The Role of Nutrition in Sickle Cell Disease

Sickle cell anemia (SCA)—the autosomal recessive disease that leads to sickling of oxygen-carrying red blood cells—affects about 100,000 people in the U.S. per year. African Americans are especially at high risk, with SCA occurring in 1 in 365 births. An additional 1 in 13 Black or African American babies are born with sickle cell trait.

Strong evidence points to nutrition as an increasingly important factor in the propagation of SCA symptoms such as poor growth, joint pain, infection, and anemia as these symptoms often led to painful crises and recurrent hospitalizations.

The Case for a Balanced Diet

A sickle crisis occurs when sickle-shaped blood cells clump together and block small blood vessels that carry oxygen to vital organs. Therefore, nutritional interventions are aimed at promoting an immuno-healthy environment that limits sickling.

A balanced diet is also important in combatting hypermetabolism, a phenomenon where people with sickle cell anemia use nutrients more quickly than they replenish them.

A study in the Journal of Social, Behavioral, and Health Sciences explains that people with SCA are in a state where their body breaks down nutrients quicker than they build them up, resulting in higher than normal energy requirements.

In other words, patients with sickle cell anemia have greater than average requirements for both calories and micronutrients and therefore need to eat more to avoid being deficient in immune-boosting nutrients.

If you have sickle cell anemia your metabolism may be working on overdrive, and therefore undernourished, but you may be unaware of this until you develop symptoms. To help limit frequent sickle cell crisis you may need to eat more foods with high amounts of vitamins and essential minerals because your nutrients are consumed more quickly than the average person.

In SCA, nutrients are quickly diverted away from bodily processes that support growth and other essential functions to making more healthy red blood cells. As a result, some people with sickle cell need as much as three times the recommended amount of micro- and macronutrients of a person without sickle cell disease.

All in all, it suffices to say that people with SCA expend more energy on a cellular level than those who do not have the disease and should therefore eat a balanced diet that not only suits their health needs, but also provides energy to the mitochondria and other proteins in the body. 

Start Early

The energy intake for people with sickle cell anemia can be especially poor, especially during a sickle cell crisis. Because these crises frequently occur in children it is not uncommon to see hospitalized children present with poor growth, lean body mass, and reduced fat-free mass.

Therefore, parents and healthcare professionals need to create a diet that will meet the daily recommended dietary that best supports their child's growth and overall health needs. A diet high in fruits, vegetables, whole grains, and legumes usually provides enough vitamins and nutrients to support the health needs of people with sickle cell anemia.

Additional supplementation with a multivitamin, amino acids such as arginine, and folic acid can also help to prevent nutritional deficiencies and decrease the likelihood of disease exacerbation.

It’s important to keep in mind that hypermetabolism may play a role in your child’s increased nutritional needs so emphasizing proper nutrition as they grow is vital. To ensure that your child is getting all the nutrients they need, make sure their balanced diet meets the following criteria:

High-Calorie, Nutrient-Dense Diet

The average energy intake of sickle cell patients is typically below optimal values even when the disease is not symptomatic and it drops to roughly half the recommended levels during times of illness, especially when hospitalization is required.

As a result, children with SCA are at risk for impaired growth and significantly lower fat and fat-free mass, though obesity is also a risk, especially in female adolescents.

Nutritional Micro Supplementation

Standard nutritional assessment methods used to calculate energy needs typically underestimate resting energy expenditure in persons with SCA. A careful nutritional assessment and the possible addition of energy supplements are sometimes needed.

According to the journal Nutrition & Metabolism, a high protein diet can lessen the likelihood of infection. Moreover, the amino acid, arginine, has been shown to have specific benefits for SCA patients due to the effects of nitric oxide—one of its byproducts—which works to reduce pain and vaso-occlusive crisis (VOC) and increase muscle function.

Additional micronutrient supplements of note include zinc for improved growth and immunity, chromium for carbohydrate metabolism and energy, manganese and vitamin D for bone health as well as energy, magnesium for cellular hydration balance, and the B vitamins for their crucial roles as enzyme cofactors, neurotransmitter precursors, and amino acid metabolism.

Plant compounds or flavonoids such as Vitamins A, C, and E have also been shown to have some benefits, as these are the natural antioxidants that play a role in lessening inflammation and pain.

Fluids to Maintain Hydration

Sickling of red blood cells increases in SCA patients who exercise in the heat without consuming fluids, climb to high altitudes, or experience other physical stressors compared with those who maintain well-hydrated status. Therefore, it is very important that all people with sickle cell disease remain adequately hydrated.

Preventative Care

Reducing recurrent painful episodes and hospital visits is the primary focus of clinical care in SCA patients. Comprehensive and multidisciplinary care is essential. A balanced diet plus education about the disease are the pillars of prevention.

If you have sickle cell anemia you are at higher risk of acquiring an infection. Therefore, healthcare professionals encourage anyone with sickle cell anemia to get vaccines for the prevention of influenza, meningococcal disease, and pneumococcal disease.

Also, a diet that contains at least 1 milligram (mg) of folic acid daily and an iron-free multivitamin is recommended to ensure adequate uptake in nutrients.

SCA treatments such as hydroxyurea and oral antibiotics are also beneficial, but parents and caregivers should be aware of the side effects that are often experienced with taking these medications.

Still, combining the interventions of a proper diet, nutrient supplementation, medication, periodic blood transfusions, and creating a stress-free environment may limit or prevent SCA exacerbations.

Hematopoietic cell (bone marrow) transplantation is currently the only curative option for SCD patients, but the use of this therapy is still limited.

A Personalized Nutritional Plan 

It is important that every person with sickle cell disease meet with a team consisting of a hematologist, primary care physician, and nutritionist 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 sickle cell achieve optimal nutrition and immune status, ultimately reducing one's chances of getting sick, acquiring an infection, or even death morbidity by supporting a strong baseline level of nutritional and immune health.

Although a personalized nutritional plan may help to limit SCA symptoms, it is not a cure-all. Sickle cell crises can be life-threatening. If you or someone you know is experiencing symptoms associated with a sickle cell crisis, call 911 and a healthcare professional immediately.