Monitoring of physiologic features and treatment aspects of children on home invasive mechanical ventilation
Respiration Artificial; Monitoring Physiologic; Ventilators Mechanical; monitoring; pediatric long-term ventilation; tracheostomy ventilation
Pediatric home invasive mechanical ventilation patients are a small but resource-intensive cohort, requiring close monitoring and multidisciplinary care. Patients are often dependent on their ventilator for life support, with any significant complications such as equipment failure, tracheostomy blockage, or accidental decannulation becoming potentially life-threatening if not identified quickly. This review discusses the indications and variations in practice worldwide, in terms of models of care, including home care provision, choice of equipment, and monitoring. With advances in technology, optimal monitoring strategies for home, continue to be debated: In-built ventilator alarms are often inadequately sensitive for pediatric patients, necessitating additional external monitoring devices to minimize risk. Pulse oximetry has been the preferred monitoring modality at home, though in some special circumstances such as congenital central hypoventilation syndrome, home carbon dioxide monitoring may be important to consider. Children should be under regular follow-up at specialist respiratory centers where clinical evaluation, nocturnal oximetry, and capnography monitoring and/or poly(somno)graphy and analysis of ventilator download data can be performed regularly to monitor progress. Recent exciting advances in technology, particularly in telemonitoring, which have potential to hugely benefit this complex group of patients are also discussed.
Chawla J; Tan HL
Pediatric Pulmonology
2024
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.26901" target="_blank" rel="noreferrer noopener">10.1002/ppul.26901</a>
Postdischarge Health Resource Use in Pediatric Survivors of Prolonged Mechanical Ventilation for Acute Respiratory Illness
Child; Infant; Pediatrics; Respiration; Aftercare; Pneumonia; Critical illness; Discharge; Health resources; Healthcare; Intensive care unit; Respiratory distress syndrome
We aimed to identify characteristics associated with postdischarge health resource use in children without medical complexity who survived an episode of prolonged mechanical ventilation for respiratory illness. We hypothesized that longer durations of mechanical ventilation, noncomplex chronic conditions, and severe acute respiratory distress syndrome (ARDS) would be associated with readmission or an Emergency Department (ED) visit. In this retrospective cohort, we evaluated children without a complex chronic condition who survived a respiratory illness requiring ≥3 days of mechanical ventilation and who had insurance eligibility within the Colorado All Payers Claims Database. We used insurance claims to characterize health resource use and multivariable logistic regression to identify characteristics associated with readmission or an ED visit during the postdischarge year. We evaluated 82 children, median age 12.8 months (interquartile range [IQR]: 4.0-24.1), 20 (24%) with a noncomplex chronic condition and 62 (76%) without any chronic conditions. Bronchiolitis (60%) and pneumonia/aspiration pneumonitis (17%) were the most common etiologies of respiratory failure and 47 (57%) patients had severe ARDS. Forty-six (56%) patients had an ED visit or readmission. Among the 18 readmitted patients, 16/18 (89%) readmissions were for respiratory illness. Forty (49%) patients had ≥2 outpatient pulmonary visits and 45 (55%) filled a pulmonary medication prescription. In analyses controlling for age, illness severity and mechanical ventilation duration, severe ARDS was predictive of ED visit or readmission (odds ratio [OR]: 5.53 [95% confidence interval [CI]: 1.79, 19.09]). Children who survive prolonged mechanical ventilation for respiratory disease experience high rates of postdischarge health resource use, particularly those surviving severe ARDS.
Vo M; Miller K; Bennett TD; Mourani PM; LaVelle J; Carpenter TC; Scott Watson R; Pyle L L; Maddux AB
Pediatric Pulmonology
2022
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.25934" target="_blank" rel="noreferrer noopener">10.1002/ppul.25934</a>
Palliative Care in Pediatric Pulmonology
palliative care; quality of life; bronchopulmonary dysplasia; communication; pulmonary hypertension; cystic fibrosis; psychosocial support; neuromuscular disease; lung transplant; pediatric pulmonology; primary palliation
Children with End Stage Lung Disease (ESLD) are part of the growing population of individuals with life-limiting conditions of childhood. These patients present with a diverse set of pulmonary, cardiovascular, neuromuscular, and developmental conditions. This paper first examines five cases of children with cystic fibrosis, bronchopulmonary dysplasia, neuromuscular disease, pulmonary hypertension, and lung transplantation from Texas Children's Hospital. We discuss the expected clinical course of each condition, then review the integration of primary and specialized palliative care into the management of each diagnosis. This paper then reviews the management of two children with end staged lung disease at Hospital Civil de Guadalajara, providing an additional perspective for approaching palliative care in low-income countries.
