Sickle Cell Anemia in Pediatrics



Michelle Smith | 2013Minor Case StudySickle Cell Anemia in PediatricsTable of Contents TOC \o "1-3" \h \z \u Introduction PAGEREF _Toc349503323 \h 4Social History PAGEREF _Toc349503324 \h 4Normal Anatomy and Physiology of Applicable Body Functions PAGEREF _Toc349503325 \h 4Present Medical Status and Treatment PAGEREF _Toc349503326 \h 5Theoretical Discussion of Disease Condition PAGEREF _Toc349503328 \h 5Usual Treatment of the Condition PAGEREF _Toc349503329 \h 6Nutrition Treatment PAGEREF _Toc349503330 \h 6Medical Treatment PAGEREF _Toc349503331 \h 6Patient’s Symptoms upon Admission Leading to Present Diagnosis PAGEREF _Toc349503332 \h 7Laboratory Findings and Interpretation PAGEREF _Toc349503333 \h 7Medications PAGEREF _Toc349503334 \h 8Observable Physical and Psychological Changes in Patient PAGEREF _Toc349503335 \h 8Treatment PAGEREF _Toc349503336 \h 8Medical PAGEREF _Toc349503337 \h 8Surgical PAGEREF _Toc349503338 \h 8Medical Nutrition Therapy PAGEREF _Toc349503339 \h 9Nutrition History and Analysis of Previous Diet (24 hour recall) PAGEREF _Toc349503340 \h 9Current Prescribed Diet & Responses PAGEREF _Toc349503341 \h 9Nutrition-Related Problems PAGEREF _Toc349503342 \h 9Evaluation of Present Nutritional Status and other Nutrients to Address PAGEREF _Toc349503343 \h 9Goals, Interventions, Monitoring, and Evaluation PAGEREF _Toc349503344 \h 10Patient’s Nutrition Education Process PAGEREF _Toc349503345 \h 10General Conditions upon Discharge PAGEREF _Toc349503346 \h 10Prognosis PAGEREF _Toc349503347 \h 11Summary & Conclusion PAGEREF _Toc349503348 \h 11Appendix A - Medications PAGEREF _Toc349503349 \h 12Bibliography PAGEREF _Toc349503350 \h 13IntroductionThe patient chosen for this case study, EV, is a 16 year old African American male who has been diagnosed with sickle cell crisis pain. The height of the patient is 5 feet 5 inches (165.1 cm) and he weighs 115.5 pounds (52.5 kg). The patient was chosen for the pediatric case study because of his sickle cell anemia diagnosis which has an impact on nutrition. The patient was admitted to the hospital on February 27th and was discharged March 3rd. Social History This is a single male who is currently in juvenile detention but usually lives at home with his mother. He is Christian with no special religious dietary guidelines to follow.Normal Anatomy and Physiology of Applicable Body FunctionsNormally, red blood cells have an iron-rich protein called hemoglobin inside that carries oxygen to the body. Normal red blood cells have a disc-like shape allowing them to move freely through blood vessels without getting stuck or easily breaking apart (1, 2). Normal red blood cells live about 120 days in the bloodstream and then die (2).Present Medical Status and Treatment Theoretical Discussion of Disease ConditionSickle cell anemia is an inherited disease that is named for the abnormal sickle or crescent shape of the red blood cells (1-3). Sickle cell anemia is caused by an abnormal type of hemoglobin, hemoglobin S, that changes the shape of the red blood cells to the sickle shape (1,2,4). These sickle-shaped cells are fragile and only live about 10 to 20 days and the bone marrow cannot make new red blood cells fast enough to replacing the dying ones (2, 4). These abnormal cells also deliver less oxygen to the cells of the body. These abnormal blood cells are also sticky causing them to get stuck more easily in small blood vessels and often break into pieces. These pieces can disrupt blood flow causing oxygen flow to cells to decrease even more (1,4). This block in blood flow can cause pain and organ damage, increasing the risk for infection (2). People can carry the sickle cell trait but show no symptoms of sickle cell anemia when they inherit the sickle cell gene from only one parent. Sickle cell disease is much more common in people of African and Mediterranean descent but is also seen in people from South and Central America, the Caribbean, and the Middle East (1,2). One of every 12 African Americans carry the sickle cell trait while 1 in 500 African Americans have the disease (2).Symptoms of sickle cell anemia are not usually seen until after a person