Thalassaemia and Sickle Cell Disorder
Research has recently been carried out by York, Loughborough and De Montfort Universities with the objective of allowing the uninterrupted education of children who suffer from Thalassaemia and Sickle Cell Disorder. The full guidance is available from www.sicklecelleducation.com and www.sicklecellanaemia.org.
What is Sickle Cell Disorder (SCD)?
Sickle cell disorder (SCD) is a collective name for a series of serious inherited chronic conditions that can affect all systems of the body. These sickle cell disorders are associated with episodes of severe pain called sickle cell painful crises. Many systems of the body can be affected, meaning that different key organs can be damaged and many different symptoms can occur in many different parts of the body.
What is Beta-Thalassaemia Major?
Beta-thalassaemia major is a serious inherited blood condition in which the red blood cells are nearly empty of haemoglobin, the key part of the blood that carries oxygen around the body. The first life-saving step of treatment involves children having blood transfusions every 3-4 weeks for the rest of their lives. This extra blood introduces extra iron into the body that the body cannot get rid of easily. The second step of treatment involves drugs that get rid of the excess iron.
Both these disorders are inherited and cannot, like a cold, be caught from another person. A person may be a carrier of either disease, in which case they have one normal and one affected gene. Such a person will normally be perfectly healthy and may not know they have the trait unless they have a blood test. If two carriers have children together there is a one in four chance that each child could have sickle-cell anaemia and a one in two chance that a child could be a carrier.
Certain factors have been identified as more likely to precipitate a painful sickle cell crisis. These include infections, colds and/or damp conditions, pollution, dehydration, strenuous exertion, stress, sudden changes in temperature, alcohol, caffeine, and smoking.
Young people with SCD need to be well hydrated to reduce the likelihood of becoming ill. This means that they need a ready supply of fresh drinking water available at all times and need to be able to access drinking water in class.
People with SCD produce large quantities of dilute urine and need to go to the toilet more often so will need frequent toilet breaks.
A child with SCD may experience severe anaemia. This may mean they feel tired, lethargic and unable to concentrate. It is possible that a sufferer will find everyday activities such as climbing stairs extremely tiring. Young people with betathalassaemia major are likely to be tired towards the end of their 4 week cycle of transfusions. It is important that teachers do not mistake serious medical symptoms of SCD or betathalassaemia major for laziness.
A child with SCD will need to avoid hard, physical exercise that could precipitate a sickle cell crisis. For children with SCD, cold or wet weather, or exposure of the skin to cooling wind, may all be a trigger to episodes of illness. Obligatory sports and gym sessions out of doors in cold and wet weather is a potent stimulant to crisis for some children. It is important to listen to the child and parent, and follow advice from their specialist medical teams about this.
Young people with SCD and thalassaemia may catch infections more easily. Safe storage and dispensing of any antibiotic drugs prescribed for a student with sickle cell disorder or thalassaemia is essential. Young people with SCD need to avoid activities that require outdoor work in cold or damp conditions as well as under-heated classrooms, especially mobile classrooms. They may need to wear a coat in class and should be allowed to stay inside at break times in cold or wet and windy weather.
A student with SCD or betathalassaemia major will have an individual health care plan, which should be reviewed yearly. As both SCD and beta-thalassaemia major have numerous possible complications affecting many systems of the body, it is important, where possible, to include a specialist sickle cell or thalassaemia nurse in drawing up this plan.