This section includes more information about sickle cell disorders, including links to information leaflets made for patients and families. You will also find information about treatment options and complications.

General information

Here you will find information about the condition and our general service information leaflets.

• What is sickle cell disorder
• NHS – sickle cell disease
• Travel advice (please note – we are happy to provide travel letters but require 2 weeks notice either via text, email or over the phone.)
• About our service at UHBW

Supportive Treatments

Hydroxycarbamide is a tablet which is offered to most patients with HbSS/HbS Beta-thalassaemia, and some patients with HbSC. This works by increasing the amount of fetal haemoglobin inside the body, which is a different type of haemoglobin that helps to protect against sickling. Hydroxycarbamide is offered to most patients over the age of 9 months as there is good evidence that it can help to reduce complications.

After starting hydroxycarbamide, patients need a blood test around 2 weeks after starting/on dose increase and then once every 3 months once the dose is stable. This is to ensure that we keep the blood counts within a safe range. The dose is increased gradually until we get to the right dose for the patient.

• Hydroxycarbamide
• How hydroxycarbamide works

Blood transfusions can be used to manage sickle cell disorders and can be given either as an emergency (if the person is very unwell) or routinely to prevent complications. This may be a simple blood transfusion or as a red cell exchange (where the sickle blood cells are temporarily replaced with donor red cells which do not sickle).

Blood transfusion is not offered to everyone with sickle cell disease as there are risks associated with this. The benefits of transfusion (such as treating or preventing severe complications) must outweigh the risks. The risks can include transfusion reactions including forming antibodies (the body’s immune response to donor blood). This is more likely to happen in people with sickle cell disorders for several different reasons. Transfusion-associated infections such as HIV and hepatitis are now very rare.

• Blood transfusion
• Sickle cell society – what is a red cell exchange

Curative Treatments

Stem cell transplant is a curative treatment for sickle cell disorders. Patients with more severe complications may be eligible.

Patients who may be appropriate for stem cell transplant would have their siblings (brother/sister) ‘typed’. This is to find out if they are a match. If they are a close match, the patient can be referred to the transplant team to discuss receiving a stem cell transplant from their sibling.

If the patient does not have a sibling who is a close match, they may still be eligible for a ‘half match’ (haploidentical, i.e. mother, father, brother, sister) transplant. This is only available through a clinical trial called the REDRESS trial at present.

A stem cell transplant can lead to some short term as well as long term complications. These are dependent on the type of transplant and will be discussed between the transplant team and the patient.

Patients who are referred for transplant require additional testing to ensure that they’re fit enough to go through with the procedure. They may require chemotherapy before receiving the donor stem cells. During this process their blood counts will drop while the donor cells settle into the bone marrow. This process can make the individual very prone to severe infection. The patient will need to remain in hospital around 3-4 weeks depending on blood count recovery.

• Anthony Nolan – sickle cell disease and stem cell transplant
• REDRESS trial

Gene therapy

For patients who do not have a matched sibling donor, gene therapy has been approved since February 2025. This is a potentially curative treatment that uses patient’s own stem cells, which are gene-edited to produce more fetal haemoglobin. This leads to a reduction in patient’s sickle cell percentage to a level of a sickle carrier. Gene therapy is available for all patients who have more than 2 painful crises per year, and patients who would be able to tolerate high dose chemotherapy.

NHS England » Revolutionary gene-editing therapy for sickle cell ‘offers hope of a cure’ for NHS patients

Painful crisis

Painful crisis is the most common complication of sickle cell disorders. This happens when red cells are triggered to turn into a sickle shape and cause blockages in small vessels. This causes acute (sudden onset and short-term) pain. This can happen due to stress on the body including cold weather, overexertion, dehydration or infection.

All patients should have a personalised pain plan, which is developed by their medical team. This gives them some advice on how to manage their pain and gives advice for hospital teams on how to manage their pain if they attend the hospital for a painful crisis. Many patients need to attend hospital for management of a painful crisis, particularly if they are not able to control their pain with the pain killers that they have at home.

