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Alexa AngeloMedical Nutrition TherapyCase Study 3 Cystic FibrosisNovember 13, 2012I. Understanding the Disease and Pathophysiology1. Define cystic fibrosis. Cystic fibrosis is a disease characterized by abnormally thick mucus secretions from the epithelial surfaces of various organ systems, including the respiratory tract, the gastrointestinal tract, the liver, the genitourinary system, and the sweat glands. Cystic fibrosis is caused by an abnormal mutation of the cystic fibrosis transmembrane conductance regulator (CFTR), which is a type of protein classified as an ATP-binding cassette (ABC) transporter. Mutations of this protein causes the prevention of the CFTR to function properly. In patients with CF, the CFTR’s failure to function properly causes thick viscous secretions that eventually lead to obstruction of the glands and ducts in the affected organs (1, pg.663)2. Describe the most common populations affected by this disease, including age, gender, and ethnicity. Cystic fibrosis is the second most common life-shortening, inherited disorder occurring in childhood in the United States. The most common population affected by cystic fibrosis are Caucasians of Northern Europe descent. It occurs equally with female and male babies. . This disease is an autosomal recessive disorder affecting the CFTR gene on Chromosome 7. What does this mean? Describe what is currently understood about the genetic characteristics of this disease. (CFTR), which is a type of protein classified as an ATP-binding cassette (ABC) transporter. Mutations of this protein causes the prevention of the CFTR to function properly. The CFTR proteins are responsible for the transport of sugars, peptides, inorganic phosphate, chloride, and metal cations across the cellular membrane. One in 31 Americans are carriers of the defective gene that causes CF. In order to have CF, the individual must inherit the defective gene from both parents. There is a 25% chance that a child with get CF when both parents carry the gene, 50% chance that the child will carry the defective gene, and 25% chance that the child will be a non-carrier. (1, pg.663)4. How is this disease diagnosed? List at least three methods that are used. Along with a complete medical history and physical examination, the diagnoses of CF uses the following tests: sweat chloride test (a test to measure the amount of chloride in the sweat by stimulating the skin to produce a large amount of sweat that is then absorbed by a special filter paper and analyzed for chloride content), a blood test to confirm any mutations of the CFTR gene, sputum cultures to test for infections typical in CF, pancreatic function tests (tests to measure pancreatic function, including serum amylase or lipase, a test for the amount of fat in the stool, and an X-ray of the anatomical features of the pancreas and common bile duct), and also pulmonary function tests. In May 2005, the FDA approved the first DNA-based test to detect CF. This test directly analyses human DNA to find the genetic variations indicative of the disease and is used for the diagnoses of children with the disease and adults who are carriers of the disease. (1, pg.664)5. For each of the following organs or organ systems, describe the most common physical changes that occur as a result of the abnormality of the CFTR gene. Explain how these changes may affect Lily’s nutritional status.a. Respiratory: Respiratory problems that may occur include coughing or increased mucus in the sinuses and lungs, fatigue, nasal congestion, and recurring pneumonia. b. Reproductive: Later in adulthood, men may experience infertility due to CF. c. Pancreatic: Pancreatic problems may include poor digestion, poor absorption of fat and fat-soluble vitamins, and loss of bile and salts. Malabsorption of calcium, phosphorous, magnesium, and vitamins D and K are of primary concern. (1, pg.664)d. Gastrointestinal: Gastrointestinal problems may include severe constipation and pain in the stomach from this constipation, increased bloating and gas (distended belly), nausea and loss of appetite, foul smelling stools and also weight loss. The constipation is a nutritional concern as well as the loss of appetite. With a loss of appetite runs the risk of malnutrition and weight loss. 6. Lily was admitted and diagnosed with bacterial pneumonia. Why is this the most common hospitalization for patients with CF? Explain. When cystic fibrosis patients get buildup of thick mucus in the lungs and sinuses, it causes an ideal breeding ground for bacteria and fungi. This causes the frequent bouts of pneumonia. II. Understanding the Nutrition Therapy7. What are the most common nutritional consequences of cystic fibrosis?Some of the most common nutritional consequences include poor weight gain and poor growth (due to poor intake and lack of appetite), pancreatic insufficiency which can lead to the malabsorption of calcium, phosphorous, magnesium, and vitamins D and K, increased nutrient needs in patients with lower than normal BMI, poor bone mass, low serum nutrient statues, and high infection rates. (1, pg.664)8. Describe the major modifications for carbohydrate, protein, and fat intake that would be needed as components of nutrition therapy for CF. For a patient with cystic fibrosis, adequate kilocalories to support normal growth and development are essential. Energy intake should be calculate based on the patterns of weight gain and growth in the child. Children without respiratory infections are comparable to healthy children and the energy RDA should be 100% to 110 %. However, if the patient has growth deficits, malabsorption, or lung disease, the RDA should be 110% to 200% of the RDA. Fat absorption for patients with cystic fibrosis is higher than normal patients because of the need to compensate for any fat malabsorption. The recommended fat intake for a patient with CF is 35% to 45% rather than the normal 25% to 35%. Medium chain triglycerides are best because they require less bile salt for solubilization and requires less lipase activity than long-chain fatty acids. Protein intake should be 15% to 20% of total kcal for the patient and is essential to ensure growth and maintenance of protein stores. Carbohydrates should constitute the rest of the diet and should be distributed throughout the day.