Mechanical insufflation-exsufflation for airway mucus clearance.

Cough is an important component of airway clearance, particularly in individuals with intrinsic pulmonary disease, weakness of respiratory muscles, or central nervous system disease that impairs breathing. The use of assisted cough to enhance airway clearance in individuals with neuromuscular disease is essential to produce and maintain peak cough flow above a minimum and thereby avoid retained secretions that cause infection, inflammation, and respiratory failure. Periodic insufflation of the lung above a reduced vital capacity is also important, to maintain range of motion of the thoracic cage and avoid progressive respiratory disability. Mechanical insufflation-exsufflation is a therapy in which the device (the CoughAssist In-Exsufflator is the only currently marketed insufflation-exsufflation device) gradually inflates the lungs (insufflation), followed by an immediate and abrupt change to negative pressure, which produces a rapid exhalation (exsufflation), which simulates a cough and thus moves secretions cephalad. Mechanical insufflation-exsufflation is used with patients with neuromuscular disease and muscle weakness due to central nervous system injury. Insufflation-exsufflation decreases episodes of respiratory failure, particularly during upper-respiratory-tract infection, and provides greater success in weaning from mechanical ventilation than do conventional methods. Alternatives to insufflation-exsufflation that can produce sufficient peak cough flow for airway clearance include (1) insufflation to maximum insufflation capacity (via breath-stacking with a bag and mask, a volume ventilator, or glossopharyngeal breathing) followed by a spontaneous cough, and (2) manually assisted cough with an abdominal thrust. The effectiveness of insufflation-exsufflation in patients with obstructive lung disease, such as chronic obstructive pulmonary disease or asthma, and in pediatric patients, is less clear.

The effect of a first-generation antihistamine on sputum viscoelasticity in cystic fibrosis.

Tenacious airway secretions are responsible for much of the lung damage in cystic fibrosis (CF). Label warnings on potential secondary effects of some antihistamines include possible drying or thickening of lower airway secretions, suggesting that they are detrimental to individuals with airway disease. We studied the effects of cyproheptadine hydrochloride (CH) on sputum weight, viscoelasticity, and transportability in CF patients participating in a pilot trial of CH as an appetite stimulant to assure no potential adverse secondary effects on mucus clearance. Sixteen clinically stable subjects were randomized to receive either CH (2 mg QID for 1 week followed by 4 mg QID for 11 weeks) or placebo. Sputum was obtained by voluntary forced cough and expectoration prior to starting CH or placebo and at 4 weeks. Viscoelasticity was measured by rheometry and cough transportability by simulated cough machine. Sufficient paired sputum for rheologic analysis was obtained on four placebo and seven CH subjects and for cough transportability analysis on three placebo and six CH subjects. Weight on all specimens was obtained prior to both analyses. There were no significant differences in sputum weight wet, measures of mucus viscoelasticity (rheology), or cough transportability of mucus between baseline and 4 weeks in patients on placebo or CH. From this limited study, CH, a first-generation antihistamine, appears to have no adverse effects in sputum viscoelasticity or cough transportability in CF patients.

Parent-reported physician diagnosis is an important factor in asthma management: an elementary school survey.

Parent surveys seem to provide sufficient information for asthma case selection. In this study, elementary school children were identified for an asthma education program through a screening questionnaire, followed by a comprehensive family survey. The data were analyzed by groups according to those with parent-reported physician diagnosis or no physician diagnosis. Those with an asthma diagnosis had less nighttime symptoms, less family stress, and more asthma follow-up visits, and were prescribed appropriate medications and peak flow meters more often than those students without a physician diagnosis of asthma. A physician commitment to or recognition of an asthma diagnosis is an important factor in subsequent asthma care.

Long-term trial of cyproheptadine as an appetite stimulant in cystic fibrosis.

