The disorder affects the red blood cells which contain a special protein called haemoglobin (Hb for short). The function of haemoglobin is to carry oxygen from the lungs to all parts of the body.
People with Sickle Cell Anaemia have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA). When sickle haemoglobin gives up its oxygen to the tissues, it sticks together to form long rods inside the red blood cells making these cells rigid and sickle-shaped. Normal red blood cells can bend and flex easily.
Blocked blood vessels
Because of their shape, sickled red blood cells can’t squeeze through small blood vessels as easily as the almost donut-shaped normal cells. This can lead to these small blood vessels getting blocked which then stops the oxygen from getting through to where it is needed. This in turn can lead to severe pain and damage to organs.
Who Gets SCDs?
The different kinds of SCD and the different traits are found mainly in people whose families come from Africa, the Caribbean, the Eastern Mediterranean, Middle East and Asia.* In Britain SCD is most common in people of African and Caribbean descent (at least 1 in 10-40 have sickle cell trait and 1 in 60-200 have SCD). It is estimated there are over 6,000 adults and children with SCD in Britain at present. There are other inherited conditions that mainly affect other groups, e.g. Cystic Fibrosis in Europeans, and Tay-Sachs disease in Jewish people.
Thalassaemia is a group of inherited blood disorders where the part of the blood known as haemoglobin is abnormal.
The abnormality means that the affected red blood cells are unable to function normally, which leads to anaemia (a red blood cell deficiency).
Red blood cells
Red blood cells are very important because they contain a substance called haemoglobin, which carries oxygen from the lungs to the rest of the body.
Haemoglobin is produced in the bone marrow (a spongy material found inside larger bones) using the iron that the body gets from food.
In thalassaemia, haemoglobin production is abnormal, leading to anaemia and a reduced oxygen-carrying capacity. If your body doesn’t receive enough oxygen, you’ll feel tired, breathless, drowsy and faint.
If left untreated, the most serious types of thalassaemia can cause other complications, including organ damage, restricted growth, liver disease, heart failure and death.
Who is affected by thalassaemia?
In England, beta thalassaemia major (BTM) is thought to affect around 1,000 people, with an estimated 214,000 carriers.
It most commonly affects people of Cypriot, Indian, Pakistani, Bangladeshi and Chinese origin.
In the UK, 8 out of 10 babies born with BTM have parents of Indian, Pakistani or Bangladeshi ancestry.
Sickle Cell and Thalassaemia: A guide to school policy [pdf]
A new law (Section 100 of the Children and Families Act 2014) places a duty on schools and academies to make arrangements for supporting pupils with medical conditions. Research has shown that schools struggle to support young people with sickle cell, but the Sickle Cell Society can offer help and advice. Working with university researchers our advisors have overseen the development of a Guide to School Policy for Sickle Cell.
“Teachers are faced with many different possible medical conditions and it is not reasonable to expect them to remember details of all of them. At the same time young people with sickle cell dislike initiatives that draw attention to them as different from their peers,” said Professor Simon Dyson of De Montfort University, who led the team of researchers. “What was needed was a policy that supported the student with sickle cell but which operated in the background without overloading teachers with information”.
The Guide to School Policy for young people with sickle cell is based on examples of good practice and contains a template for drawing up an individual health care plan.
For more information go to www.sicklecellsociety.org There is a great video on the website about Sickle cell and how a young person feels about the condition.