CPGs/ Recommendations |
ACOG
[20]
|
NHLBI [21]
|
NICE [22]
|
RCOG
[23]
|
Preconception care |
Preconception care |
Preconception care |
Preconception care |
Preconception care |
Genetic screening
|
Individuals of African, Southeast Asian, and
Mediterranean descent are at increased risk for being carriers of
hemoglobinopathies and should be offered carrier screening and, if both
parents are determined to be carriers, genetic counselling.
|
If the partner of a man or woman with SCD has unknown SCD or
thalassemia status, refer the partner for hemoglobinopathy screening.
After testing, refer couples who are at risk for having a potentially
affected fetus and neonate for genetic counseling.
|
Not Mentioned
|
Women and men with SCD should be encouraged to have the hemoglobinopathy
status of their partner determined before they embark on pregnancy. If
identified as an ‘at risk couple’, as per National
Screening Committee guidance, they should receive counselling and advice
about reproductive options.
|
Penicillin prophylaxis |
Not mentioned |
Mentioned for the
pediatric but not for the pregnant women population. |
Not mentioned |
Penicillin prophylaxis or the equivalent should be
prescribed |
Vaccination status updated pre-pregnancy
|
Not Mentioned
|
Mentioned in general for the adult population but not specifically for
pregnant women
|
Not Mentioned
|
Women should be given H. influenza type b and the conjugated
meningococcal C
Vaccine as a single dose if they have not received it as part of primary
vaccination. The pneumococcal vaccine should be given every 5 years.
Hepatitis B vaccination is recommended and the woman’s immune status
should be determined preconceptually. Women with SCD should be advised
to receive the influenza and ‘swine flu’ vaccine annually.
|
Vitamin supplementation |
Pregnant patients with SCD need
increased prenatal folic acid supplementation. The standard 1 mg of
folate in prenatal vitamins is not adequate for patients with
hemoglobinopathies; 4 mg per day of folic acid should be prescribed
because of the continual turnover of red blood cells. |
Folic acid
supplementation should be used whenever considering or at risk of
pregnancy to prevent neural tube defects. |
Not Mentioned |
Folic acid
(5 mg) should be given once daily both preconceptually and throughout
pregnancy. |
Medication review
|
Hydroxyurea has been shown to reduce the frequency of painful crises in
non-pregnant patients with severe SCD. However, the use of hydroxyurea
is not recommended during pregnancy because it is teratogenic.
|
In females who are pregnant or breastfeeding, discontinue hydroxyurea
therapy.
|
Not Mentioned
|
Hydroxycarbamide (hydroxyurea) should be stopped at least 3 months
before conception.
Angiotensin-converting enzyme inhibitors and angiotensin receptor
blockers should be stopped before conception.
|
Antenatal care |
Antenatal care |
Antenatal
care |
Antenatal care |
Antenatal care |
Antenatal hemoglobinopathy screening
|
Not mentioned
|
Not mentioned
|
Not Mentioned.
But a link to the ‘NHS Sickle Cell and Thalassaemia Screening Programme’
was provided.
|
If the woman has not been seen preconceptually, she should be offered
partner testing. If the partner is a carrier, appropriate counselling
should be offered as early as possible in pregnancy – ideally by 10
weeks of gestation – to allow the option of first-trimester diagnosis
and termination if that is the woman’s choice.
|
Medications during pregnancy
|
Not mentioned
|
Not clearly mentioned for women during pregnancy.
|
Mentioned under research recommendations
|
If women have not undergone a pre-conceptual review, they should be
advised to take daily folic acid and prophylactic antibiotics (if not
contraindicated). Drugs that are unsafe in pregnancy should be stopped
immediately.
Iron supplementation should be given only if there is laboratory
evidence of iron deficiency.
Women with SCD should be considered for low-dose aspirin 75 mg once
daily from 12 weeks of gestation in an effort to reduce the risk of
developing pre-eclampsia.
Women with SCD should be advised to receive prophylactic
low-molecular-weight heparin during antenatal hospital admissions.
|
Blood transfusion or prophylactic exchange transfusion for
pregnancies
|
Recommended just to keep of Hb S to approximately 40%
While simultaneously raising the total haemoglobin concentration to
about 10 g/dL.
|
Not Mentioned
|
Not Mentioned
|
Routine prophylactic transfusion is not recommended during pregnancy
for women with SCD.
If acute exchange transfusion is required for the treatment of a
sickle complication, it may be appropriate to continue the transfusion
regimen for the remainder of the pregnancy.
Blood should be matched for an extended phenotype including full
rhesus typing (C, D and E) as well as Kell typing.
