Sickle cell disease/disorder and its relationship with nutrition have been under-recognized and underused in its management. Despite the many years of research put into determining the relationship between nutrition and sickle cell diseases/disorder, the overall outcome has shown a gross lack of awareness and knowledge of nutrition in SCD. I am writing this article as part of my advocacy for the awareness month of sickle cell disease. Based on my research and opinion, I would like to take time to explain the importance of nutrition in sickle cell patient.
PATHOPHYSIOLOGY OF SICKLE CELL DISEASE (SCD)
Sickle cell disease is an inherited genetic condition that involves defects in the shape and function of haemoglobin in the blood. This increases the likelihood of blockages in the blood vessels and disrupted blood flow, which can cause serious complications.
TYPES OF SCD
According to the CDC, there are various types of Sickle cell disease. They are;
- HbSS -: This is the most known form of SCD also known as sickle cell anaemia. People with this inherit two sickle cell gene from each parent. This is the most severe form of the disease.
- HbSC -: people with this inherit a sickle cell gene (S) from a parent and abnormal haemoglobin (C) from the other. This is a milder form of sickle cell disease.
- HbS beta+ thalassemia -: people with this inherit a sickle cell gene from a parent and a beta-thalassemia gene from the other. People with beta + gene have less severe symptoms.
- HbS beta zero thalassemias -: people with this inherit a sickle cell gene from a parent and a beta-thalassemia gene from the other. People with beta zero gene have more severe symptoms.
- HbS (D, E or O) -: These types of sickle cell disease are rarer and usually don’t have severe symptoms.
- HbAS -: people with this inherit a normal cell gene (A) from one parent and a sickle cell gene (S) from the other parent.
TREATMENT OPTIONS OF SCD
The sickle cell gene comes with a lot of complications such as acute chest syndrome, anaemia, pain, hand-foot syndrome, vision loss, leg ulcers, deep vein thrombosis and pain, etc.
Treatment is varied depending on the complications experienced;
- Pain medication like NSAIDs is used to treat pain.
- Hydroxyurea to prevent complications by preventing the blood cell from sickling.
- Folic acid to help to make new red blood cells
- Antibiotics to treat infections
- Vaccinations to prevent diseases that would be risky for those with sickle cell disease
- Blood transfusions to treat complications and help prevent stroke
- Bone marrow transplant for some patients with severe symptoms
NUTRITION IN THE MANAGEMENT OF SICKLE CELL DISEASE
NUTRITION AND DISEASE RELATED MALNUTRITION
Growing up as a kid with SCD (HbSS), I was very positive that healthy eating (nutrition) was a major factor in my recovery, even though I didn’t know the chemistry behind it.
In a study published on the 23 of August 2018 by BMC Nutrition, shows that children with SCD have a low intake of energy and micronutrients particularly calcium and the antioxidant nutrients, vitamin C and E. Age trend showed that meeting nutrient requirements declined with increase in age and children with the genotype SS had a lower tendency to meet recommended daily allowances for nutrients.
SCD patient has a higher energy and nutrient requirement than an average person. They have an even higher energy and nutrient requirement during pain crisis/episodes. There is a higher energy need in sickle cell patient because of the high amount of resting energy expenditure (REE).
Sickle cell patient during the crisis has been seen to have disease-related malnutrition like micronutrient deficiencies (zinc, iron, arginine, glutamine, calcium, vitamin C, vitamin D, vitamin B, folates etc) and protein-energy malnutrition evident by a lean body mass. These micronutrient deficiencies cause a significant depreciation in blood-antioxidant status in these patients, and the resulting oxidative stress may precipitate vaso-occlusion related acute chest syndrome.
DIET FOR SCD
Diet for children and adults with sickle cell disease should be able to meet up with their high energy and nutrient needs. A Mediterranean style diet alongside the diet micronutrient supplementation is needed to reach maximum dietary requirement.
Finally and most importantly, every sickle cell patient should always hydrated. as a kid and an adult, water has always been my companion. Drinking water promotes healthy blood flow and reduces the chance of our red blood cells sickling and sticking together. It keeps our blood cells supple so they can move more freely within our blood vessels. A sickle patient should drink at least 12 or more cups of water per day
NUTRITION AND SCD AWARENESS
People should feel empowered to take charge of their own health. It is important for people who have SCD to work with health care providers who understand the disease. Build a strong national partnership network with ties to community-based organizations with the potential to reach affected populations. SCD patients should ignore myths about the disease. People who have SCD can live long and productive lives. No stigma should be attached to having SCD or sickle cell trait. People with the sickle cell trait should take different action step that makes people understand their genetics even before dating. People with SCD and caretakers of SCD patient should advocate and create awareness of the diseases. NGO’s should create mechanisms for facilitating peer-to-peer education and counselling, Create educational products for print, DVD, podcast, and Web placement.