Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person’s parents; the most common type is known as sickle cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain (“sickle cell crisis”), anemia, swelling in the hands and feet, bacterial infections and stroke. Long-term pain may develop as people get older. The average life expectancy in the developed world is 40 to 60 years. Sickle cell disease occurs when a person inherits two abnormal copies of the β-globin gene (HBB) that makes haemoglobin, one from each parent. This gene occurs in chromosome; several subtypes exist, depending on the exact mutation in each haemoglobin gene. An attack can be set off by temperature changes, stress, dehydration, and high altitude. A person with a single abnormal copy does not usually have symptoms and is said to have sickle cell trait. Such people are also referred to as carriers (Kumar, 2019).
Causes of Sickle Cell Disease
Sickle cell disease is a genetic condition that is present at birth. It is inherited when a child receives two sickle cell genes one from Father and mother, (William, 2016).
Types of sickle cell disease
There are several different types of sickle cell disease. According to Matthew (2013), the main ones include:
- Sickle Cell Anaemia (SS): When a child inherits one substitution beta globin genes (the sickle cell gene) from each parents, the child has Sickle Cell Anaemia (SS).
- Sickle Haemoglobin- C Disease (SC): Individuals with Sickle Haemoglobin-C Disease (SC) have a slightly different substitution in their beta globin genes that produces both haemoglobin C and haemoglobin S. Sickle Haemoglobin-C disease may cause similar symptoms as sickle cell anaemia but less anemia due to a higher blood count level.
- Sickle Beta-Plus Thalassemia: Individuals with Sickle Beta Thalassemia (SB) disease also contain substitutions in both beta globin genes. The severity of the disease varies according to the amount of normal beta globin produced. When no beta globin is produced, the symptoms are almost identical to sickle cell anaemia, with severe cases needing chronic blood transfusions.
- Sickle Haemoglobin-D Disease: haemoglobin D, which is a different substitution of the beta globin gene, has been found to interact with the sickle haemoglobin gene. Individuals with Sickle Haemoglobin-D disease (SD) have moderately severe anaemia and occasional pain episodes.
- Sickle Haemoglobin-O Disease: Haemoglobin O, another type of substitution in the beta globin gene, also interacts with sickle haemoglobin. Individuals with Sickle Haemoglobin- O disease (SO) can have symptoms of sickle cell anaemia. (Matthew, 2013).
Signs and Symptoms of Sickle Cell Disease
Signs of sickle cell disease usually begin in early childhood. The severity of symptoms can vary from person to person. Sickle cell disease may lead to various acute and chronic complications, several of which have a high mortality rate. (Matthew, 2013; Rock, 2016).
Early symptoms according to Rock (2016) may include:
- Jaundice, or a yellowing of the skin and the whiteness of the eyes.
- Pain and swelling in the hands and feet.
Further symptoms and complications according to Matthew (2013) may include:
- episodes of pain
- acute chest syndrome
- vision loss
- enlarged spleen
- leg ulcers
- deep vein thrombosis
- liver, heart, or kidney damage
- malnutrition (in young people)
- infertility (in males)
- Priapism, which refers to a prolonged and painful erection
- pulmonary hypertension, which is high blood pressure in the blood vessels that supply the lungs
- heart failure
- bone and joint damage, which occurs due to low blood supply
- a higher risk of infections, which may have severe symptoms
- Fever .
Diagnosis of Sickle Cell Disease
Sickle Cell Disease is diagnosed with a simple blood test, most often is found at birth during routine newborn screening tests at the hospital. In addition, SCD can be diagnosed before birth. Because children with SCD are at an increased risk of infection and other health problems, early diagnosis and treatment are important, (Garry, 2017).
Complications of Sickle Cell Disease
Garry (2017) stated all major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly. These complications may include the following:
- Increased infections
- Leg ulcers
- Bone damage
- Early gallstones
- Kidney damage and loss of body water in the urine
- Eye damage
- Multiple organ failure
Treatment of Sickle Cell Disease
Jadavji (2014) suggest that the following drugs may help reduce the risk of complications:
- Hydroxyurea (Hydrea): This helps ensure the supply of oxygen to the body. It is not safe to use during pregnancy. Research has not proven the benefits of this medication in children younger than 9 months of age.