is 4 months old (1,2). There are painful episodes called crises that almost all patients will experience. These crises can last from hours to days and cause pain in the bones of the back, long bones, and chest (1,2). When the anemia becomes more severe, the patient will experience fatigue, paleness, rapid heart rate, shortness of breath, and yellowing of eyes and skin called jaundice (1,2,4).With time, the spleen will stop working causing bone, gallbladder, lung, and urinary tract infection. Other symptoms include delayed growth, delayed puberty, and painful joints caused by arthritis (1).Usual Treatment of the ConditionNutrition TreatmentBasal energy requirements are higher in adolescents with sickle cell anemia than in healthy control subjects therefore energy intake may need to be increased. Energy deficits are common and nightly tube feedings can help improve nutritional status (3).Medical TreatmentPatients with sickle cell anemia need ongoing treatment to limit the number of crises. Folic acid supplements should be taken to make new red blood cells. Treatment for sickle cell anemia crisis includes blood transfusions, pain medications, antibiotics to prevent bacterial infections, and fluids. Medical treatments for complications of sickle cell anemia include dialysis or kidney transplant for kidney disease, counseling for psychological complications, gallbladder removal with gallstone disease, hip replacement for avascular necrosis of the hip, surgery for eye problems, treatment for overuse or abuse of narcotic pain medications, and wound care for leg ulcers (1,4). Sickle cell anemia can be cured by bone marrow or stem cell transplants. This is not currently an option for most patients as they are unable to find well-matched donors (1, 2).Patient’s Symptoms upon Admission Leading to Present DiagnosisThe patient was admitted to the hospital with sickle cell anemia-related pain crisis occurring in his right leg. On a scale of 1 to 10, he rated his pain an 8.Laboratory Findings and InterpretationClinical indicators to diagnose and monitor patients with sickle cell anemia include bilirubin, blood oxygen, complete blood count, hemoglobin, serum creatinine, serum potassium, and sickle cell test (1,3).Below is a list of the lab values out of the normal range for the patient that are related to his diagnosis.Lab ValueNormal Value RangeBilirubin1.60.1-1.5RBC3.054-5.65Hemoglobin9.212.6-16.7Hematocrit 25.836.9-48.5Sickle cells1+NegativeCreatinine0.360.6-1.2MedicationsA list of the medications the patient was on when at the hospital is provided in Appendix A. Also listed are the uses and possible side effects that relate to the patient. Observable Physical and Psychological Changes in PatientThe major physical change seen in the patient was that he seemed very small for his age, both in height and weight. TreatmentMedicalThe only medical treatment for this patient has been providing him with pain medication as needed for his pain. SurgicalNo surgeries have been needed for this patient. Medical Nutrition TherapyNutrition History and Analysis of Previous Diet (24 hour recall)A 24 hour recall was obtained from the patient relating to what he eats when not in juvenile detention. BreakfastSnackLunchDinner4 waffles with butter and syrup4 slices bacon2 c OJHam & cheese sandwich on white bread with mayoSnack bag of potato chipsCan of pepsi2 McChicken sandwiches10 piece nuggets with sweet chili sauceSmall friesHi-C2 drumsticksWhite rice with corn and carrotsKool-aidBelow is the nutrient analysis of his 24 hour recall.NutrientsAverage EatenTotal Calories3968 CaloriesProtein (g)***172 gProtein (% Calories)***17% CaloriesCarbohydrate (g)***483 gCarbohydrate (% Calories)***49% CaloriesDietary Fiber22 gTotal Fat34% CaloriesSaturated Fat9% CaloriesMonounsaturated Fat13% CaloriesPolyunsaturated Fat9% CaloriesLinoleic Acid (g)***34 gLinoleic Acid (% Calories)***8% Caloriesα-Linolenic Acid (g)***2.8 gα-Linolenic Acid (% Calories)***0.6% CaloriesOmega 3 - EPA28 mgOmega 3 - DHA84 mgCholesterol443 mgMineralsAverage EatenCalcium1340 mgPotassium4325 mgSodium**7287 mgCopper1860 ?gIron21 mgMagnesium389 mgPhosphorus2212 mgSelenium214 ?gZinc17 mgVitaminsAverage