Sickle cell disease is the name for a group of inherited conditions that affect the red blood cells. The most serious type is called sickle cell anaemia. In sickle cell anaemia, the protein in red blood cells (haemoglobin) becomes distorted when oxygen leaves, which creates a red blood cell that is the shape of a crescent instead of the usual smooth, disk shape. This can then lead to cells sticking together and not moving through the blood vessels. These blockages can cause pain (sickle cell crisis).

Sickle cell disease affects 1 in every 2000 births in England. High-risk ethnic groups include people from African or African-Caribbean backgrounds.

Symptoms

• Sickle cell crisis, which is a painful episode that can last for days or weeks.
• Being more prone to serious infections
• Anaemia
• Tiredness
• Shortness of breath

Causes

Sickle cell is inherited; it is caused by a change in the genes that help produce haemoglobin. Both parents must have the gene for the child to inherit sickle cell disease.

• Drinking plenty of fluids and staying warm to reduce painful episodes.
• Exercise regularly.
• Eating food rich in folic acid and calcium.
• Using paracetamol or ibuprofen when treating crises from home.
• Daily antibiotics and having regular vaccinations.
• Regular blood transfusions can be used in cases where your symptoms are continuing or getting worse.
• Emergency blood transfusions in cases of severe anaemia.
• Medications such as hydroxycarbamide which can reduce sickling, preventing the development of a crisis.

Myth 1: Sickle cell trait is the same as sickle cell disease

This is untrue!

People who have sickle cell trait only have one gene of sickle cell disease. This means they are a carrier of sickle cell and don’t have symptoms. They can pass this gene onto their children who may develop sickle cell disease. Often, people with sickle cell disease can live a life without any symptoms. However, sometimes stress, dehydration or high altitude can trigger symptoms. 

Often, people with sickle cell disease can live a life without any symptoms. However, sometimes stress, dehydration or high altitude can trigger symptoms.

Myth 2: Sickle cell disease is always visible

This is not the case.

People with sickle cell disease often do not show signs of their illness even though they may often be in pain or dealing with chronic fatigue. It is usual for people who deal with ongoing pain and fatigue to be good at masking it, but just because you cannot see their symptoms visually does not mean that they are not real.

Drink plenty of water

If you have sickle cell diseases, especially sickle cell anaemia, being dehydrated can bring on a sickle cell crisis, as red blood cells begin sticking together.

Wear warm clothes and avoid extreme temperature changes

Both the cold and also extreme temperature changes (such as going into a cold swimming pool) can trigger a sickle cell crisis.

Use heat to soothe pain during crisis

Items such as heated blankets and other devices can really help when applied to the location of the pain.

Ensure you are up to date with your your vaccinations

Those with sickle cell disease are more vulnerable to infection, so it is extra important to take measures to protect yourself from potential infection, such as vaccination.

Ask for help

There are charities, helplines and support groups available for those who have sickle cell disease. It is important to reach out for support and information to better manage your condition and the symptoms that come along with it.

Sickle Cell Society

www.sicklecellsociety.org/resources/

Help Line: 0780 973 6089 (Available Monday-Wednesday)

National charity that supports and advocates for those with Sickle Cell Disease. They have information on a wide range of topics for all age groups, covering things such as treatments, breaking barriers and new research. They also run specific children activities.

Let’s Keep Talking

www.letstalksicklecell.co.uk

Website created to support you in working with your Healthcare Team. There is lots of information on here to empower you to manage your condition.

NHS Conditions Sickle Cell

www.nhs.uk/conditions/sickle-cell-disease/

NHS conditions pages contain information about the causes, symptoms and treatment of Sickle Cell disease. It also includes information about recovery from stroke and about the rehabilitation services you might be entitled to.

NICE (National Institute for Health and Care Excellence)

https://cks.nice.org.uk/topics/sickle-cell-disease/

Topic finder on Sickle Cell Disease.