Assessing efficacy of high-frequency chest wall oscillation in patients with familial dysautonomia.
OBJECTIVE
To determine the benefits of daily use of high-frequency chest wall oscillation (HFCWO) in familial dysautonomia (FD) patients with lung disease.


METHODS
Pulmonary function tests, chest radiographs, and blood tests were performed on entry to the study. A retrospective chart review of 12 months prior to entry provided baseline data regarding respiratory illnesses, medications, doctor visits, hospitalizations, and absenteeism. Daily logs provided prospective data on these parameters as well as HFCWO usage. Evaluations were performed at 1, 3, 6, 9, and 12 months for pulse oximetry, spirometry, and log review. At the exit evaluation, blood tests and chest radiographs were repeated.


METHODS
Fifteen FD patients with history of lung disease requiring daily inhalation therapy (7 female and 8 male; age range, 11 to 33 years) were enrolled in a 1-year clinical trial of HFCWO therapy. Two subjects withdrew after 3 months and 6 months, respectively. Each individual served as his/her own control.


RESULTS
Oxygen saturation improved by 1 month (median, 97.5%; interquartile range [IQR], 96 to 98%; vs median, 94%; IQR, 89 to 96%) and was sustained at exit evaluation (median, 98%; IQR, 98 to 98%) [p = 0.004]. Median FVC and peak expiratory flow rate (PEFR) were the pulmonary function measures with sustained improvement from baseline to exit (p = 0.02 and p = 0.03, respectively). When retrospective and prospective data were compared, all measured health outcomes improved significantly, including pneumonias (p = 0.0156), hospitalizations (p = 0.0161), antibiotic courses (p = 0.0005), antibiotic days (p = 0.0002), doctor visits (p = 0.0005), and absenteeism (p = 0.0002).


CONCLUSIONS
In this limited study of FD patients, HFCWO effected significant improvements in all measured health outcomes and oxygen saturation; FVC and PEFR were the pulmonary function measures demonstrating sustained improvement.
Giarraffa
Philip
P
Dysautonomia Center, New York University Medical Center, New York University School Medicine, NY, USA.
Berger
Kenneth I
KI
Chaikin
Alice A
AA
Axelrod
Felicia B
FB
Davey
Cynthia
C
Becker
Brian
B
D000293:Adolescent; D000328:Adult; D035641:Chest Wall Oscillation; D002648:Child; D004402:Dysautonomia, Familial / Q000453:epidemiology*; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000453:epidemiology / Q000628:therapy*; D008297:Male; D010092:Oximetry; D010100:Oxygen / Q000097:blood; D010366:Peak Expiratory Flow Rate; D011446:Prospective Studies; D012008:Recurrence; D012129:Respiratory Function Tests; D040443:Students, Public Health; D016896:Treatment Outcome; D014797:Vital Capacity
D010100:Oxygen