SICKLE CELL ANEMIA

Kaitlyn Kavan ISU Dietetic Intern ‘18

OSF St. Joseph Clinical Mini Case Study
 OUTLINE

o Define sickle cell disease & related problems

o Determine the affected population

o Discover the complications of sickle cell anemia

o Discuss medical interventions

o Deliver medical nutrition therapy strategies

o Disclose pertinent assessments & recommendations

 SICKLE CELL ANEMIA

o One form of sickle cell disease, a genetic disorder of the blood

o Anemia: a deficiency in the size or number of red blood cells
o Hemolytic

o “Hemoglobin S disease”
o Defective hemoglobin synthesis
o Partial or total deoxygenation of hemoglobin S molecules
o Sickle-shaped red blood cells
o Poorly carry oxygen
o Impacts oxygen & carbon dioxide exchange
o Oxygen deprivation → organ damage
o Catch in the capillaries
o Blood vessel obstruction → vaso-occlusion
o Sickle cell crisis
 POPULATION

o Sickle cell anemia affects:

o ~1 out of every 500 African Americans in United States
o Individuals of African, Middle Eastern, East Indian, & Mediterranean origin
o Typically diagnosed around one year of age
o Some complications include:
o Stroke
o High blood pressure
o Retinopathy
o Acute & chronic pulmonary dysfunction
o Hemochromatosis of the heart & liver
o Negative impact on gallbladder → gallstones, spleen, & kidneys
o Aseptic necrosis of the hips & shoulders
o Skin ulcerations
o Delayed growth & sexual maturation
o Shortened life span
 MY INSPIRATION

o 21 YOM
o Medical problem: Sickle cell crisis
o Dietetic trigger: BMI < 19
o 6’ 3” 150# BMI – 18.75 kg/m2
o General diet
o LOS: 4 days
o Previous SJMC visits
o September, 2015 o September, 2016
o November, 2015 o November, 2016
o January, 2016 o December, 2016
o March, 2016 o February, 2017
o May, 2016 o June, 2017
o August, 2016 o July, 2017
o Dietetic triggers: BMI < 19 or LOS
 SICKLE CELL CRISIS

o Occlusion of small blood vessels

o Pain in abdomen, arms, & legs

o Medical interventions:
o Pain relief
o Blood exchange transfusion
o Lessen likelihood of pressure ulcers, infections, & renal failure
o Ensuring patient properly oxygenated
o Zinc supplementation

o Hydroxyurea: chemotherapy drug

o Vitamin K: prior to procedures
 MEDICAL NUTRITION THERAPY

o Boosting calories & protein

o Intake may be low due to pain, medications
o Higher metabolic rate
o Well-balanced meals, adequate fiber & fluids, low sodium, omega-3 fatty acid consumption
o Consumption of foods high in:
Micronutrient Sources
Zinc* Oysters, poultry, beans, nuts, whole grains, dairy
Copper* Sunflower seeds, lentils, almonds, dried apricots
Folate* (B Vitamin) Fortified cereal, spinach, black-eyed peas, brown rice
Vitamin A Carrots, spinach, kale, cantaloupe, mango, oatmeal
Vitamin D Fish, liver, egg yolks, fortified foods such as dairy
Vitamin E Vegetable oils, nuts, leafy greens, fortified cereals
Calcium Dairy, Ca-fortified drinks, beans, canned fish
*Recommend vitamin & mineral supplement containing 50-150% of these minerals, excluding iron.
 PATIENT INTERVIEW

o Usual questions:
o Appetite: current & prior to admission
o Intake: current & prior to admission
o Weight: UBW, any trends, clothes fit as normal
o Symptoms of nausea, vomiting, constipation, diarrhea
o Any chewing or swallowing issues, other barriers to consumption
o Any ulcers/wounds, & how they are healing
o Familiarity to supplements
o Additional questions:
o Vitamin & mineral supplement regimen
o Sources of protein
o Alcohol consumption

o Interest in education?
o Boosting calories & (plant) protein; sources of vitamins/minerals, meal preparation
 SITUATION & BACKGROUND

o Situation
o Low BMI
o LOS of 7 days
o Background
o Patient interview & chart information
o Sickle cell anemia specific laboratory values:
o Hemoglobin ↓
o Folic acid ↓
o Iron ↑
o Renal labs
o BUN
o Creatinine
o Albumin
 ASSESSMENT

o Malnutrition

o Inadequate protein-energy intake

o Inadequate oral intake
o Inadequate fluid intake

o Increased nutrient needs

o Underweight
o Unintended weight loss

o Food- and nutrition-related knowledge deficit

 ESTIMATED ENERGY NEEDS
& RECOMMENDATIONS

o Kilocalories
o 30-35 kcal/kg CBW
o Protein
o 0.8-1.0 g PRO/kg CBW
o Fluid
o 2-3 quarts (~1890-2840 mL/day)
o Recommend nutritional supplement
o Multivitamin/mineral supplement
o Folic acid & zinc supplements
o Omega-3 fatty acids: supplement, increased dietary intake
o Low sodium
o Low iron: if hemochromatic/receiving transfusions
o Recommend vegetable proteins
o Avoid liver, iron-fortified items, alcohol, sports drinks
*Nightly tube feedings may be considered to meet needs, avoid excess iron
 RESOURCES

Escott-Stump, S. (2012). Nutrition and diagnosis-related care. (7th ed.). Philadelphia,

PA: Wolters Kluwer Health/Lippincott Williams & Wilkins.

Mahan, L. K., Escott-Stump, S., & Raymond, J. L. (2012). Krause’s food and the
nutrition care process. (13th ed.). St. Louis, MO: Elsevier Saunders.

Steinberg, M. H., Barton, F., & Castro, O. (2003). Effect of hydroxyurea on

mortality and morbidity in adult sickle cell anemia. Journal of the American
Medical Association, 289(13), 1645-1651.
 SICKLE CELL RELATED
RECOMMENDATIONS
Estimated Nutrition Needs
o 30-35 kcal/kg CBW
o 0.8-1.0 g PRO/kg CBW
o 2-3 quarts fluids (~1890-2840 mL/day)

o Supplements
o Recommend nutritional supplement; provide cafeteria menu
o Multivitamin/mineral supplement
o Folic acid & zinc supplements
o Omega-3 fatty acids: supplement, increased dietary intake
o Low sodium
o Low iron: if hemochromatic/receiving transfusions
o Recommend vegetable proteins
o Avoid liver, iron-fortified items, alcohol, sports drinks

*Nightly tube feedings may be considered to meet needs, avoid excess iron