Quality of locally designed surveys in a quality improvement collaborative: review of survey validity and identification of common errors.

OBJECTIVE: Surveys are a commonly used tool in quality improvement (QI) projects, but little is known about the standards to which they are designed and applied. We aimed to investigate the quality of surveys used within a QI collaborative, and to characterise the common errors made in survey design. METHODS: Five reviewers (two research methodology and QI, three clinical and QI experts) independently assessed 20 surveys, comprising 250 survey items, that were developed in a North American cystic fibrosis lung transplant transition collaborative. Content Validity Index (CVI) scores were calculated for each survey. Reviewer consensus discussions decided an overall quality assessment for each survey and survey item (analysed using descriptive statistics) and explored the rationale for scoring (using qualitative thematic analysis). RESULTS: 3/20 surveys scored as high quality (CVI >80%). 19% (n=47) of survey items were recommended by the reviewers, with 35% (n=87) requiring improvements, and 46% (n=116) not recommended. Quality assessment criteria were agreed upon. Types of common errors identified included the ethics and appropriateness of questions and survey format; usefulness of survey items to inform learning or lead to action, and methodological issues with survey questions, survey response options; and overall survey design. CONCLUSION: Survey development is a task that requires careful consideration, time and expertise. QI teams should consider whether a survey is the most appropriate form for capturing information during the improvement process. There is a need to educate and support QI teams to adhere to good practice and avoid common errors, thereby increasing the value of surveys for evaluation and QI. The methodology, quality assessment criteria and common errors described in this paper can provide a useful resource for this purpose.

Severe lung disease in children with cystic fibrosis missed in newborn screening.

BACKGROUND: Cystic fibrosis (CF) is now routinely diagnosed through newborn screening (NBS), but the tests employed in the USA have been evolving for two decades as missed cases become recognized and lab methods improve in association with more knowledge about CF genetics. New Jersey was among the first states to implement CF NBS in 2001 when it introduced the original two-tiered method that combined measurements of immunoreactive trypsinogen (IRT) with detection of the principal pathogenic variant (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. OBJECTIVE: With continuation of the IRT/DNA (F508del) algorithm for two decades and identification of screening false negative children, we decided to examine the condition of some missed cases with special attention to their respiratory status. METHODS: To strengthen the arguments for quality improvement in New Jersey's CF NBS program, we reviewed and evaluated false negative cases to determine the potential extent of preventable patient suffering as a consequence of delayed diagnoses. RESULTS: Five children with CF who had false negative screening results were studied in detail. In each case there was a different cause of the negative screening results. They all had clinically significant/severe lung disease, ranging from chronic cough with CF pathogens on respiratory culture at a young age to respiratory failure. CONCLUSION: This case series highlights the consequences of false negative screening results, which served as the impetus to upgrade New Jersey's CF NBS algorithm. Implemented changes include lowering the IRT cutoff to 70 ng/mL and expanding to a 139 variant CFTR panel. In 2023, a floating IRT cutoff is anticipated to be implemented.

Use of medical marijuana in cystic fibrosis patients.

BACKGROUND: The usage and attitudes towards medical marijuana in Cystic Fibrosis (CF) patients is unknown. Through the use of a survey we aim to clarify rates and reasons for use. METHODS: An anonymous survey was sent out to six centers in the Mid-Atlantic region of the United States. Use of and reason for medical marijuana was assessed, along with attitudes of the perceived utility of medical marijuana. RESULTS: A total of 637 surveys were sent out, and 193 surveys were returned (30.3% return rate). Three did not give consent, and one was empty, for a total of 189 completed surveys. 31 subjects (16.5%) reported having used marijuana for medical purposes in their lifetime, with 29 (15.4%) of these in the past year. The most used forms were edible and vaporized. The most common indications for usage were pain and stress. 28 out of 31 found marijuana to be a great deal effective for their symptoms. 21 of the 31 rated marijuana very important or important to their health. There were two reported side effects, both mild. Of 156 subjects who responded to the question if they would be interested in medical marijuana if available, 72 (46.2%) replied yes. CONCLUSION: The use of marijuana for medical reasons was 15.4% in the past year in this sample CF population, although more expressed interest if it was available through prescription. Side effects were rare. CF physicians are going to have to familiarize themselves with advantages and disadvantages of medical marijuana as there is a great deal of interest within the community, and legalization becomes more common.

Daily spirometry in an acute exacerbation of adult cystic fibrosis patients.

To help answer the question of length of intravenous antibiotics during an acute exacerbation of cystic fibrosis (CF), we had subjects to follow daily home spirometry while on intravenous antibiotics. CF patients, 18 and older, with an acute exacerbation requiring intravenous antibiotics had a daily FEV1. The average time to a 10% increase over their initial sick FEV1 was calculated, as well as the time to a new baseline. A total of 25 subjects completed the study. Ten of the 25 subjects did not have a sustainable 10% increase in FEV1. Of the 15 subjects with a sustainable 10% increase in FEV1, it took 5.2 days (±4.5) after day 1, while a new baseline was achieved on average at 6.6 days (±4.8) after day 1. Given the wide range of time to a 10% improvement and new baseline, it is recommended there should be flexibility in length of intravenous antibiotics in CF, not by a preset number.

Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis.

