Sickle Cell Disease

What is Sickle Cell Disease?

• Sickle Cell Disease (SCD) is a serious blood condition.
• Sickle Cell Disease is passed on from birth-parents to their children, like eye and hair colour is passed on.
• There are three main types: Sickle cell anaemia, Haemoglobin Sickle Cell disease and Sickle Beta Thalassemia – (you can find out more from the Sickle Cell Society website or your Sickle Cell Team).
• Sickle Cell Disease is particularly common in people with an African or Caribbean family background and it is also found in people who originate from the Mediterranean, Asia and the Middle East.

How Sickle Cell Disease affects the body?

Day to day:

• Sickle Cell Disease can cause tiredness, pain, more likely to get illnesses such as colds and flu, and infections.
• It can make a person need to pass urine (wee) more often, and feel more cold than others.

More serious symptoms:

• High temperature/Fever
• Breathlessness
• Pain – can be anywhere in the body
• Headache
• Swelling (sudden swelling of tummy is a serious emergency)
• Anaemia – dizziness
• Jaundice – a yellow tinge to the eyes and/or skin
• Symptoms of Stroke – something going wrong inside the brain – facial weakness, arm weakness, slurred speech

What can cause these symptoms (triggers)?

• Illness and Infections
• Cold or damp weather
• Not drinking enough
• Strenuous exercise
• Not enough oxygen (such as high altitude – e.g. Ski trip or flights)
• Stress
• Exhausted / over tired
• Sudden changes in temperature
• Alcohol, caffeine, smoking and non-prescription drugs.
• General anaesthetics

Sickle Cell Crisis:

• The red blood cells carry oxygen around the body. In a Sickle Cell crisis they become a sickle shape (see picture below) and clog up blood vessels.
• This causes lack of blood and oxygen, to the part of the body supplied by those vessels.
• It can happen anywhere in the body, and can cause extreme pain and damage to those parts of the body.

What can help to avoid symptoms:

• Drink plenty through the day, and more in hot weather.
• Exercise – don’t overdo it – rest as needed.
• Dress for the weather, for example, in cold weather wrap up warm, wear hat, gloves, scarf.
• Go to the toilet as needed – do not ‘hold on’ as this can cause problems.
• Have a toilet pass at school.
• If gets wet hair or clothes, dry hair and change into dry clothes as soon as possible. Change of clothes to be kept to school.
• Pain relief to be taken as soon as pain starts, as advised by Sickle Cell Clinic.
• May go swimming if pool is heated. Keep towel at the poolside – get dry immediately on leaving the water, including hair, and dressed in appropriate warm clothing.
• School trips must be discussed and carefully planned with Parent, Child or Young Person and School.
• If going abroad, Sickle Cell Nurses to be informed (check with parents). They will supply travel letter, check list, last clinic letter – to be kept with the travel documents.
• May not be able to travel if have had a crisis within 2 weeks of travel date.

Getting help in an emergency:

• If any symptoms – ask for help straight away.
• To have pain medicine given by responsible adult.
• To have a drink – at room temperature.
• Keep warm.
• Rest
• If not getting better, get medical attention.
• In an emergency, DIAL 999 TO CALL AMBULANCE – Tell them this is a sickle cell crisis.

For further information, speak to your Sickle Cell Team, Doctor or School Nurse or visit Sickle Cell Society – Supporting People Affected by Sickle Cell Disorder www.sicklecellsociety.org