Page 27 - Delaware Medical Journal - May/June 2019
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CASE REPORT
INTRODUCTION
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.2-5 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
failure.1 Staphylococcus aureus usually reside below the mucus and can grow uncontrollably in infants and children with CF. 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, agents, chest physiotherapy, and inhaled medications to improve mucus clearance.8
States have CF, and about 1,000 new 1 percent of CF patients are diagnosed
as a newborn, and 75% of cases are detected by two years of age.9 Half of the individuals with CF are 18 years of age or older.1,9 High allele frequencies (1:20) are observed in a Caucasian population, with 9-11
The Amish community was a part of the Swiss-German migration to North America in the 17th century.10 Their approximate They hold different lifestyle philosophies relative to health care beliefs, insurance, and religion. They use traditional farming transportation.
Figure 3 Physical activity classification in both groups
TABLE 1: Demographics of the Amish and non-Amish groups.
Baseline Characters (n=25)
Amish Group (n=9)
Non-Amish Group (n=16)
Sex (% male)
33%
37.50%
Average Age
7
7.75
% With Insurance
55%
100%
% With Electricity*
11%*
100%*
% With Well Water*
100%*
31%*
% Given Oral Supplements
88%
50%
* indicates statistically significant difference for numbers between Amish and non- Amish groups (p<0.05)
Estimating the number of Amish
affected with CF is challenging, as this population is primarily understudied and underreported.14 Previous research from Ohio in one Amish community reported amongst the population was 1 in every 569 live births. There were no reported in the second Amish community. However, higher rates of CF exist amongst the Amish people compared to the general population.
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 one second (FEV1), forced
of airway microbial colonization, and changes in antibiotic treatment regimen compared to the non-Amish community.
MATERIALS AND METHODS
Patients and study design
Study protocol approval was obtained from Lutheran Hospital’s institutional review board before initiation.
This single site, open-labeled, non- interventional, retrospective case-
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