07 / 05 / 2021

Managing fertility challenges in patients with Sickle Cell Disease (SCD)

Women with Sickle Cell Disease (SCD) should be seen before conception (per conception consultation) by a sickle cell specialist care giver to receive information about how this condition affects pregnancy and effect of pregnancy on the patient with this disease, and also how to improve overall outcome for mother and the baby.

Primary care physicians do play a key role in preconception screening, and also the provision of contraceptive advice. This consultation should include optimization of management and screening for end organ damage.

The assessment for chronic disease complication should include:

Blood pressure and urinalysis should be performed to identify women with hypertension and/or proteinuria.

Retinal screening since proliferative retinopathy is common in patient with sickle cell disease.

Screening for iron overload in women who have had multiple transfusion in the past or who have a high ferritin level. Cardiac magnetic resonance imaging may be helpful to assess body iron.

Screening for pulmonary hypertension with echocardiography

Screening for red cell antibodies, these may indicate an increased risk of haemolytic (destruction of red blood cell) disease of the new born.

Sickle Cell Disease is a chronic, lifelong condition and there are recommendations for clinical care which apply to all patients, including women planning to become or those already pregnant.

Women with SCD should receive not only the general preconception care which is given to all women but also additional advice about vaccinations, medication and crisis avoidance. Those with low pain threshold are advised to seek medical help for management of pain crisis. Medical review at least once yearly by a sickle cell physician for the monitoring of chronic disease complications while getting information on the treatment process is recommended.

Information that is particularly relevant for women planning to conceive includes:

The role of dehydration, cold, hypoxia (an absence of enough oxygen in the tissue to sustain bodily functions), over exertion and stress in the frequency of sickle cell crises, and how to avoid them.

How nausea and vomiting in pregnancy can result in dehydration and the precipitation of crises and pain.

The risk of worsening anaemia (reduction in the healthy red cells in blood) the increased risk of crises and acute chest syndrome (ACS).

The risk of increased infection (especially urinary tract infection) during pregnancy.

The increased risk of having a growth-restricted baby, which increases the likelihood of fetal distress, induction of labour and caesarean section.

The chance of their baby being affect by Sickle Cell Disease.

An up-to-date assessment for chronic disease complications that may be related to pregnancy and child birth.

Vaccination status should be determined and updated before pregnancy.

Penicillin prophylaxis or the equivalent should be prescribed. Penicillin prophylaxis and vaccinations are usually monitored and administered in primary care, but should be reviewed by the physician haematologist/obstetrician during pregnancy.

Patient with Sickle Cell Disease have reduced spleen function (hyposplenic) which makes them at a high risk of infection, in particular from encapsulated bacteria such as Neisseria meningitides, Streptococcus pneumonia and Haemophilus influenza. Those with this disease should be advised to receive the influenza and ‘swine flu’ vaccine annually.

Hepatitis B vaccination is recommended and the woman’s immune status should be determined preconception.

Vitamin supplements recommended for SCD patients.

Folic acid is recommended in all pregnant women to prevent neural tube defects. Folic acid at a dosage of at least 1 mg daily is recommended for women with SCD outside pregnancy in view of their haemolytic anaemia, which puts them at increased risk of folic deficiency.

Folic acid 5 mg daily should be prescribed before, during and throughout pregnancy to reduce the risk of neural tube defect and to compensate for the increased demand for folate during pregnancy.

What medications should be reviewed before preconception?

It is importance that a patient with SCD visits the doctor to identify the medications you are presently using to know if they should be stopped before attempting pregnancy.

Hydroxycarbamide (hydroxyurea): This is the medication used to increase red blood Cells), should be stopped at least 3 months before conception. Hydroxycarbamide has been demonstrated to decrease the incidence of acute painful crises in individuals with severe clinical manifestations of SCD, however studies have proven that they are not safe for the development of fetus.

Angiotensin-converting enzyme inhibitors (medication which help to relax blood vessel) and angiotensin receptor blockers (medication which helps to enlarge blood vessel) should be stopped before conception.