pneumococcal polysaccharide vaccine (PPV23) at 2 years.
    • See the CKS topic on Immunizations - pneumococcal for more information. 
  • Influenza vaccine annually from the age of 6 months.
    • See the CKS topic on Immunizations - seasonal influenza for more information.
  • Meningitis ACWY vaccine — the schedule depends on the age the child is first diagnosed or presents: 
    • Aged under 1 year — two doses at least 4 weeks apart during their first year plus a booster dose 8 weeks after vaccines scheduled at 1 year of age.
    • Aged 1–2 years — one dose at least 8 weeks after the vaccines scheduled at 1 year of age.
    • Aged 2 to under 10 years — one dose.
    • Aged 10 years and over — one dose.
• Older children and adults diagnosed with sickle cell disease, regardless of previous vaccination, should receive:
  • PPV23 vaccine every 5 years. 
    • See the CKS topic on Immunizations - pneumococcal for more information.
  • One dose of MenB and MenACWY, followed by an additional MenB vaccine dose 4 weeks later. 
  • Influenza vaccine annually.  
    • See the CKS topic on Immunizations - seasonal influenza for more information.
• Other vaccines that may be appropriate include: 
  • Hepatitis B vaccine if they have not received it, or have previously received it but are non-immune.   
    • See the CKS topic on Hepatitis B for more information.

What antibiotic treatment should I prescribe to protect against infection in a person with sickle cell disease?

• Penicillin prophylaxis should be offered to all children with sickle cell disease, started by 3 months of age and continued until the child is 5 years old. 
• The recommended doses of phenoxymethylpenicillin are:  
  • Under 1 year — 62.5 mg twice a day.
  • 1–5 years — 125 mg twice a day.
  • Over 5 years — 250 mg twice a day.
• If the person is allergic to penicillin, oral erythromycin should be given. The recommended doses are: 
  • 1 month to 2 years — 125 mg twice a day.
  • 2–8 years — 250 mg twice a day.
  • Over 8 years — 500 mg twice a day.
• Lifelong prophylactic antibiotics should be offered to people considered at continued high risk of pneumococcal infection.
  • Prescribe antibiotic prophylaxis for adults who prefer to continue.

How should I prescribe folic acid for a person with sickle cell disease?

• Folic acid supplementation is unlikely to be necessary on a regular basis in adults with sickle cell disease with a balanced diet. 
• Consider prescribing folic acid for people with a diet that does not contain adequate folic acid, or in whom there is proven folate deficiency. 
  • Seek specialist advice before prescribing folic acid to determine the optimum dose.
• A dose of 5 mg folic acid once a day is recommended for pregnant women and for women trying to conceive.

What should I advise on malaria prophylaxis for people with sickle cell disease?

• Explain that sickle cell disease does not protect against malaria and that malaria is likely to be severe because of splenic hypofunction.
• Advise that all adults and children travelling to an area where malaria is endemic must take adequate prophylaxis, and adherence is very important.
  • See the CKS topic on Malaria prophylaxis for more information.

What interventions are available in secondary care to prevent chronic complications in people with sickle cell disease?

