Learning Outcomes
By the end of this section, you should be able to:
- 10.2.1 Discuss the impact of nutrition on congenital hematologic disorders.
- 10.2.2 Discuss the impact of nutrition on cancers.
Congenital Hematologic Disorders
Sickle cell disease (SCD), a genetic hemoglobinopathy with chronic anemia, is the most common inherited blood disorder in the United States (McCaskill et al., 2018) and affects the shape of hemoglobin, resulting in blocked blood flow. Individuals with SCD are more prone to micronutrient and macronutrient (carbohydrate, fat, and protein) deficiencies related to hyperreactive hematopoiesis, the physiologic response caused by chronic anemia. Nutrient deficiencies (Table 10.5) further increase systemic oxidative stress (disturbance in the balance between the production of free radicals and antioxidant defenses) and contribute to severe and frequent sickle cell vaso-occlusive crises. These deficiencies can cause the following in people with SCD (Umeakunne & Hibbert, 2019):
- Delayed growth and development
- Decreased quality of life
- Chronic inflammation
- Painful vaso-occlusive crises
- Increased occurrence of stroke, pulmonary hypertension, heart disease, and renal disease
As with SCD, thalassemia is a genetic hemoglobinopathy, characterized by less oxygen-carrying protein and fewer red bloods cells. There are multiple genetic forms ranging in severity. Thalassemia carries the same nutritional risks as SCD does.
Table 10.5 outlines additional micronutrient deficiencies and recommended interventions.
| Micronutrient Deficiency | Recommended Foods/Interventions |
|---|---|
| Vitamin D* | Milk, yogurt, cheese |
| Vitamin A | Carrots, sweet potatoes, kale, spinach |
| Vitamin C | Kiwis, oranges |
| Vitamin E | Mustard greens, sunflower seeds |
| Zinc | Nuts, fish, whole grains, dairy products |
| Folic acid | 1 mg daily supplement Leafy green vegetables |
| Vitamin B12 | Steamed shrimp, baked snapper, whole grain breads |
| Omega-3 fatty acids | Meats, beans, nuts, seeds, vegetable oils |
| *Vitamin D deficiency is five times more likely to occur in children with SCD than in children without SCD and is associated with increased hospitalizations (McCaskill et al, 2018). | |
Genetic Hemochromatosis
Genetic hemochromatosis causes excessive absorption of dietary iron secondary to decreased expression of the peptide hormone hepcidin. It is the most common autosomal recessive disorder in White individuals, affecting one in 300–500 people of northern European descent (Porter & Prashanth, 2023). It is twice as common in males as females and can result in multiple organ dysfunction. Excessive intestinal dietary intake of heme and nonheme iron is primarily managed by dietary modifications (Milman, 2021). These modifications are extensive and require nutritional counseling and reinforcement by health care professionals:
- Consider a vegetarian or semivegetarian diet.
- Consume 21 oz of fruit and vegetables daily; fresh is best.
- Avoid red meat from mammals; instead, choose lean, white poultry.
- Avoid processed meat. Consume no more than 7 oz of meat from poultry per week. Consider additional protein sources such as fish, eggs, and legumes.
- Choose whole grains; avoid iron-enriched grains.
- Beverages should consist of water, green or black tea, coffee, and low-fat milk. Avoid alcoholic beverages.
Hemophilia
Hemophilia is an inherited X-linked blood disorder resulting in decreased levels of clotting factor VIII or IX. Occurring in 1 in 5,000 male births, this deficiency results in spontaneous bleeding, often in joints, and severe bleeding following injury or trauma (CDC, 2023a). Nutritional considerations include managing weight and maintaining iron levels to avoid IDA. Obesity is common secondary to a sedentary lifestyle to avoid injury-induced bleeding; however, consequences of decreased physical activity include more stress on the joints and muscle strains that can result in musculoskeletal bleeding (Lim et al., 2020). A recommended diet includes iron-rich foods such as lean red meat, leafy green vegetables, broccoli, dried beans, grains, and raisins. It is important to consume foods containing vitamin C, such as orange juice, to enhance iron absorption.
Effect of Misshapen Red Blood Cells on Blood Flow
The American Society of Hematology (2009) offers a video that demonstrates how blood flow is affected by misshapen red blood cells.
