Sickle-cell anaemia - Treating sickle cell anaemia

If your child is diagnosed with sickle cell anaemia, they'll be referred to a care team in a specialist sickle cell centre.

Sickle cell centres are specialist units usually based in large hospitals. The staff at the centres have a high level of expertise in treating people with sickle cell anaemia.

A detailed plan (see below) outlining future medical care will be drawn up and you'll be given information and support to help you manage your child’s condition.

Read more about care plans and living with a long-term condition.

Your care team

Sickle cell anaemia is a complex disorder that requires a number of different healthcare professionals working together as a team. Members of your care team may include a:

paediatrician – a doctor who specialises in the treatment of children
haematologist – a doctor who specialises in treating blood disorders
clinical psychologist
pharmacist
physiotherapist
social worker
specialist nurse – who will usually act as the first point of contact between you and the rest of the team

Your child’s care plan

The objectives of the care plan are to:

try to prevent sickle cell crises (episodes of severe pain) occurring
provide adequate pain relief when a crisis does occur
reduce the risk of serious complications developing, such as infections and stroke
treat other associated symptoms of sickle cell anaemia, such as anaemia (a lack of red blood cells) or priapism (a persistent and painful erection)
plan for urgent assessment at hospital if care at home isn't providing adequate symptom relief

Preventing a sickle cell crisis

Following lifestyle advice, such as drinking plenty of fluids, can help reduce the risk of experiencing a sickle cell crisis.

Some children (and adults) will continue to experience episodes of pain despite their best preventative efforts. In such circumstances, a medication called hydroxycarbamide may be recommended.

Hydroxycarbamide

Hydroxycarbamide is usually recommended if a person with sickle cell anaemia has recurring episodes of a sickle cell crisis. This is usually more than six episodes in the past 12 months.

Hydroxycarbamide may also be recommended if:

testing shows that a person is at increased risk of having a stroke (see below)
a person has severe symptoms of anaemia that aren't responding to other treatments

Hydroxycarbamide was originally designed as a type of chemotherapy medication to treat cancer, but researchers found that it stimulated the production of foetal haemoglobin.

Foetal haemoglobin is found in unborn babies. It's gradually replaced by adult haemoglobin as a child gets older.

As foetal haemoglobin isn't affected by the sickle cell mutation, it's able to take over the role of adult haemoglobin to some extent. This helps reduce the risk of someone with the condition experiencing a sickle cell crisis.

Hydroxycarbamide has also proved effective in preventing acute chest syndrome (a life-threatening lung condition). Therefore, the medication may also be recommended if a person has had two or more episodes of acute chest syndrome.

Hydroxycarbamide is available in capsule form and people with sickle cell anaemia are usually required to take one tablet every day.

A short-term side effect of hydroxycarbamide is that it can lower the amount of other blood cells including:

white blood cells – these cells help fight infection; having a low number of them can make you more vulnerable to infection
platelets – these cells help the blood to thicken and form clots; having a low number of them can make you bleed and bruise more easily

If you're taking hydroxycarbamide, regular blood tests will usually be recommended to monitor your blood cells. If your blood cells drop below a certain level, the dosage may need to be adjusted.

Other side effects can include:

diarrhoea
constipation
feeling sick

As a precaution, the use of hydroxycarbamide isn't recommended during pregnancy. This is because high doses of the medication may increase the risks of the baby being born with birth defects.

There's no evidence that much lower doses pose a similar risk. However, as a precaution, sexually active men and women are advised to use a reliable method of contraception while taking hydroxycarbamide.

The use of hydroxycarbamide has also been linked to an increased risk of developing leukaemia (cancer of the white blood cells) in later life. However, this risk is thought to be extremely small.

In most cases the potential benefits of using hydroxycarbamide outweigh the risks, but you should discuss any concerns that you have with your child’s (or your) care team.

Pain management

Although sickle cell crises can be distressing, most episodes can be managed at home.

Over-the-counter painkillers, such as paracetamol, can be used to control symptoms of mild pain. Always follow the dosage instructions and ensure the recommended amount isn't exceeded.

If your child’s pain is more troublesome, your GP may prescribe stronger painkillers, which usually contain a combination of paracetamol and codeine.

Other ways of easing your child’s pain are described below.

Ensure that your child has plenty to drink. Fluids help thin the blood and clear out the sickle cells that are clogging the blood vessels.
Place your child in a warm bath. Make sure that the water isn't too hot and don't let it get too cold because changes in temperature could trigger another crisis.
Use a warm towel or a heated pad to gently massage the affected body part. Many pharmacists sell electrical pads that can be used for this purpose.
Use suitable distractions. For example, reading them a story, watching a film or playing their favourite computer game will help to take their mind off the pain.

If your child’s pain is severe, take them to your nearest accident and emergency (A&E) department. If they're too unwell to travel by car, dial 999 to request an ambulance.

Hospital treatment for a painful episode is usually only needed if the pain is uncontrolled or there’s no access to analgesia (pain relieving medication).

Once your child has been admitted to hospital, they may need to stay in for several days until their pain has been effectively controlled.