- L-glutamine oral powder (Endari): This helps reduce the number of sickle cells. It is suitable from the age of 5 years.
- Voxelotor (Oxbryta): This helps boost levels of healthy haemoglobin. It is suitable from the age of 12 years.
- Crizanlizumab-tmca: This can help reduce pain by preventing blood cells from sticking to blood vessels. It is suitable from the age of 16 years
- Narcotic: this can helps to relief pain during sickle cell pain crisis.
- Folic acid supplement: It helps the bone marrow to new red blood cells.
Dietary Management of Sickle Cell Disease
Table 1: Food Requirement in Dietary management of Sickle Cell Disease
|Food group||Examples of Foods To Be Taken|
|Grain||These include whole grain breads, rice, pastas, cereals and crackers, etc. It is recommended that at least half of the grains that you eat should come from whole gains linked to various health benefits. Grains also contain folate which enhances the production of new red blood cells. Grains are also rich in vitamins B, magnesium and selenium, which can help strengthen the immune system.|
|Vegetable||Vegetables, particularly dark green leafy such as spinach, carrots, broccoli, tomato, green beans are packed full of vitamins and minerals such as fibre, folate, potassium, etc. It is recommended to add orange and dark green leafy veggies to diet. It will help protect against infections, heal wounds, keep eyes and skin healthy. It will also help maintain healthy blood pressure.|
|Fruits||It’s important to eat a variety of fruits, including apples, oranges, bananas, grapes, melon, berries, kiwi, plums, peach, etc. These fruits can provide similar vitamins, minerals and fibre as vegetables.|
|Milk||Milk contains calcium and vitamin D, which are essential for strong and healthy bones. Milk contains valuable nutrients, including protein and potassium, which can help improve heart health. Opt for low-fat milk, yogurt and cheese.|
|Meat and Beans||Foods such as lean chicken, pork, fish and beef, nuts, seeds, peas and beans are great sources of protein, zinc, iron and omega-3 fatty acids. For instance, the human body needs protein and zinc for energy and growth, iron to help carry oxygen in the blood. Similarly, omega 3 fatty acids found in certain fish, nuts and seeds can help reduce inflammation and decrease risk of chronic diseases, including heart disease.|
|Oils||Liquid vegetable oils such as olive, canola, corn oil, and nuts are rich in omega-3 fatty acids and vitamin E, which works as an antioxidant to help to prevent disease.|
|Water||It is ideal that water should be taken at least 8-10 glasses on a daily basis; to help prevent dehydration.|
|Exercise regularly, but not excessively.|
(Source: Zidane etal., 2020)
Sickle cell disease is a debilitating genetic disease but symptoms can be alleviated with early diagnoses and with general improvement of health status through health education, regular medical follow-up and which can also be prevented with pre-marital counseling.
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Jadavji, P.C.G. (2014). Treatment of sickle cell disease. Canadian Medical Association Journal, 132 (7), 814–5. PMC 1345873. PMID 3978504.
Kumar K. (2019). Sickle Cell Disease. National Heart, Lung, and Blood Institute. Archived from the original on 6 March 2019. Retrieved 8 March, 2019 from http://www.usa.gov
Matthew, B. (2013). Types of sickle cell disease. British Journal of Haematology, 160 (6), 754–65. PMID 23293942.
Miller, G. (2017). Psychological therapies for sickle cell disease and pain. The Cochrane Database of Systematic Reviews, (5), CD001916.
Rock J. (2016). Signs and Symptoms of Sickle Cell Disease. National Heart, Lung, and Blood Institute. Archived from the original on 4 March 2016. Retrieved 8 March, 2016, from http:P//www.nhlbi.nih.gov
Williams, S. (2016). Causes of Sickle Cell Disease. National Heart, Lung and Blood Institute. Archived from the original on 24 March 2016. Retrieved 8 May 2016. from http://www.nhlbi.nih.gov
Zidane , H., Fitterman, S., & Shekelle, R. (2020). Dietary treatment of sickle cell disease: (A clinical practice guideline from the American Academic of Dietetics). Retrieved from Website: www.cdrnet.org.