Baumann T; Das S; Jarrell JA; Nakashima-Paniagua Y; Benitez EA; Gazzaneo MC; Villafranco N
Children
2021
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.3390/children8090802" target="_blank" rel="noreferrer noopener">10.3390/children8090802</a>
Telemedicine in children with medical complexity on home ventilation during the COVID-19 pandemic
Adolescent; Child; Chronic Disease; COVID-19; Home Care Services; Hospitalization; mechanical ventilation; noninvasive ventilation; Pandemics; Artificial Respiration; respiratory technology; SARS-CoV-2; Telemedicine; telemonitoring
Children with medical complexity (CMC) are patients with one or more complex chronic conditions dependent on medical technologies. In our unit (Pediatric Pulmonology and Respiratory Intermediate Care Unit, Department of Pediatrics, "Bambino Gesù" Children's Hospital and Research Institute), we regularly follow-up CMC patients, particularly children on long-term, invasive (IMV) or noninvasive (NIV), ventilation. Children suffering from chronic diseases and with medical complexity have lost the possibility to go to the hospital during the COVID-19 pandemic. The aim of this article is to describe our experience with telemedicine (teleconsultation [TC] and telemonitoring of ventilator [TM]) in CMC on ventilation. We presented 21 children on long-term ventilation (NIV or IMV) whose planned hospital admission was postponed due to lockdown. A total of 12 healthcare problems were detected during scheduled TCs. Only one problem was not solved by our remote intervention. Specifically, TM has allowed us to change the ventilator parameters and to monitor patients on ventilation remotely. In conclusion, the use of telemedicine in CMC ventilated patients resulted in a feasible tool to avoid in-person visits during the pandemic.
Onofri A; Pavone M; De Santis S; Verrillo E; Caggiano S; Ullmann N; Cutrera R
Pediatric Pulmonology
2021
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.25289" target="_blank" rel="noreferrer noopener">10.1002/ppul.25289</a>
Shared decision making for children with chronic respiratory failure-It takes a village and a process
tracheostomy; chronic respiratory failure; shared decision making; long-term mechanical ventilation
BACKGROUND AND OBJECTIVES: Shared decision making (SDM) before nonurgent tracheostomy in a child with chronic respiratory failure (CRF) is often recommended, but has proven challenging to implement in practice. We hypothesize that utilization of the microsystem model for analysis of the complex ecosystem in which SDM occurs will yield insights that enable formation of a reproducible, measurable SDM process. METHODS: Retrospective chart review of a case series of children with CRF in whom a SDM process was pursued. The process included a palliative care consult, a validated decision aid and 12 key questions designed to elucidate information integral to an informed decision. Investigators reviewed a single hospital admission for each child, focusing on the 3 core elements of a medical microsystem-the patient, the providers, and information. RESULTS: Twenty-nine patients who met inclusion criteria ranged in age from 0 to 19.5 years (median 1.7) and remained in the hospital from 10 to 316 days (median 38). Patients were medically complex with multiple and varied respiratory diagnoses, multiple and varied comorbidities, and varying psychosocial environments. 14/29 children received tracheostomies. Each child encountered a mean of 6.2 medical specialties, 1.9 surgical specialties and 8.5 nonphysician led services. Answers to 12 key questions were not documented systematically and often not found in the electronic medical record. CONCLUSION: A unique SDM microsystem is formed around each child but not optimally utilized. Explicit recognition of these microsystems would enable team formation and an SDM process comprised of measurable steps and communication patterns.
