Sickle Cell Disease in Pregnancy

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Introduction

Pregnancy in the Sickle Cell Disease (SCD) patient is associated with poor obstetric outcomes for both the mother and her baby.

Sickle cell complications during pregnancy leads to high maternal and perinatal mortality.

Common pregnancy complications of the mother with sickle cell disease include miscarriages, frequent vaso-occlusive crises, severe anaemia, recurrent urinary tract infections, acute chest syndrome, preterm labour and pulmonary embolism.

In the foetus, complications include intrauterine growth restriction, prematurity and intrauterine foetal death.
Care by a multidisciplinary team consisting of the Obstetricican, Physician specialist, Haematologist, Clinical Pharmacist, Anaesthetist, Nurse and Midwife ensures good health outcomes for these patients.

Causes of sickle cell disease

(See ‘Sickle Cell Disease‘)

Symptoms of sickle cell disease

  • Recurrent Jaundice
  • Bone and joint pain (recurrent episodes)
  • Sub-fertility
  • Anaemic complications (easy fatiguability, palpitations)

Signs of SCD

(See ‘Sickle Cell Disease’)

Investigations

  • Sickling test and Haemoglobin electrophoresis
  • Full blood count including reticulocyte count
  • RDT or blood film for malaria parasites
  • Midstream urine for Culture and sensitivity at booking indicated
  • Renal function test (BUN, Creatinine, electrolytes)
  • Liver function tests
  • Serum iron, TIBC and Ferritin (if possible)
  • All other antenatal screening test

Treatment for sickle cell disease in pregnancy

Treatment objectives

The treatment objectives of sickle cell disease in pregnancy include the following

  1. To identify patients with condition early during pregnancy for closer monitoring
  2. To correct anaemia
  3. To prevent known crises triggers
  4. To detect and manage promptly all associated complications and crises
  5. To deliver client safely as soon as baby is viable

Pharmacology treatment

Pre-pregnancy Care

  • Provide genetic counselling
  • Assist patient to plan pregnancy in a status of optimum health
  • Educate on potential risk and health problems of sickle cell disease in Pregnancy

Care during Pregnancy

At booking
  1. Assess patient carefully for risk of frequent crises, and obstetric complications previous
  2. Examine for nutritional status, anaemia, jaundice, and hepatosplenomegally
  3. Perform ultrasound to date pregnancy and exclude foetal abnormality and growth retardation
  4.  Perform the above listed investigations to screen for and detect complications
  5. Manage identified problems at booking (e.g. urinary tract infection, malaria etc.) appropriately and promptly
Follow up visits
  1. If low risk, schedule follow up visits monthly until 2nd trimester, then 2 weekly until 28th week and thereafter weekly
  2. If high-risk, schedule visits 2 weekly until third trimester, then weekly until term
  3. At review visits, enquire about maternal well-being, symptoms of crises, infection and foetal activity.
  4. Examine for pallor, jaundice, fever and proteinuria. Check for foetal growth and compare to gestational age

Labour and delivery

  1. Stress and pain of labour and delivery can trigger crisis.
  2. Risks of crisis is increased if dehydration, fever, infection, hypothermia develop during labour.
  3. Care during labour/delivery is targeted at preventing these triggers
  4. All women with Sickle cell Disease must deliver in hospital, and the most experienced midwife and obstetrician should be involved in their care
  5. In uncomplicated pregnancies, spontaneous vaginal delivery must be aimed at until 40 weeks (term).
  6. Induction must be undertaken only if the woman has not delivered after this time.
  7. For the high risk women with recurrent complications, deliver as soon as baby is viable
  8. At admission in labour;
    • Labour should be monitored closely with partograph
    • Check for haemoglobin level
    • Group and cross-match a unit of blood on standby
    • Maintain good hydration with IV fluids
    • Provide adequate pain relief (See ‘Analgesia in labour’)
    • Provide prophylactic antibiotics (See ‘Antibiotic prophylaxis in labour and puerperium below’ )
  9. Vaginal delivery should be the aim unless there are obstetric reasons for cesarean section
  10. Prolonged second stage labour should be avoided by offering assisted delivery (vacuum)
  11. Active Management of Third Stage of Labour (AMTSL) is recommended to prevent PPH

Postpartum care

  1. All Sickle Cell Disease patients must be hospitalized and monitored closely for at least 72 hours postpartum.
  2. Check for pallor, puerperal sepsis, acute chest syndrome, vaso-occlusive crises and urinary tract infection
  3. Continue with prophylactic antibiotics for at least 72 hours postpartum
  4. Counsel women and their support persons to avoid crises triggers such as stress, pain, lack of sleep, fatigue etc.
  5. Encourage early ambulation (especially after operative delivery)
  6. Review client at 7 days postpartum and thereafter weekly until 6 weeks
  7. Refer patient to physician for continuing care
  8. Counsel on family planning and offer contraception

Baby

  • The baby should be screened for sickle cell disease
  • Closely monitor the babies with associated complications e.g. IUGR, prematurity etc.

Sickling crises in pregnancy

  • Admit all sickle cell patients with sickling and/or complications during pregnancy to a hospital
  • Avoid NSAIDs e.g. Diclofenac in the 3rd trimester obstetric
  • Opiods like pethidine and morphine for pain management should be used with care during labour due to respiration depression effects on baby (See Analgesia in Labour)
  • Identify and treat underlying triggers e.g UTI, malaria, dehydration etc.
  • Blood transfusion is recommended if haemogloblin level is than less 7g/dl at 36 weeks and/or during crises (See ‘Sickle Cell Disease’)
  • If in vaso-oclusive crises maintain hydration with IV fluids (crystalloids) 2-4 litres in 24 hours, and provide analgesia (See ‘Sickle Cell Disease’)
  • Acute chest syndrome (ACS)- foetal monitoring is essential during this crisis (See ‘Acute Chest Syndrome’)

Pharmacological treatment

A. Antenatal treatments

(See Pharmacological treatments under ‘Antenatal Care’)

And

Multivitamin, oral, once daily

B. Antibiotic prophylaxis in labour and puerperium

Ceftriaxone, IV/IM,

  • 1 g daily for 72 hours

Or

Amoxicillin + Clavulanic Acid, IV,

  • 1.2 g stat.

Then

Amoxicillin + Clavulanic Acid, oral,

  • 625 mg 12 hourly for 72 hours

Referral Criteria

All patients with sickle cell disease with obstetric complications should be referred to a specialist for continuing care.

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