Appetite stimulants have been used to help overcome decreased appetite and malnutrition in children and adults with various chronic illnesses, including cystic fibrosis (CF). Stimulants have included megestrol acetate (MA), cyproheptadine hydrochloride (CH), cannabinoids, hydrazine sulfate, anabolic hormones, and growth hormone. Many of these, including MA, have substantial side effects and may not be suitable for prolonged use. We previously studied the effects of CH on weight gain in a short-term (12 week) trial in CF with good results compared to placebo. Side effects were few, and weight gain was significant. In this study, we sought to determine the effects of CH over a longer term in order to assess its suitability for prolonged use. Sixteen CF children and adults enrolled in the original short-term study subsequently entered this study, and 12 completed the 9-month trial. All patients receiving placebo in the original short-term study received CH 4 mg up to four times a day in the long-term study continuation, and those receiving CH in the short-term study continued on the drug. No pill counts were done, and patients were queried at quarterly visits as to their CH use. Anthropometrics and spirometry were also done quarterly, and antibiotic use was quantified. Subjects who had changed from placebo (CH2 group) gained weight significantly over 3-6 months, and those continuing on CH (CH1 group) generally maintained previously gained weight over the duration of the study. Select spirometric measures improved in both groups but not significantly, and side effects were mild. CH appears to be an effective appetite stimulant in CF, and generally maintains its effect over time with an acceptable side-effect profile.

The effect of therapeutic horseback riding on balance in community-dwelling older adults: a pilot study.

Equine assisted activities (hippotherapy and therapeutic riding) improve balance in patients with disabilities such as cerebral palsy, but have not been systematically studied in older adults, at risk of falls due to balance deficits. We conducted a 10-week, single blind, controlled trial of the effect of a therapeutic horseback riding course on measures of balance in community-dwelling adults 65 years and older. Nine riders and six controls completed the trial. Controls were age matched to riders and all participants were recruited from the local community. Both groups showed improvements in balance during the trial, but did not reach statistical significance. Sample size was small, participants had relatively high initial balance scores, and controls tended to increase their physical activities, likely influencing outcomes. No adverse events occurred and the supervised therapeutic riding program appeared to be a safe and effective form of exercise to improve balance in older adults. A power analysis was performed to estimate numbers of participants needed for a larger study.

Pulmonary function and sputum production in patients with cystic fibrosis: a pilot study comparing the PercussiveTech HF device and standard chest physiotherapy.

STUDY OBJECTIVE: To compare the PercussiveTech HF (PTHF) device (Vortran Medical Technology 1; Sacramento, CA) to standard manual chest physiotherapy (CPT) with respect to acute changes in pulmonary function, sputum production, and pulse oximetry in patients with cystic fibrosis (CF). DESIGN: Randomized crossover. SETTING: University-affiliated, community-based CF center. PARTICIPANTS: Ten clinically stable patients with CF (age range, 10 to 21 years; mean age, 15.3 years) with Shwachman scores from 55 to 95 (mean 75). INTERVENTIONS: Two treatment regimens were used: 2.5 mg of albuterol delivered via updraft nebulizer followed by standard CPT, and 2.5 mg of albuterol delivered via the PTHF device without CPT. RESULTS: Outcome measures included pulmonary function test (PFT) results 4 h after treatment and quantitative sputum production during the 4 h after treatment. Pulse oximetry was performed during treatment. A patient satisfaction questionnaire was administered at the end of the study. No PFT parameters were significantly changed 4 h after CPT or PTHF, although there was a trend to decreasing residual volume after both treatments. There was a trend for more sputum production after PTHF compared to CPT, but this did not reach statistical significance. There were no episodes of hemoglobin-oxygen desaturation during or after either treatment. One patient had minor hemoptysis after CPT. No adverse effects occurred after PTHF. Eight patients found the PTHF device easy to use, and six patients would prefer the PTHF device to CPT. CONCLUSIONS: The PTHF device appears to be a safe and effective method of airway clearance in CF patients in this small pilot study.

Cyproheptadine is an effective appetite stimulant in cystic fibrosis.

