World Sickle Cell Day 2022

What is Sickle Cell Disease (SCD)

Sickle Cell Disease (SCD) is a group of inherited red blood cell disorders that are present at birth. It is inherited when a child receives two sickle cell genes—one from each parent.  Worldwide there are about 300,000 babies born with SCD every year. Furthermore, the majority in low-income countries will pass away before their fifth birthday.

Normally, healthy red blood cells are round and move through small blood vessels to carry oxygen to all parts of the body. The red blood cells in someone with SCD become hard and sticky and look like a C-shaped farm tool called a “sickle”. These sickle cells die early, which causes a constant shortage of red blood cells in the body. In addition, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such as infection, acute chest syndrome, and stroke. 

What are the risk factors? 

SCD is most prevalent amongst people whose ancestral lineage links to sub-Saharan Africa, Central, South America, India, and the Caribbean regions. In addition to the Middle East and Mediterranean regions. According to the Centers for Disease Control and Prevention (CDC), SCD affects up to 100,000 Americans. More specifically, in African Americans, the prevalence of SCD is 1 in 500, while the sickle cell trait is present in up to 1 in 13. Among Hispanic Americans, the prevalence is 1 in 36,000; while in White Americans the risk of being born with SCD is approximately 300 times less than African Americans.

Treatment for Sickle Cell disease

Treatment plans and options all include:

• Analgesics: Acetaminophen, acetaminophen-opioid combinations, and nonsteroidal anti-inflammatory medications (NSAIDs) are all appropriate for mild and temporary pain.
• Antibiotics: Infections cause a significant degree of SCD-induced mortality. Patients who have febrile episodes, even without other symptoms, should always be evaluated. Hence, the need for empiric broad-spectrum antibiotic coverage (e.g., ceftriaxone) should be considered. Meningitis, bacteremia, osteomyelitis, urinary tract infections, and acute chest syndrome require specific antibiotic regimens.
• Blood Transfusions: Transfusions may be episodic or regularly scheduled and of the simple or exchange type.

Nutritional Considerations 

People with sickle cell disease often experience episodes of fatigue, pain, and infections on a frequent basis. SCD is often associated with vitamin D deficiency, as well as a poor appetite. Both of these can lead to delayed growth and development in children. As a result, it may necessitate a higher intake of certain nutrients, such as calories and protein.

A diet emphasizing whole foods, such as fruits, vegetables, whole grains, and legumes will provide a greater amount of essential nutrients than what may be seen in a typical Western diet. In addition,  an appropriate supplementation (1-3 times the recommended intakes for most essential nutrients) can prevent deficiency and may decrease the likelihood of disease exacerbation. Here are some nutritional considerations that could be used to help a person with SCD live a better quality of life: 

1. High-calorie, nutrient-dense diet

The average energy intake of sickle cell patients is typically below the suggested allowance for calories during the quiescent, or inactive, phase of the disease, and it drops to roughly half the recommended levels during times of illness requiring hospitalization.  Therefore, children with SCD are at risk for impaired growth and significantly lower fat and fat-free mass. Having said that, obesity is also a risk, especially in female adolescents. A careful nutritional assessment and the possible addition of energy supplements are indicated.

2. Adequate fluid consumption to maintain hydration. 

Sickling of erythrocytes (looking like a crescent or “C Shape”) increases in SCD patients who exercise in the heat without consuming fluids, compared with those who maintain well-hydrated status.

3. Micronutrients

When testing the blood levels of individuals with SCD, several vitamins and minerals are often low. Especially the vitamin D, vitamin C, vitamin E, vitamin A and carotenoids, vitamin B6, magnesium, and zinc. These apparent deficiencies, if left untreated, can cause significant reductions in blood-oxidation statuses in the person, resulting in oxidative stress and possible vaso-occlusion-related acute chest syndrome.

Studies have shown that when a person with SCD supplements with vitamin A, C, E, and D, zinc, and magnesium or even treatment with a combination of high-dose antioxidants, there can be a reduction of irreversibly sickled cells. In addition, antioxidant plant phenols, such as flavonoids, may also reduce the oxidative stress in SCD. 

4. Omega-3 fatty acid supplements

The serum phospholipids of children with SCD contain a reduced percentage of both the alpha-linolenic acid and the long-chain omega-3 polyunsaturated fatty acids (eicosapentaenoic acid [EPA] and docosahexaenoic acid [DHA]) when compared with healthy controls. This is important to understand because these long-chain omega-3 fatty acids increase the fluidity of red blood cell membranes, which may prevent sickle cell crisis.

Tips for eating a balanced diet: 

• Drink plenty of non-caffeinated fluids each day; ask your doctor or registered dietitian/clinical nutrition specialist (CNS)  how much fluid you should be drinking. 
• Keep food with you when you are away from your home. This will help to ensure that you have a healthy snack with you at all times. 
• Try to have foods from at least 3-to 4 of the food groups each time that you eat; this helps you to eat a balanced variety of foods throughout the day. 
• Consult your registered dietitian (RD) or clinical nutrition specialist (CNS) for specific recommendations for weight loss or weight gain as appropriate. 

See this sample meal plan below for examples of how to choose balanced meals: 

• Breakfast: whole-wheat toast with peanut butter, banana, and glass of milk (or non-dairy milk)
• Lunch: tuna fish sandwich on whole-wheat bread with a mixed green salad and water
• Snack: low-fat Greek yogurt mixed w/ 1-2 tablespoons nuts and fruit, water
• Dinner: grilled salmon, sauteed vegetables, salad with olive oil and vinegar and water
• Snack: cheese and whole-grain crackers, water 

(Note: your RD or CNS can help make meal plans that are specific to your individual energy and nutrient needs) 

Remember

Good nutrition can help increase the chances of healthy growth in children with SCD and may reduce the risk of complications in both children and adults. It is important that every person with SCD meets with a team consisting of a hematologist, primary care physician, and a registered dietitian (RD) or clinical nutrition specialist (CNS) to come up with a personalized diet that best meets their metabolic and energy requirements. 

A personalized diet, in combination with treatment, can help people with SCD achieve optimal nutrition and immune status. Furthermore, they ultimately reduce their chances of getting sick, acquiring an infection, or even death by supporting a strong baseline level of nutritional and immune health.

Even though a personalized nutritional plan may help a person have a better quality of life due to limiting SCD symptoms, it is by no means a cure-all or “magic pill”.  SCD crises can be life-threatening. So, if you or someone you know is experiencing symptoms associated with a sickle cell crisis, call 911 and a healthcare professional immediately.