(1, pg. 668)9. Is Lily at risk for electrolyte imbalances? Specifically, address her sodium and chloride requirements. Is there additional information from Lily’s history that outs her at risk for changes in her sodium and chloride levels?People with cystic fibrosis should consume more sodium, especially in the summer months when sodium excretion is higher or if they live in places with higher climates. Lily’s sodium intake should be higher due to her participation in sports. Some pharmacies have sodium solutions with chloride in them, but usually sodium intake is not a problem in adolescents or adults because of the amount of sodium in the food supply. Lily’s lab values show normal sodium and chloride levels.( 1, pg.666)10. What is Pancrease? Lily mentioned that she did not know how much to take. What are the recommendations?Pancrease is a form of pancreatic enzymes that help with fat absorption and are dosed by units of lipase/kg/meal, or units of lipase/gram fat ingested. A weight-based method has been developed as a practical way to determine the dose of enzymes per meal. In a newly diagnosed patient, dosages of 500 unites lipase/kg/meal should be used, and half of that before snacks. In symptoms improve, the dose should be lowered. If symptoms do not improve, the dosage should be increased. . Nutrition AssessmentA. Evaluation of Weight/Body Composition11. Assess Lily’s weight and height. Plot her height and weight on the appropriate growth chart. Calculate her BMI. Calculate her %UBW. Explain what each of these assessments provides and why one or more provides the most relevant information for Lily. Lily’s weight and height on the growth chart show that she falls in the 10th percentile for her age group. Lily’s BMI was calculated by using the equation: [Weight (lb)/ height (in2)] x 703= (102/4225) x 703= 16.97 or BMI ~ 17%UBW= Actual weight/Usual weight= 102/112= 91%The growth charts provide information that compares Lily to other children her age. According to the growth charts, Lily falls within the 10th percentile. This is very low. Lily’s BMI is below what it should be; a BMI below 18.5 is classified as underweight. Her %UBW is 91%, which means she is below her usual body weight. All of these assessments provide evidence that Lily is underweight and needs to increase her energy intake to prevent malnutrition and further weight loss.. Calculation of Nutrient Requirements12. Determine Lily’s energy and protein requirements. You see that she typically runs 5 to 7 miles 3 to 4 times per week as well as taking a dance class 3 times per week for 1 hour. Make sure this is taken into account when calculating her energy requirements. Your recommendations for Lily should include the appropriate macronutrient and micronutrients based on the requirements for an adolescent with cystic fibrosis.Harris Benedict Equation:REE= 655 + (9.56 x wt (kg)) + 1.85 x ht (cm)) – 4.68 x age (yrs))REE= 655 + (9.56 x 46.4) + 1.85 x 142.2 – 4.68 x 14 REE= 1,279 or ~ 1,300 kilocalories1,300 x 2.0= 2,600 kilocaloriesRounding up to 3,000 to help her gain weightFAT: 3,000 x .40 = 1,200 kcal from fat 1,200/9 = 133 g fat/dayProtein: 3,000 x .15 = 450 kcal from protein450/ 4 = 113 g protein/dayCHO: 3,000 x .45= 1,350 kcal/day1,350/4 = 338 g CHO/dayVitamin supplementation should consist of the following: AgeVitamin A (IU)Vitamin E (IU)Vitamin D (IU)Vitamin K (IU)>810,000200-400400-8000.3-0.5C. Intake Domain 13. Analyze Lily’s nutritional intake according to the usual dietary intake. Attached your computerized analysis for this assessment.Dietary intake:24 hour recall:14. Compare your analysis to her estimated nutritional needs.I estimated that Lily should be consuming 3,000 kilocalories to help her gain some weight that she has lost and also allow her to perform her every day activities without losing any more weight. However, both Lily’s dietary intake and also 24-hour recall total energy requirements were below the amount of kilocalories she needs to maintain her weight, which is 2,600 kilocalories. In her dietary intake, she was consuming too much fat and not enough carbohydrates. Her 24-hour dietary recall was divided correctly along the macronutrients, but was only half of the energy intake she should be consuming. 15. Identify three specific vitamins and minerals that are needed in increased amounts during adolescence. Explain why they are of special importance for an adolescent. Will Lily’s CF affect the metabolism of these nutrients? Do Lily’s diet history and 24- hour recall indicate that she consumes adequate amounts of these nutrients?Calcium, Vitamin D, and Vitamin K are three vitamins and minerals that are especially of concern during adolescents. These nutrients help in the support of bone growth and bone health and are important during the adolescent years for a child. Lily’s CF will affect the absorption of these nutrients because of pancreatic problems that will slow down the absorption of fat soluble vitamins. Lilly did not consume enough of any of these nutrients. In order to help prevent this, the use of a multivitamin will be continued to be administered, as well as the addition of more fortified dairy products as well as more leafy green vegetables. 