Sickle cell disease

This is really aimed at secondary care, but there are some things we need to be aware of. A useful guideline to read if you have an affected patient.

Preconception Counselling

  • Ask women about intentions regarding pregnancy so they can have proper preconception management.
  • Vaccinations – Hib / MenC (if not had in childhood). Pneumococcus every 5 yrs. Hep B. Flu annually.

Medications advice

  • Hydroxyurea - stop at least 3m before conception
  • ACEi / A2RB – need to stop.
  • NSAIDS only between 12 and 28/40 (adverse effects on fetal development)
  • Crises triggers – dehydration (eg vomiting in pregnancy) / cold / hypoxia / overexertion / stress. Warn women to avoid where possible.
  • Penicillin (they are hyposplenic) – ensure women are on this.
  • Folic Acid – 5mg throughout pregnancy
  • Partner’s Status – ensure this is checked and counselling offered if a high risk phenotype (see the guideline).

Management during Pregnancy

  • If the woman falls pregnant without preconception planning, then ensure that all the above are done as soon as possible.
  • At booking do the blood pressure, MSU and sats.
  • Care will be with the haematologists / obstetricians throughout the pregnancy.
  • Iron – don’t treat with iron unless iron deficiency is proven (as they are often iron overloaded).
  • Aspirin 75mg – should be used from 12/40 (as small increase risk of pre-eclampsia).
  • Viability scan – should be offered at 7 to 9/40 in addition to the dating scan.
  • MSU monthly throughout pregnancy as high risk from an untreated UTI.
  • Crises – If there is any doubt about what is going on, or if this could be a crises, then seek advice. Anyone with chest pain, fever, SOB or not responding to simple analgesia needs referral

Contraception

  • Progesterone only methods are preferred as there is a small increase risk of VTE, but COC can be used too.

Louise Hudman

I'm a freelance GP locum in Winchester & Southampton locum chambers, and Pallant Medical Chambers Clinical Guidelines Lead Partner.

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