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Hayley Kurtz Sodexo Dietetic Internship March 6, 2014

The Role of the Registered Dietitian in the Nutritional Management of Cystic Fibrosis Disease Progression. Hayley Kurtz Sodexo Dietetic Internship March 6, 2014. Agenda. Cystic Fibrosis CFTR Gene & Organs Diagnosis Symptoms & Complications Medical Treatment Medical Nutrition Therapy

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Hayley Kurtz Sodexo Dietetic Internship March 6, 2014

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  1. The Role of the Registered Dietitian in the Nutritional Management of Cystic Fibrosis Disease Progression Hayley Kurtz Sodexo Dietetic Internship March 6, 2014

  2. Agenda • Cystic Fibrosis • CFTR Gene & Organs • Diagnosis • Symptoms & Complications • Medical Treatment • Medical Nutrition Therapy • Presentation of Patient • Critical Comments • Summary

  3. CFTR Gene • Cystic fibrosis transmembrane conductance regulator (CFTR) gene • CF related to defects in CFTR gene makeup • CFTR gene produces CFTR protein • Made up of 1,480 amino acids • Each domain acts as a channel transporting chloride across cell membranes

  4. Classifications of Mutations According to Their Effect on the CFTR Protein • Class I, II and III mutations generally lead to complete loss of function and a more severe disease. • Class IV and V cause a reduction in function and have a milder effect.

  5. CFTR Gene Mutations Affect: • Sinus • Pancreas • Lungs • Sweat glands • Reproductive tract

  6. Pancreas • Exocrine and endocrine cells • CFTR’s role

  7. Pancreas • CFTR protein defect: absence or dysfunction of CFTR channel in pancreatic ductules

  8. Lungs • Expands and contracts ~20 times per minute to supply oxygen to tissues • Functions of CFTRproteinchannel: • Transports chloride in and out of cells • Transports sodium in and out of the airway surface liquid • Bicarbonate neutralizes chloride ions

  9. Lungs Healthy Lungs CF Lungs

  10. Diagnosis of Cystic Fibrosis • Newborns inherit two copies of the CFTR gene, one from each parent

  11. Diagnostic Testing • Prenatal testing • Sweat-chloride test • Chest x-ray • Sinus x-ray • Lung function test • Sputum culture

  12. Symptoms

  13. Complications • Buildup of thick, sticky mucus • Excessively salty skin • Pancreatic insufficiency • Malnutrition • Chronic lung infections • Bronchiectasis • Pneumothorax

  14. Medical Treatment Thoracic Expansion Exercises Forced Expiration Technique Autogenic Drainage Percussion Vibrations Huffing

  15. Medical Treatment

  16. Medical Nutrition Therapy

  17. Medical Nutrition Therapy • Multidisciplinary team • Pulmonologist/Pediatric Pulmonologist • Registered Dietitian • Social Worker • Critical Care Specialist • Pulmonary Surgeon • Respiratory Care Practitioner • Registered Nurse • Primary Care Physician • Endocrinologist, Gastroenterologist & Cardiologist

  18. Medical Nutrition Therapy • Early detection • Monitor growth and nutritional status • Lung function and malnutrition interrelated

  19. Nutrition Assessment

  20. Nutrition Assessment • Indicators for nutritional status: • Genetic potential • Growth charts • Weight • Puberty development

  21. Nutritional Failure in CF Patients

  22. Calculating Daily Energy

  23. Dietary Reference Intakes

  24. Pancreatic Enzyme Replacement • Starting dose: • 500 units/kg/meal • 250 units/kg/snack • Increasing gradually until 10,00 units/kg/day is reached • Do not exceed 10,000 units/kg/day

  25. Laboratory Data • Blood glucose • Arterial blood gases • Lipase • Amylase • Sodium • Chloride

  26. Nutrition Diagnosis

  27. Nutrition Support

  28. Presentation of R.L.

  29. Medical & Social History of R.L. • Age: 26 • Sex: Female • Race: Caucasian • Allergies: NKFA • Height: 63in (160cm) • Weight: 81.9 lbs (37kg) • BMI: 14.5 • PMH: CF, Dehydration, Malnutrition, Depression, Bronchiectasis, Pulmonary HTN • Nutrition support: PEG tube receiving nocturnal feeds of enteral nutrition (Perative) & bolus feeds PRN at home • Family hx: Brother passed away from CF • Treated at Penn-Presbyterian for the last 7 years by a Pediatrician who specializes in CF

