Sickle Cell Disease in Obstetrics

• Mothers with sickle cell disease have historically been over-represented in MBBRACE reports, although improving care of these patients has improved outcomes
• They remain at higher risk of a number of complications:

• Antenatal exacerbations are common inc. painful crises, acute chest syndromes and infections
• They can be precipitated by the physiological changes in pregnancy e.g.:
• Higher metabolic demand
• Greater susceptibility to infection
• Pro-thrombotic state
• Physiological anaemia
• Aorto-caval compression and vascular stasis

• Routine anaesthetic assessment at or before 36 weeks to:
• Identify end-organ damage and relevant complications of sickle cell disease
• Ensure compatible RBC can be sourced; generation of red cell alloantibodies may limit number of suitable units
• Allow full discussion of labour analgesia and operative anaesthesia (if necessary)
• May require ante-natal aspirin and LMWH; need to consider timing of the latter with regards to neuraxial intervention

• Pregnant patients with sickle cell are more frequently anaemic, yet immediate correction is not warranted unless:
• Hb <50g/L
• Hb fallen by >20g/L
• Haemodynamic compromise
• Risk of end-organ sequelae


• Prophylactic transfusion can reduce the incidence of painful crises during the third trimester, but:
• It does not affect foetal outcome
• It increases the risk of acute transfusion reactions, alloimmunisation (12 - 29%) and delayed, haemolytic transfusion reactions
• It may be considered in those with multiple pregnancies, with a history of complication from previous pregnancy or previously on hydroxycarbamide


• Exchange transfusion, which aim to reduce HbS levels to <30%/HbA levels to >40% should only be instigated by Haematology

• Acute painful crisis (57%)
• Management involves assessing for underlying trigger, which may be infection, acute chest syndrome and/or dehydration (e.g. hyperemesis)
• Analgesia with a blend of simple painkillers and strong opioids is required, although NSAIDs are contra-indicated
• Other standard care including VTE prophylaxis, management of opioid-related side-effects and joint Haematology/Obstetric input


• Acute chest syndrome (7 - 20%)
• More likely in the latter stages of pregnancy
• Presents with cough, chest pain, SOB and hypoxia
• Investigations include FBC (inc. reticulocyte count), ABG, CXR, urinary antigens, sputum ± blood culture and nasal swabs for 'flu, Covid and other common viruses
• Management involves broad-spectrum antibiotic coverage, HDU care with appropriate respiratory support and haematology input to consider exchange transfusion


• Acute stroke
• May be an infarct (although thrombolysis contraindicated) or haemorrhagic
• Urgent Stroke team and Haematology review is required; may need exchange transfusion

• Labour on a unit experienced in managing patients with sickle cell disease and its complications
• Ideally deliver before 40 weeks as risk of complications increase with advancing pregnancy
• No absolute indication for any one mode of delivery although prolonged duration of labour increases risk of complications too
• Ensure:
• Cross-matched red cell units available if needed
• Triggers for sickle crisis are avoided e.g. maintain sats >94%, keep warm and hydrated
• Low threshold for antibiotics if concern re: maternal infection
• Appropriate VTE prophylaxis

• Epidural analgesia is ideal owing to:
• Higher rates of opioid tolerance in this patient population
• Reduces sickle-related pain in the lower body
• Facilitates post-partum analgesia if operative delivery required
• Pethidine is avoided

• Higher rate of LSCS
• There is an increased risk of acute sickle complications after LSCS, regardless of whether regional or general techniques are used
• Maintenance of:
• Normothermia e.g. theatre temperature warmed crystalloid, appropriate warming devices
• Normoxia, which may necessitate supplementary oxygen
• Adequate perfusion e.g. fluid, maintenance of blood pressure with vasopressor infusion as standard
• Meticulous positioning and pressure care
• Standard antibiotic and anti-emetic prophylaxis

• Central neuraxial blockade unsurprisingly remains the preferred mode of anaesthesia, carrying the same benefits as for any patient and an ability to monitor for acute chest crises intraoperatively
• No evidence use of vasopressor increases risk of acute crisis via vasoconstriction/venous stasis
• General anaesthesia remains second line
• Putative benefits from controlled ventilation and avoiding vasoconstrictors
• Higher risk of bleeding from uterine atony in an already-anaemic population at high risk of transfusion complications

• None of the commonly used drugs are contraindicated by the presence of sickle cell disease alone
• Oxytocin bolus + infusion is the preferred first line agent
• Complications arising from sickle cell disease may contra-indicate use of some agents, e.g.:
• Carboprost - renal impairment, chest disease
• Ergometrine - pulmonary hypertension

• A dearth of fully cross-matched red cells mean 'least incompatible' units may have to be used
• This may necessitate concurrent use of IVIg and methylprednisolone to prevent delayed haemolytic transfusion reactions
• TXA is safe to give
• Sickle cell is not an absolute contraindication to cell salvage yet it is not routinely used in such patients, although it has been used in the non-obstetric sickle cell patient

• HDU care as the early puerperium is a high-risk period for complications - acute crisis after delivery may occur in up to 25% of patients
• Extended stay on the delivery suite for monitoring
• Optimised multi-modal analgesia, which may include use of epidural catheters in an ongoing fashion
• LMWH VTE prophylaxis