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.