Good nutrition is very important for individuals with sickle cell disease. Vitamin deficiency and diet have a profound effect on the outcomes and experiences of the disease. In a recent study that followed 60 people with sickle cell disease for nine years, the authors reported that over 80% of the complications of sickle cell disease could be related to diet alone. In this study, there was no difference between the income and social status of the three groups being studied: the control group, the vitamin-sufficient sickle cell group, and the vitamin-deficient sickle cell group. Diet was the only difference between the two sickle cell groups. Those who made poor nutritional choices had a much more severe course of disease over the nine years. The average age of the participants was 26 years and even though all of the nutritional deficiencies were corrected, and related diseases were treated, the outcome was still poor for those who had a poor diet. As such, it is important and never too late to change your diet and your lifestyle.
Vitamin deficiency is common in people who have sickle cell disease. The goal is to reach a normal level of particular vitamins through a nutritious, balanced diet. In doing so, you may not need to take vitamin supplements. However, taking a regular dietary supplement (without over-consuming supplements alone) may be an option if you are vitamin deficient and cannot reach adequate levels through diet alone. If you are taking dietary supplements or thinking about taking supplements of any kind, let your primary care physician know. Some supplements interact with prescribed medications. There have been no reports of how much of any vitamin should be taken by people who have sickle cell disease even if they have a deficiency.
Zinc is one of the most important dietary supplements for men and individuals with sickle cell disease generally. Zinc is a mineral that plays a role in puberty, growth, and development in men. Zinc deficiency is common in sickle cell disease, and in men, causes low levels of testosterone (the male sex hormone) and can have an effect on pain. Besides effects on growth and development, zinc is an important vitamin that supports good immunity.
Zinc can be taken as a supplement and is found in chickpeas, lentils, peanuts, and all types of beans and tree nuts especially cashews. Taking too much zinc can decrease copper which can lead to anemia and muscle weakness. Balance is key when taking zinc as a supplement.
Vitamin D primarily affects bone strength and development. For those with sickle cell disease, vitamin D deficiency is associated with asthma, other breathing problems, and pain, which may be increased in people who are vitamin D deficient. Vitamin D deficiency is common in sickle cell disease with some studies reporting up to 65% of adults who have sickle cell disease having vitamin D deficiency. Some studies recommended that all people who have sickle cell disease be supplemented with vitamin D.
Vitamin D can be supplemented with a vitamin or foods that contain vitamin D like salmon and tuna. Milk is fortified with vitamin D.
The B vitamins
B vitamins are often known for their role in producing new red blood cells for those with sickle cell disease. Recent reports show that B vitamins are also important to maintain the health of blood vessels (veins, arteries, and capillaries) in sickle cell disease. These vitamins, particularly Folate, B12, and B6, may decrease inflammation and blood clotting in sickle cell disease by reducing the effect of an amino acid called homocysteine, which is involved in vascular disease. In the general population, B vitamin deficiencies have been associated with cardiac disease and cerebrovascular disease (a disease that affects blood flow and the blood vessels in the brain).
B vitamins and folate are found in spinach and other green vegetables, milk, fish, and in the same types of foods that contain zinc.
A Nutritious Diet
Vitamins are only one part of nutrition. An overall nutritious diet will give you the best defense against development and male-related problems. Cooking at home rather than eating out increases the nutritional value of foods. Using less salt and eating fresh vegetables and fruit every day is part of a nutritious diet. Protein in the diet is important for people who have sickle cell disease; however, do not eat too much red meat as it has been associated with cardiovascular disease. If you are a vegetarian or a vegan, you have to make sure you are eating the right kind of vegetables, grains, and legumes to have a diet with enough of the right kinds of protein.
Low to moderate levels of exercise can be very beneficial for individuals with sickle cell disease. Once you feel fatigued during exercise, stop and rest. Exercise is a gradual process that starts with low impact and low-level exercise. Any exercise, even as simple as walking, is good for your health.
Before starting an exercise program, consult with your primary care provider. Strenuous exercise can cause problems in those with sickle cell disease and sickle cell trait. Prolonged strenuous exercise is generally not recommended. Very strenuous exercise without rest and adequate hydration can lead to severe dehydration, hemolysis, exertional heat stroke, heat stress, and rhabdomyolysis (breakdown of muscle), which has been life-threatening in athletes who have sickle cell trait. These risks can be decreased substantially with good hydration and rest periods.
You should be guided by your ability to tolerate exercise and the recommendations of your health care provider. If possible, have an evaluation by a physical therapist to help you tailor an exercise plan.
Growth and Development
Most young men who have sickle cell anemia have normal puberty. Still, many young men with sickle cell anemia may experience a delay in puberty. There can also be more severe growth delay around the time of puberty, particularly in developing countries. Some males may also experience long-term issues with pubertal development, which requires an evaluation by a primary health care provider.
Men who have sickle cell anemia have sperm abnormalities as well as decreased sperm counts compared to men who do not have sickle cell disease. These changes may not affect fertility, even though there are no fertility studies in males with sickle cell anemia. Hydroxyurea further decreases sperm counts, but again, the decrease may not affect fertility. Research has also reported that about 25% of men who have sickle cell anemia have low testosterone levels (hypogonadism). In a 12-month study, 7 men with low testosterone were treated to increase their testosterone levels. They had an improved quality of life without causing priapism, which can be related to testosterone levels.
Priapism is very common in adolescents and men who have sickle cell anemia and disease. About a third of men report that they have experienced priapism at least once. Priapism can lead to erectile dysfunction due to scarring in the soft part of the penis. This can occur after severe episodes of priapism that are extremely painful and require emergent care. Erectile dysfunction can also occur in men who have never had a severe episode of priapism, but who have multiple episodes of priapism that are self-limiting, and they felt that medical treatment was not necessary. All acute events should be reported and treated emergently and aggressively. Unfortunately, no medications have been proven in studies to prevent priapism in sickle cell anemia; it has been recommended not to use medication to reduce priapism.
The recommended primary treatment is to treat individual episodes. Surgical treatment is considered a last resort.
Over a third of people who are hospitalized with sickle cell disease have depression. Depression is not related to the severity of sickle cell disease nor is it related to income. It is related to the reported quality of life. Depression can be from mild to severe. Many people, perhaps men, in particular, feel that they would not want anyone to know they were depressed or seeing a counselor for depression or other emotional problems. The sickle cell community and those who care for those with sickle cell disease realize how your life is affected. Counseling is encouraged for people who have a chronic illness and in particular sickle cell disease, which is so unpredictable and at times severe and life-threatening. A counselor can be a social worker, a trusted member of your church, or even a family member. The most effective counselor could be a psychologist – someone who is trained to listen and help people with their life problems.
From our Medical Director, Dr. Keith Quirolo