SICKLE CELL: A PUBLIC HEALTH ISSUE

By Kadie Emma Deen

Sickle cell disease is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells, which leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. Several health problems may develop, such as attacks of pain, anaemia, swelling of the hands and feet.


According to the National Institutes of Health, symptoms of sickle cell disease vary. In some people, they are mild, in others severe and requiring hospitalisation. The most common signs and symptoms are linked to anaemia. Anaemia is a condition in which blood has a lower than the usual number of red blood cells. People with anaemia do not have enough red blood cells, which deliver oxygen. As a result, they may feel tired or weak. Fatigue is one of the most common symptoms of sickle cell anaemia. Severe or long-lasting anaemia can damage the heart, brain, lungs, kidney, spleen, and other organs of the body. Very severe anaemia may even cause death.


Many people with sickle cell disease live with chronic pain, especially in their bones. However, sudden pain that can occur anywhere in the body is also a common symptom of sickle cell disease. This pain is called a "sickle cell crisis." Sickle cell crises often affect the bones, lungs, abdomen, and joints. Other symptoms of sickle cell disease include: episodes of pain, acute chest syndrome, stroke priapism, weak immune system, leg ulcers, vision problems {leading to blindness}, necrosis, jaundice, shortness of breath, headache, organ damage.


Early diagnosis of sickle cell disease is critical because many complications can be prevented with early diagnosis and treatment. Sickle cell disease and sickle cell trait can be diagnosed with a simple blood test; hence, the reason why I am pushing for the “know your genotype” campaign. If I were to have an audience with the president of Sierra Leone, I would suggest that the country embarks on this campaign to ensure the population are aware of their status to get the right help, because health is wealth and a healthy nation is a wealthy nation. It is all well and good to invest in people, but investing in people who are not healthy or have hidden health issues can have dire consequences in the future. A 1955 article in the West African Medical Journal by Rose and Suliman, and a 1995 article by Wurie et al. in the same journal note that the frequency of the trait is 20-25% based on limited survey, which represents 10-16 SS per 1,000 live births. This translates to 3160 to 4946 of new additions of sickle cell haemoglobinopathy to the population annually (Roberts et al., Ghana Medical Journal, Dec 2015). There-fore, it is imperative for the entire nation to take part in this “know your genotype” campaign to:


1 Identify how many people are living with the defected gene {sickle cell Trait}


2 Raise awareness nationwide


3 Educate and train health professionals on how to treat patients with the disease


The best way to fight sickle cell is to prevent it through knowing your genotype; hence a pilot-testing scheme using the sickle cell test kit is critical, this will give instant results of what type of sickle cell the person has, and anyone can use the equipment, as its ideal for use in rural communities.

According to research, cases of sickle cell are expected to increase by 30% worldwide by 2050 most of them in Africa but can be prevented if we take the necessary steps.


The goals of treating sickle cell disease are to prevent or relieve pain; prevent infections, organ damage, and strokes; treat anaemia, and control complications.


Some doctors and clinics specialise in treating people who have Sickle cell disease. Haematologists specialise in treating adults and children who have blood diseases and disorders.


Treating Pain

Mild pain is often treated with over-the-counter medicine and heating pads. Severe pain may need to be treated in a hospital. The usual treatments for acute (short-term) pain crisis are fluids and pain-controlling medicines. Liquids help prevent dehydration, a condition in which the body doesn't have enough fluids. Fluids are given either by mouth or through a vein.


Preventing Pain

Those with more severe sickle cell anaemia may benefit from the daily administration of a medicine called hydroxyurea. This medicine may help reduce the number of painful crisis. Hydroxyurea is used to prevent painful crisis, not to treat them when they occur.


Preventing Infection

Bacterial infections can be a significant complication of sickle cell disease, but often they can be prevented or treated. If a child who has sickle cell disease shows early signs of an infection, such as a fever, difficulty breathing, or localised bone pain, treatment should be given right away.


Preventing Complications

Complications from sickle cell disease can include gallstones, lung crisis (acute chest syndrome), pulmonary hypertension, and stroke, leg ulcers that do not heal, and eye damage. Blood transfusions are commonly used to treat worsening anaemia and sickle cell complications.

People with sickle cell disease should have regular check-ups to detect eye damage. Moreover, a simple ultrasound test of the head can identify children at high risk for strokes.


Recent Developments

Research on bone marrow transplants, gene therapy, and new medicines for sickle cell anaemia is ongoing. The hope is that these studies will provide better treatments for sickle cell disease.


Kadie is an international advocate raising awareness for sickle cell disorder. She is living with the condition and as a mother she is inspired to support kids with sickle cell as she knows what it feels like to have the condition.


She is dedicating her time to raise awareness in Sierra Leone and the Gambia. She has a registered charity in Sierra Leone and the UK. Her Goal is to encourage the nation of Sierra Leone and the Gambia to know their Genotype.


She also aims to improve the health and well-being of people with sickle cell by:


Raising awareness

Educating/advocating

Improve access to health care solutions

Empower women and youths


Having spent my life living with an inherited blood disorder (sickle cell) my faith, courage, determination, passion and hope in the midst of adversity keeps me going. The pain is invisible but the illness is real.


Kadie Emma Deen

www.sicklesmart.org


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