Management of sickle cell disease in pregnancy GT61

Question 1

If woman is HbSA (sickle cell trait), what symptoms may she suffer from in pregnancy

Answer 1

usually asymptomatic. Increased risk UTI and microscopic haematuria

Question 2

how many children are born with sickle cell disease each year? Where are most of these children born?

Answer 2

300 000 born each year.

2/3 of these children are born in Africa

Question 3

in the UK, how many individuals are affected with sickle cell? How many children are born each year with Sickle cell?

Answer 3

12000-15000 affected individuals in UK

each year approx 300 infants born with Sickle cell

Question 4

in the uk, how many pregnancies occur in women with sickle cell per year?

Answer 4

100-200

Question 5

what are the 9 main clinical presentations of sickle cell disease?

Answer 5

anaemia, acute pain crisis, stroke, pulmonary hypertension, renal dysfunction, retinal disease, leg ulcers, kidney stones, avascular necrosis

Question 6

what are the maternal risks of sickle cell disease (HbSS)?

Answer 6

spont miscarriage, antenatal hospital admission, maternal mortality, pre-eclampsia, c/s, VTE, infection

Question 7

what are the main risks associated with HbSC?

Answer 7

increased painful crises, FGR, antepartum hospital admission, post partum infection

Question 8

what are the increased risks to fetus in HbSS?

Answer 8

FGR- thus increasing likelihood fetal distress, IOL and c/s, prem labour, perinatal mortality,

Question 9

when planning to conceive, what information is important to tell pt regarding change to their sickle cell disease?

Answer 9

Increased freq sickle cell crises- dehydration, stress

worsening anaemia, Acute chest syndrome, increased risk infection- UTI, chance of baby being infected with SCD

Question 10

how should women with sickle cell be screened for pulmonary hypertension?

Answer 10

cardiac echo- tricuspid regurge jet >2.5m/sec= high risk pulmonary hypertension. should be done if not had this in last 1 year

Question 11

what screening tests are done pre-conceptually in women with sickle cell?

Answer 11

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

Question 12

if the partner is carrier or affected by the following haemaglobinopathies, then pre-natal disgnosis for fetus is advised

Answer 12

HbS, beta thal, O-Arab, HbC, D-Punjab

Question 13

what bacteria are patients with sickle cell susceptible to? what prophylaxis should be given?

Answer 13

encapsulated bacteria- strep pneumonia, neisseria meningitides. Should be given penicillin if hyposplenic

Question 14

which vaccinations are reccomended for women with sickle cell, prior to pregnancy

Answer 14

H.Influenza b, conjugated meningococcal C- as a single dose.
Hep B vaccine
pneumococcal vaccine every 5 years.
influenza and swine flu vaccines- yearly

Question 15

What vitamin supplements should be given to women with sickle cell

Answer 15

folic acid 5mg OD. preconception and throughout pregnancy

Question 16

in women with sickle cell, which medications need to be reviewed before pregnancy?

Answer 16

hydroxycarbamide (hydroxyurea) stopped 3/12 prior to conception. Teratogenic.
ACEi/ ARB

Question 17

what should be done if woman becomes pregnant on hydroxycarbamide?

Answer 17

stop. level 3 ultrasound for structural abnormality. TO not advised

Question 18

in pregnancy, are women with HbSS treated with the same ante-natal care as women with HbSC?

Answer 18

YES. HbSC has fewer adverse outcomes, but still an increased risk of painful crises, FGR, antenatal hospitalisation, postnatal infection

Question 19

in woman with sickle cell, if partner is carrier/ affected by haemoglobinopathy, by what gestation should they be seen in ANC for counselling?

Answer 19

ideally by 10/40 to allow 1st tri diagnosis or TOP

Question 20

is sickle cell considered a ‘mild’ or ‘high’ risk factor for pre-eclampsia?

Answer 20

sickle cell is mild. Aspirin prophylaxis given according to NICE guidelines

Question 21

if NSAIDs are required in pregnancy, what gestation is safe to take them?

Answer 21

between 12/40 and 28/40

Question 22

in women with sickle cell, how often should BP, urine and MSU for culture be checked?

Answer 22

BP, urinalysis at every appointment.

MSU for culture every month

Question 23

in women with sickle cell, what is the recommended schedule of USS during pregnancy?

Answer 23

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

Question 24

why is routine prophylactic blood transfusion not recommended in women with sickle cell?

