Sickle cell anaemia can cause a wide range of symptoms though not everyone with the condition will experience all the symptoms.
Sickle cell crisis (episodes of pain)
Episodes of pain during a sickle cell crisis are one of the most common and upsetting symptoms of the condition.
A sickle cell crisis (also known as a vaso-occlusive episode or VOE) is triggered when the abnormal blood cells block the small blood vessels that supply the body’s tissues.
This causes the cells in the affected tissue to be damaged, resulting in swelling (inflammation) which irritates nearby nerve endings.
During a sickle cell crisis, younger children may develop painful swelling in their hands or feet. This is often the first noticeable symptom.
As a child grows older, pain can affect any area of the body, although the most common areas are:
- sternum (breastbone)
- long bones in the legs and arms
People with sickle cell anaemia describe the pain as aching, throbbing, sharp and shooting, penetrating, or burning.
Symptoms can be severe and episodes can last from a few minutes to several weeks with an average duration of around five to seven days.
Children may experience from one or two episodes to 14 episodes a year .
A sickle cell crisis can often occur for no apparent reason, though there are certain triggers, such as:
- sudden changes in body temperature; either the result of an infection causing a high temperature or a change in the outside environment
- dehydration (a lack of water in the body)
- the body suddenly becoming short of oxygen as a result of exercise, sudden exertion or stress
In older children and adults with sickle cell anaemia, potential triggers include:
- illegal drugs such as cocaine and cannabis
It is important to try and learn what triggers set off an episode so they can be avoided.
Anaemia ( lack of red blood cells) is a common symptom of sickle cell anaemia. The abnormal cells have a shorter life-span and are not replaced quickly enough.
- fatigue (feeling tired all the time and having a general lack of energy)
- dyspnoea (shortness of breath)
- palpitations (irregular heartbeat)
Your child’s body is usually able to compensate for the lack of red blood cells by an increase in heartbeat, although symptoms of fatigue may persist.
This can make participating in physical activities, such as sports, more difficult.
A more serious type of anaemia, known as aplastic crisis, can occur if your child develops a type of common childhood infection called slapped cheek syndrome (also known as parvovirus B19).
The combination of infection and pre-existing anaemia can lead to a sharp and sudden drop in the number of red blood cells inside your child’s body.
Symptoms of an aplastic crisis include:
- very pale skin
- fever (high temperature) of 38C (100.4F) or above
- tachycardia (rapid heartbeat)
Vulnerability to infection
Children with sickle cell anaemia are more vulnerable to infection, particularly if under three years of age. This is because the abnormal blood cells can disrupt the normal functions of the spleen.
The spleen is an organ located in the abdomen (the lower left side of the body, behind the stomach). It plays an important role in filtering harmful bacteria and viruses from the blood.
Children with sickle cell anaemia are particularly vulnerable to three types of infection:
As younger children with sickle cell anaemia have an increased risk of developing an infection, it is often recommended that they take daily doses of antibiotics as a precaution.
Additional vaccinations such as the Pneumovax (which protects against some types of bacteria that can cause pneumonia) and the meningococcal vaccines (which protect against meningitis and septicaemia), as well as annual flu vaccinations, may be recommended.
Jaundice and gallstones
Jaundice (yellowing of the skin and the whites of the eyes) is a common symptom of sickle cell anaemia. Jaundice occurs because the rapid breakdown of abnormal red blood cells leads to a build-up of a waste product in the body called bilirubin.
The build-up of bilirubin often leads to the formation of small crystals called gallstones. Most gallstones do not cause symptoms but occasionally they can block the bile duct which drains bile from the liver into the bowel. This can trigger jaundice.
More commonly, the stone can get stuck in the gallbladder, causing it to become swollen leading to abdominal pain and nausea.
Avascular necrosis (AVN) is the medical term used to describe the loss of bone tissue due to a restriction of blood supply.
In children with sickle cell anaemia, AVN usually affects the hip. The first sign of this can be a limp or pain in the groin. AVN can lead to persistent hip pain that can last for several months. In serious cases, your child may be advised to avoid putting any weight on the affected leg, and may need to use crutches or a wheelchair.
Less commonly, AVN can affect the shoulder joint or elbow.
A leg ulcer is a painful, open sore that develops on the lower leg, usually close to the ankle. Leg ulcers often develop after a skin infection or injury, such as a cut or puncture wound.
Children with sickle cell anaemia have an increased risk of developing leg ulcers. This is because the lack of normal red blood cells means their leg does not receive the nutrients it needs to keep skin and surrounding tissue healthy.
Red blood cells provide the oxygen required for physical development. In sickle cell anaemia, it is likely that your child will experience delayed growth compared with other children.
Many children with sickle cell anaemia will also experience puberty at a later stage (around 13-14 years of age for girls and 13½-14½ years of age for boys).
The average age of puberty in children who are unaffected by sickle cell anaemia is 11 years of age for girls and 12 years of age for boys.
Priapism is the medical term that describes a persistent and painful erection of the penis. Episodes of priapism can sometimes last for several hours.
Priapism can affect teenage boys and men who have sickle cell anaemia because the abnormal blood cells can cause blood to become trapped in the penis.
Priapism does not usually develop as a result of sexual stimulation, and it can often occur for no apparent reason, usually during a sickle cell crisis.
Teenage boys may be reluctant to mention that they are experiencing priapism due to feelings of embarrassment.
If you have a teenage son with sickle cell anaemia, it is very important you emphasise the importance of seeking immediate treatment for priapism.
This is because episodes of priapism that last more than two hours can cause permanent damage to the penis, and may affect the ability to obtain an erection in the future.
When to seek urgent medical advice
Due to the risk of life-threatening conditions, it is important to look out for any signs or symptoms that your child’s health has suddenly worsened.
Signs and symptoms to look out for are:
- fever (high temperature) of 38C (100.4F) or above
- severe pain that develops during a sickle cell crisis that cannot be controlled using over-the-counter painkillers, such as paracetamol or ibuprofen
- breathing difficulties
- severe abdominal pain or swelling of the abdomen
- severe headache, stiff neck or dizziness
- changes in mental state, such as appearing confused or drowsy
- episodes of priapism (see above) that last longer than two hours
- seizures (fits)
If your child develops any of the above symptoms, immediately phone your GP for advice. If this is not possible, take your child to your local accident and emergency (A&E) department.
You can take them by car if they are well enough to travel. However, if this is not the case, dial 999 and ask for an ambulance.
It is very important when requesting an ambulance or visiting A&E that you inform all ambulance, medical and nursing staff that your child has sickle cell anaemia. This is so that they are aware of the potentially serious nature of your child’s condition.