Sickle Cell Disease: A Complete Guide to Living Well
Sickle cell disease is an inherited condition that affects red blood cells and can cause pain, swelling and tiredness. It's usually lifelong, but treatment and good everyday care can help manage it (NHS). This guide gives you the big picture and links to our detailed guides on each topic. You might also see it called sickle cell anaemia (or anemia), which is technically the most common type.
What is sickle cell disease?
Red blood cells carry oxygen around your body using a protein called haemoglobin. In sickle cell disease, a change in the gene for haemoglobin means red blood cells can become stiff and crescent (sickle) shaped. These cells break down early, causing anaemia, and they can get stuck in small blood vessels, blocking blood flow. That blockage causes pain and, over time, damage to organs (NHLBI).
It affects millions of people worldwide, with an estimated 7.7 million living with it in 2021 (WHO).
Read more: Is sickle cell curable, contagious or a disability?
How do you get it?
You inherit it. A child has sickle cell disease when they get a sickle gene from one parent and a sickle, C or beta-thalassaemia gene from the other. People with one sickle gene and one usual gene have sickle cell trait. They're carriers, not ill with the disease.
Read more: Sickle cell trait vs sickle cell disease · Genotype compatibility for marriage · Free genotype checker
Types of sickle cell disease
The main types are HbSS (sickle cell anaemia), HbSC, and HbS beta-thalassaemia, with rarer types such as HbSD and HbSE. They vary in how severe they tend to be.
Read more: Types of sickle cell disease explained
Symptoms
The main symptoms include pain in the bones, joints, muscles or chest, feeling weak and tired, swelling in the hands and feet, breathing problems, more frequent infections and stomach pain (NHS). Symptoms can start from around six months old and usually come and go.
Sickle cell crises
A crisis is a sudden episode of severe pain or other symptoms. It can last hours, days or weeks. Common triggers include dehydration, sudden changes in temperature and stress (NHS), as well as infection, overexertion and low oxygen.
Read more: Crisis triggers · How to write a crisis plan · Free crisis plan template · Being heard in A&E
- you think you're having a crisis and have severe pain, breathing difficulties or are being sick
- you have sickle cell disease and a high temperature
- you have an erection that is painful or does not go away
(NHS)
Treatment
You'll usually be cared for by a specialist haematology team. Treatments include (NHS):
- painkillers, with stronger options for severe pain
- antibiotics such as penicillin, to help prevent serious infections, especially in children
- vaccinations, including a yearly flu vaccine
- hydroxycarbamide (hydroxyurea), which can help reduce pain crises
- blood transfusions and red cell exchange
- stem cell transplants and gene therapy, which can cure sickle cell disease for some people
Read more: Gene therapy and cure: Casgevy, Lyfgenia and cost
Living well day to day
The NHS recommends drinking plenty of fluids, avoiding sudden temperature changes, keeping vaccinations up to date, gentle to moderate exercise and breathing exercises to relax, and not drinking alcohol or smoking (NHS).
Read more: Water intake · Cold weather · Hot weather · Diet · Exercise · Flying and travel · Mental health
Keeping track and working with your care team
Regular check-ups matter, because sickle cell disease raises the risk of complications such as stroke, eye problems and damage to the lungs, heart and kidneys (NHS). Keeping a record of your pain, crises and triggers makes those appointments far more useful.
Read more: How to track sickle cell pain · Free printable pain diary · Preparing for a haematology appointment · Best apps and tools
School, work and your rights
Sickle cell disease often counts as a disability in law, which gives you protection and the right to reasonable adjustments.
Read more: Sickle cell at school · Sickle cell at work · PIP and disability benefits
Support
You don't have to manage this alone. In the UK, the Sickle Cell Society offers information, support groups and advocacy. Your haematology team, GP and others living with sickle cell are all part of your support network.
This guide is general information, not medical advice. Always follow the plan agreed with your own care team. Read our medical disclaimer.
