Pregnancy nightmare for sickle cell disease

In November 2021, Sally Magdaline lost her sister, Maureen.

Maureen, who died aged 32 while giving birth, had sickle cell disease. This year, Sally got pregnant. The 29-year-old lived with dread because she, too, is a sickle cell disease warrior. She feared history would repeat itself.

Throughout her pregnancy, all she thought of was how she was going to escape the jaws of death. Sally’s mother, Lilian Okuku, had wanted her not to bear children. Jolted by what had befallen Maureen, she requested Sally to have her uterus removed so she could not go through the pain. Sally refused.

“When she told me she was pregnant at 16 weeks, admitted and needed blood, I did not sleep. I cried to God and even asked my daughter to abort. But she refused,” says Ms Okuku. “I lost my first daughter as I watched when her pregnancy was in the 34th week. Given her condition, the doctors were against a Caesarean section. They wanted her to give birth normally.”

Maureen had been induced to start labour and she died as she pushed out her baby. She lost a lot of blood even after being transfused.

“Had they acted faster, my daughter would not have died. Because of that, I really feared for Sally. I saw death immediately my child told me she was expecting,” remembers Ms Okuku.

Sally’s journey was not easy either. When she was 16 weeks pregnant, she had pelvic pains then she went to a dispensary close to her home. She was, however, referred to Pumwani Maternity Hospital as the medics categorised her pregnancy as high-risk. At the hospital, she needed an emergency admission. Her haemoglobin levels were at 5.5 grams per decilitre — which the doctors deemed critical.

The hospital did not have the expertise and enough blood to handle her condition, and so she was then referred to the Kenyatta National Hospital (KNH).The family had to do a blood drive to save her life. She was lucky and was discharged when her haemoglobin levels were at 9.4 grams per decilitre. The haemoglobin levels of a healthy person are around 13 grams per decilitre.

Haemoglobin is a protein that helps carry oxygen throughout the body. People with sickle cell disease produce abnormal haemoglobin.

At the hospital, Sally was asked to choose between her life and that of her unborn child. They wanted her to terminate the pregnancy but she refused.

At four months, she landed in a crisis when her haemoglobin level reduced to 6.5 grams per decilitre. She was rushed to Juja Hospital but they could not handle her situation. She was again referred to KNH, where she was admitted for three weeks. She got transfused again and discharged when her haemoglobin levels were at 10 grams per decilitre. She was stable.

For two months, she was okay, going for her normal check-ups after every two weeks as instructed by the doctor. At six months, she got another attack and started bleeding. Her life was on the line. She was having one of the worst sickle cell crises of her life. Her haemoglobin level was at six. She was admitted at Ruaraka Hospital for two weeks.

When her condition was worsening with no improvement, doctors recommended that she be taken to KNH. She was admitted for three weeks. She got a transfusion of three pints of blood. During her entire pregnancy, Sally was in and out of hospital so many times that she considered the brightly-lit wards her second home.

At seven months, another crisis happened. This time, she requested the nurses to remove the child. She was tired. The nurses refused and instead induced her. It did not work.

She was taken for a Caesarean section. She lost a lot of blood and had a transfusion of five pints of blood in addition to supplements.

“Apart from just the crisis, I was in so much pain and I was ever on bed rest. When not in hospital wards, I was in my bed at home. I was tired of that life and I needed to rest,” she tells Healthy Nation at their home in Nairobi. “This is not an easy journey. You must be ready. With the right support system, doctors and family, one can make it. If it weren’t for my doctors, I don’t think I would have made it.”

Sally is not ready to give birth again any time soon. She is, nonetheless, thankful to God that despite her delicate condition, she gave birth to her son safely. “I am a testimony. It is possible, but under total care,” she says.

Dr John Ongech, a consultant obstetrician/ gynaecologist, says that with the help of haematologists, he has seen thousands of women with sickle cell disease give birth normally without complications. He admits that pregnancy for a sickle cell patient is a high-risk situation.

However, he notes that with special care and monitoring, a woman can give birth to as many as four children. “Whenever I have a patient who has the condition, I always walk the journey with a haematologist. I have helped so many and none of them has died. The success rate of delivery of women with the condition is high,” he said.

Dr Ongech made it clear that it is not automatic that women with the condition have to die. “Even a normal pregnancy can be risky. They (women) just need to have a specialist and special care and they are good to go,” he said.

Ms Okuku is a happy grandmother. However, she has a message to young men and women getting into marriage. “In the past, this disease was known in Western and Nyanza region but now it is all over. Had I known that we (my husband and I) were both carriers, we would not have married,” she says, advising the public to make it a habit of testing for hereditary conditions before getting into marriage.

“Even though I am happy that my daughter made it, the journey was painful. I cannot wish it even on my worst enemy. My lastborn son is also a warrior; he has a long way to go. I continue hoping for the best,” she said.