This can usually be achieved by trying a number of different pain relief medications and carrying out regular assessments to help determine how effective each medication has been.

The treatment should take into account the needs and preferences of the individual and, where possible, they or you should be given the opportunity to make informed decisions about their treatment and care.

Severe pain can be treated by giving your child injections of a strong opiate-based painkiller, such as morphine.

Some parents are concerned that regular use of opiate-based painkillers will cause their child to become addicted. Your specialist or staff at the treatment clinic will carefully regulate the dosage to minimise the chances of addiction.

People with sickle cell anaemia are no more likely to become addicted to opiates than anyone else.

Reducing the risk of infection

Due to the increased risk of developing serious infections, such as meningitis, it's recommended that children take daily doses of antibiotics, usually penicillin. If your child is allergic to penicillin, an alternative antibiotic, such as erythromycin, can be used.

Ideally, your child should take penicillin for the rest of their life or, at the very least, until they reach adulthood. The long-term use of penicillin won't pose any risks to your child’s health.

It's vitally important that your child’s vaccinations are up to date. As well as the routine childhood vaccinations, such as meningitis C and tetanus, your child will probably need some additional vaccinations, such as an annual flu vaccination and the hepatitis B vaccine.

As children with sickle cell anaemia often require blood transfusions, there's a small risk that they could develop blood-borne viruses, such as hepatitis B. The hepatitis B vaccination will prevent this.

Reducing the risk of a stroke

A stroke is one of the most serious complications of sickle cell anaemia. Reducing the risk of a stroke will be an important part of your child’s treatment.

Your child’s risk of a stroke can be assessed using a test known as a transcranial doppler (TCD) scan. The scan uses ultrasound to measure the blood flow through the brain. A fast blood flow could be caused by narrowed blood vessels that are clogged by abnormal red blood cells. This is a major risk factor for having a stroke.

If your child is diagnosed with sickle cell anaemia, it's recommended that they have an annual TCD scan from the age of three.

The results of the scan will show whether your child has a high, moderate, or low risk of having a stroke. If the results show a high or moderate risk, a further TCD scan will be carried out within two months.

If the results of the second scan still indicate a high stroke risk, regular blood transfusions to improve blood supply to the brain will usually be recommended.

Chelation therapy

Regular blood transfusions can help reduce your child’s risk of having a stroke by 90%. However, blood transfusions can also leave excess iron in the body.

This can cause a number of potentially dangerous complications, including liver damage, delayed physical and sexual development and heart disease.

As a result, people who have regular blood transfusions must also be treated to remove excess iron from their body. This treatment is known as chelation therapy.

Deferasirox is a medication used during chelation therapy. It works by locking onto or binding the iron molecules in the body before releasing them through urine or stools. Deferasirox is available in tablet form and most people need to take one tablet a day.

Your child will need regular blood and urine tests to measure the amount of iron in their body so that the effectiveness of chelation therapy can be assessed.

Treating associated symptoms

Anaemia

In most cases, if your child eats a healthy, balanced diet they should get enough nutrients to compensate for their lack of healthy red blood cells.

Dietary supplements, such as folic acid (which helps stimulate the production of red blood cells), are usually only required if your child has a restricted diet, such as a vegetarian or vegan diet.

Never give your child iron supplements without consulting your GP first.

The type of anaemia associated with sickle cell isn't due to an iron deficiency and increasing your child’s iron levels could be dangerous, particularly if they've been receiving blood transfusions.

Gallstones

If your child has pain due to gallstones, it's usually recommended they have their gallbladder surgically removed. This type of operation is known as a cholecystectomy.

Avascular necrosis

Avascular necrosis is the death of bone cells caused by an interruption to the blood supply.

Mild cases of avascular necrosis can be treated with painkillers to relieve pain, and a type of medication called bisphosphonates to prevent further loss of bone tissue.

More severe cases may require referral to an orthopaedic surgeon (a surgeon who specialises in treating bones and joints) because surgery may be needed to repair bone damage.

Leg ulcer

A leg ulcer can be treated by cleaning the ulcer with sterile (germ-free) water and dressing it with a bandage. Read more about treating leg ulcers.

Delayed growth

Most children with sickle cell anaemia should ‘catch up’ on growth and reach a normal height by early adulthood.

In more severe cases, zinc supplements may be recommended. Zinc is a metal that plays an important role in stimulating bone and muscle growth.

If your child doesn't show any physical signs of puberty by the time they've reached 14 or 15 years of age, they will be referred to an endocrinologist (a doctor who specialises in treating hormonal conditions). A short course of hormonal medication may be prescribed to help trigger puberty.

Read more about the treatment of delayed puberty.

Priapism

If your child develops priapism (a persistent and painful erection), the self care techniques listed below may help relieve the symptoms.

Make sure your child drinks plenty of fluids.
Encourage your child to urinate regularly.
Use over-the-counter painkillers to help relieve pain.
A warm shower or bath can help encourage blood circulation.

Take your child to your nearest accident and emergency (A&E) department if an episode of priapism lasts more than two hours.

Treatment for priapism includes medication to stimulate blood circulation or using a needle to drain blood from the penis.