2011 Sickle Cell Disease: Swap BSH Flashcards
What are the significant maternal haemoglobinopathies that can lead to sickle cell disease?
- HbSS (homozygous, worst)
- HbSC
- HbSD
- HbSE
- HbSO
- HbS/Lepore
- HbS/β-thalassemia
What is the patholophsiilogy of sickle cell disease?
- Abnormal haemoglobin
- Rigid, fragile, sickle-shaped RBCs
- Haemolytic anaemia
- Vaso-occlusion
- Complications & crises related to 4
What are the complications of sickle cell disease?
- Acute chest syndrome
- Pulmonary hypertension
- Stroke
- Renal dysfunction
- Retinal disease
- Leg ulcers
- Cholelithiasis
- Avascular necrosis
What obstetric complications are at increased risk in sickle cell disease?
- Hypertension & PET
- VTE
- UTIs
- Preterm birth
- Low birth weight
- Stillbirth
- Maternal mortality
What aspects of preconceptual care should be discussed at the annual sickle cell review?
- Partner testing
- Reproductive options: non-intervention, prenatal diagnosis, PGD
- Review of complications
- Medication review
What medications should be recommended preconceptionally/antenatally in SCD?
- Folic acid 5mg OD preconceptually & throughout
- Vitamin D
- Daily antibiotic prophylaxis
- Vaccinations: annual flu, 5-yearly pneumococcal
- Aspirin from 12-36/40
Which medications should be stopped prior to conceiving in SCD?
- ARBs & plan for ACEis
- Hydroxycarbamide
- Iron chelators
What haemoglobinopathy screening should be offered antenatally in SCD?
- Partner screening if not preconceptual
- Prenatal diagnosis: FFD, CVS, amnio; with option of termination
What schedule of ultrasound scanning should be offered in SCD?
Serial growth scans 4-weekly from 24/40
Which women with SCD should blood transfusions be considered for?
- Medical, obstetric or fetal problems
- Previously on hydroxycarbamide
- Multiple pregnancy
- On long-term transfusions for stroke prevention or severe sickle complications
- Worsening anaemia
- Acute complications eg stroke, acute chest syndrome
Why should NSAIDs be avoided from 31/40?
Risk of premature closure of PDA
What recommendations are made for pain management in SCD?
- Agreed pain Mx plan
- Standard except avoid NSAIDs
- If admitted, MDT, thromboprophylaxis, careful fluid & oxygen balance
What proportion of women with SCD have acute pain a) in pregnancy, b) postnatal?
a) 57%
b) 22%
What proportion of pregnant women with SCD develop acute chest syndrome?
10%
What are the characteristics of acute chest syndrome?
- Fever
- Respiratory symptoms
- New pulmonary infiltrate on CXR
How should acute chest syndrome in SCD be managed?
- Admit
- Specific treatment pathway
- CXR, FBC
- Rule out infective causes & consider PE
- Antibiotics covering for atypically even if cultures negative
- Analgesia
- Incentive spirometry
- Top-up or exchange transfusion
What are the main differentials for acute neuro impairment in SCD?
- Stroke
- PET/eclampsia
Which infections should be considered in acute anaemia in SCD?
- Parvovirus B19 = erythrovirus
- Malaria
How does parvovirus infection manifest in SCD?
- Red cell maturation arrest
- Aplastic crisis
- Retuculocytopenia
How much is the risk of pregnancy-related VTE increased in SCD?
1.5-5x
How should VTE risk be managed in SCD?
- Risk assess early pregnancy, hospital admission, intrapartum, postpartum
- Consider 28/40 to 6/52 PN
- Consider throughout if additional RFs
- Give during hospital admission
At what gestation is birth recommended in SCD?
38-40/40
What mode of birth is recommended in SCD?
- Vaginal birth inc VBAC unless contraindications
- Plan positions if hip replacement following AVN
- CS if obstetric indication
Which intrapartum analgesic should be avoided in SCD & why?
Pethidine
Risk of seizures
What are the UKMEC ratings in SCD?
- Progesterone-only: 1
Also may reduce sickle pain - Copper IUCD: 2, due to blood loss
- Combined: 2, due to VTE/CV risk