  30. Previous PPMC Admissions

  31. PPMC Admission • November 11, 2013: Admission • Dx: CF exacerbation, 9% wt loss x 1 month, dehydration, malnutrition, change in cough sputum, FEV1 12-13% • November 12, 2013: R.L. declared as VIP • Initial assessment

  32. Day OneAssessment • 81.9 lbs/37kg • 63in/160cm • BMI: 14.5 • %IBW: 70% • %UBW: 91% • 9% wt loss x 1 month • Abnormal labs: Glucose 134mg/dL, BUN 6mg/dL, Magnesium 1.6mg/dL • Medications: Vit D, Vibramycin, Iron Sulfate, Prevacid, Reglan, MVI, Pancrealipase

  33. Day OneDiagnosis

  34. Day OneNutrition Requirements • Increased needs per CF, intubated and extubated needs dependent of medical status: • Intubated: 1100-1290kcals/day (30-32kcals/kg) • Extubated: 1480-1850kcals/day (40-50kcals/kg) • Protein: 56-74g/day (1.5-2.0g/kg) • Fluid: 1100-1290mL/day (30-32mL/kg)

  35. Day OneNutrition Prescription • Intubated: Perative @ 40mL/hr x 24 hrs + bolus flushes 60mL q6hrs to provide 1248kcals, 64g protein, 1120mL • Extubatedw/ bipapx 12 hrs: Perative @ 100mL x 12 hrs + bolus flush 200mL x 1 daily to provide 1560kcals, 80g protein, 1150mL • Extubatedw/ bipap >12 hrs: Recommend post-pyloric feeds (J tube) Perative @ 55mL/hr x 24 hrs + bolus flush 110mL x 1 daily to provide 1716kcals, 88g protein, 1150mL

  36. Day TwoFollow up • Remains on bipap • Medical staff requesting TPN recommendations 2/2 patient requiring bipap >12 hrs • Medical staff did not take my recommendation of post pyloric feeds via J-tube while on bipap >12 hrs • Spoke with medical staff to replete electrolytes prior to initiating TPN • Identified patient with severe weight loss may cause risk of refeeding

  37. Day TwoNutrition Intervention • TPN recommendations • Initiate TPN day 1: 340kcals Dextrose, 54 g AA • Continue to monitor electrolytes, if depleted, replete prior to advancing TPN (waiting 24-48 hrs of electrolytes WNL) • TPN day 2: 340kcals Dextrose, 75g AA, 166kcals Lipids • TPN day 3: 510kcals Dextrose, 75 g AA, 246kcals Lipids • Goal: 544kcals Dextrose, 75g AA, 370kcals Lipids • 1214 kcals, 914 NPC, GIR 3 meeting 82% estimated kcal needs, 101% estimated protein needs

  38. Day FourFollow up • Remains on bipapx 24 hrs, NPO • Electrolytes WNL • TPN initiated • Day 1 TPN recommendations

  39. Day 8Follow up • Remains on bipapx 24 hrs, NPO • Labs not being drawn • TPN has not advanced • Stage I pressure ulcer on nose r/tbipap • Full code changed to DNAR-B

  40. Day 10Follow up • Remains on bipapx 24 hrs • Rapid pulmonary function deterioration • TPN still not advanced • R.L. passed away in the MICU this evening

  41. Critical Comments • Stable at previous admissions • Appropriate clinical judgment • Noncompliant with TF and PO dietary intake • Weight loss during initial assessment • Medical team decided against nutrition recommendations

