Management of sickle cell disease in pregnancy GT61 Flashcards
If woman is HbSA (sickle cell trait), what symptoms may she suffer from in pregnancy
usually asymptomatic. Increased risk UTI and microscopic haematuria
how many children are born with sickle cell disease each year? Where are most of these children born?
300 000 born each year.
2/3 of these children are born in Africa
in the UK, how many individuals are affected with sickle cell? How many children are born each year with Sickle cell?
12000-15000 affected individuals in UK
each year approx 300 infants born with Sickle cell
in the uk, how many pregnancies occur in women with sickle cell per year?
100-200
what are the 9 main clinical presentations of sickle cell disease?
anaemia, acute pain crisis, stroke, pulmonary hypertension, renal dysfunction, retinal disease, leg ulcers, kidney stones, avascular necrosis
what are the maternal risks of sickle cell disease (HbSS)?
spont miscarriage, antenatal hospital admission, maternal mortality, pre-eclampsia, c/s, VTE, infection
what are the main risks associated with HbSC?
increased painful crises, FGR, antepartum hospital admission, post partum infection
what are the increased risks to fetus in HbSS?
FGR- thus increasing likelihood fetal distress, IOL and c/s, prem labour, perinatal mortality,
when planning to conceive, what information is important to tell pt regarding change to their sickle cell disease?
Increased freq sickle cell crises- dehydration, stress
worsening anaemia, Acute chest syndrome, increased risk infection- UTI, chance of baby being infected with SCD
how should women with sickle cell be screened for pulmonary hypertension?
cardiac echo- tricuspid regurge jet >2.5m/sec= high risk pulmonary hypertension. should be done if not had this in last 1 year
what screening tests are done pre-conceptually in women with sickle cell?
BP, urine- for HTN/ nephropathy
retinal screening- to look for proliferative nephropathy
screening for iron overload- ferritin, T2 cardiac MRI
screening for red cell antibodies
if the partner is carrier or affected by the following haemaglobinopathies, then pre-natal disgnosis for fetus is advised
HbS, beta thal, O-Arab, HbC, D-Punjab
what bacteria are patients with sickle cell susceptible to? what prophylaxis should be given?
encapsulated bacteria- strep pneumonia, neisseria meningitides. Should be given penicillin if hyposplenic
which vaccinations are reccomended for women with sickle cell, prior to pregnancy
H.Influenza b, conjugated meningococcal C- as a single dose.
Hep B vaccine
pneumococcal vaccine every 5 years.
influenza and swine flu vaccines- yearly
What vitamin supplements should be given to women with sickle cell
folic acid 5mg OD. preconception and throughout pregnancy
in women with sickle cell, which medications need to be reviewed before pregnancy?
hydroxycarbamide (hydroxyurea) stopped 3/12 prior to conception. Teratogenic.
ACEi/ ARB
what should be done if woman becomes pregnant on hydroxycarbamide?
stop. level 3 ultrasound for structural abnormality. TO not advised
in pregnancy, are women with HbSS treated with the same ante-natal care as women with HbSC?
YES. HbSC has fewer adverse outcomes, but still an increased risk of painful crises, FGR, antenatal hospitalisation, postnatal infection
in woman with sickle cell, if partner is carrier/ affected by haemoglobinopathy, by what gestation should they be seen in ANC for counselling?
ideally by 10/40 to allow 1st tri diagnosis or TOP
is sickle cell considered a ‘mild’ or ‘high’ risk factor for pre-eclampsia?
sickle cell is mild. Aspirin prophylaxis given according to NICE guidelines
if NSAIDs are required in pregnancy, what gestation is safe to take them?
between 12/40 and 28/40
in women with sickle cell, how often should BP, urine and MSU for culture be checked?
BP, urinalysis at every appointment.
MSU for culture every month
in women with sickle cell, what is the recommended schedule of USS during pregnancy?
7-9/40= viability scan
11-14/40= routine first tri scan
20/40= anomaly scan
from 24/40= serial growth scans every 4 weeks
why is routine prophylactic blood transfusion not recommended in women with sickle cell?
prophylactic transfusion decreased incidence of painful crises but did not influence maternal or fetal outcome in singleton pregnancy
in women with sickle cell, under what circumstances is blood transfusion indicated ante-natally
- acute anaemia- ‘top-up’ transfusion.