• The detailed management of sickle cell disease in secondary care is beyond the scope of this guidance.
• Parents or carers will receive comprehensive advice on caring for children with sickle cell disease which should be reinforced in primary care if necessary, including:
  • When to seek urgent medical assistance. 
  • How to examine their child for pallor and an enlarging spleen. 
  • The importance of keeping warm, avoiding sudden changes in temperature, and avoiding dehydration (by maintaining a good fluid intake). 
  • The importance of adherence to prophylactic antibiotics and the immunization schedule.
  • When to seek early medical advice, for example if the child has a fever, respiratory symptoms, signs of infection, unusual pallor, an enlarged (or enlarging) spleen, signs of stroke (weakness, tingling, or loss of speech), or painful crisis and dactylitis. 
  • To report any concerns, for example about a child's development or any deterioration in school achievement. 
  • To seek advice about intended travel to a foreign country, so that travel immunization and malarial prophylaxis are provided. 
• Management in secondary care may include:
  • Regular psychological support for children — cognitive behavioural therapy may also be offered to children experiencing frequent pain episodes and emotional difficulties. 
  • Elective blood transfusion — for example, for people with pulmonary hypertension, acute priapism, or severe anaemia. 
  • Hydroxycarbamide treatment — for example, for people who have had recurrent hospital admission for acute chest syndrome or acute painful crises. 
  • Surgery — for example, to relieve pain due to avascular necrosis, or splenectomy for people who have had two or more episodes of acute splenic sequestration. 
  • Stem cell transplantation — this is the only cure for sickle cell disease and may be considered in children and some adults with severe sickle cell disease. 

How should I manage people with sickle cell trait?

As sickle cell trait is usually asymptomatic, management consists mainly of providing appropriate advice.

• Explain to people with sickle cell traits and/or their family/carers that:
  • They should very rarely have symptoms. However, they are at risk of a vaso-occlusive episode if they become oxygen deprived. They should therefore:
    • Be careful at high altitudes (for example, long haul flights, mountain climbing). 
    • Inform the anaesthetist that they are sickle cell carriers, if they are going to have an anaesthetic. 
  • Other known triggers are high atmospheric pressure (such as scuba diving) and exercise — advise them to stay hydrated.
  • They have a 1 in 2 chance of passing the sickle haemoglobin gene to their child. If the other parent is also a carrier, there is a 1 in 4 chance that their child will have sickle cell disease. 
  • It is important to have malaria prophylaxis if they are visiting an area where malaria is endemic. See the CKS topic on Malaria prophylaxis for more information. 
• Refer children and adults with haematuria. 
• Refer children and adults urgently if they present with symptoms suggestive of renal medullary cancer — haematuria, weight loss, loin pain, fever, and abdominal pain.
  • Refer using the suspected cancer pathway referral (for an appointment within 2 weeks).

Which contraception is suitable for women with sickle cell disease?

When advising on contraception, consider the potential harms in the context of the benefits (that is, preventing potentially serious complications caused by pregnancy in a woman with sickle cell disease).

• In general, women with sickle cell disease without complications can use any method of contraception. Options include:
  • Combined hormonal contraceptives (pill, patch, or vaginal ring). See the CKS topic on Contraception - combined hormonal methods for detailed information on these methods of contraception. 
  • Progestogen-only contraception (pill, implant, or injectable). See the CKS topic on Contraception - progestogen-only methods for detailed information on these methods of contraception. 
  • Intrauterine contraception (levonorgestrel intrauterine system [LNG-IUS] or copper intrauterine devices [Cu-IUD]). See the CKS topic on Contraception - IUS/IUD for detailed information on these methods of contraception. 
  • Surgical sterilization. See the CKS topic on Contraception - sterilization for detailed information on this method of contraception. 
• Advise the woman that:
  • Sole use of barrier methods or behaviour based methods of contraception may not be an appropriate choice for them due to higher typical-use failure rates of these methods of contraception. For more information, see the CKS topics on Contraception - barrier methods and spermicides and Contraception - natural family planning. 
  • Long-acting reversible contraception (such as Cu-IUD, LNG-IUS, progestogen-only injectables, and the progestogen-only implant) are more effective at preventing unwanted pregnancy than user-dependent methods. 
• Be aware that:
  • Combined hormonal contraceptives are contraindicated in women with pulmonary hypertension. 
  • There may be an increased risk of menorrhagia with the Cu-IUD. 
  • Surgical sterilization in women is associated with increased risk of pulmonary, cardiac, and neurological complications, and a possible increased risk of wound infections. Women with sickle cell disease who desire sterilization should receive appropriate counselling.
  • In men with sickle cell disease, there is no medical reason to deny vasectomy.