In this article
- What is sickle cell disease?
- How do babies get sickle cell disease?
- How do I find out if I’m a carrier of sickle cell?
- How do I find out if my unborn baby has sickle cell?
- What are the symptoms of sickle cell disease?
- How is sickle cell disease treated?
- How can I care for my baby?
- Is there a cure?
- Where can I get help and support?
What is sickle cell disease?Sickle cell disease is a genetic blood disorder that’s passed down through families. Both parents need to carry the sickle cell gene for their baby to have it.
One baby in every 2,000 babies is born with sickle cell disease in the UK. It’s most common among people of Asian, African, Caribbean, Mediterranean, or Middle Eastern descent (Patient UK 2012b).
Sickle cell disease causes the body to sometimes make abnormal haemoglobin. Haemoglobin is an iron-rich protein in red blood cells, which carries oxygen to all parts of the body.
The haemoglobin in normal red blood cells makes them soft, round, and flexible, and able to move easily around the body.
At times when people with sickle cell disease produce abnormal haemoglobin, the red blood cells become hard, sticky, and sickle-shaped (like a crescent). These sickle cells can get stuck in the blood vessels, blocking the flow of blood and causing pain (NHS 2012, Patient UK 2012a).
When this blockage happens, it's known as a sickle cell crisis.
Sickle cells can get stuck in the spleen, too. The spleen is an organ that filters the blood for infections. This means people with sickle cell disease are more prone to illnesses. Sickle cells also die off more quickly than normal red blood cells, which can make your baby anaemic (NHS 2012 Patient UK 2012a). Anaemia is the medical term for having a reduced number of red blood cells.
How do babies get sickle cell disease?For your baby to have it, both parents must carry the abnormal haemoglobin gene responsible for sickle cell (Meremikwu 2009, NHS 2012, Patient UK 2012a). This means you may have sickle cell disease yourself, or it may mean you are just a carrier. If you’re a carrier of the sickle cell gene, you won’t show any signs or have any symptoms.
The diagram above shows that there is a one-in-four chance of a baby having sickle cell if both parents are carriers (NHS 2012b). But that does not mean that one in four of those parents’ babies will definitely have sickle cell.
The chance of having a boy or girl is about one in two. But we all know families that have all boys or all girls. The same thing happens with inherited conditions, so one family may have more than one baby with sickle cell, while another family may have none with the disease.
How do I find out if I’m a carrier of sickle cell?In England, all pregnant women are given a screening to find out if they are a carrier. If you live in an area of the UK where sickle cell is common, such as parts of London, you will automatically be offered a blood test in early pregnancy. However, if sickle cell is not very common where you live, you will initially be screened with a questionnaire (NHS 2012).
If you carry the sickle cell gene, a blood test will be offered to your baby's father, too (NHS 2012b).
How do I find out if my unborn baby has sickle cell?There are three ways of finding out if your unborn baby has sickle cell:
- Amniocentesis: Your doctor will test the amniotic fluid surrounding your baby when you’re between 15 weeks and 18 weeks pregnant.
- Chorionic villus sampling (CVS): Your doctor will take a small sample of the placenta for DNA testing when you’re between 10 weeks and 12 weeks pregnant.
- Fetal blood sampling: Your doctor will take a sample of blood from the umbilical cord. This can be done from 16 weeks of pregnancy. (NHS 2012b, OMIN 2013)
It’s up to you whether you have your baby tested during pregnancy. It can be a hard decision, because, unfortunately, these tests do carry some risk of miscarriage. Your doctor should clearly explain the risks and benefits of the test to help you make your choice.
All newborn babies are screened for sickle cell, so if your baby isn’t tested during pregnancy, it can be picked up after she’s born. Your midwife will prick your baby’s heel and take a tiny amount of blood to be tested for sickle cell and other conditions.
Finding out your baby has sickle cell disease can come as a shock. You may feel frightened and that you don't know what to expect. You may feel responsible for your baby's condition.
It's perfectly natural to have these feelings, but the reality is that neither you nor your baby's father is to blame. The important thing is that you'll have plenty of expert support to help you and your baby as she grows.
What are the symptoms of sickle cell disease?Sickle cell disease causes a range of symptoms, including:
Episodes of pain: This is the most common symptom of sickle cell (Meremikwu 2009, NHS 2012a). Your baby may experience sudden pain, also known as a sickle cell crisis. This happens when the sickle cells get stuck in the small veins, preventing normal blood flow. These episodes of pain can last from a few hours to a few weeks. The episodes may occur once a year or twice a year, or more than 10 times a year. Your baby may have:
- pain in her arms, legs, back, and stomach
- swelling in her hands and feet
- stiff, painful joints
- bone pain
- a fever (NHS 2012a, Maakaron 2013)
Anaemia: This means your baby doesn’t have enough red blood cells. It's because her sickle cells die early, before they can be replaced with new red blood cells. Your baby’s anaemia will probably be quite mild and won’t cause any problems (Patient UK 20012). Your baby may:
- become tired easily
- get short of breath quickly when she's active
- have a sudden irregular heartbeat (palpitations), particularly after a spell of activity
- look pale, with pale palms and lips (NHS 2012a, NHS 2012b)
Sickle cell disease can also cause:
Infections: These happen because your baby's sickle cells aren’t flexible enough to fit through her spleen. The spleen is an organ that filters the blood for infections. Your baby's body may be less able to fight off bacteria and viruses. Some infections can be serious, such as, pneumonia, meningitis, septicaemia and bone infections (Meremikwu 2009, NHS 2012a, Patient UK 2012).