Kevill K; Ker G; Meyer R
Pediatric Pulmonology
2021
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.25416" target="_blank" rel="noreferrer noopener">10.1002/ppul.25416</a>
Healthcare utilization and costs of pediatric home mechanical ventilation in Canada
Adult; Child; Female; Humans; Male; Child Preschool; Middle Aged; Quality of Life; Longitudinal Studies; Canada; Prospective Studies; pediatrics; Health Care Costs; Health Expenditures; Patient Acceptance of Health Care; Tracheostomy; healthcare costs; healthcare utilization; home mechanical ventilation; long-term mechanical ventilation; ventilator assisted individual; Ambulatory Care/economics; Caregivers/economics; Home Care Services/economics; Respiration Artificial/economics
BACKGROUND: Children using home mechanical ventilation (HMV) live at home with better quality of life, despite financial burden for their family. Previous studies of healthcare utilization and costs have not considered public and private expenditures, including family caregiver time. Our objective was to examine public and private healthcare utilization and costs for children using HMV, and variables associated with highest costs. METHODS: Longitudinal, prospective, observational cost analysis study (2012-2014) collecting data on public and private (out-of-pocket, third-party insurance, and caregiving) costs every 2 weeks for 6 months using the Ambulatory Home Care Record. Functional Independence Measure (FIM), WeeFIM, and Caregiving Impact Scale (CIS) were measured at baseline and study completion. Regression modeling examined a priori selected variables associated with monthly costs using Andersen and Newman's framework for healthcare utilization, relevant literature, and clinical expertise. Data are reported in 2015 Canadian dollars ($1CAD = $0.78USD). RESULTS: Forty two children and their caregivers were enrolled. Overall median (interquartile range) monthly healthcare cost was $12 131 ($8159-$15 958) comprising $9929 (89%) family caregiving hours, $996 (9%) publicly funded, and $252 (2%) out-of-pocket (<1% third-party insurance) costs. With higher FIM score (lower dependency), median costs were reduced by 4.5% (95% confidence interval: 8.3%-0.5%), adjusted for age, sex, tracheostomy, and daily ventilation duration. Note: since the three cost categories did not sum to the total statistically derived median cost, the percentage of each category used the sum of median public + caregiver lost time + private out-of-pocket + third-party insurance as the denominator. CONCLUSIONS: For HMV children, most healthcare costs were due to family caregiving costs. More dependent children incur highest costs. The financial burden to family caregivers is substantial and needs to considered in future policy decisions related to pediatric HMV.
Nonoyama ML; Katz SL; Amin R; McKim DA; Guerriere D; Coyte PC; Wasilewski M; Zagorski B; Rose L
Pediatric Pulmonology
2020
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.24923" target="_blank" rel="noreferrer noopener">10.1002/ppul.24923</a>
Development and validation of a novel informational booklet for pediatric long-term ventilation decision support
children; noninvasive ventilation; tracheostomy; mechanical ventilation; decision making shared; respiration artificial
OBJECTIVES: To provide accessible, uniform, comprehensive, and balanced information to families deciding whether to initiate long-term ventilation (LTV) for their child, we sought to develop and validate a novel informational resource. METHODS: The Ottawa Decision Support Framework was followed. Previous interviews with 44 lay and 15 professional stakeholders and published literature provided content for a booklet. Iterative versions were cognitive tested with six parents facing decisions and five pediatric intensivists. Ten parents facing decisions evaluated the booklet using the Preparation for Decision Making Scale and reported their decisional conflict, which was juxtaposed to the conflict of 21 parents who did not read it, using the Decisional Conflict Scale. Twelve home ventilation program directors evaluated the booklet's clinical sensibility and sensitivity, using a self-designed six-item questionnaire. Data presented using summary statistics. RESULTS: The illustrated booklet (6th-grade reading level) has nine topical sections on chronic respiratory failure and invasive and noninvasive LTV, including the option to forgo LTV. Ten parents who read the booklet rated it as helping "Quite a bit" or more on all items of the Preparation for Decision Making Scale and had seemingly less decisional conflict than 21 parents who did not. Twelve directors rated it highly for clinical sensibility and sensitivity. CONCLUSIONS: The LTV booklet was rigorously developed and favorably evaluated. It offers a resource to improve patient/family knowledge, supplement shared decision-making, and reduce decisional conflict around LTV decisions. Future studies should validate it in other settings and further study its effectiveness.
