1. Marion Viglietta UMD College Park Dietetic Intern

2.  Overview of Primary Ciliary Dyskinesia  Assessment of Patient  PES Statement and Needs  Plans & Goals  Evaluation and Follow-Up

3.  Autosomal recessive genetic disorder  Often seen in siblings  Immotile cilia  Chronic recurrent lung infections  Treatment much like CF Nebulizers, bronchodilators, nasal steroids, etc. No pancreatic insufficiency (PI)  Near normal life expectancy

4.  14 y/o F admitted with Primary Ciliary Dyskinesia (PCD) exacerbation and 6.9 kg weight loss since 06/2011 admission  PMH: PCD, FTT, pHTN, Brconchiectasis, Left Lower Lobectomy, Reflux  Social Hx: Family from Algeria Anthropometrics Height159.9 cm25 th - 50 th %ile Weight38.8 kg<3 rd %ile BMI15.2<3 rd %ile

5.  PES: Inadequate oral food/beverage intake related to high calorie needs from PCD exacerbation and severely decreased lung function as evidenced by 6.9 kg weight loss in 9.5 months and patient BMI < 3 rd percentile

6. Growth Chart

7. MedicationsDosage Albuterol Ceftazidime Vitamin D4000 IU daily Lansoprazole Megestrol400 mg daily Methylprednisone MVI1 tab daily Tobramycin Labs Na139mEq/L K3.6mEq/L Cl102mEq/L CO 2 24mmol/L BUN13mg/dL Creatinine0.4mg/dL Glucose102mg/dL Vitamin D16ng/mL Vitamin A28ng/mL Zinc54mcg/dL Prealbumin15.8

8. Needs Energy2483 kcalDRI x 2; 64 kcal/kg Protein58.2 g1.5 g/kg Fluid1880 mLHolliday-Segar

9. 1.Oral nutrition: High-calorie, high-protein diet with chocolate Pediasure TID (750 kcal) to supplement. Monitor and encourage PO intake. Nurses to record food and beverage intake. 2.Increase Megace dosage to 400 mg daily to promote PO intake 3.Check weight daily to assess growth. Goal is weight gain on this admission. 4.If PO intake does not improve, Megace dosage may need to be increased to 800 mg daily 5.Medical team strongly believes patient is a candidate for G tube

10. DateMealFood 4/16/12Breakfast3 pancakes, oatmeal, cereal Afternoon SnackTurkey and cheese sandwich, sour cream and onion chips Dinner1 McChicken, small fries, 1 hot dog, 1 chicken tender, ½ serving mashed potatoes, 1 lasagna, 1 green beans Evening Snack1 cookie 4/17/12Breakfast2 pieces French toast, 1 pack Lucky Charms, Boost shake Morning SnackBoost Shake Lunch½ piece pizza, ½ turkey sandwich, portion spaghetti and meatballs, 1 large cookie, ¾ bag sour cream and onion chips, 2 hot dogs Afternoon Snack¼ large bag sour cream and onion chips Dinner2 pieces chicken, 1 Boost shake, 1 serving mac and cheese, 1 serving egg noodles, 3.5 bag sour cream and onion chips, broccoli and cauliflower serving

11.  Patient with 2.7 kg weight gain in 4 days  PO intake increasing  Megace effective  Monitor/encourage PO intake  Monitor weight gain

13. Block, R.W., Krebs, N.F, et al (2005) Failure to Thrive as a Manifestation of Child Neglect Pediatrics Official Journal of the American Academy of Pediatrics 116;1234 DOI: 10.1542/peds.2005-2032 Frequently Asked Questions Primary Ciliary Dyskenesia Foundation website 2011 Mackerness, K. J., Jose, P. J., & Bush, A. A. (2009). Differences in airway inflammation in cystic fibrosis and primary ciliary dyskinesia. Pediatric Asthma Allergy & Immunology, 22(4), 163-168. doi:10.1089/pai.2009.0022

14. Questions?