Blood used for transfusion in pregnancy should be cytomegalovirus
negative.
|
Ultrasound Scanning and fetal surveillance during pregnancy
|
Pregnancies in women with sickle cell disease are at increased risk for
spontaneous abortion, preterm labor, IUGR, and stillbirth. For this
reason, a plan for serial ultrasound examinations and antepartum fetal
testing is reasonable.
|
Fetal surveillance, which includes growth ultrasounds and antepartum
testing (non-stress tests, biophysical profiles, and contraction stress
tests), may lead to planned early delivery and can reduce but not
eliminate risks (not mentioned as a recommendation).
|
Not mentioned
|
Women should be offered a viability scan at 7–9 weeks of gestation.
Women should be offered the routine first-trimester scan (11–14 weeks
of gestation) and a detailed anomaly scan at 20 weeks of gestation. In
addition, women should be offered serial fetal biometry scans (growth
scans) every 4 weeks from 24 weeks of gestation.
|
Acute painful crisis
|
Major complications (e.g., worsening anemia; intrapartum complications
such as hemorrhage, septicemia, and cesarean delivery; painful crisis;
and chest syndrome) may require intervention with an exchange
transfusion (not mentioned as a recommendation).
Painful crises in pregnancy as well as in the non-pregnant
patient are managed with rapid assessment of the level of pain and
prompt administration of analgesia.
|
Not clearly mentioned for women during pregnancy.
|
For pregnant women with an acute painful sickle cell episode, seek
advice from the obstetrics team and refer when indicated.
Offer all patients regular paracetamol and NSAIDs (non-steroidal
anti-inflammatory drugs) by a suitable administration route, in
addition to an opioid, unless contraindicated (Not clearly mentioned
for women during pregnancy).
The use of NSAIDs should be avoided during pregnancy, unless the
potential benefits outweigh the risks. NSAIDs should be avoided for
treating an acute painful sickle cell episode in women in the third
trimester. See the ’British National Formulary’ for details of
contraindications.
|
Women with SCD who become unwell should have sickle cell crisis
excluded as a matter of urgency.
Pregnant women presenting with acute painful crisis should be rapidly
assessed by the multidisciplinary team and appropriate analgesia
should be administered. Pethidine should not be used because of the
associated risk of seizures.
Women admitted with sickle cell crisis should be looked after by the
multidisciplinary team, involving obstetricians, midwives,
hematologists and anaesthetists.
The requirement for fluids and oxygen should be assessed, and fluids
and oxygen administered if required.
Thromboprophylaxis should be given to women admitted to hospital with
acute painful crisis
|
Intrapartum care |
Intrapartum care |
Intrapartum care |
Intrapartum care |
Intrapartum care |
Timing and mode of delivery
|
Not mentioned
|
Not mentioned
|
Not mentioned
|
Pregnant women with SCD who have a normally growing fetus should be
offered elective birth through induction of labour, or by elective
caesarean section if indicated, after 38+0 weeks of gestation.
SCD should not in itself be considered a contraindication to
attempting vaginal delivery or vaginal birth after caesarean section.
Blood should be cross-matched for delivery if there are atypical
antibodies present (since this may delay the availability of blood),
otherwise a ‘group and save’ will suffice.
In women who have hip replacements (because of avascular necrosis) it
is important to discuss suitable positions for delivery.
|
Optimal mode of analgesia and anaesthesia
|
Not mentioned (analgesia mentioned with painful crisis).
|
Not clearly mentioned for women during pregnancy.
|
Not mentioned
|
Women with SCD should be offered anaesthetic assessment in the third
trimester of pregnancy.
Avoid the use of pethidine, but other opiates can be used.
Regional analgesia is recommended for caesarean section.
|
Postpartum care |
Postpartum care |
Postpartum
care |
Postpartum care |
Postpartum
care |
Neonatal screening |
Not mentioned |
Not mentioned |
Not
mentioned |
In pregnant women where the baby is at high risk of SCD
(i.e. the partner is a carrier or affected), early testing for SCD
should be offered. Capillary samples should be sent to laboratories
where there is experience in the routine analysis of SCD in newborn
samples. This will usually be at a regional centre. |
VTE prophylaxis |
Not Mentioned |
Not clearly mentioned for
women during pregnancy. |
Not mentioned |
Low-molecular-weight heparin
should be administered while in hospital and 7 days post-discharge
following vaginal delivery or for a period of 6 weeks following
caesarean section. |
Contraception
|
Not Mentioned
|
Progestin-only contraceptives (pills, injections, and implants),
levonorgestrel IUDs, and barrier methods have no restrictions or
concerns for use in women with SCD.
If the benefits are considered to outweigh the risks, combined
hormonal contraceptives (pills, patches, and rings) may be used in
women with SCD.
|
Not Mentioned
|
Progestogen-containing contraceptives such as the progesterone only
pill, injectable contraceptives and the levonorgestrel intrauterine
system are safe and effective in SCD.
Estrogen-containing contraceptives should be used as second-line
agents.
Barrier methods are as safe and effective in women with SCD as in the
general population.
|