Welcome to the Sickle Cell Association of Ontario
What is the sickle cell disease ?
Sickle cell disease is the overall name that is given to a specific group of inherited conditions that negatively impact the red blood cells in a human body. Of these conditions, the most serious and dangerous is known as anaemia. Whilst sickle cell disease has been reported in all nationalities, it is particularly prevalent in people of Asian, African, Caribbean, Eastern Mediterranean and Middle Eastern origins – particularly those who have Caribbean and African heritage.
Haemoglobin is the substance within our red blood cells that gives blood its colour and appearance. The purpose of haemoglobin is to move oxygen out of the lungs and transport it to the rest of the body. The narrow blood vessels known as capillaries are where the transportation of oxygen takes place, and in many people these are not any larger than the width of a red blood cell. In those who have regular haemoglobin A (HbA), the red blood cells remain round and flexible. This ensures that they have the required space they need in order to fit through the capillaries and do their job.
Those who suffer with sickle cell anaemia for example, instead have a specific type of haemoglobin known as Ha haemoglobin S (HbS) or Sickle haemoglobin, which differs greatly from typical haemoglobin. The name of sickle cell is given due to the unnaturally sickle tool shaped red blood cells that the disease produces. Not only do these distorted red blood cells die quicker than regular ones, but they are not as flexible and can easily become stuck in the capillaries, which normal shaped red blood cells have no issue with. When this occurs, that part of the body where the red blood cells can’t reach becomes starved of oxygen. The results can vary from mild discomfort to moderate, or even unbearable pain in a multitude of areas, and even the possibility of permanent damage to the tissue that was deprived of the oxygen.
Unfortunately, there is no current cure for Sickle cell disease, and thus it is considered a lifelong condition. However, there are long-term treatments that can be utilised in order to help manage much of the associated issues that come with the condition. Mainly these include painkillers (for the associated pain), and antibiotics (to help ward off any potential infections), as well as blood transfusions (for particular cases where the production of new blood cells via bone marrow breaks down). There have been some reported successes using a treatment drug known as Hydroxyurea, which has been noted to reduce a lot of the symptoms and crises associated with the condition, but does have a few known side effects. Folic acid supplements can also be used as it is known to help promote the production of red blood cells.
Anyone who has sickle cell must also be aware of the potential triggers that could exacerbate the issue. These include conditions that are damp and cold, excessive stress, smoking and drinking, and infections. Whilst it is difficult to avoid certain stressors to the condition, maintaining a healthy and active lifestyle with a good diet will help to combat much of the issues associated with the condition.
Helpful sickle cell disease resources
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