EatenVitamin A341 ?g RAEVitamin B63.3 mgVitamin B123.2 ?gVitamin C303 mgVitamin D2 ?gVitamin E10 mg ATVitamin K92 ?gFolate744 ?g DFEThiamin2.8 mgRiboflavin2.7 mgNiacin53 mgCholine476 mgCurrent Prescribed Diet & ResponsesThis patient is currently on a solid, regular, adolescent diet order. He is eating great and loves the food. Nutrition-Related ProblemsThis patient did not have a nutrition-related problem. An example of a possible problem could be inadequate protein-energy intake related to decreased ability to consume sufficient protein and energy as evidenced by estimated energy intake less than recommended levels.Evaluation of Present Nutritional Status and other Nutrients to AddressPatients with sickle cell anemia require 120-150% higher energy needs. EV had an EER of 2768 with an active activity factor. EER x 1.2-1.5 = 3320-4150 kcals. His protein needs were 1.1-1.4 g pro/kg BW which was 58-74 g pro/day. Based on the analysis of his 24 hour recall plus a reported a 10# weight gain in the last month, the patient is getting enough calories and protein in his diet without the need for supplements. His diet is low in vitamin D, therefore, he may want to have his vitamin D levels checked and possibly take a vitamin D supplement if it is low.Children with sickle cell anemia have lower vitamin B6 concentrations and are at risk for nutritional deficiencies. Inadequate dietary intakes of folate are common whereas vitamin B12 intakes are usually adequate. Suboptimal vitamin A intake, low serum vitamin D status, and zinc deficiency are also common in children with sickle cell anemia. Supplementing the diet with missing nutrients and correcting malnutrition are important objectives. A multivitamin-mineral supplement should be recommended. Avoid one with excess iron if the patient is receiving transfusions (3). Goals, Interventions, Monitoring, and Evaluation The goal for this patient is to consume > 50% of meals. The intervention for the patient includes a general healthful diet and the patient will be monitored and evaluated by checking the amount of their food intake. Other possible interventions for children with sickle cell anemia may be to include a liquid supplement if the patient isn’t able to eat enough calories and protein to sustain growth or to add a multivitamin-mineral supplement to replace missing nutrients. Patient’s Nutrition Education ProcessLiterature was provided on how to eat high calorie and high protein meals with suggestions of adding butter and sauces to vegetables and sandwiches. The patient verbalized his understanding and had no barriers to learning. He denied having any other questions at the time of the visit.General Conditions upon DischargeUpon discharge the patient was no longer experiencing pain crises from the sickle cell anemia. His appetite was still good, eating 100% of meals. PrognosisThe patient has a very good prognosis. His needs are very high but he is still able to eat enough calories and protein to promote weight gain and growth. Summary & Conclusion I have learned a lot about sickle cell anemia with this case study. I was unaware just how high the needs for these patients were. I didn’t know about the pain crises they experience because of the disease. It was amazing to see how much a boy had to eat and yet only gained 10# in one month. DrugUseSide EffectsKetorolacShort-term relief of moderately severe painHeadache, dizziness, drowsiness, diarrhea, constipation, gas, sores in mouth, sweatingTylenolPain reliefRash, hives, itching, hoarseness, difficulty breathing or swallowingAppendix A - MedicationsBibliographyPubMed Health. The sickle cell anemia page. Available at: . Accessed March 7, 2013.National Heart, Lung, and Blood Institute. Explore sickle cell anemia page. Available at: . Accessed March 7, 2013.Escott-Stump S. Nutrition and Diagnosis-Related Care. 7th ed. Baltimore, Md: Lippincott Williams & Wilkins; 2012.Centers for Disease Control and Prevention. Sickle Cell Disease page. Available at: . Accessed March 7, 2013. Medline Plus. The drugs, supplements, and herbal information page. Available at: . Accessed March 7, 2013. ................
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