Pediatric Case StudyPrimary Ciliary Dyskinesia

Marion VigliettaUMD College Park Dietetic

Intern

Objectives Overview of Primary Ciliary

Dyskinesia Assessment of Patient PES Statement and Needs Plans & Goals Evaluation and Follow-Up

Primary Ciliary Dyskinesia 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

Initial Assessment 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

AnthropometricsHeight 159.9 cm 25th - 50th %ile

Weight 38.8 kg <3rd %ile

BMI 15.2 <3rd %ile

PES Statement

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 < 3rd percentile

Growth Chart

Labs & Medications

Medications DosageAlbuterolCeftazidimeVitamin D 4000 IU dailyLansoprazoleMegestrol 400 mg dailyMethylprednisoneMVI 1 tab dailyTobramycin

LabsNa 139 mEq/LK 3.6 mEq/LCl 102 mEq/LCO2 24 mmol/LBUN 13 mg/dLCreatinine 0.4 mg/dLGlucose 102 mg/dLVitamin D 16 ng/mLVitamin A 28 ng/mLZinc 54 mcg/dLPrealbumin

15.8

Needs

Needs

Energy 2483 kcal DRI x 2; 64 kcal/kg

Protein 58.2 g 1.5 g/kg

Fluid 1880 mL Holliday-Segar

Plans/Goals1. 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

Date Meal Food

4/16/12 Breakfast 3 pancakes, oatmeal, cerealAfternoon Snack Turkey and cheese sandwich, sour cream and

onion chips

Dinner 1 McChicken, small fries, 1 hot dog, 1 chicken tender, ½ serving mashed potatoes, 1 lasagna, 1 green beans

Evening Snack 1 cookie4/17/12 Breakfast 2 pieces French toast, 1 pack Lucky Charms,

Boost shake

Morning Snack Boost ShakeLunch ½ piece pizza, ½ turkey sandwich, portion

spaghetti and meatballs, 1 large cookie, ¾ bag sour cream and onion chips, 2 hot dogs

Afternoon Snack ¼ large bag sour cream and onion chips

Dinner 2 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

Nutritional Evaluation Patient with 2.7 kg weight gain in 4

days PO intake increasing Megace effective

Monitor/encourage PO intake Monitor weight gain

Follow Up Plans…

Health of the PatientPCD

FTTBronchiectasis Left Lower Lobectomy

Improper management of care

ReferencesBlock, 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

Questions?