  42. Summary

  43. References • U.S. National Library of Medicine. Genetics Home Reference: CFTR. National Institute of Health.http://ghr.nlm.nih.gov/gene/CFTR. Published January 6, 2014. Accessed January 8, 2014.   • Cystic Fibrosis Foundation. Living with Cystic Fibrosis. http://www.cff.org/treatments/. Accessed December 22, 2013. • Brown, MB, Haack, KKV, Pollack, BP, et al. Low abundance of sweat duct Cl− channel CFTR in both healthy and cystic fibrosis athletes with exceptionally salty sweat during exercise. American Journal of Physiology. http://ajpregu.physiology.org/content/300/3/R605. 2011; 30: R605-R615. DOI: 10.1152/ajpregu.00660.2010.   • National Heart, Lung, and Blood Institute. How is cystic fibrosis diagnosed? National Institute of Health.http://www.nhlbi.nih.gov/health/health-topics/topics/cf/diagnosis.html. Published December 26, 2013. Accessed December 29, 2013.    • US National Library of Medicine. The role of cftr in bicarbonate secretion by pancreatic duct and airway epithelia. National Institute of Health. http://www.ncbi.nlm.nih.gov/pubmed/20224219. Published 2009. Accessed December 29, 2013.   • Mayo Clinic Staff. Cystic fibrosis: disease complications. Mayo Clinic. http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/complications/CON-20013731. Published June 13, 2012. Accessed December 22, 2013. • American Lung Association. Cystic fibrosis: state of lung disease in diverse communities. http://www.lung.org/assets/documents/publications/solddc-chapters/cf.pdf. Published 2010. Accessed January 7, 2014.

  44. References • Lucile Packard Children’s Hospital Pediatric Pulmonogists Team. Pulmonary medicine and cystic fibrosis. Stanford Children’s Health. http://www.lpch.org/clinicalSpecialtiesServices/COE/PulmonaryCareCF/team.html. Accessed January 4, 2014. • Accurso FJ, Castellani C, Cutting GR, et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: cystic fibrosis foundation consensus report. J Pediatrics. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2810958/pdf/nihms125156.pdf. 2008; 153(2): S4-S14. DOI: 10.1016/j.jpeds.2008.05.005. • America Thoracic Society. To sweat or not to sweat: is that enough to diagnose cystic fibrosis? American Journal of Respiratory and Critical Care Medicine.http://www.atsjournals.org/doi/pdf/10.1164/rccm.201208-1510EDD. 2012; 186: 700-701. DOI: 10.1164/rccm.201208-1510ED. • Stanford University Psychology Department. Human genome, cystic fibrosis. Stanford University. http://www.stanford.edu/class/psych121/humangenome-CF.htm#sweat. Published 2010. Accessed December 22, 2013. • Johns Hopkins Cystic Fibrosis Center. Science of cf: cftr function. Johns Hopkins Medicine. http://www.hopkinscf.org/what-is-cf-teen/science-of-cf-teen/cftr-teen/function-teen/. Published 2013. Accessed December 22, 2013. • US National Library of Medicine. Medline plus: glucose test. National Institute of Health. http://www.nlm.nih.gov/medlineplus/ency/article/003482.htm. Published June 6, 2012. Accessed January 6, 2014. • US National Library of Medicine. Medline plus: cystic fibrosis lung transplant. National Institute of Health. http://www.nlm.nih.gov/medlineplus/ency/article/000107.htm. Published August 8, 2010. Accessed January 6, 2014.

  45. References • Chenoweth, R. H., Garwick, A. E., Lawler, M. R., et al. Normal and therapeutic nutrition 17th edition. NY, NY: Macmillan Publishing Company; 1986. • Feranchak, A. P., Quinton, H., Stallkings, V. A., et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency. Journal of American Dietetic Association. May 2008; 108: 1-8. • Academy of Nutrition and Dietetics. Nutrition care manual: cystic fibrosis nutrition therapy. Eatright.org; 2014. Chicago, IL 2014. https://www.nutritioncaremanual.org/content.cfm?ncm_content_id=89545&highlight=cystic%20fibrosis. Accessed January 9, 2014. • Ittenbach, R. I., Olsen, I. E., Schall, J. I., et al. Evaluation of formulas for calculating total energy requirements of adolescents and children with cystic fibrosis. The American Journal of Clinical Nutrition. 2007; 85: 144-151. • Kains, D. and Wilchanski, M. Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies. National Institute of Health. 2012; 6: 151-161. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3392141/. Accessed January 9, 2014. • Baker, R. D., Borowitz, D., and Stallings, V. Consensus report on nutrition for pediatric patients with cystic fibrosis. Journal of Pediatric Gastroenterology and Nutrition. 2010; 35: 246-259. http://www.cff.org/UploadedFiles/treatments/CFCareGuidelines/Nutrition/Consensus-Report-on-Nutrition-for-Pediatric-Patients-with-CF-JPGN-Sep-2002.pdf. Accessed January 11, 2014. • Pohl J. Nutrition in cystic fibrosis. Practical Gastroenterology Journal. 2010; 8:20-27.

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