Sickle Cell Disease (SCD) is now one of the most common serious genetic conditions in England. Approximately 14,000 people live with the disease in the UK, this is 1 in 4600 people. SCD is a complicated disease where the red blood cells (hemoglobin) turn into an atypical hemoglobin molecule called Hemoglobin S, this distorts the red blood cell and turns it into a sickle shape. Sickle shaped red blood cells do not deliver oxygen effectively causing long term health problems.
People who have SCD will normally be diagnosed from birth, but this does rely on the fact that they have been screened in the first few days of life. As they grow up if the disease is not well managed they are at risk of a Sickle Cell Crisis.
What is a Sickle Cell Crisis?
A Crisis is a serious medical emergency caused by the blood cells joining together to form a clot. Like any clot in the body it can travel through the network of blood vessels causing devastating consequences. Sickle Cells patients are 300% more likely than anyone else to have a stroke and are more at risk for heart attacks, pulmonary embolisms and lung injury.
Many factors can effect why a Crisis occurs in the first place such as heat, being outside in the cold, overexertion, stress, infection and illness.
How to recogonise a Crisis? Signs and Symptoms
- Fatigue
- Pain in a joint or soft tissue
- Difficulty in breathing
- Any Stroke or heart related symptoms
- Unconscious
What to do?
If you suspect a Sickle Cell Crisis in the person with SCD that you are caring for then call 999 immediately and state SCD. Treat them for the symptoms that they are presenting, do not move them and do not give them anything to eat or drink. The ambulance service treat this emergency as a high priority call with a 18 minute response time.
Although there is currently not a cure for Sickle Cell Disease the long term prognosis for SCD is that with good management and specialist care they can go onto lead a normal life, with a life expectancy of 40 years old.
