Identifying and Integrating Parent Priorities for Psychosocial Support Services in a Pediatric Cystic Fibrosis Clinic.

Engaging parents early in the development of psychosocial support programs in cystic fibrosis (CF) clinics may enable services and care team recommendations to be tailored appropriately. This pilot study identified psychosocial priorities of parents of children with CF related to treatment adherence, parent/child mental health, and CF-related communication. Forty parents of children with CF (2 months to 17 years) completed an anonymous 17-item survey during routine clinic visits that assessed priorities related to psychosocial services. Elements of a quality improvement framework were used to develop the survey and determine recommendations based on findings. Parents reported the most interest in support related to improving adherence to respiratory therapies and helping children complete treatments independently. Other priority areas included services that helped children cope with feelings of isolation or abnormality due to CF and strategies to improve communication with the care team. Additionally, the majority of families indicated that they preferred receiving psychosocial services during routine clinic visits, followed by periodic parent workshops. Based on survey results, the psychosocial team at our center developed a survey/response model (e.g., roundtables, workshops) that may serve useful for other CF care teams as they identify the priorities of parents and adapt to their needs.

Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis.

BACKGROUND: Variation in CF pulmonary outcomes is multifactorial, but a significant component appears to be dependent upon differences in CF Center care. Previous investigations suggest that high performing CF centers are more consistent and proactive in the treatment of pulmonary exacerbations. We incorporated this approach into a program that could be bundled and shared with other CF Centers. METHODS: The reorganization of CF pulmonary care at the Children's Hospital of Richmond included the development of a pulmonary algorithm to define a standard response to changes in lung function and run charts to track process and outcome measures. We calculated the rolling average of the best percent predicted FEV1 (ppFEV1) over the previous 12 months as our primary outcome measure. RESULTS: The mean of the best ppFEV1 in the previous 12 months rose from 87% predicted (65% predicted for those 13-18 years, 97% predicted for those 6-13 years) in January 2013 to 98% predicted (95% predicted for those 13-18 years, 110% predicted for those 6-13 years) in January 2018. The ppFEV1 difference between children 6-13 years and adolescents 13-18 years dropped from 34 to 14 during that time. CONCLUSIONS: Improvements in pulmonary outcomes can be accomplished rapidly using basic quality improvement principles, including interdisciplinary team goal setting, standardized and proactive approaches that ensure consistent recognition and treatment of pulmonary exacerbations, and the use of data to follow the effectiveness of the process. We believe that the steps involved would be easy for other CF Centers to adapt to their own settings.

Secretory hyperresponsiveness and pulmonary mucus hypersecretion.

The term bronchial hyperresponsiveness is generally used to describe a heightened airway smooth muscle bronchoconstrictor response measured by bronchoprovocation testing. However, the airway also responds to inflammation or bronchoprovocation with increased mucus secretion. We use the term "secretory hyperresponsiveness" to mean increased mucus secretion either intrinsically or in response to bronchoprovocation. This is not the same as retained phlegm or sputum. Unlike smooth muscle contraction, which is rapidly reversible using a bronchodilator, mucus hypersecretion produces airflow limitation that reverses more slowly and depends upon secretion clearance from the airway. Certain groups of patients appear to have greater mucus secretory response, including those with middle lobe syndrome, cough-dominant ("cough-variant") asthma, and severe asthma. Secretory hyperresponsiveness also is a component of forms of lung cancer associated with bronchorrhea. An extreme form of secretory hyperresponsiveness may lead to plastic bronchitis, a disease characterized by rigid branching mucus casts that obstruct the airway. Secretory hyperresponsiveness and mucus hypersecretion appear to be related to activation of the extracellular-regulated kinase 1/2, signaling through the epidermal growth factor receptor, or secretory phospholipases A2. Recognizing secretory hyperresponsiveness as a distinct clinical entity may lead to more effective and targeted therapy for these diseases.

The future in paediatric respirology.

The authors were given the charge of providing a vision of the future in paediatric respirology. Themes selected for being ripe for this visionary analysis include bronchopulmonary dysplasia (BPD), asthma, cystic fibrosis (CF), lung infections, obstructive sleep disordered breathing (OSDB) and pulmonary diagnostics and monitoring. A profound reduction or elimination of BPD is seen. Given the strong genetic component of this disease, genetic biomarkers will likely be identified that will permit much earlier recognition of BPD susceptibility and potentially the ability to modify disease course by altering gene expression. The ultimate prevention of BPD will be to prevent prematurity, but recognition of both the genetic basis of BPD and the inflammatory background should lead to improved prevention and therapy. A clear understanding and definition of asthma phenotypes will lead to more specific and targeted therapy, earlier detection and prevention, better monitoring of severity and adherence to therapy, lower mortality and decreased inappropriate diagnosis of asthma. The greatest opportunities in asthma care will likely come through tools to improve adherence to effective therapy. Also, areas are identified where better therapies are needed such as in patients with severe mucus hypersecretion (secretory hyperresponsiveness) especially in those with life-threatening asthma. The future of CF is easier to foresee with early successes seen in clinical trials. After the expected ability to correct the CF transmembrane regulator, care will need to change and additional research will be needed. Additionally, the face of CF is changing with more adults than children presently having the disease. This will necessitate changes to our approach to treating this disease in a fortunately aging population. If we are going to affect the worldwide lung health of children, we will need to address respiratory infections particularly pneumonia, tuberculosis and HIV-associated infections. Preventive, diagnostic and treatment strategies will shape the future face of these problems. The availability of inexpensive, readily available, and rapid molecular techniques to identify true infection (including HIV and tuberculosis) may permit earlier use of effective therapy while preventing the inappropriate use of antibiotics for common viral diseases. Sleep medicine will continue to be an important aspect of paediatric pulmonology. The evaluation of OSDB cannot rely on full-night attended polysomnography due to limited access. Identifying reliable markers of end organ dysfunction in children with OSDB may permit more rapid identification of patients in need of intervention like CPAP and assisted breathing. In addition, management options, as an alternative to adenotonsilectomy, are listed with a call for further research. Pulmonary diagnostics and monitoring will see the development and refinement of tools like the lung clearance index and the analysis of exhaled gases, volatiles and dissolved biomarkers of inflammation as techniques that might help clinicians identify both the initiation of inflammation while it is more amenable to therapy, and to identify more readily the early changes associated with chronic lung diseases in children. The authors hope that these visionary articles will generate comments, arguments, inspiration, and perhaps even motivate funding agencies.