Sickle Cell Anemia Flashcards
1
Q
Mx in pregnancy (include acute pain crisis)
A
autosomal recessive - HbS - sickling of Hb in deoxygenated state
risk to mum - pain crisis/acute chest syndrome/infections/VTE/PET/APH
risk to baby - inheritance/IUGR/PTL/PTB/IOL/CS
Targeted Hx
- previous dx & rx (e.g. transfusions)
- previous hospitalisation - crisis/ACS
- medications - eg ACEI
- ?splenectomy - HBV/Hib vax/Penicillin
- Tertiary - high risk
- MDI - MFM/Haem/Genetics
- Partner screen +/- carrier testing +/- Genetic counselling
- Ed - triggers - dehydration/stress/over-exertion
- LDA + LMWH based on VTE/PET risks
- high dose FA entire preg
- Regular ANC - screen for PET + Serial G/S
- Timing - IOL from 38/40 (risk abruption/PET/crisis)
- IP - CEFM/warm/hydration/epi/avoid long labor
- PN - VTE proph/avoid triggers/haem F/U, paed F/U ?testing for SCA
SCA crisis mx
- targeted HEI (DDx - ACS/Dehydration/Sepsis)
- resuscitation/stabilisation
- analgesia + oxygenation
- fetal wellbeing
- find & rx reversible cause
- follow-up
