Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi
Part 11 of 11 in Diagnosis and Management of Sickle Cell Disease
Sickle Cell Disease and Pregnancy: Managing a High-Risk Combination
November 24, 2024
Pregnancy in a woman with sickle cell disease is a genuinely high-risk combination, and both maternal and fetal morbidity and mortality are elevated. Every patient should be counselled on this risk before she decides to start a family, since a vaso-occlusive crisis occurring during labour or childbirth can be particularly difficult to manage.
Managing Treatment Through Pregnancy
Hydroxyurea cannot be given during the first three months of pregnancy, which can be challenging since it is often central to a patient's usual disease control. Instead, the priority through pregnancy is keeping the patient well hydrated and warm, and doing everything possible to prevent a vaso-occlusive crisis. In the second and third trimesters, hydroxyurea can be restarted if required, and blood transfusion can also be given if needed.
Why a Multidisciplinary Approach Is Essential
Sickle cell disease in pregnancy needs a genuinely multidisciplinary approach, with the pregnancy closely supervised by a gynaecology specialist working alongside the haematology team managing the underlying disease. Given the elevated risk to both mother and baby, this close, coordinated supervision through pregnancy and delivery is what allows the risk to be managed rather than simply accepted.
This article is based on a Jivo Masterclass session conducted by Dr. Divya Doval, Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi. The article has been summarised with the assistance of an AI tool from the original masterclass recording. Watch the full Masterclass recording
This guide is based on a live Jivo Masterclass: Dr. Divya Doval taught doctors across Africa on November 24, 2024.
FROM THE LIVE Q&A
Moderator
What is the overall cost of bone marrow transplant at your facility?
Dr. Divya Doval
If it's a matched sibling donor, an HLA-matched sibling, it is somewhere between 20,000 and 24,000 US dollars. If a matched sibling is not present and it is a half-match transplant, the price goes up to around 35,000.
Frequently Asked Questions
What is the role of urine alkalisation in the management of vaso-occlusive crisis?▼
Alkalinisation of the urine prevents acidosis and infections, and once the urine is alkaline, crystallisation is prevented as well. Overall, it helps with the pain.
What is the management of sickle cell disease in pregnancy?▼
Once a patient is pregnant, hydroxyurea cannot be given for the first three months, which can be challenging, so we make sure the patient is well hydrated and stays warm, and we try to prevent all sorts of crisis through the pregnancy. In the second and third trimester, one can start hydroxyurea if required, and transfusions can be done if needed. Morbidity and mortality are high, and every patient should be counselled before starting a family that the risks for both mother and baby are high, since a crisis during labour or childbirth can be quite difficult to manage.
What is the symptomatic treatment for chronic jaundice in sickle cell disease, especially in children who have never had a blood transfusion?▼
This jaundice happens because the red blood cells are breaking down. It is chronic, and these red cells will keep breaking, so a lot of patients will have a high bilirubin, but there isn't a specific treatment for the jaundice itself. We can reduce the underlying haemolysis by starting hydroxyurea, but the jaundice itself does not otherwise affect most patients. If there is chronic jaundice, we do need to find the cause by checking for hepatitis B and C and other infective causes, to make sure it isn't due to something else.
From an epidemiological point of view, what makes Africa more affected than other countries in the world?▼
I think the whole geographical distribution is just a genetic basis. A certain gene pool has that gene mutation, so depending on the geographical area, that population is more susceptible to carrying the mutation and having the disease.
What is the role of consanguineous marriage in sickle cell disease?▼
As I mentioned, the inheritance is autosomal recessive. In consanguineous marriages, where the two partners may be related in some way, the chances of both parents being carriers are quite high, since it may run in the family. When both parents are carriers, the chance of having a child with sickle cell disease is 25 percent, so consanguineous marriage increases the risk of autosomal recessive diseases like this one.
In This Series: Diagnosis and Management of Sickle Cell Disease
- 1.Diagnosis and Management of Sickle Cell Disease
- 2.Sickle Cell Disease Explained: Inheritance, Haemoglobin S and Why Cells Sickle
- 3.Diagnosing Sickle Cell Disease: From Newborn Screening to Confirmatory Testing
- 4.Pain Crisis and Acute Chest Syndrome: The Two Most Common Sickle Cell Emergencies
- 5.Stroke Risk in Sickle Cell Disease: Screening and Preventing a Devastating Complication
- 6.How Sickle Cell Disease Damages the Spleen, Liver, Kidneys and Eyes
- 7.Priapism, Infections and Aplastic Crisis in Sickle Cell Disease
- 8.Hydroxyurea and Newer Drug Therapies for Sickle Cell Disease
- 9.Blood Transfusion and Iron Overload Management in Sickle Cell Disease
- 10.Bone Marrow Transplant and Gene Therapy: Curing Sickle Cell Disease
- 11.Sickle Cell Disease and Pregnancy: Managing a High-Risk Combination