Comparison of Homozygous for Delta F508 Mutation Nutritional Status, Lung Function Outcomes, Bacterial Growth, Antibiotic Treatments and Therapeutic Outcomes between the Amish and Non-Amish in a Single Pediatric Cystic Fibrosis Centre: A Retrospective Case-Controlled Study

Rajeswary Padman Padmalingam,Tina Laturner,Alyaa Hussien,Gordon Bokhart,Abeer Omar,Chelsea Hamilton,Rachel Mccafferty,Denise Gilham,Ron Jones

doi:10.35248/2572-0775.19.4.151

Abstract

There exists evident differences in the health and the care received of the Amish people in comparison to the general population. Amish patients were 100% homozygous for delta F508 mutation versus 12% in the non-Amish population (P=0.057). The Amish community was prescribed dietary supplements at a rate of 89% versus 50% for non-Amish (P=0.087). BMI > 75th percentile was 51.25% in Amish patients and 61.25% in non-Amish patients (P=0.31). Mean FEV1 value was 83.6% versus 100% while mean FEF 25-75 value was 58% versus 86% for Amish and nonAmish people respectively. There was substantial variance in the number of episodes of the same infection per patient between Amish and non-Amish population (1 IQR 0–1 versus 0 IQR 0–1; p=0.012). The rate of antibiotic prescribing per patient in the Amish group was 7.9 perceptions versus 2.2 prescriptions. The study aims to create the best possible treatment and the highest quality of life for patients with cystic fibrosis. Our manuscript creates a paradigm for future studies on the delta F508 mutation, nutrition supplements, body mass index (BMI), forced expiratory volume in 1 second (FEV1), forced expiratory flow (FEF 25-75), airway microbial organisms and changes in antibiotic treatments in Amish compared to the non-Amish group, as well as developing a platform for an Amish community health program.

Highlights

Cystic Fibrosis (CF) is a progressive, hereditary illness that causes persistent lung infections and curbs the ability to breathe over time [1]
The aim of this study was to explore the clinical manifestations and outcomes in the Amish community with CF regarding the delta F508 mutation, nutrition, Body Mass Index (BMI), forced expiratory volume in 1 second (FEV1), forced expiratory flow (FEF 25-75), patterns of airway microbial colonization and changes in antibiotic treatment regimen compared to the non-Amish community
The primary outcomes investigated included homozygous for delta F508 mutation, nutrition, Body Mass Index (BMI), Forced Expiratory Volume in 1 second (FEV1), Forced Expiratory Flow (FEF 25-75), airway microbial organisms and changes in antibiotic treatments in Amish compared to the non-Amish group

Summary

Introduction

Cystic Fibrosis (CF) is a progressive, hereditary illness that causes persistent lung infections and curbs the ability to breathe over time [1]. Despite the same genetic mutations, the disease expression is variable and depends on environmental, cultural, behavioral, psychosocial, and economic background [2,3,4,5]. Pulmonary disease is the most common cause of morbidity and mortality in patients with CF [6]. Mucus secretion increases and traps bacteria causing infections, expanded lung damage, and respiratory failure [1]. Pseudomonas aeruginosa often affects patients throughout adulthood [7]. With the advent of new bacterial culture methods, it is evident that the airways of patients with CF are colonized recurrently with polymicrobial infections [7]. Treating infections in CF is multifarious, comprising antibiotics, anti-inflammatory agents, chest physiotherapy, and inhaled medications to improve mucus clearance [8]

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