- exchange transfusion for acute chest syndrome or acute stroke.
- transfusion regimen before pregnancy
- Twin pregnancies- prophylactic transfusion
in what percentage of sickle cell women will alloimmunisation (formation of red cell Ab to red cell Ag) also occur?
18-36%
why might a woman with sickle cell be on a blood transfusion regimen prior to pregnancy?
for primary or secondary prevention of stroke, or prevention of severe disease complications
what is the most frequent complication of sickle cell in pregnancy? What percentage of women will present with this?
painful crisis.
27-50% women with have this during pregnancy
in sickle cell, what is the most frequent cause of hospital admission in pregnancy?
acute painful crisis
in sickle cell, how is ‘mild’ painful crisis managed in pregnancy?
what is criteria for referring women to hospital?
rest, oral fluids, paracetamol, weak opioids, NSAIDs (btwn 12-28/40)
referral if- pain not settling, febrile, atypical pain, chest pain, SOB,
if woman presents with painful sickle crisis, what are initial investigations?
FBC, reticulocyte count, renal function.
other tests depend on clinical presentation- CXR, urine culture, LFT
what analgesia should be avoided in women with sickle cell disease, and why?
PETHIDINE. because risk of toxicity and associated seizures.
where should a woman with acute painful crisis be admitted in early pregnancy? what about in late pregnancy?
early pregnancy- medical ward
later on in preg- LEVEL 2 antenatal bed
in acute painful sickle crisis, in what time frame should initial analgesia be given?
initial analgesia within 30 minutes of arrival, effective analgesia by 1 hour
how often should observations be done in pregnant women with sickle cell presenting with acute painful crisis?
initial- every 20 mins= Resp rate, sedation, pain score
if admitted- 2 hourly= resp rate, pain score, sedation, O2sats
what is optimal fluid intake of woman treated for acute sickle crisis?
60ml/kg/24hrs
after acute pain, what is the next most common complication of sickle cell disease? in what percentage of pregnancies is this reported?
Acute chest syndrome. reported in 7-20% pregnancies
what are characteristics of acute chest syndrome?
tachypnoea, chest pain, cough, SOB, new infiltrate on CXR
what is treatment of acute chest syndrome?
IV ABx, O2, blood transfusion- exchange transfusion in severe hypoxia where Hb is maintained.
which teams should be involved with pregnant woman with acute chest syndrome?
obstetrics, haematology, critical care
what type of infection is associated with acute anaemia in women with sickle cell?
what investigation may help with diagnosing this?
erythrovirus infection. Causes reticulocytopenia (due to red cell maturation arrest and aplastic crisis).
Ix= reticulocyte count- if low may indicate infection.
Tx= blood transfuion, isolation
what is the risk to fetus if woman develops erythrovirus infection (in sickle cell disease)
vertical transmission of erythrovirus infection, causing hydrops
in pregnant women with sickle cell disease, who have normally growing fetus, when should labour ad delivery be planned?
delivery between 38-40/40
in women with sickle cell disease, what are the increased risks to the disease in the intrapartum period?
increased frequency sickle cell crisis, acute chest syndrome.
increased risk painful crisis with long labour
in women with sickle cell disease, during labour what observations are rquired? at what O2 saturation should ABG and Oxygen therapy be started?
regular o2 sats, BP, RR.
if O2 sats <94%- do ABG and give O2
what is the recommended type of analgesia in labour for women with sickle cell?
regional anaesthesia.
what VTE prophylaxis is given to women with sickle cell disease postnatally?
vaginal delivery - 10/7 LMWH.
c/s- 6/52 LMWH
what is the percentage risk of sickle cell crisis post-natally? after what type of anaesthetic is it more common?
25%. More common after GA
what type of longer term contraception is recommended in women with sickle cell disease?
progesterone only contraceptive
in women with sickle cell, with which type of contraceptive are women less likely to have painful episode?
DMPA
in women with sickle cell, which contraceptives are deemed UK MEC category 2, which are UK MEC category 1?
COC, copper IUD= UK MEC cat 2
POP, depot, mirena= UK MEC 1