Acute chest syndrome: This happens if your baby has an episode of pain in her lungs. It may also happen if she has lung infection, such as pneumonia (Maakaron 2013). The symptoms are the same for both conditions, so it’s difficult to tell without tests in hospital (NHS 2012b). Acute chest syndrome affects about 40 per cent of people with sickle cell and can be serious (Maakaron 2013). It can cause:
- a fever
- a cough
- chest pain
- shortness of breath
If you think your child has acute chest syndrome, take her to a GP or hospital straight away.
Your baby’s symptoms may begin to show when she’s around six months (NHS 2012b). She will need help to cope with these symptoms throughout her life. On occasion, she may need to go to hospital. Some symptoms can be severe or even life-threatening if they are not treated.
However, symptoms do tend to come and go. Most of the time your baby may feel fine. You should be able help your baby stay well at home with the help of your doctor.
How is sickle cell disease treated?Sickle cell disease is treated in a variety of ways. The right care can help to limit how often your baby feels unwell as she goes through life.
Your baby will be referred to a specialist sickle cell care team, which will probably be based in a hospital (NHS 2012a). The team may include a paediatrician, a blood specialist called a haematologist, and expert nurses.
This team will give your baby frequent check-ups as she grows. The team can help you with looking after your baby, and can show you how to care for her at home. With all this support around her, your baby should be able to have a fairly normal childhood.
If your baby has a mild to moderate episode of pain, you can:
- Give your baby infant paracetamol or ibuprofen. Your doctor may also recommend a stronger medicine, such as codeine, which he can give you on prescription (NHS 2012a, Patient UK 2012).
- Apply warmth to your baby’s painful areas. You can do this by massaging and rubbing your baby's skin. You can also use hot water bottles and deep-heat creams (NHS 2012b).
- Give your baby plenty of fluids (NHS 2012a, Patient UK 2012). This can be breastmilk, or if she is formula-fed, offer extra water, too. Older babies and toddlers can have diluted fruit juice. Extra fluids will help to clear the sickle cells in her blood vessels.
Other treatments may include:
- Daily antibiotics, probably penicillin, to control infections (NHS 2012a, OMIN 2013, Patient UK 2012). If your baby does develop an infection, take her to the doctor straight away for treatment.
- A daily folic acid supplement. This will help her to make extra red blood cells (NHS 2012a).
If your baby is very unwell, and needs frequent specialist care, you may find it helpful to live close to the hospital.
How can I care for my baby?Try not to do things that will trigger your baby's symptoms. Your sickle cell care team at the hospital will give you a plan to help you look after your baby. Here are some things that may be recommended:
- Giving your baby plenty to drink, particularly in hot weather. Dehydration encourages sickle red blood cells to form.
- Ensuring your baby has all the routine immunisations, as well as the flu and hepatitis B vaccines (NHS 2012b).
- If your baby has started solids, giving her foods rich in nutrients, such as fruit, vegetables, oily fish, and whole grains.
- Keeping your baby warm. Being cold can cause her red blood cells to start sickling, leading to a crisis (Meremikwu 2009, NHS 2012b, Patient UK 2008a).
- Making sure your baby grows up to be active. However, it is best if she does not over-exert herself. Sports that involve rests, such as sprints or hockey, may be better than sports such as long-distance running.
- Keeping your baby relaxed and calm. Getting stressed can lead to a crisis.
Is there a cure?In most cases, sickle cell disease can’t be cured. However, some people can have a bone marrow transplant from a suitable donor. This provides a lasting cure. Giving normal bone marrow to someone with sickle cell disease means they’ll produce healthy red blood cells. But even if a donor is found, it can be a complicated and risky procedure (NHS 2012b, Patient UK 2012).
Where can I get help and support?There are support groups available for people with the disease. For more information visit:
- Sickle Cell Society
- The Sickle Cell and Thalassaemia Centre
- NHS Sickle Cell and Thalassaemia Screening Programme
You can also share your experiences and find support in the BabyCentre community.
Last reviewed: August 2014
ReferencesOMIN. 2013. Sickle cell anaemia. Online Mendelian Inheritance in Man. [Accessed July 2014]
Maakaron JE. 2013. Sickle cell anaemia. eMedicine. emedicine.medscape.com [Accessed July 2014]
Meremikwu MM. 2009. Sickle cell disease. BMJ Clinical Evidence. Published online March 2009.
NHS. 2012. Sickle cell anaemia. NHS Choices, Health A-Z. www.nhs.uk [Accessed July 2014]
Patient UK. 2012a. Patient information: Sickle cell disease and sickle cell anaemia. www.patient.co.uk [Accessed July 2014]
Patient UK. 2012b. Sickle cell trait and sickle cell screening tests. www.patient.co.uk [Accessed July 2014]