Clinical Case Study: Cystic fibrosis Carrie Draney

Patient Profile: KS 24 Years Old Female Caucasian Single LDS Lives with her family

Past Medical History Cystic Fibrosis Pancreatic Insufficiency Kidney Stones PEG placement

KS Previous Admits Cystic fibrosis exacerbation w/ pseudomonas MRSA Pancreatic insufficiency Uses PERT with very meal Nephrolithiasis

Weight trends: slightly variable but overall stable PEG tube replacement (original tube placed 1 year earlier) Home G-tube feeding of Nutren 1.0 not tolerated Started 2 bottles of Naked juice each night Provides: 840 kcals w/60g protein Weight trends: slightly variable but overall stable

Cystic Fibrosis Autosomal recessive disorder Different mutations in the CFTR gene Controls the flow of water and chloride ions Exocrine glands are affected Respiratory tract, sweat, salivary glands, intestines, pancreas, liver, reproductive tract Secrete abnormally thick mucus that obstructs glands and ducts in various organs

Nutritional Effects Profound effect on the digestive system 85-90% patients experience pancreatic insufficiency Maldigestion of food Malabsorption of nutrients Decreased bicarbonate production Decreased bile acid reabsorption (fat malabsorption) Meconium ileus Thicker meconium when born causing GI blockage

Prognosis Once thought to be a pediatric disease 42% of cases live past 18 years of age Median predicted survival age is close to 40

Present Illness Chief complaint: Increasing shortness of breath and cough related to cystic fibrosis Presented with: increasing cough and sputum production but w/o fevers or chills. Complaints of weight decreasing No GI complaints of N/V, diarrhea, or constipation

Physical Examination Vital Signs: saturation were 91% on room air Normal is 95-100% on room air Lungs: clear to auscultation and percussion Abdomen: Bowel sounds present. PEG site looked well maintained Metabolic: Presented with eosinophilia

Lab values Vitamin K: 0 undetectable Vitamin A: 100 ug/dL Sputum cultures: Pseudomonas + Albumin: 3.7 g/dL Prealbumin: 18 mg/dL HgbA1c: 5.6% Check glucose and HgbA1c because often diabetes is related to cystic fibrosis.

Nutritional Status Pt. had no complaints about food and was very knowledgeable about her nutritional needs Complaints of minor weight loss Current weight 48.5kg/106.9lbs BMI 17.3kg/m2

Treatments- Medication 14 day treatment plan Meropenem, tobramycin, cefazolin (Antibiotics) Prednisone Therapeutic Multivitamin w/ Mineral PERT Cholecalciferol (Vitamin D3) Vitamin A Zinc Gluconate Vitamin K Prednisone to decrease inflammation related to CF. Patient tolerated prednisone treatments.

Nutrition Assessment Anthropometrics Day 2 Ht: 5’ 5’’ BMI: 17.8kg/m2 Admit weight: 48.5kg/106.9lbs Previous admit weight: 49.9kg/108.9lbs Day 6: 49.9kg/110lbs BMI: 17.8kg/m2 IBW: 85%

Biochemical Undetectable vitamin K levels Albumin: 3.7 g/dL WNL Prealbumin: 18 mg/dL Measurement indicates PO meets minimal nutrition needs.

Nutrition focused Physical Findings Petite Skin unremarkable with G-tube in place

Estimated Needs Using Admit wt: 48.5kg Needs based on weight repletion Kcals Kcals/kg Protein Protein g/kg mL Fluid 1ml/kcal Based on 2180-2425 45-50 58-68g 1.2-1.4 2180 ABW 48.5kg

Nutrition Diagnosis PES: Increased nutrient needs related to cystic fibrosis as evidenced by BMI of 17.5kg/m2 and mild weight reduction.

Nutrition Intervention Regular diet w/café options Pt. advised to eat any food from outside the hospital MVI w/ mineral supplement Vitamin A, Vitamin D, Vitamin K Nocturnal TF w/ 2 bottles naked juice Has tried other oral supplements and has had severe intolerance. Weight stayed the same when she switched from Nutren to Naked Juice and feels better.

Nutrition Monitoring and Evaluation Monitor pt. PO intake Daily wt. check using a standing scale Obtain weekly prealbumin measurement

Appropriateness of Nutrition Care KS received appropriate treatment for infections and CF exacerbation Nutritional care was appropriate. Pt. was very aware of dietary needs for CF. Future admits are expected

References Cystic Fibrosis Foundation. About cycstic fibrosis. https://www.cff.org/What-is-CF/About-Cystic- Fibrosis. Accessed October 21, 2015. Mueller DH. Medical nutrition therapy for pulmonary disease. In: Mahan LK, Escott-Stump S, ed. Krause’s Food, Nutrition, & Diet Therapy. 12th ed. Philadelphia: Elsevier; 2008: 790-794. Pagana KD, Pagana TJ. Manual of Diagnostic and Laboratory Test. 5th ed. St. Louis, MO: Elsevier;2014.