If you are having a painful crisis, contact your local haematology team for advice. If you require emergency attention, depending on your location either contact your local haematology unit, attend the local emergency department or call 999.

You should go directly to the emergency department if any of the following apply:

• you have chest pain with shortness of breath
• you are pregnant (contact your midwife in the first instance)
• your pain does not feel like your usual sickle crisis
• you have weakness affecting your limbs
• Pain management – SDEC
• How to use incentive spirometer (this is offered to people admitted to hospital with a painful crisis to prevent lung complications)

Infections

People with sickle cell disorders are more susceptible to infections by certain bacteria called ‘encapsulated bacteria’ as their spleen does not work as it should. This can make you prone to infections such as pneumonia, meningitis and osteomyelitis (bone infection). To prevent this, most people with sickle cell disorders will be recommended to take lifelong preventative antibiotics (usually penicillin V) and keep up to date with an enhanced vaccination schedule including:

• 5 yearly pneumonia vaccine (pneumovax)
• Meningitis B and ACWY vaccination
• Yearly influenza vaccination
• Antibiotics

Sleep problems

Sickle cell disease can make you more prone to developing a sleep-related breathing problem called obstructive sleep apnoea. This can reduce the amount of oxygen your body receives overnight, which can lead to morning headaches, daytime sleepiness and can precipitate painful crisis and other health complications related to sickle cell disease if left undiagnosed.

Your doctor will usually arrange for an overnight blood oxygen level test if you experience snoring or other symptoms suggestive of sleep apnoea.

• CDC – checklist for sleep and breathing related problems

Eyes

Sickle cell disorders can affect the eyes by blocking the blood supply to the small vessels at the back of the eye (called the retina). This causes a chronic (long-term) condition called sickle retinopathy. Individuals with HbSC are more prone to eye complications.

People with sickle cell disorders should have their eyes checked by an ophthalmologist at least once per year to screen for this condition.

• RNIB sickle retinopathy information
• Sickle eye project

Pregnancy

Patients with sickle cell disorders should let their haematology team know if they are planning a pregnancy. This is important as the team can review their medications, medical history and blood results to ensure that their health is optimised going into the pregnancy.

The team may also suggest a referral to a genetic counsellor depending on whether the partner carries a sickle gene or another unusual haemoglobin gene. There are several options available when planning a pregnancy, and more options can be given to you if you seek advice before you or your partner become pregnant.

• Information for those at risk of having a baby with sickle cell disease
• Patient information – Sickle cell disease and pregnancy
• South West Maternal Medicine Network – pregnancy advice for Parents & Families
• Sickle cell carriers and pregnancy

Priapism

Priapism is a problem which causes an unwanted and painful erection of the penis and can occur typically from the onset of puberty. This happens because sickle cells block the normal blood flow from the penis, causing blood to become stuck. This may need to be treated in hospital if self-help strategies don’t work within two hours (see the leaflet below). Some people who suffer priapism require long term medications to prevent this problem from coming back.

If your priapism episode does not settle within 2 hours, you should attend the emergency department straight away. (Bristol patients should attend Southmead rather than the BHOC as the urology team are based in Southmead)

• Priapism in adults
• Sickle cell society – priapism

Stroke

Stroke occurs in sickle cell disease when sickled cells block the small vessels supplying blood to the brain. This can cause problems such as difficulty speaking, weakness in an arm or a leg and/or drooping of the face as well as many other possible symptoms affecting the brain.

If you think you may be suffering from a stroke, call 999. Stroke is a medical emergency and urgent treatment is essential.

• Stroke association – stroke in sickle cell disease
• Sickle cell & Young Stroke Survivors

If you feel that any information is missing from this page, please submit this via the ‘contact us’ page and we will endeavour to include your suggested additions in our next update.