Answer 24

prophylactic transfusion decreased incidence of painful crises but did not influence maternal or fetal outcome in singleton pregnancy

Question 25

in women with sickle cell, under what circumstances is blood transfusion indicated ante-natally

Answer 25

1. acute anaemia- ‘top-up’ transfusion.
2. exchange transfusion for acute chest syndrome or acute stroke.
3. transfusion regimen before pregnancy
4. Twin pregnancies- prophylactic transfusion

Question 26

in what percentage of sickle cell women will alloimmunisation (formation of red cell Ab to red cell Ag) also occur?

Answer 26

18-36%

Question 27

why might a woman with sickle cell be on a blood transfusion regimen prior to pregnancy?

Answer 27

for primary or secondary prevention of stroke, or prevention of severe disease complications

Question 28

what is the most frequent complication of sickle cell in pregnancy? What percentage of women will present with this?

Answer 28

painful crisis.

27-50% women with have this during pregnancy

Question 29

in sickle cell, what is the most frequent cause of hospital admission in pregnancy?

Answer 29

acute painful crisis

Question 30

in sickle cell, how is ‘mild’ painful crisis managed in pregnancy?
what is criteria for referring women to hospital?

Answer 30

rest, oral fluids, paracetamol, weak opioids, NSAIDs (btwn 12-28/40)
referral if- pain not settling, febrile, atypical pain, chest pain, SOB,

Question 31

if woman presents with painful sickle crisis, what are initial investigations?

Answer 31

FBC, reticulocyte count, renal function.

other tests depend on clinical presentation- CXR, urine culture, LFT

Question 32

what analgesia should be avoided in women with sickle cell disease, and why?

Answer 32

PETHIDINE. because risk of toxicity and associated seizures.

Question 33

where should a woman with acute painful crisis be admitted in early pregnancy? what about in late pregnancy?

Answer 33

early pregnancy- medical ward

later on in preg- LEVEL 2 antenatal bed

Question 34

in acute painful sickle crisis, in what time frame should initial analgesia be given?

Answer 34

initial analgesia within 30 minutes of arrival, effective analgesia by 1 hour

Question 35

how often should observations be done in pregnant women with sickle cell presenting with acute painful crisis?

Answer 35

initial- every 20 mins= Resp rate, sedation, pain score

if admitted- 2 hourly= resp rate, pain score, sedation, O2sats

Question 36

what is optimal fluid intake of woman treated for acute sickle crisis?

Answer 36

60ml/kg/24hrs

Question 37

after acute pain, what is the next most common complication of sickle cell disease? in what percentage of pregnancies is this reported?

Answer 37

Acute chest syndrome. reported in 7-20% pregnancies

Question 38

what are characteristics of acute chest syndrome?

Answer 38

tachypnoea, chest pain, cough, SOB, new infiltrate on CXR

Question 39

what is treatment of acute chest syndrome?

Answer 39

IV ABx, O2, blood transfusion- exchange transfusion in severe hypoxia where Hb is maintained.

Question 40

which teams should be involved with pregnant woman with acute chest syndrome?

Answer 40

obstetrics, haematology, critical care

Question 41

what type of infection is associated with acute anaemia in women with sickle cell?
what investigation may help with diagnosing this?

Answer 41

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

Question 42

what is the risk to fetus if woman develops erythrovirus infection (in sickle cell disease)

Answer 42

vertical transmission of erythrovirus infection, causing hydrops

Question 43

in pregnant women with sickle cell disease, who have normally growing fetus, when should labour ad delivery be planned?

Answer 43

delivery between 38-40/40

Question 44

in women with sickle cell disease, what are the increased risks to the disease in the intrapartum period?

Answer 44

increased frequency sickle cell crisis, acute chest syndrome.
increased risk painful crisis with long labour

Question 45

in women with sickle cell disease, during labour what observations are rquired? at what O2 saturation should ABG and Oxygen therapy be started?

Answer 45

regular o2 sats, BP, RR.

if O2 sats <94%- do ABG and give O2

Question 46

what is the recommended type of analgesia in labour for women with sickle cell?

Answer 46

regional anaesthesia.

Question 47

what VTE prophylaxis is given to women with sickle cell disease postnatally?

Answer 47

vaginal delivery - 10/7 LMWH.

c/s- 6/52 LMWH

Question 48

what is the percentage risk of sickle cell crisis post-natally? after what type of anaesthetic is it more common?

Answer 48

25%. More common after GA

Question 49

what type of longer term contraception is recommended in women with sickle cell disease?

Answer 49

progesterone only contraceptive

Question 50

in women with sickle cell, with which type of contraceptive are women less likely to have painful episode?

Answer 50

DMPA

Question 51

in women with sickle cell, which contraceptives are deemed UK MEC category 2, which are UK MEC category 1?

Answer 51

COC, copper IUD= UK MEC cat 2

POP, depot, mirena= UK MEC 1