16. From information gathered within the intake domain, list possible nutrition problems using the diagnostic term.- Inadequate energy intake NI-1.4- Inadequate mineral intake (Calcium 1) NI-5.10.1-Inadequate vitamin intake (D3, K5) NI-5.9.1D. Clinical Domain17. After reading the physician’s history and physical, identify the signs and symptoms that are consistent with Lily’s admitting medical diagnoses. The major sign that is consistent with her medical diagnosis is the fact that Lily has cystic fibrosis, and has been hospitalized before due to respiratory infections. Lily has a cold, and her pharynx is reddened and she has postnasal drainage. She is experiencing decreased breathing sounds, percussion hyperresonant, rhonci and rales present which indicate respiratory distress. She said she is experiencing a lack of appetite as well.18. Evaluate each of the medications that Lily takes as an outpatient. Determine the function of each medication and identify any nutritional implication.MedicationFunction of MedicationNutritional ImplicationsPancreaseHelps absorption of fatAllows Lily to absorb fats and fat soluble vitamins more easilyPrevacidDecreases amount of acid made in the stomachWill prevent Lily from experiencing heartburn and allow her to eat more acidic thingsHumabidThins mucous membranes by thinning mucus in the air passages to make it easier to cough up the mucus and clear the airwaysThis will help Lily breathe and hopefully allow her to eat peacefullyProventil PRNRelaxes and opens the air passages to the lungs to make breathing easierThis will hopefully help Lily breathe and allow her to eat peacefully19. Biochemical: Evaluate Lily’s laboratory values. In the following table, list any laboratory values that are abnormal. What is the most probable cause of the abnormality?Abnormal LabNormal ValueReason for AbnormalityNutritional ImplicationsTransferrin 219 mg/dL250-380 mg/dLPossible acute anemiaLow iron intake; need to increase iron intakeMagnesium 1.6 mg/dL1.8 to 3 mg/dLLow intake in dietary magnesiumNeeds to increase magnesium intakeHba1c 6.3 mg/dL3.9- 5.2 mg/dLImbalance of glucoseNeed to stabilize glucose intakeWBC 13 x10^3/mm34.8-11.8Infection of lungNeed to get Lily curedHGB 11.5 g/dL12 -15 mg/dLPossible acute anemiaLow iron intake; need to increase iron in dietHCT 33%37-47%Infection of lungMust cure LilyFerritin 19 mm320-120 mm3Possible acute anemiaLow iron intake; need to increase iron intake20. List possible nutrition problems within the clinical domain using the diagnostic term.- Impaired nutrient utilization NC- 2.1- Underweight NC-3.1E. Behavioral –Environmental Data21. After reading the history and physical as well as the nutrition history, identify factors that may impact the success of Lily’s current medical and nutritional care for her cystic fibrosis.Lily has a history of getting pneumonia. She is a very active child and because of her cystic fibrosis, she requires even more energy requirements. If Lily does not start meeting her energy requirements, she may become even more underweight and is putting herself at risk for malnutrition. This is a critical time in Lily’s life and she needs to be consuming the right amount of macro and micronutrients in order to promote the growth of her young body. In order to do this, she must start eating the right foods that are high caloric and nutrient dense. This will help her start to gain weight and also prevent her from becoming malnourished. IV. Nutrition DiagnosisSelect two high-priority nutrition problems and complete PES statements for each.Lack of energy intake related to loss of appetite as evidenced by low BMI and 24-hour diet recall.Inadequate vitamin and mineral intake related to poor knowledge of nutrition and lack of appetite as evidenced by 24-hour diet recall.V. Nutrition Intervention23. For each of the PES statements that you have written, establish an ideal goal (based on the signs and symptoms) and an appropriate intervention (based on the etiology).- Help increase appetite by administering small meals throughout the day with a goal on consuming more energy and gaining weight.- Educate Lily on the importance of eating the correct amount of macronutrients and micronutrients and how they can help her feel better with a goal of her consuming >110% of the RDA for macronutrients and micronutrients24. What might be different about your nutrition interventions if Lily were a young teenager of different ethnicity and/or religion? Give an example and explain.If Lily were young, I may supplement her diet with Pediasure or some other form of nutrient dense shake that would help her consume the correct amount of macro and micro nutrients. If Lily were of a different ethnicity or religion, I would talk to her about her knowledge of cystic fibrosis and of nutrition because people of African descent rarely get this disease, and it may be more frightening to someone whose race rarely gets the disease than a race (Caucasian) who do have knowledge of the disease because it is prevalent among the race. References:1. Nelms, M. N. (2011). Nutrition therapy and pathophysiology (2nd ed.). Belmont, CA: Wadsworth, Cengage Learning.2. Cystic fibrosis - PubMed Health. National Center for Biotechnology Information. Retrieved November 10, 2012, from 3. . . ................
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