Definition and causes
Seglcellesygdom is a hereditary disease, caused by a defective formation of the iltbindende substance hemoglobin in the red blood cells. Due. of the abnormal type of hemoglobin are given the red blood cells shaped like a crescent (usually the round). This form means that the red blood cells have problems to get through the smallest blood vessels (kapillærer). This creates a disturbance in blood circulation, resulting in a reduced oxygen supply to tissues. The red blood cells are destroyed, and this kind of hemolytic anemia called sickle cell disease.
Seglcellesygdom is widespread in Africa, the Middle East, Southeast Asia and among the afroamerikanske population. You can have the disease in various degrees. The light degrees of the disease is not as debilitating, and the disease at the same time provide protection against Malaria . In Denmark there is the disease almost exclusively of immigrants.
Symptoms of seglcellesygdom
- The first signs of the disease seen 3-4 months after birth as a hand-foot syndrome. This manifests itself as fever and fluid and soreness in the hands and feet.
- There is the general signs of anaemia and the development of pain in the long rørknogler, in the spine and chest. During pregnancy, and by infections, especially in the airways and mellemøret can these pain worse.
- The risk of developing blood clots is greater, and there are bad circulation particularly in underbenene, which manifests itself as skinnebenssår.
Precautions and diagnosis
The diagnosis can be made by taking a blood sample and look at the red blood cells under a microscope, where the characteristic seglcelleform will be clear.
As the red blood cells are quickly destroyed, you have to folinsyretilskud the determination of the disease.
To avoid infections, it may be beneficial to be vaccinated against bacteria, which provides respiratory.
Know Monday a family member who has the disease, and has family from Africa, the Middle East or Southeast Asia, it is a good idea to be examined by a doctor.
Consideration of Seglcellesygdom
There is, as mentioned varying degrees of the disease, but most are symptombehandlet with blood , Which replace the destroyed red blood cells. Do we have periods where the disease flaring up, we can deal with the injection of fluid in the bloodstream and pain killer.
Is the severity of the disease high, is the only treatment method to give a bone marrow .
The forecast is highly variable. Without opportunities for modern transfusionsbehandling is forecast significantly lower, and in particular many children in the affected areas die of the disease. If you have access to blood and modern medical treatments, improve the forecast significantly.
Prevention of seglcellesygdom
Screeningsundersøgelser of pregnant women is recommended if one or both parents are known to the disease.