As sickle cell anaemia can block the flow of oxygen to parts of your child’s body, it can cause serious complications. These are described below.
Stroke
A stroke is one of the most serious complications of sickle cell anaemia. It can happen when the sickle cells block the flow of oxygen to your child’s brain.
The main stroke symptoms can be remembered with the word FAST: Face-Arms-Speech-Time.
- Face: may have dropped on one side, the person may not be able to smile or their mouth or eye may have drooped
- Arms: the person with suspected stroke may not be able to lift one or both arms and keep them there because of arm weakness or numbness
- Speech: may be slurred or garbled, or the person may not be able to talk at all despite appearing awake
- Time: dial 999 immediately if you see any of these signs or symptoms
Some children with sickle cell anaemia may experience a transient ischaemic attack (TIA), or ‘mini-stroke’. A TIA occurs when the supply of blood to the brain is temporarily interrupted.
Symptoms of a TIA are similar to those of a stroke, and last from a few minutes to a few hours before disappearing completely. However, do not ignore a TIA because it is a serious warning there is a problem with the brain’s blood supply.
If your child has a TIA, contact your GP, local hospital or out-of-hours service immediately to arrange a specialist assessment.
Strokes can be treated by blood transfusions. Blood transfusions are not effective for treating strokes in people who do not have sickle cell anaemia because the causes are usually different, such as a blood clot or burst artery.
Once your child’s condition has stabilised, it is usually recommended they continue receiving regular blood transfusions to reduce the risk of further strokes.
Acute chest syndrome
Acute chest syndrome is a life-threatening lung condition. The exact cause is unknown, though it is thought an infection in the lungs, which are already damaged by sickle cell anaemia, can cause problems with air circulating there.
The symptoms of acute chest syndrome include:
- high temperature (fever) of 38C (100.4F) or above
- chest pain
- cough
- breathing difficulties
- more rapid breathing than normal
If you think your child has developed acute chest syndrome, dial 999 immediately and ask for an ambulance.
Acute chest syndrome requires emergency medical treatment with antibiotics, blood transfusions, oxygen and intravenous fluids (where liquids are pumped directly into your child’s veins to help rehydrate their body).
Splenic sequestration crisis
Splenic sequestration crisis (SCC) is a complication that can affect sickle cell anaemia patients between the age of six months and three years.
SSC occurs when the sickled red blood cells become trapped inside the spleen. This causes the spleen to swell and at the same time leads to a dangerous drop in the number of red blood cells in the rest of the body.
Symptoms of SSC include:
- unusually pale skin
- feeling very tired
- shortness of breath
- irritability
- rapid heartbeat (tachycardia)
- abdominal pain, usually in the left side of your child’s abdomen
- swelling of the spleen (you should be able to check for this by gently touching the upper left side of your child’s abdomen, just underneath their rib cage)
If you think your child has experienced SCC, immediately contact your GP or a member of their sickle cell care team for advice. If this is not possible, call your local out-of-hours service or NHS Direct (0845 46 47).
Some episodes of SSC are minor and resolve themselves without the need for treatment. However, more severe episodes will require a blood transfusion in hospital.
Vision problems
In cases of sickle cell anaemia, blood vessels that lead to the eyes can get blocked by sickled cells causing damage to the retina, known as retinopathy. The retina is a light-sensitive layer of cells that processes light coming into the eye and sends signals to the brain.
Symptoms of retinopathy can include:
- floaters (coloured spots that ‘float’ in your field of vision)
- blurred vision
- vision blocked by patches or streaks
- reduced night vision
- most seriously, sudden vision loss
If your child complains of any deterioration in their vision, contact your sickle cell care team for advice.
Retinopathy can be treated using laser surgery to repair damage to the blood vessels and retina. Your child should have eye examinations at least once a year.
Pulmonary hypertension
Pulmonary hypertension is high blood pressure in the blood vessels that carry blood from the heart to the lungs. This is a common complication in teenagers and adults with sickle cell anaemia affecting over half of them.
It is a serious medical condition as it results in damage to the right-hand side of the heart making the heart less efficient at pumping blood around the body and getting oxygen to the muscles. This leads to symptoms such as:
If it is not treated, pulmonary hypertension can cause heart failure, which is when the heart struggles to pump enough blood around the body. This can be fatal.
Because of this risk your care team may recommend regular tests to check the blood pressure inside these vessels. This can be done using a machine called an echocardiogram.
An echocardiogram is a type of ultrasound scan that can be used to see how well your heart is working. An ultrasound is a procedure that uses high-frequency sound waves to create an image of part of the inside of your body, such as your heart. This can be used to estimate the pressure in your pulmonary arteries.
Treatment options for people with sickle cell anaemia who develop pulmonary hypertension include hydroxycarbamide and regular blood transfusions. (These treatments are not used in people with pulmonary hypertension who do not have sickle cell anaemia).
In some case additional medications such as iloprost and sildenafil, designed to widen blood vessels and reduce pressure, can be tried.
Read more about the treatment of pulmonary hypertension.
Kidney problems
The kidneys are designed to filter waste products out of the blood. But due to the ‘stickiness’ of blood in people with sickle cell anaemia the kidneys can become damaged over time.
This can lead to a number of problems, such as:
And in the past around 1 in 3 adults with sickle cell anaemia experienced end stage kidney disease (also known as kidney failure) which is where the kidneys lose most or all of their functioning abilities. Recent advancements in treatment should improve these figures.
Treatment options for kidney failure include:
Depression
Living with a condition as unpredictable as sickle cell anaemia can cause anxiety and stress, often triggering depression.
Research has found around half of people living with sickle cell anaemia will experience depression at least once during their life.
It can be difficult to spot depression in children, particularly in teenagers, because they are often reluctant to talk about their feelings with their parents.
Signs your child may be depressed include:
- no longer showing interest or taking pleasure in activities they used to enjoy
- an inability to make or keep friends
- lack of energy
- problems sleeping or, alternatively, sleeping much more than usual
- changes in their diet, either eating much more or much less than usual,
- a sudden worsening of behaviour, such as getting into trouble at school or frequent rule breaking at home
- a general lack of enjoyment in their day-to-day life
If you are concerned your child may be depressed, encourage them to talk about their feelings in a calm, non-judgemental way while stressing additional help and support is available if needed.
Mild to moderate depression in children and young people is often treated with talking therapies such as cognitive behavioural therapy (CBT).
CBT is based on the principle that the way a person feels is partly dependent on the way that they think about things. It teaches children and young people to behave in ways that challenge negative thoughts, for example confronting feelings of hopelessness.
More severe depression is usually treated using a combination of talking therapies, such as CBT, and antidepressant medications, such as fluoxetine.