When Ms Okuku realised that the last three of her five children had sickle cell disease, she removed her uterus at the age of 42. She swore never to give birth. “I was afraid that if I continued to give birth, I would bring forth more children with the condition,” she said. The disease is a genetically inherited condition that is transferred to children if both parents are carriers of the sickle cell trait.

In Kenya, approximately 6,000 infants are born with sickle cell disease each year. Fifty to 80 percent of them die before their fifth birthday.

There is limited data and gaps in knowledge on the reproductive health and needs of women with sickle cell disease. Blood transfusion is a lifeline for sickle cell disease warriors, though a perennial shortage puts many lives at risk. Transfusion lowers the amount of haemoglobin S (Hgb S) red blood cells in the body and increases the number of normal red blood cells. Hgb S cells are the abnormal sickle-shaped components that give the disease its name.

When the Hgb S cells are fewer in the bloodstream, they are less likely to build up and block blood vessels. Having sickle cell disease makes red blood cells become rigid and sickle-shaped, blocking the flow of blood and oxygen to the body and causing intense pain and other complications.

A paper published in Blood, a journal by the American Society of Haematology, says that compared to healthy women, pregnant women with a severe form of sickle cell disease are six times more likely to die during or following pregnancy. They also have a higher risk for stillbirth, high blood pressure, and preterm delivery.

It is for this reason that 27-year-old Millicent Makori has lived in fear of being further weakened by the disease in case she gets pregnant. She has had a rough journey with the disease. She has vowed never to give birth and is willing to start the journey of adopting a child. “I cannot imagine that even when I am having my normal menstruation, at times during my heavy bleeding I have to get transfused. What about giving birth where I may lose a lot of blood?” asked Ms Makori, who hails from Kisii County.

“It is not that I am not willing to have a child. I love children and would really wish to be a mother. But with my condition, I am better off not giving birth.”

Even without pregnancy, she explains to Healthy Nation how she has had recurrent painful crises, severe body pains, frequent hospitalisation, and blood transfusions. With all that, she is not willing to push her body any further. “Keeping a stable health status has not been easy. I have lived on borrowed blood. I am afraid things might get worse with pregnancy,” says Ms Makori who, for the last three months, has been bedridden and has undergone surgery to repair her hipbone.

Ms Makori has lost friends to the disease and she is still hoping and praying that she lives longer. “I am really avoiding anything that will put me into crisis,” she says.

Dr Valerie Magutu, a clinical pathologist with the Haematology and Transfusion Unit at the University of Nairobi, is asking the government to allocate more funds for the provision of blood.“Pregnant women with sickle cell disease require blood. At times, getting blood can be challenging. With good special care and sufficient blood, the women can get through their pregnancy safely,” she says.

“They just need to inform their care provider of their condition and alert them once they have decided to get pregnant so that they can walk the journey together. Several have made it. Pregnancy is not a death sentence to women with the condition, provided the care is given throughout,” Dr Magutu notes.

To better estimate pregnancy-related complications in women with sickle cell disease, Prof Eugene Oteng-Ntim, the senior study author and honorary senior lecturer at King’s College London and a team of researchers from the London School of Hygiene and Tropical Medicine examined 21 published observational studies, comparing outcomes in women with and without sickle cell disease.

The studies included 26,349 pregnant women with sickle cell disease and 26,151,746 pregnant women who shared attributes with the sickle cell population, such as ethnicity or location but were otherwise healthy. Investigators classified the sickle cell population based on genotype, or form of the disease, including 1,276 women with the classic form (HbSS genotype), 279 with a milder form (HbSC genotype), and 24,794 whose disease genotype was unreported.

Thirteen of the studies originated from high-income countries, and the remaining were from low- to median-income countries. Based on their analysis, researchers concluded that pregnant women with classic sickle cell disease living in a low-income country were at the highest risk for complications.

When researchers compared these women to healthy pregnant women, they observed that women with the most severe form of sickle cell disease in both high- and low-income countries were six times more likely to die during or shortly after pregnancy.

More work needed

Researchers also noted that women with severe sickle cell disease had an increased risk of high blood pressure during pregnancy (known as pre-eclampsia), stillbirth, preterm delivery, and delivering smaller-than-average infants.“Our report suggests that, even in developed countries with advanced care, there continues to be a much higher maternal mortality rate in women with sickle cell disease compared to the general population, strongly suggesting that more work must be done to improve outcomes for these patients and their families,” said Prof Oteng-Ntim.

“By improving care providers’ ability to more accurately predict adverse outcomes, this analysis is a first step toward improving universal care for all who suffer from this disease,” he added.

The study suggested that with early prenatal care and careful monitoring throughout the pregnancy, a woman with sickle cell disease can have a normal pregnancy and delivery. However, many women start their clinics very late. The study says: “There is a need to create awareness and educate the population on the importance of listening to their doctors on the risks to their health and that of their unborn baby while planning to start a family.

