Sickle cell disease (SCD) is an inherited condition affecting red blood cells, causing them to become sickle-shaped and fragile. This leads to hemolytic anemia and recurrent vaso-occlusion in the microvasculature due to increased red blood cell adhesion and retention. This article provides a comprehensive overview of dietary recommendations for individuals with SCD to help manage the condition and improve their quality of life.
Understanding Sickle Cell Disease
Sickle cell disease (SCD) is an autosomal recessive condition where red blood cells become sickle-shaped and fragile. This abnormal morphology is caused by a single change in the amino acid sequence of the β-globin chain, resulting in Hemoglobin S (HbS).
Individuals heterozygous for the HbS gene are carriers of the sickle cell trait and usually do not exhibit symptoms. However, if both parents are carriers or have another abnormal hemoglobin gene, their offspring could be affected. Other SCD variants include hemoglobin SC and hemoglobin Sβ-thalassemia, which may present with less severe symptoms than homozygous HbS.
Symptoms and Complications
Acute signs and symptoms of SCD can include:
- Pain in the hands and feet
- Fever
- Serious bacterial infections
- Priapism
- Chest pain
- Shortness of breath
- Fatigue
- Pallor
- Tachycardia
- Jaundice
- Urinary symptoms
Chronic complications may include:
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- Delayed growth/puberty
- Retinopathy
- Chronic lung and kidney disease
- Cardiovascular disease
- Avascular necrosis
- Bone infarcts
- Leg ulcers
Risk Factors and Diagnosis
SCD is most prevalent among individuals with ancestry linked to sub-Saharan Africa, Central and South America, the Caribbean, India, and the Middle East and Mediterranean regions. In the United States, it affects approximately 100,000 Americans, with a higher prevalence in Black Americans (1 in 500) and Hispanic Americans (1 in 36,000).
Prenatal screening is available through chorionic villous sampling, and universal newborn screening is performed in every US state using electrophoresis to detect hemoglobin F and hemoglobin S.
General Nutritional Considerations
Patients with sickle cell anemia typically have greater-than-average requirements for both calories and micronutrients. During sickle cell crises, energy intake can be especially poor. Children frequently hospitalized for SCD commonly show poor linear growth, lean body mass, and reduced fat-free mass. For reasons that are poorly understood, many patients are deficient in essential micronutrients. A diet emphasizing fruits, vegetables, whole grains, and legumes will provide a greater proportion of essential nutrients than a typical Western diet, and appropriate supplementation (1-3 times the recommended intakes for most essential nutrients) can prevent deficiency and may decrease the likelihood of disease exacerbation.
Good nutrition is very important to promote health and prevent complications from sickle cell disease.
High-Calorie, Nutrient-Dense Diet
The average energy intake of sickle cell patients is typically below the suggested allowance for calories during the quiescent phase of the disease, and it drops to roughly half the recommended levels during times of illness requiring hospitalization. As a result, children with SCD are at risk for impaired growth and significantly lower fat and fat-free mass, though obesity is also a risk, especially in female adolescents. Standard nutritional assessment methods used to calculate energy needs typically underestimate resting energy expenditure in persons with SCD. A careful nutritional assessment and the possible addition of energy supplements are indicated.
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Adequate Fluid Consumption
Maintaining hydration is critical for SCD patients. Sickling of erythrocytes increases in SCD patients who exercise in the heat without consuming fluids, compared with those who maintain well-hydrated status. The Centers for Disease Control and Prevention (CDC) recommend that people with SCD drink 8-10 glasses of water per day. Avoid drinks with caffeine (soda, coffee) and alcohol. These drinks make the kidneys pull more water into the urine and can cause dehydration. Other fluids may include: flavored water, milk, and fruit juice.
Specific Dietary Recommendations
Macronutrient Needs
- Protein: Protein is essential for growth, repair, and immune function. High protein diets (35% of energy from dietary protein) have shown to improve weight gain and reduce inflammatory proteins.
