In this interview with Sade Oguntola, Professor Bosede Afolabi, the Director of the Centre for Clinical Trials, Research, and Implementation Science at the University of Lagos, College of Medicine, and Head of the Experimental and Maternal Medicine Unit at the Lagos University Teaching Hospital (LUTH), discusses the information that women with sickle cell disease and their partners should be aware of in order to have safe pregnancies.
CAN women with sickle cell get pregnant? Does it carry any risk?
Women with sickle cell disease, preferably referred to as sickle cell warriors, can get pregnant. In the few available studies, they do appear to have a higher risk of miscarriage. Also, from the studies that my team and I did, it appears that their ovarian reserve is lower, age for age, than their haemoglobin AA counterparts. However, they can and do get pregnant. And we look after a lot of pregnant women with sickle cell disease in the Lagos University Teaching Hospital (LUTH), where I work.
Can they have healthy pregnancies, therefore?
They can have healthy pregnancies. However, at least two-thirds of them have complicated pregnancies. Pregnancy, unfortunately, is a situation where they have more frequent crises and more frequent complications, including acute chest syndrome. In pregnancy, they are more likely to have hypertensive diseases of pregnancy like preeclampsia, low birth weight babies and many other issues. They also have a higher likelihood of dying from childbirth, unfortunately. So yes, some pregnancies can be healthy; at least a third of sickle cell warriors in our environment tend to go through pregnancy easily or fairly easily. But a majority of them do have complications during pregnancy.
What are the challenges their babies are likely to face?
Their babies are more likely to be small in weight for their age at birth. They’re more likely to have growth restriction during pregnancy, which means their growth is not as optimal as babies of women who don’t have sickle cell disease. And also, they’re more likely to be born preterm. And finally, because of all these things, their babies do have a higher incidence of stillbirth and death, either during pregnancy or immediately after birth.
How does sickle cell affect pregnancy and childbirth generally?
Generally, it affects their pregnancy by causing them to have more frequent crises and more infections, including malaria. They stand a higher risk of having preterm delivery, ending up in a caesarean section, even though they can have normal vaginal deliveries, and a host of other complications. We prefer them, if possible, to have normal deliveries because it is less stressful on their bodies in total.
However, sometimes, because some of them have had what we call avascular necrosis of the hip, they sometimes have difficulty with their gait and managing the position for a vaginal birth. Some of them have some other reasons, like preeclampsia or severe crises earlier than the ideal time for childbirth, in which case they may not be able to dilate quickly enough to have a normal delivery, and we may need to deliver them quickly. So, for some of these reasons, whether obstetric (i.e., pregnancy-related) or due to the sickle cell itself, we sometimes have to deliver them by caesarean section. But the ideal, if everything goes well, is to deliver them vaginally, and yes, they can push.
What we try to do is to reduce the pain that they have during labour so that they are more comfortable. And when it comes to the second stage, that is, the time when they’re supposed to push, we also try to reduce their pain as much as possible and sometimes even assist them with the pushing by using instrumental delivery, i.e., forceps or vacuum, to help pull the baby out. Sickle cell definitely affects their pregnancy quite significantly.
They talk about painless childbirth; can they have pain-free labour?
An epidural is preferable if it’s available in a centre. In my centre – the Lagos University Teaching Hospital (LUTH), for example – we try to ensure that women going for vaginal delivery who have sickle cell disease have an epidural in labour so that they can deliver as pain-free as possible and smoothly. So yes, that’s the best for them if they are going for vaginal delivery so that the stress of the pain and duration of the labour will not trigger them into having more crises and more problems.
How will their sickle cell treatment plan change during pregnancy to ensure they have healthy babies, free of congenital malformations?
We do want to ensure that they are on the right medication, especially in the first three months of pregnancy. Some pregnant women with sickle cell disease have been on hydroxyurea, which is a drug that helps reduce crises. And when they get pregnant, we say that they should stop. However, it is a good thing to check if hydroxyurea affects babies in that way.
Animal studies have been done that show that hydroxyurea can cause problems in the unborn foetus. However, these animal studies used doses of hydroxyurea that are 10 to 100 times higher than what human beings use. We know that hydroxyurea really helps these women. So, in the guideline that WHO just released, which I was privileged to be the chair of, we decided that we should consider recommencing hydroxyurea after the first trimester (first three months of pregnancy) in somebody who has been using it before, while making sure to balance the benefits versus the risks. This relates especially to women that the drug has helped in the past by helping to reduce their frequency of crises.