BACKGROUND: Vitamin D deficiency is common in CF. Whether vitamin D affects pulmonary function in CF is unknown. METHODS: Data were abstracted from clinically stable CF patients who had pulmonary function studies and serum 25-hydroxyvitamin D [25(OH)D, ng/ml] levels drawn within 2 months of each other. Findings were adjusted for multiple variables known to affect pulmonary function in CF. RESULTS: Enrollees totaled 597. Overall mean 25(OH)D level was 29.6±12.8 ng/ml (SD). Serum 25(OH)D levels showed a significant correlation with forced expiratory volume in 1s (FEV1) % predicted (r=0.20, p<0.0001) and forced vital capacity % predicted (r=0.13, p=0.0019). Multivariate analysis revealed that serum 25(OH)D remained an independent predictor of FEV1 % predicted even after controlling for multiple other factors known to affect CF lung function. CONCLUSIONS: Serum 25(OH)D levels are significantly associated with pulmonary function in CF. Further study is required to determine whether this association is causal.

The impact of re-education of airway clearance techniques (REACT) on adherence and pulmonary function in patients with cystic fibrosis.

BACKGROUND: Our centre's median forced expiratory volume in one second (FEV1) reported in the 2005 Cystic Fibrosis (CF) Foundation Patient Registry was below the national median. The focus of our quality improvement initiative was to improve lung function through re-education of airway clearance techniques (REACT). AIM: The global aim was to improve the median FEV1 in our patients. The specific aim was to encourage adherence to airway clearance techniques (ACT). To achieve these goals we implemented the REACT programme for patients. METHODS: Educational sessions introduced the concept of improving clinical outcomes and the importance of airway clearance in achieving optimal lung function. The REACT programme utilised an anonymous survey, in-clinic questionnaire and ACT demonstration to assess knowledge, practices and barriers to ACT. Patients were then categorised as non-adherent or adherent with correct or incorrect technique. Improper techniques were corrected. All patients were re-educated on the rationale for ACT. RESULTS: Our surveys revealed that 43% of patients had barriers to ACT and 53% were non-adherent. Following implementation of REACT, median FEV1 increased from 84% to 92% (national median 91-94%) from 2005 to 2010 for patients aged 6-17. For patients 18 and older, median FEV1 increased from 56% to 64% (national median 62-65%) from 2005 to 2010. CONCLUSIONS: By introducing a programme focused on technique and adherence, we were able to improve median FEV1 in patients with CF. Sustained improvement of FEV1 was accomplished by continued use of the REACT programme.

The use of complementary and alternative medicine among pediatric cystic fibrosis patients.

HYPOTHESIS: The goal of this paper is to assess the use of complementary and alternative medicine (CAM) use among children with cystic fibrosis (CF) and the communication with the primary care physician. MATERIALS AND METHODS: The paper is based on data from surveys mailed to 97 patients recruited from Monmouth Medical CF Center. The questionnaire was anonymous and included 47 questions referring to demographic data (age, sex, number of years since diagnosed, state of residence, use of pancreatic enzymes, overall lung function and general health) as well as questions referring to all types of CAM practices: whole medical systems, manipulative practices, body mind medicine, biologically based practices, and energy medicine. RESULTS: The response rate was 50.5%, with 81% being pediatric patients under 18 years old. The results showed that 77% of patients use some form of CAM when the definition of CAM therapy included prayer specifically for health reasons. When prayer was excluded from the definition, the use of CAM decreased to 65%. The most commonly used CAM therapies were biologically based practices (53%) followed by body-mind medicine (49%), manipulative practices (14.5%), and whole medical systems (8%). Most of the patients interviewed did not consult a natural practitioner but considered that CAM treatments were beneficial. CONCLUSIONS: The present survey provides current data describing the use of CAM in patients with CF and adds to the increasing body of evidence about CAM use in the United States. Further research is needed to explore the relationship and effectiveness of specific types of CAM practices and their benefits on symptom relief and overall quality of life of CF patients.

Palivizumab use in subjects with congenital heart disease: results from the 2000-2004 Palivizumab Outcomes Registry.

The Palivizumab Outcomes Registry prospectively collected data on 19,548 subjects who received respiratory syncytial virus (RSV) prophylaxis with palivizumab during the 2000-2004 RSV seasons. We evaluated the characteristics of enrolled registry subjects with congenital heart disease (CHD) over the four RSV seasons and examined additional information on these subjects collected in the 2002-2004 seasons. The percentage of registry subjects with CHD increased from 4.8% (102/2116) in the first season to 11.4% (688/6050) in the last season. Across all four seasons, 1500 subjects with CHD were enrolled, 71% of whom had acyanotic CHD. The proportion with cyanotic CHD increased from 19.6% (20/102) in the 2000-2001 season to 37.5% (258/688) in the 2003-2004 season, while the proportion of all CHD in the registry more than doubled during this time. The cumulative RSV hospitalization rate was 1.9% among patients with CHD who received prophylaxis. Among subjects with cyanotic and acyanotic CHD, hospitalization rates were 2.6% and 1.6%, respectively. Prospective data collected in the Palivizumab Outcomes Registry provide the largest published dataset available on infants with CHD receiving palivizumab and show low hospitalization rates and use consistent with prelicensure clinical trial data and revised American Academy of Pediatrics guidelines.