Chronic pulmonary infection and intestinal malabsorption often lead to malnutrition in children and adults with cystic fibrosis (CF). Appetite stimulants, along with provision of adequate calories, may aid in overcoming nutritional deficits, allowing a better prognosis. We undertook a trial of cyproheptadine hydrochloride (CH) to determine its effectiveness as an appetite stimulant in 18 adults and children with CF. This was a 12-week, randomized, double-blind, controlled trial of CH vs. placebo. Eighteen subjects with documented CF (sweat or genetics positive), minimum age of 5 years, and ideal body weight for height <100% were entered, and 16 completed the study. Subjects were seen at baseline and every 4 weeks. Measures included baseline demographics, Shwachman score, anthropometrics (weight, height, body mass index, skin folds, and body composition by bioelectric impedance analysis), spirometry, caloric intake, days of oral (PO) and intravenous (IV) antibiotics, and a symptom and satisfaction survey. Subjects in the CH group showed significant increases in weight (mean 3.45 kg vs. 1.1 kg in the placebo group), height, BMI percentiles, ideal body weight/height, weight for age z-scores, and fat and fat-free mass. There were no changes or differences in PO or IV antibiotic use or spirometric changes. No significant side effects except transient mild sedation occurred in the CH group. Patient acceptance was good. In conclusion, CH appears to be an effective appetite stimulant with minimal side effects in children and adults with CF.

Effect of therapeutic horseback riding on balance in community-dwelling older adults with balance deficits.

OBJECTIVES: Falls are an important cause of morbidity in older adults. Equine-assisted activities including therapeutic riding (TR) benefit balance and neuromuscular control in patients with neurological disabilities but have not been systematically studied in older adults at greater risk for falls due to balance deficits. The effect of an 8-week TR program on measures of balance and quality of life in community-dwelling older adults with established balance deficits was evaluated. DESIGN: This was a pretest-post-test single-group trial of a TR program on measures of balance and quality of life. SETTING: The study was conducted at a Professional Association of Therapeutic Horsemanship (PATH) International Premier riding center. SUBJECTS: The subjects comprised 9 adults (5 female, 4 males) with a mean age 76.4 years (range 71-83 years). INTERVENTIONS: This included an 8-week observation period followed by an 8-week TR program consisting of 1 hour per week of supervised horseback riding and an 8-week follow-up period. SUBJECTS received balance testing at weeks 0, 8, 16, and 24 using the Fullerton Advanced Balance Scale (FABS), and quality of life was measured at weeks 8 and 16 using the Rand SF (short form) 36 quality-of-life measure. OUTCOME MEASURES: OUTCOME MEASURES were change in the FABS and Rand SF 36. RESULTS: There was no significant difference in balance scores between the start and end of the observation period. There was a significant improvement in the balance score and perception of general health from the start to the end of the intervention period, and no significant difference between the end of the intervention and the end of study, suggesting that improvements may have been sustained. CONCLUSIONS: TR is a safe activity for older adults with mild to moderate balance deficits and leads to both improvements in balance and quality of life. Longer and larger studies to assess the benefit of equine-assisted activities on improvements in balance and reduction in fall risk are needed.

Evaluation of genetic counseling among cystic fibrosis carriers, Michigan Newborn Screening.

OBJECTIVE: A quality improvement (QI) strategy to improve the rate of genetic counseling (GC) services was initiated in cystic fibrosis (CF) care Center E in 2010. This statewide study was conducted to determine: (1) GC rates before and after implementation of the QI strategy at Center E; (2) characteristics associated with not receiving GC; and (3) topic areas addressed during GC. METHODS: The retrospective study included 1,097 CF carriers born from 2008 to 2011 identified through Michigan's Newborn Screening Program. Rate of GC services was determined for Center E and the other four CF centers before and after the QI change. Bivariate and multivariable logistic regression was used to determine associations between select characteristics and not receiving GC. Topic areas discussed during GC sessions were assessed using frequency tables. RESULTS: Rate of GC services in Center E increased from 23% in 2008-2010 to 91% in 2011, while at the other centers approximately 92% received GC services across those years. In 2008-2010, being seen at Center E and black race were significantly associated with increased likelihood of not receiving GC services in adjusted analyses. In 2011, neither characteristic was associated with receipt of GC. Of 16 target topic areas, all were discussed in 85% of GC sessions. CONCLUSIONS: Implementing a QI strategy of providing sweat test results at the GC appointment within Center E resulted in more CF carriers receiving comprehensive GC services. Center-specific procedure differences should be assessed to increase rate of GC services following a positive CF newborn screen.