Edwards JD; Panitch HB; George M; Cirrilla AM; Grunstein E; Wolfe J; Nelson JE; Miller RL
Pediatric Pulmonology
2020
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.25221" target="_blank" rel="noreferrer noopener">10.1002/ppul.25221</a>
Autonomic Dysregulation in Young Girls With Rett Syndrome During Nighttime in-Home Recordings
Pediatrics; patterns; autonomic dysregulation; cardiorespiratory dysregulation; disorder; disturbances; dysfunction; hyperventilation; mecp2 mutations; mice; nervous-system; Respiratory System; breathing difficulties; Rett syndrome; trajectory; characteristics; irregular breathing; increased breathing frequency
This study was designed to specifically characterize the autonomic phenotype of cardiorespiratory dysregulation during the nighttime in young girls with MECP2 mutation-confirmed Rett Syndrome (RS), studied in their home environment. Computerized breath-to-breath and beat-to-beat characterization of at-home continuously recorded respiratory inductance plethysmography of chest/abdomen and ECG (VivoMetrics, Inc.) was obtained during overnight recordings in 47 girls with MECP2 mutation-confirmed RS and 47 age-, gender-, and ethnicity-matched screened controls (ages 2-7 years). We determined that although the breathing and heart rate appear more regular during the night compared to the day, young girls with RS demonstrate apparent nocturnal irregularities. Comparing daytime versus nighttime, breathing was more irregular, with an increased breathing frequency (and irregularity), mean amplitude of respiratory inductance plethysmography sum (AMP)/T-I, and heart rate and decreased AMP in girls with RS. Comparing girls with RS versus controls during nighttime recording, breathing was more irregular, with an increased breathing frequency (and irregularity), mean AMP/T-I, and heart rate. An increased uncoupling between measures of breathing and heart rate control indicates malregulation in the autonomic nervous system, and is apparent during the day as well as the night. This uncoupling may represent a mechanism that renders the girls with RS more vulnerable to sudden death. Pediatr Pulmonol. 2008;43:1045-1060. (C) 2008 Wiley-Liss, Inc.
Weese-Mayer D E; Lieske S P; Boothby C M; Kenny A S; Bennett H L; Ramirez J M
Pediatric Pulmonology
2008
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.20866" target="_blank" rel="noreferrer noopener">10.1002/ppul.20866</a>
The feasibility and validity of forced spirometry in ataxia telangiectasia
Adolescent; Ataxia Telangiectasia/di [Diagnosis]; Ataxia Telangiectasia/pp [Physiopathology]; Case-Control Studies; Child; Preschool; Feasibility Studies; Female; Humans; Male; Reproducibility of Results; Spirometry; Young Adult; breathing difficulties; Ataxia Telangiectasia; trajectory; characteristics; lung deterioration
OBJECTIVES: To explore the feasibility and validity of forced spirometry in patients with ataxia telangiectasia (A-T). STUDY DESIGN: Twenty-eight patients (aged 3.7-19.3 years) performed spirometry on 47 occasions. Parameters studied were technical quality and relation to: predicted values, pulmonary illness. RESULTS: Start of test criteria for correct expiratory effort was significantly prolonged (183 +/- 115 ms; P < 0.001). The rise-time to peak flow in children free of respiratory symptoms (Group-FRS; n = 8) increased by 16.2 +/- 12.5 ms/year above recommended and in children having recurrent infections (n = 8) 30.4 +/- 16.1 ms/year, P < 0.01. Expiration-time was significantly shorter than requested (1.21 +/- 0.47 sec) and was ended abruptly in 57% of the patients. FEV(1) could not be established by 8/20 patients. The intra-subject reproducibility met criteria (4.4 +/- 2.7%, 5.2 +/- 2.8%, 2.9 +/- 3.2%, 6.3 +/- 5.3%, for FVC, FEV(0.5), PEF, FEF(25-75), respectively). Group-FRS showed yearly deterioration in FVC of 2.2%, while patients with hyper-reactive airways (Group-HRA; n =12) had a deterioration rate of 3.6%/year. FEV(0.5) deterioration rate was similar in both groups (2.2 and 2.0, respectively), but baseline values in Group-HRA were significantly lower than those of Group-FRS (P = 0.029) in similar young ages, indicating airway obstruction at early ages in Group-HRA. FEV(0.5) values deterioration also correlated with body mass index (P< 0.017). CONCLUSION: Forced spirometry in A-T patients is reproducible and has a distinct pattern, although curves do not meet other recommendations for acceptable criteria. The study insinuates that a rapid deterioration in lung function occurs in A-T patients with recurrent respiratory infection, suggesting that early intervention may prevent further deterioration or improve their lung function. Further studies are needed to confirm our results.Copyright © 2010 Wiley-Liss, Inc.