- Carbohydrates: Choose complex carbohydrates like whole grains, fruits, and vegetables over simple sugars to provide sustained energy. Grains include wheat, oat, rice, cornmeal, and barley. Foods made from grain include bread, tortillas, pasta, oatmeal, and breakfast cereals.
- Fats: Focus on healthy fats like omega-3 fatty acids found in fish oil, flaxseeds, and walnuts. These fats can improve red blood cell membrane fluidity and reduce inflammation.
Micronutrient Needs and Supplementation
Blood levels of several vitamins and minerals, including vitamin A and carotenoids, vitamin B6, vitamin C, vitamin E, magnesium, and zinc, are often low in individuals with SCD. These 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. Studies indicate supplementation of zinc, magnesium, and vitamins A, C, and E or treatment with a combination of high-dose antioxidants can reduce the percentage of irreversibly sickled cells.
- Folate: Folate is very important for healthy red blood cells. People with sickle cell disease break down red blood cells too quickly. They need more folate. Because folate is so important, most children with sickle cell disease will be given a 1 mg (1000 µg) folate supplement to take every day. Folate is also found in a variety of foods. The richest sources are dark green leafy vegetables, beans, and peas. Some rich sources of folate include leafy green vegetables, such as broccoli, spinach, and asparagus, certain fruits, such as papaya and strawberries, and peanuts. Foods fortified with folate may include cereals, rice, bread, pasta, flour, and cornmeal.
- Zinc: Zinc has been shown to decrease pain, infection, leg ulcers, and improve growth. It also may decrease the amount of time a person with sickle cell disease stays in the hospital with pain. Children with sickle cell disease may need more zinc than children without sickle cell disease. The richest sources of zinc are meat, fish, and seafood. Other sources of zinc in food include beans, eggs, legumes, dairy products, and whole grains. They may be a good source of zinc for young children. The amount of zinc your child needs will depend on their age: about 5 to 11 mg of zinc each day.
- Vitamin D: Vitamin D is important for bone health, immune function, and reducing pain in people with sickle cell disease. Vitamin D is found in food. It can also made by the body when the skin is exposed to the sun. People may have low levels of vitamin D in their blood if they have naturally dark skin, if they don’t spend a lot of time outside in the sun, or if they don't eat many foods that have vitamin D. The foods with the most amount of natural vitamin D are fish (sardines, salmon, swordfish), fish oils (cod liver oil), mushrooms, and egg yolks. The amount of vitamin D your child needs from the foods they eat will depend on how much they make in their skin. Many people with sickle cell disease may have vitamin D deficiency. It will be checked in your child’s blood often. Your doctor may give your child a vitamin D supplement to take if the amount of vitamin D in their blood is low.
- Vitamin E: Vitamins E is important for red blood cells and to reduce inflammation. The more red cells break down, the more the body needs vitamin E. Foods that have a lot of vitamin E include nuts, vegetable oils, and wheat germ. The amount of vitamin E your child needs will depend on their age: about 7 to 15 mg of vitamin E each day.
- Magnesium: Magnesium may be important for reducing painful episodes in patients with sickle cell disease. Magnesium is found in a wide variety of foods including seeds, nuts, dark green leafy vegetables, legumes, dairy, nuts, whole grains, and even chocolate. The amount of magnesium your child needs will depend on their age: about 300 to 400 mg of magnesium each day.
- Omega-3 Fatty Acids: The serum phospholipids of children with SCD contain reduced proportions of both the parent (alpha-linolenic acid) and the long-chain omega-3 polyunsaturated fatty acids (eicosapentanoic acid [EPA] and docosahexanoic acid [DHA]), compared with healthy controls. These long-chain omega-3 fatty acids increase the fluidity of red blood cell membranes, which may prevent sickle cell crisis.
Dietary Choices
A high calorie, nutrient-dense, balanced diet rich in fruits and vegetables and low in added sugars, saturated fat, and sodium may also benefit people with SCD. The table below offers examples of foods a person may include in a diet for SCD based on the DGA:
- Fruits: A variety of fruits, such as berries, apples, bananas, and oranges.