Evaluation of serum concentrations achieved with an empiric once-daily tobramycin dosage regimen in children and adults with cystic fibrosis.

OBJECTIVES: To assess the ability of an empiric once-daily dosing (ODD) tobramycin regimen to achieve desired serum concentrations in patients with cystic fibrosis (CF); to determine an optimal dosage regimen, using pharmacodynamic parameters; and to evaluate clinical response, adverse effects, and patient/parent satisfaction with ODD. METHODS: This was a prospective single-center trial in patients with CF who are 5 years of age and older requiring intravenous antibiotics. Tobramycin, 10 mg/kg every 24 hours, was infused over 60 minutes, and two serum concentrations were analyzed using 1-compartment pharmacokinetic modeling. Simulations were performed to identify dosage regimens that maximized desired pharmacodynamic parameters. Other data included demographics, symptoms, spirometry, adverse events, and satisfaction with ODD. RESULTS: A total of 14 children and 11 adults completed the study. Empiric doses resulted in mean peak tobramycin concentrations of 28.7 ± 5.5 mg/L and undetectable trough concentrations. Only 42% of patients achieved desired peak serum concentrations (20-30 mg/L) with the empiric regimen. A regimen of 12 mg/kg every 24 hours would achieve modified pharmacodynamic goals with an acceptable peak range of 20 to 35 mg/L. Forced expiratory volume in 1 second improved in 15 of 20 (75%) patients with ODD. Two patients experienced reversible vestibular adverse effects attributed to tobramycin. All patients were satisfied or very satisfied with ODD because of convenience and ease of use. CONCLUSIONS: An empiric tobramycin regimen of 10 mg/kg every 24 hours did not achieve desired serum concentrations for most patients, although all patients demonstrated clinical improvement. Desired tobramycin concentrations with modified pharmacodynamic goals could be achieved by using an empiric dosage of 12 mg/kg every 24 hours. Prospective evaluation of this regimen with individualized patient monitoring is needed to ensure safety and efficacy and to monitor the effects on microbial resistance patterns.

Predictors of insufficient sweat production during confirmatory testing for cystic fibrosis.

Michigan's Newborn Screening (NBS) Program began statewide screening for cystic fibrosis (CF) in October 2007. Confirmatory sweat testing is performed in infants having initial immunoreactive trypsinogen concentrations ≥ 99.8th percentile or ≥ 96 th percentile and at least one CF mutation identified by DNA analysis. Some infants fail to produce a sufficient quantity of sweat (QNS-quantity not sufficient) to test for CF, meaning disease confirmation is delayed and sweat testing is later repeated. In this study, we evaluate predictors of QNS results. Information from the linked birth certificates and NBS diagnostic confirmation data were used. The study population was resident infants born in Michigan in 2008 who underwent a sweat test. Bivariate analyses revealed that preterm birth, low birth weight, CF care center, and race were significantly associated with QNS sweat testing results. Adjusted analyses indicated that preterm infants were 2.4 times more likely to have QNS results (95% CI 0.9, 6.4). When age at time of test, accounting for gestational age (gestational age at delivery plus postdelivery age of life=corrected age), was used in the multivariable model, infants <39 weeks were 7.4 times more likely to have QNS results (95% CI 2.5, 21.8). Waiting to sweat test until an infant is aged 39 weeks or more (corrected age) would likely reduce the rate of QNS results, thereby reducing the burden of repeat sweat testing on families and healthcare providers. Further research is necessary to understand the impact of potential delays in diagnosis/treatment relative to postponing sweat testing.

Making airway clearance successful.

Airway clearance therapy (ACT) requires a substantial time, effort and resource commitment on the part of families and patients with cystic fibrosis (CF). Lack of compliance with caregiver recommendations and adherence to a proposed therapeutic plan occurs more than 50% of the time with ACT and is particularly common in adolescents. There is a large menu of ACT devices and techniques from which to choose. Increasing adherence and compliance requires an effort on the part of the CF team, including regular visits to the CF center, matching the patient to the device or technique based on lifestyle and disease severity, selecting appropriate outcome measures and monitoring adherence and compliance. Successful ACT also requires the CF team to understand individual patient situations, individualize ACT programs and accept a realistic level of patient participation.