Understanding sickle cell anaemia treatments is important for people to stick with their treatments and manage them properly.
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It considers recent measures that have developed in gene and stem cell therapy - especially the former which look promising.
Now, it's important to say these measures, whether stem cell or gene therapy are recent developments available in the higher income countries.
Treatment Challenges for Sickle Cell Anaemia
In lower and middle-income countries, access to cure may still be distant, but at least there is some hope.
Nigeria has the world's highest burden for Sickle Cell Anaemia.
Presently, what we have available in countries like Nigeria is access to treatments that could provide relief of symptoms, treat/prevent anaemia and prevent crises.
Even people awaiting the successful completion of clinical trials for gene therapy in high-income countries require treatments to manage their health and control their symptoms.
Sickle Cell crises are severely painful attacks that can happen most commonly in the abdomen or long bones of the arms and legs.
They are caused by sickled red cells blocking the blood supply to organs.
This affects the flow of oxygen to those areas causing sudden pain in the affected areas.
If they occur in the abdomen, they cause severe pain and vomiting.
When the blockage of blood vessels happens in the lungs, in addition to severe chest pain, there is difficulty breathing.
The best way to manage crises is to prevent them when possible.
This includes avoiding conditions and activities that can provoke sickle crises and medication that reduces their occurrence.
Activities/Conditions that could provoke Sickle Cell crises
- Excessive exercise or exertion - such as very strenuous, prolonged exercise; or military drills.
- Any type of infection; even minor illnesses like viral infections.
- Dehydration: insufficient fluid makes the blood thick/sticky. When the red cells are sticky, this increases the chance of blocking the blood vessels and leading to crises.
Treatments to prevent infection
- A. People with SCA should receive vaccines for:
- Pneumonia (pneumococcal)
- Meningitis (meningococcal),
- Influenza or Flu (influenza and Haemophilus influenzae type b).
- B. Children typically take Penicillin by mouth from age 4 months until age 6 years
- This to prevent severe pneumococcal bacterial )infections occurring.
Treatments that Reduce Crisis
- A drug called Hydroxyurea (Hydroxycarbamide) increases the production of a form of haemoglobin found mainly in fetuses (Hb F).
- Hb F reduces the number of red blood cells becoming sickle-shaped. Therefore, it reduces the frequency of sickle cell crises and acute chest syndrome.
Preventing Severe Anaemia
People with SCA have sickle red cells.
Sickle red cells are fragile. Their abnormal shape and stickiness contribute to the blockage of blood vessels, which leads to crises and other complications like stroke.
The fragility of the cells means they do not live very long.
Compared to healthy red cells which naturally live for 90-120 days, sickle red cells only live for 10-20 days.
When a lot of red blood cells die quickly before they can be replaced by new ones, a condition known as haemolytic anaemia develops.
Hence this is the 'normal' state of the blood cells in a person with SS.
Depending on the volume of cells lost, they may be well, or very ill.
To reduce the loss of red blood cells, people with SCA take Folic acid.
Folic acid is a vitamin that helps the body make new red blood cells.
If their red blood cell levels are very low, they will require a blood transfusion to prevent the anaemia from getting worse and leading to serious illness.
However, despite these preventive measures, people with SCA can fall seriously ill.
It is important to note that illness can start from age 4-6 months when levels of the natural foetal haemoglobin, which prevents sickle red cells even in the person with SS genotype begin to drop.
Therfore, when a person with SCA falls ill, they may often require hospital treatment, sometimes as an emergency.
Additionally, they may also require:
- Fluids are given in the vein (intravenously) to treat dehydration
- Powerful pain-relieving drugs to treat painful crises
- They may require Oxygen therapy (as you recall that this is one of the direct causes leading to crises)
- Many times people with SCA are already anaemic, but illness further worsens their anaemia. They would, therefore, require a blood transfusion to prevent developing stroke, heart attack or lung damage.
- If the illness has been provoked by an infection like pneumonia or urine infection, this would be treated.
So these are the current treatments for problems associated with Sickle Cell Anaemia.
- Revolutionary Treatment for Sickle Cell Disease Shows Promise in Clinical Trial
- Encouraging Results Seen with Novel Approach to Gene Therapy for Sickle Cell Disease
- What are stem cells?
Editing By AskAwayHealth
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