Sickle cell anaemia is a genetic disease, which affects haemoglobin in the red blood cells. Haemoglobin is responsible for transporting oxygen around the body. Healthy red cells are flexible and look like oval biconcave disks (similar to a doughnut). When someone is suffering from sickle cell anaemia, their red blood cells are abnormal, rigid and in sickle-like shape.

Rigid blood cells affected by sickle cell disease
Blood cells affected by sickle cell anaemia

 

 

It affects oxygen transportation because the cells have more difficulty in moving along the vessels due to their abnormal shape. This can lead to other problems with health.

 

In England, around 250,000 people have a ‘sickle cell gene’ and it is usually diagnosed in infancy. Whenever two parents have that gene, there is a 25% chance that the child will have sickle cell anaemia, and will need to be referred to a specialist medical centre.

 

Unfortunately, this illness is life-threatening and complications can happen that may lead to severe infections, attacks of severe pain and stroke.

 

When you notice that your casualty starts to develop the following problems, you need to seek medical advice:

 

A child with sickle cell anaemia may require additional help and attention, especially with physical exercise (They may become tired very quickly, have problems with breathing, have low energy levels). It’s good practice and advisable to speak with the parents or carers and get as much information as possible with regards how to treat that child if they become unwell.

 

What is the mechanism of the illness?

Abnormal blood cells can block small blood vessels, which supplies the tissues with oxygen. Tissues becoming swollen and it can irritate nearby nerves, causing pain.

 

 

Are the symptoms different for adults and kids?

Yes, it may be different. Typical symptoms for children is painful swelling of the hands or feet. For adults, the pain can be allocated everywhere in the body (mostly in: pelvis, abdomen, ribs, spine, long bones, breastbone).

 

 

What is the pain like?

Patients describe the pain as a sharpshooting one, penetrating, burning, aching and throbbing. Can last for a couple of minutes or even several weeks (mostly 5 to 7 days).

 

 

How often will sickle cell crisis appear?

Children may have those symptoms several times a year (2 – 14).

 

 

Are there any triggers which can cause a sickle cell crisis?

Yes. Try to avoid: dehydration, stress, sudden changes in body temperature, shortage of oxygen (intensive physical exercises). All of those can cause a sickle cell crisis. Adult patients should also refrain from drinking alcohol, smoking cigarettes and taking recreational drugs like cannabis or cocaine.

 

 

Are there any other medical issues that I should be aware of?

Yes. A child with sickle cell anaemia is more vulnerable to various infections, like pneumonia, meningitis or osteomyelitis (bone infection). When taking care of a child with sickle cell anaemia, you should pay additional attention to avoid any infections.

Following vaccinations may also be recommended: annual flu vaccination, against meningitis and septicaemia.

 

Sickle Cell Anaemia is covered in our Paediatric First Aid courses in London here at Siren Training.

 

Siren Training offers a specialist First Aid course taught by registered firefighters and Emergency service staff.