First trial of its kind for pregnant women with sickle cell disease

In the first trial of its kind in the UK, Guy's and St Thomas' has assessed the health benefits for pregnant women with sickle cell disease having regular blood transfusions.

Published in Blood Advances, the new study is helping improve treatments for women with this condition. It paves the way for future studies to show whether pregnant women with sickle cell disease would choose to have planned Serial Prophylactic Exchange Blood Transfusions and if regular transfusions can help them and their baby remain healthier.

Professor Eugene Oteng-Ntim, clinical director of women's services at Guy's and St Thomas' and study lead, said: "I'd like to thank all the women who took part in the study. You have shown that women with sickle cell disease are willing to get involved in studies while pregnant and to undergo planned regular exchange blood transfusions. You have given us confidence that a larger, more detailed clinical trial that would give us clearer answers about the impact of treatments during pregnancy is possible."

Sickle cell disease is a common inherited condition that causes anaemia, severe pain episodes, and long-term health issues like lung and kidney problems, strokes, and high blood pressure in the lungs. People with the condition produce unusual red blood cells, shaped like a crescent, that can cause problems. Around 110 women with sickle cell become pregnant each year.

While in the UK most children with sickle cell disease now live into adulthood and can start families, pregnancies for women with the condition are still high risk as there can be complications caused by sickle cell disease.

Sickle cell complications may increase due to the added pressures of pregnancy, resulting in more frequent hospital and critical care admissions.

Treatments that can control the disease, like hydroxycarbamide and others, are currently not recommended in pregnancy. In the UK, the standard care for pregnant women with sickle cell disease is to have blood transfusions if they become unwell due to the effects of the condition.

Serial Prophylactic Exchange Blood Transfusions are performed using a process called apheresis. A cannula (hollow needle) is placed in each arm, which allows the woman's blood to be passed through the apheresis machine, and for sickle cells to be removed. The machine returns plasma and its contents back to the woman and adds donated healthy red blood cells at the same time.

Professor Eugene Oteng-Ntim said:

As an NHS Trust that treats a large number of sickle cell patients, it's important to us that we provide the highest quality care to pregnant women and keep them and their baby as safe as possible. Through research studies in our maternity service and by offering research opportunities to pregnant women with sickle cell disease, together we can develop understanding and improve treatments for women with the condition.

"We hope that a larger study in the future will tell us whether regular planned blood transfusions improve the outcomes for the mother and their baby, by reducing complications such as preterm delivery and low birth weight."

One of the women who took part in the TAPS2 feasibility trial at Guy's and St Thomas' was Dr Ore-Ofe Ajeigbe who had regular planned exchange blood transfusions. She had blood tests before each blood transfusion as part of her involvement in the trial.

She said: "I would go into hospital for blood tests and 2 days later, I would go in for my transfusion, which took 2 hours each time. For the procedure I had to lie on a bed; in one arm my blood would be coming out and in the other arm the donor's blood enters my body at the same time. I had monthly appointments with the consultant at the hospital and in addition, the research team contacted me after each appointment."

Her final blood transfusion was in October 2022 and she gave birth to her healthy baby girl IseOluware in November 2022.

Ore-Ofe said:

I cannot thank the Guy's and St Thomas' team enough. They looked after me, and the consultant leading this trial cared about me and my family. He knew I had concerns and called me to reassure me about the transfusions, he really did go above and beyond for me. I never felt like I was a number or just another patient. I will never forget what they did for me.

John James, Sickle Cell Society Chief Executive, said: "We are pleased that the TAPS2 study has highlighted this critical area of women's health for those with sickle cell disorder. We extend our gratitude to Professor Eugene Oteng-Ntim and the team at Guy's and St Thomas' for conducting the study, and to all the pregnant women who participated. Their involvement will contribute to better understanding of safe treatment options for sickle cell during pregnancy and provide reassurance to others with the condition who are considering starting a family."

The National Institute for Health and Care Research Clinical Research Network South London supported the TAPS2 study by recruiting participants.