”Another study published last year by the America Society of Haematology said that 86 percent are likely to develop anaemia. A 10-year retrospective study was recently conducted at King Khalid University Hospital in Riyadh, Saudi Arabia. It involved 392 cases of sickle cell disease in pregnancy and 784 controls with normal haemoglobin phenotype. The women were selected and matched for age, parity, and delivery outcome. The main outcome measures were morbidity, maternal outcomes, and foetal outcomes.

"The major maternal complications in the sickle cell disease group were anaemia (86.2 percent), sickle cell crisis at 65 percent, foetal growth restriction and stillbirths accounted for 66 per cent of the perinatal mortality. Blood transfusion was indicated in 34 percent of pregnancies in the sickle cell group, preterm delivery at 15 percent and pre-eclampsia at 10 percent and bacterial infection at nine per cent.

“Pregnant women with sickle cell disease are at increased risk for pregnancy-related complications, as well as foetal morbidity and mortality. A critical need exists among hospitals for a multidisciplinary approach to the management of pregnancies complicated by sickle cell disease,” recommends the study.

Questions of sickle cell and pregnancy answered

What is sickle cell disease?

Sickle cell disease (SCD) is an inherited disorder. That means that it runs in families. It is pronounced when a child receives a sickle cell gene from each parent.

What complications can arise if I have sickle cell during pregnancy?

Most women with the sickle cell during pregnancy can have fairly normal deliveries. However, they are more at risk for several other complications during pregnancy. These can be short-lived (acute) or long-term (chronic).

Before a woman gets pregnant, it is always advisable that she has pre-pregnancy planning and close monitoring during pregnancy to reduce the risk of complications.

The biggest complication of SCD in a growing baby is anaemia. This is because the main purpose of red blood cells is to carry oxygen to the growing baby. Sickle cell anaemia can limit this. This can slow down the baby’s growth and even lead to premature delivery.

Other complications include:
· Acute chest pain
· Blood clots
· Eye damage (retinopathy)
· High blood pressure in the heart and lungs
· Joint damage (avascular necrosis)
· Kidney disease
· Stroke
· Anaemia
· Urinary tract infections
· Leg ulcers

How will sickle cell affect my pregnancy?

With good prenatal care and careful monitoring by your healthcare provider, you can have a healthy pregnancy. But having SCD means that your pregnancy will be treated as high-risk.

That doesn’t necessarily mean you’ll have problems, but the risk of complications is higher for you than for other women. Women with SCD need extra care during their pregnancy.

The baby also needs extra monitoring as the pregnancy progresses since they can be affected by pregnancy complications including:

· Preeclampsia – a condition that reduces the flow of blood through organs including the placenta, which may deprive the baby of oxygen and nutrients
· Foetal anaemia – where the baby’s red blood cell count drops before birth. Blood transfusions before and during pregnancy can make a baby’s blood incompatible with the mother’s, which can cause foetal anaemia
· Stillbirth – more common in pregnancies affected by SCD
· Preterm delivery – when a baby is born before 37 weeks. Sometimes this is necessary because it is safer for a baby to be born early.  But it can also happen for other reasons.

How is SCD handled during pregnancy?

For SCD warriors, it’s impossible for their bodies to keep up with the demand for red blood cells during pregnancy. As a result, having a blood transfusion during pregnancy is common.

About half of pregnant women with SCD have a transfusion during their pregnancy to either treat anaemia or another complication.

Pain is also one of the biggest worries with SCD, since some of the warriors have to change their medication because of their pregnancy. Pain crises happen to many women with SCD during pregnancy.

During pregnancy, most women have to stop taking the pain drug hydroxyurea and take different pain medications like acetaminophen, which is usually recommended instead. In some cases, narcotics can be prescribed for long-term pain relief during pregnancy.

With severe pain, doctors can recommend elective preterm labour. This is when labour is induced at 37 weeks. It can help prevent severe pregnancy complications later.

How can I reduce the complications associated with sickle cell in pregnancy?

To reduce the risk, you have to attend all of your prenatal appointments, even if you’re feeling well.

Other tips include:

· Drinking plenty of fluids to stay hydrated
· Avoiding triggers of acute pain episodes such as high altitudes, sudden temperature changes, dehydration, and intense exercise. Maintaining good habits can also keep you and your baby as healthy as possible
· Avoiding extreme temperatures and places with low pressure
· Getting lots of rest, getting vaccinations or vaccine boosters, and taking additional nutritional supplements, such as folic acid
· Making more frequent doctor’s visits
· After you give birth, your doctors will still monitor you for complications, especially if the baby was delivered via C-section.

Your doctors will determine when you can resume treatments that were paused. 
This decision may depend on whether you are breastfeeding.

Will my baby have sickle cell disease?

It is possible, but it is not a must that your baby will have SCD if you do. It depends on whether your baby’s biological father carries the gene for any of the types of abnormal haemoglobin that can lead to sickle cell disease.

If you have SCD and your partner does not, it is possible your child will have only one ‘S’ gene and therefore have sickle cell trait.

People with sickle cell trait are less likely to develop any symptoms of the disease and live a normal life. 

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