- Vegetables: Leafy greens, carrots, broccoli, and bell peppers. Vegetables can be cooked or eaten raw. They can be fresh, frozen, or canned. Fruit can be fresh, frozen, canned, or dried.
- Lean Proteins: Poultry, fish, beans, lentils, and tofu.
- Whole Grains: Brown rice, quinoa, whole-wheat bread, and oats.
- Dairy/Alternatives: Milk, yogurt, cheese, and fortified plant-based milk alternatives. Good choices include milk, yogurt, cheese, and milk-based desserts like pudding.
Foods to Limit
- Processed Foods: High in sodium, unhealthy fats, and added sugars.
- Sugary Drinks: Sodas, sweetened juices, and energy drinks.
- High-Fat Foods: Fried foods and excessive amounts of saturated and trans fats.
Addressing Specific Challenges
Weight Management
A person with sickle cell disease may need to gain weight. There are also supplements you can take if you are losing weight or aren’t able to gain weight by increasing calories on your own.
In 2016, 2 in 3 Americans were considered to be overweight or obese. As care improves and individuals with sickle cell disease live longer, the chance of becoming overweight also increases. Being overweight can increase your risk of having a stroke. Eating from a smaller plate will help you eat less. When serving meals at home, avoid eating buffet style with platters of food on the table. Put the correct serving size on your plate before sitting down to eat. Don’t eat from the package. Place a serving size in a separate container and put the package away before you eat. If you get hungry between meals, eat a healthy snack.
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Picky Eaters and Pica
Many young children with sickle cell disease are picky eaters, have a poor appetite, or limited interest in food. Almost every child has some food dislikes. A nutrition expert can provide suggestions for how to encourage a picky child to try new foods. Keep in mind, it is the parents’ responsibility to provide food choice at each meal. It is the child’s responsibility to decide what and how much to eat.
Some children may also have cravings for non-food items. Eating or craving non-food substances is called “pica.” Pica is common in young children with sickle cell disease. The most commonly eaten non-foods are paper, cardboard, fabric, dirt, foam, and baby powder. Eating small amounts of non-foods can be harmless. It will reduce appetite, however. And it can lead to more serious medical problems including tooth decay, constipation, intestinal blockage, and poor growth. There are many theories why children may want to eat these things. One reason is that they could have a nutrient deficiency. If your child tries to eat non-food items on a regular basis, do not be embarrassed, but do talk to your doctor. If they have a nutritional deficiency, it can be corrected. If your child has very limited food intake, your doctor might recommend meal enhancements.
Constipation
Water is the best drink for all children over 3 years of age. It is recommended that children limit drinking 100% fruit juice and other sugar-sweetened beverages to 1 cup or less per day. Juice drinks have lots of calories and sugar. Eating enough fiber is important to reduce constipation. It can also lower the chance you child will suffer from obesity or diabetes. Fiber is found in whole grain bread, nuts, fruit, and vegetables.
The Role of Nutritional Support
Good nutrition can help safeguard healthy growth in children with sickle cell disease and may reduce the risk of complications in both children and adults. A registered dietitian can advise the patient and family on how to meet macronutrient and micronutrient needs. Supplemental nutrients may be required and ordered by the physician.
Nutrition Consultation
Nutrition consultation for assessment, to advise patient regarding specific dietary recommendations, and to arrange follow-up as needed. Protein-calorie supplements, per nutrition consultant.
Anti-Inflammatory Foods
Every day pain can lead to inflammation in the body. Inflammation happens when your body releases chemicals in response to pain or injury. It is the bodies’ way of healing damaged tissue. However, every day inflammation can be harmful to the body. Eating a healthy diet is one of the best ways to reduce inflammation in the body. It can possibly decrease some of the pain your child feels. Including these foods in your child’s diet can be helpful. There are some foods that are best to eat in limited amounts. They are “once in a while” foods. They might contribute to more inflammation. You don’t have to completely cut these foods from your child’s diet, but it is best to limit them as much as possible.