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 – UHBW Patient Information Leaflet
- NHS – Sickle Cell Disease
- Travel advice – UHBW Patient Information Leaflet
- Sickle Cell Society – A parent’s guide to sickle cell disease
- About our service at UHBW
Resources for children
These resources are written for children, produced by the Sickle Cell Society.
Treatments
Hydroxycarbamide is a tablet or liquid medication which is offered to most patients with HbSS/HbS Beta-thalassaemia.
- Hydroxycarbamide – UHBW Patient Information Leaflet
- How hydroxycarbamide works in sickle cell – YouTube video
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. Most patients would receive a simple blood transfusion, but others may require a red cell exchange (where the sickle blood cells are temporarily replaced with donor red cells which do not sickle).
- Blood transfusion
- NHSBT – Information for patients with sickle cell disease who may need a blood transfusion
- Sickle cell society – what is a red cell exchange
Stem cell transplant is a potentially curative treatment for sickle cell disorders. Patients with more severe complications and a sibling who is a matched donor may be eligible.
Painful crisis
Painful crisis is a 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 helps them to know how to manage their pain and home and gives advice for hospital teams on how to manage their pain if they attend the hospital for a painful crisis.
If pain cannot be managed at home with oral pain relief medications, you should bring your child to the emergency department where they can receive medical treatment and IV pain relief. Bristol Royal Hospital for Children has a dedicated pain team.
Infections
Children with sickle cell disorders are more susceptible to infections by certain bacteria called ‘encapsulated bacteria’ as their spleen does not work as it should. To prevent this, most people with sickle cell disorders will be recommended to take lifelong preventative antibiotics (called Penicillin V) and keep up to date with an enhanced vaccination schedule.
Sleep problems
Sickle cell disorders 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.
Priapism
Priapism is a problem which causes an unwanted and painful nonsexual erection of the penis. This happens because sickle cells block the normal blood flow from the penis, causing blood to become stuck. This may occur from early puberty but can occur at any age. 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.
Stroke
Stroke occurs in sickle cell disorders 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.
As part of your child’s annual review, they will have a Transcranial doppler scan (TCD) which is an ultrasound scan of the blood vessels in the head. These scans are undertaken on children aged 2-16years old. This scan measures a child’s stroke risk and identifies children for referral for exchange transfusion therapy and establishes if the child needs further surveillance scans such as an MRI scan to monitor stroke risk.
The Transcranial doppler screening is undertaken on Ocean Unit level 6 of Bristol Royal Hospital for Children during annual review clinic appointments. Additional TCD scans are undertaken as an outpatient in ward A225 in the Bristol Royal Infirmary (BRI).
If you think your child may be suffering from a stroke, call 999. Stroke is a medical emergency and urgent treatment is essential.
Enuresis
Children and young people with sickle cell disease are more likely to have problems with bedwetting. This is mainly because the kidneys of people with sickle cell disorders are less able to produce concentrated urine and instead make large amounts of dilute urine. This dilute urine fills up the bladder quickly, resulting in the child needing to pee during the night. In addition, some sickle cell children have a smaller bladder than normal which also means that their bladders fill quickly, and they are unable to get through the night with weeing.
- Bedwetting support for children
- Eric – Supporting children and families with bladder and bowel issues
Hydration
Hydration is extremely important in children and young people with sickle cell disease as good hydration allows the blood to flow more freely. Dehydration makes blood thicker and more ‘sticky’ and sickle cell vaso-occlusion (sickling) becomes more likely.
Dactylitis
Dactylitis is where painful swelling occurs into the feet or hands. It is caused by sickled cells blocking the circulation in the tiny blood vessels in the feet and hands. It is often the first sign on sickle cell disease in babies.
Medications
Folic acid is one of the building blocks of red cells. Most patients with sickle cell disease will be given folic acid to help them make more red cells to replace any damaged red cells and reduce the symptoms of anaemia.
Vitamin D
Vitamin D deficiency is common in children and young people with sickle cell disease. Vitamin D regulates calcium levels and supports bone health.
Penicillin V
Penicillin V helps prevent life-threatening infections. Children and young people with sickle cell disease have difficulty fighting infections, particularly pneumococcal infection and they can become seriously ill. Giving Penicillin V regularly protects and helps children and young people fight these infections. It does not weaken their immune system. It should be given every day even if you child seams well.