Prevention and treatment of sickle cell disease

Sickle cell disease is a hereditary disorder.  However, individuals still have the ability to prevent passing on the disease to their children. 

Below we discuss how this can be done.

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Identify who is at risk of sickle cell

Knowing the risk factors of sickle cell disease can help you take preventative measures drastically reducing the risk of transmitting the disease to ones children.

Risks associated with this include:

The race factor

Races at a higher risk of getting sickle cell disease include:

  • Black Africans
  • Indians
  • Arabs
  • Greeks
  • Turks
  • Latin Americans

These groups of people are more likely to inherit the gene that can lead to sickle cell disease. 

The parent factor

Parents who carry the sickle cell gene each have a significant chance of having a child or children with the disease. While the possibility of having a child with sickle cell disease is only 25%, these things do not follow a set mathematical formula. That risk can affect all children born of such parents.

The malaria factor

Sickle cell disease distribution pattern appears to be the same as that of malaria. So areas where malaria infections are high, there is a higher incidence of the disorder.

There seems to be a relationship between the malaria gene and the abnormal haemoglobin gene seen in sickle cell. Interestingly, sickle cell disease appears to confer some immunity against malaria. People with the disease while they can get malaria, they are less likely to get the severe forms of malaria or die from malaria.

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Practical things that can be done

Knowing the factors that increase the risk of sickle cell disease can help you to take proactive measures to prevent the passing on of the defective genes. The best thing is to get tested and see whether you are a carrier of the sickle cell gene. This is particularly important for couples living together and considering raising children.

Newborns in high risk areas should also be tested at birth to determine whether they have sickle cell trait genes or they have sickle cell disease.

The commonest tests include

  • Red blood cells density tests
  • Sickling test
  • Antenatal sickle cell screening tests. This test is done before a child is born (while still in the womb)

A couple that finds that they are at risk of having children with sickle cell disease may want to visit a counsellor who may help them understand better the situation they are in and the options open to them. Where available, a genetics counsellor is better placed for such a task.

Screening and testing for sickle cell disease or trait is not only an important preventive measure, but is also vital in the early intervention where the tests prove the presence of sickle cell disease.

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Treatment of sickle cell disease

The early diagnosis and management of sickle cell disease cannot be overemphasised. This is important because it has a bearing on the type of life a person living with the condition will live. Early intervention helps in the prevention of sickle cell disease complications that can greatly impair the quality of life or even proof fatal at an early age. Without good control of the disease, life can be relatively short and difficult.

The management of sickle cell disease involves many measures and not just one.

They include the following:

Lifestyle Changes

Avoid things that could trigger a sickle cell disease crisis. These include:

  • Smoking
  • Heavy drinking
  • Exposure to extreme weather elements
  • Undue stress

A good diet can help you to maintain a healthy weight, a strong immunity, and a healthy haemoglobin level. A qualified nutritionist can help you to draw a meal plan to achieve this goal. Always remain well hydrated.

Supportive treatment

Treatment is usually directed at the symptoms or complications associated with sickle cell disease. The appropriate measures are taken to correct say, anaemia, infections, chronic wounds, liver disease or any other complication. Treatment may involve:

  • Medications such as folic acid, antibiotics, pain killers or some other drugs depending on the prevailing problem.
  • Blood transfusions
  • Oxygen administration when lungs have been affected.
  • Wound management
  • Bone and joint care
  • Eye care

Targeted treatment

While sickle cell disease has no cure, there are ways of reducing the severity of the condition and the risk of a crisis.

Hydroxyurea. The use of this drug can help to reduce the risk of going into a sickle cell crisis. It has however been associated with a lowered immunity that may increase your risk of getting infections. Other medications used include:

  • Hydrea
  • Vitamin E
  • Droxia
  • Glutamine

Natural management of sickle cell disease

The natural approach here is meant to help the body’s immune system to manage without the use of conventional medicine with their myriad side effects. At the very least the aim is to minimise the use of such medications. Natural products that have been tried include:

  • These help to reduce harmful metabolic wastes called free radicals. High free radical levels increase risk of sickle cell pain crisis.
  • Omega 3 fatty acids (fish oil)
  • Vitamin D
  • These are compounds found in berries. They are said to reduce the viscosity of blood in sickle cell disease
  • Suma herb extracts
  • Have regular but moderate exercises

In Nigeria Niprisan, is an herbal based medication that has been approved in the management of sickle cell disease.

Natural management of sickle cell disease

The natural approach here is meant to help the body’s immune system to manage without the use of conventional medicine with their myriad side effects. At the very least the aim is to minimise the use of such medications. Natural products that have been tried include:

  • These help to reduce harmful metabolic wastes called free radicals. High free radical levels increase risk of sickle cell pain crisis.
  • Omega 3 fatty acids (fish oil)
  • Vitamin D
  • These are compounds found in berries. They are said to reduce the viscosity of blood in sickle cell disease
  • Suma herb extracts
  • Have regular but moderate exercises

In Nigeria Niprisan, is an herbal based medication that has been approved in the management of sickle cell disease.

The future of sickle cell disease treatment

More novel treatment options are under research. They include:

  • Bone marrow transplant. Where it succeeds, it can lead to a markedly improved quality of life in a sickle cell patient. Finding a donor and the potentially serious complications associated with this procedure are limiting factors in its use.
  • Gene therapy. The idea here is to introduce a normal gene into the bone marrow, switch off the defective gene, or make the bone marrow produce a type of haemoglobin called foetal haemoglobin. This type of haemoglobin does not support formation of sickle cells
  • Nitric oxide use. This is thought to keep blood vessels more patent and hence reduces the risk of blood stagnation in sickle cell disease.
  • Foetal haemoglobin induction. Research is focusing on medications that can trigger the production of this type of haemoglobin.

In many countries in Africa, including Nigeria and Kenya, at one time or another there may be a clinical trial relevant to sickle cell disease going on. Sometimes volunteers are needed in these trials. A person with the disease may want to consider this and may benefit from the most recent and advanced treatment options currently available. Of course there are risks to consider.

References

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