Impact of Cancer on Nutrition and Hematologic Function
Advanced age is a risk factor for nutritional deficiency; the addition of chronic diseases, including malignancy, can quickly lead to malnourishment. These comorbidities can contribute to reduced tolerance of anticancer therapy, impacting survival (Stauder et al., 2020).
The prevalence of anemia in cancer can exceed 90% across all ages and is generally considered to be related to medications, infections, or disorders of hematopoiesis. Common concerns are alterations in activities of daily living affecting quality of life, decreased productivity, and inability to work. Fatigue is reported as the most common troubling symptom of cancer and is directly linked to anemia (Gilreath & Rodgers, 2020). Fatigue and lack of appetite commonly coexist, resulting in an inability to prepare meals, further exacerbating anemia.
Anemia was traditionally believed to be primarily related to the adverse effects of anticancer therapy; however, recent research has shown a direct correlation with chronic, low-grade inflammation. Functional iron deficiency (state in which iron is insufficiently incorporated into erythroid precursors even though body iron stores are apparently adequate), malnutrition, and anorexia contribute to cancer-associated anemia (Natalucci et al., 2021). See Table 10.6 for types of IDA in cancer. In contrast, iron overload can occur in clients with symptomatic anemia receiving red blood cell transfusions and can alter the function of effective hematopoiesis, contributing to anemia (Gilreath & Rodgers, 2020). Other micronutrient deficiencies affecting hematologic function in individuals with cancer include folate and vitamin B12.
Approximately 50% of adults with colon cancer have anemia due to chronic blood loss from the gastrointestinal tract (Chardalias et al., 2023). The prevalence of anemia in other cancers ranges from 29–46%, mainly related to chronic disease, chemotherapy-induced anemia, nutrient-related anemia, chronic anorexia, and gastrointestinal adverse effects such as nausea, vomiting, and diarrhea (Busti et.al., 2018).
| Iron Deficiency | Etiology |
|---|---|
| Absolute iron deficiency anemia | Associated with bleeding; total body iron stores diminish. |
| Iron sequestration* | Inflammation results in iron being unavailable for erythropoiesis. |
| Iron-restricted erythropoiesis* | Erythropoiesis therapy (epoetin) outpaces iron delivery from storage. |
| *Functional iron deficiency anemia | |
Nutritional Interventions
Chronic illness has a significant impact on nutrition and, consequently, hematologic wellness. Recommendations have evolved due to more emphasis on a holistic approach to caring for those with hemoglobinopathies and cancer.
The Mediterranean diet has gained popularity in the United States as a way to manage weight, lower cholesterol, and stabilize blood sugar. Close investigation of the constituents of this eating lifestyle reveals significant anti-inflammatory properties that translate well to chronic hematologic illness. Extra-virgin olive oil is has been found to block the proinflammatory cascade (Natalucci et al., 2021).
The following foods and substances rich in antioxidants contribute to anti-inflammatory and microbiota-modulating properties:
- Phytochemical vitamins in red, orange, and yellow fruits and dark green leafy vegetables
- Ginger
- Green tea
- Garlic
- Black pepper
- Turmeric
To avoid severe nutritional deficiencies, a high-calorie, nutrient-rich diet is essential for people living with SCD and thalassemia. Increased oral hydration is vital in SCD to decrease the frequency of painful vaso-occlusive events. Additional recommendations include daily supplements of omega-3 fatty acids, folic acid, high-dose vitamin D, and a multivitamin without iron. For clients with thalassemia specifically, drinking black tea may decrease the gastrointestinal absorption of iron (Goldberg et al., 2018; Physicians Committee for Responsible Medicine, 2020b; Srichairatanakool et al., 2020).
People with SCD and vitamin D deficiency consuming a diet rich in fish, milk, cheese, and eggs were found to have decreased hospital and emergency department visits (McCaskill et al., 2018). High-dose vitamin D replacement (greater than 3000 units per day) has been found to augment immune response and decrease the risk of respiratory infections in SCD (Umeakunne & Hibbert, 2019). Recent literature strongly correlates that vitamin D and chronic inflammation play an important role in regulating iron homeostasis, which can improve anemia (Natalucci et al., 2021).
Adults living with hemoglobinopathies should avoid drinking alcohol because it potentiates the oxidative damage caused by iron overload (Milman, 2021).