The reason for this consideration is also because there was no increase in abnormalities with the offspring of women who were taking it during their pregnancies in other observational studies that examined this. Even though we are not 100% sure about it, as proper trials have not been carried out, it’s also important that the mother herself is well enough to be able to carry a pregnancy safely. So, sometimes, when you balance the benefits of avoiding severe crises, which can occasionally lead to the death of the mother from crisis-related complications, it may sometimes be advisable to prescribe hydroxyurea after laying all the cards on the table for the woman and her partner.
Apart from that, all the other drugs that they may have been on before getting pregnant, like antibiotics that are not safe in pregnancy, may also need to be stopped or changed to other ones that are compatible with early pregnancy. Generally, in all pregnancies, we’re always very mindful of the foetus, but we must remember that the mother is also very important, and for the foetus to be well, the mother also has to be well. So it’s important not to say, Let’s cancel a particular drug. Instead, let’s find an alternative to it. If you don’t find an alternative, then you need to treat the mother. It’s very important. She can have other children. She has to be alive to be able to look after children and to bear children.
What are the signs of sickle cell crisis in pregnancy?
It is like a sickle cell crisis even when you’re not pregnant. It is that you have the very painful bone pains, pains that are difficult to describe till you experience them. You’re suddenly feeling severe pain in different places. You find it difficult to breathe; you’re feeling uncomfortable, and you are feeling very unwell. It’s important, and that’s why, my warriors, I always say to them, You must drink water. You must have water with you everywhere so that you are not dehydrated. You must make sure you are not too cold or too hot at any given time. Anytime you have any small ache, complaint, pain, or feeling of unwellness, don’t wait like you would when you’re not pregnant. Come straight to the hospital to complain because things can change for the worse very quickly. We want to treat any complications as soon as they arise instead of having to wait.
In the case of sickle cell patients or women with sickle cell, when are they supposed to start antenatal?
They need to start early. I would say as early as two months.
We talk about preconception care; is it helpful for them to be “cleared” before getting pregnant?
Pre-conception care is very important for them. There are certain things that one has to check for. Sickle cell warriors sometimes have something we call pulmonary hypertension, which is raised blood pressure in the lung area. It is important to check for it even before the pregnancy. Some of them have sickle cell nephropathy, so they need kidney checks. Also, it’s important to check the eyes and the haemoglobin level. It’s important to know how many transfusions they’ve had in the past and if they have crises frequently so that you know how to modify their treatment and how frequently you have to see them. All these things are important. Before pregnancy, they should also be on folic acid and malaria prevention medicine before they get pregnant.
Are they fit to seek care in a primary health care centre?
You cannot afford to register at a primary health facility if you are a sickle cell warrior, as it is a high-risk pregnancy condition. A tertiary centre would be ideal. They can, however, register for prenatal treatment at a general hospital in the absence of an accessible tertiary centre, which can then refer them to one, if necessary.
The need for blood transfusion is common. Intravenous fluids and oxygen therapy are frequently required. Multidisciplinary care is required, including treatment from pulmonologists (respiratory doctors), microbiologists, infectious disease specialists, haematologists, and others. You cannot find all these specialists in a PHC and not even in some general hospitals, which is why we advise them as much as possible that, at the very least, they should register for antenatal care in a general hospital and, preferably, a tertiary centre. The PHCs do not have the resources to look after sickle cell warriors in pregnancy.
What are your parting words for their partners?
Their partners need to know that these women are very special, and they must help look after their health and care about them. Don’t try and get them to have too many children. I always advise at the very most two children. After two, have some permanent, irreversible contraception like tying your tubes or your husband having a vasectomy, or at least a long-acting contraception like the levonorgestrel intrauterine system implants or the progesterone-only pills. Those progesterone-only family planning methods are good for them, as it has been demonstrated that these can lessen the amount of blood lost during menstruation. In general, the most important thing is that their partners be there for them constantly, make sure they accompany them to the hospital, make sure they get prompt care when they feel unwell, take special care of them, and—most importantly—make sure they limit their family size.
Culled from the Tribune Newspaper