Vilozni D; Berkun Y; Levi Y; Weiss B; Jacobson J M; Efrati O
Pediatric Pulmonology
2010
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.21291" target="_blank" rel="noreferrer noopener">10.1002/ppul.21291</a>
Exploring knowledge and perceptions of palliative care to inform integration of palliative care education into cystic fibrosis care
adolescent; adult; article; female; human; male; palliative therapy; clinical article; palliative care; health care personnel; priority journal; caregiver; parent; perception; communication; semi structured interview; adolescence; attitude to health; cystic fibrosis; health education; lung fibrosis; patient education; qualitative analysis
Background: Individuals with cystic fibrosis (CF) face the challenges of managing a chronic, progressive disease. While palliative care is a standard of care in serious illnesses, there are no guidelines for its incorporation into CF care. Patients with CF, caregivers, and CF care providers may lack knowledge about palliative care and perceive barriers to integrated care. Objective(s): To: 1) explore knowledge and perceptions of palliative care among patients with CF, caregivers, and CF care providers; 2) solicit opinions about incorporating palliative care into routine CF care; and 3) solicit recommendations for CF-specific palliative care education for patients and caregivers. Method(s): We conducted semi-structured interviews with adult patients with CF, parents of adolescents with CF, and CF care providers to assess knowledge and perceptions of palliative care. Discussion included suggestions for palliative care education and integration into CF care. The sample was characterized using summary statistics. Key themes were identified using qualitative content analysis. Result(s): Ten patients with CF, ten parents, and eight CF care providers participated. Many had minimal knowledge of palliative care and endorsed the association with end of life as a barrier to palliative care, but after learning more about palliative care, thought it could be helpful, and should be introduced earlier. Conclusion(s): In this single center study, many patients with CF, caregivers, and providers lacked knowledge about palliative care. These findings warrant replication in a larger, multisite study to inform palliative care educational interventions as a step toward consistent integration of palliative care into routine CF care. Copyright � 2018 Wiley Periodicals, Inc.
Dellon E P; Helms S W; Hailey C E; Shay R; Carney S D; Schmidt H J; Brown D E; Prieur M G
Pediatric Pulmonology
2019
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.24073" target="_blank" rel="noreferrer noopener">10.1002/ppul.24073</a>
Effects of a primary palliative care intervention on quality of life and mental health in cystic fibrosis
quality of life; anxiety; depression; chronic symptom burden; cystic fibrosis; primary palliative care
BACKGROUND: Despite the significant impact of chronic symptoms on quality of life with cystic fibrosis (CF), the role of palliative care in management of this disease is not well defined. The coping, goal assessment, and relief from evolving CF symptoms (CF-CARES) model is a primary palliative care intervention designed to provide chronic symptom management at all stages of the disease. The goal of this pilot study was to estimate the effectiveness of the CF-CARES intervention on improving chronic symptoms and quality of life for people living with CF. METHODS: A structured assessment was used to guide referral to supportive services intended to address burdensome symptoms. Follow-up assessments were performed approximately 3 and 6 months later. Longitudinal regression analyses of changes in symptoms and quality of life were performed for all participants regardless of utilization of supportive services. Subgroup analyses were performed for subjects participating in mental health and alternative health services. RESULTS: Forty-one subjects completed assessment and referral processes. The mean number of CF-associated symptoms decreased over time, as did respiratory symptom-related distress and depressive symptoms. Subjects utilizing alternative health services reported less psychological distress at follow-up. Among subjects with severe disease, mental health, and quality of life improved, especially for those using mental health services. CONCLUSIONS: The CF-CARES model resulted in significant mental health and quality-of-life benefits, suggesting the value of integrating symptom management interventions into routine CF care. Moreover, mental health services can play a key role in CF-specific primary palliative care, especially for those with advanced disease.
Friedman D; Linnemann RW; Altstein L L; Georgiopoulos AM; Islam S; Bach KT; St John A; Fracchia MS; Neuringer I; Lapey A; Sicilian L; Moskowitz SM; Yonker LM
Pediatric Pulmonology
2019
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.24311" target="_blank" rel="noreferrer noopener">10.1002/ppul.24311</a>
Primary palliative care skills in CF: Perspectives of adults with CF, caregivers, and CF care team members
advance care planning; hospice; clinical assessment; pain assessment; education; palliative therapy; major clinical study; lung; prognosis; caregiver; skill; conference abstract; human; child; female; male; controlled study; adult; perception; awareness; depression assessment; Kruskal Wallis test
Background: "Primary" palliative care (PC) skills for CF care teams are not clearly defined, but in general for serious illness include managing basic physical and emotional symptoms, and having basic discussions about prognosis, goals, suffering, and code status. Complex symptom management and addressing conflicts around goals and decisions are considered "specialty" PC skills for which PC consultation may be appropriate. We aimed to understand primary PC skills of CF care teams from the perspectives of team members, individuals with CF, and family caregivers. Methods: CF care team members ("providers"), adults with CF ("patients"), and family caregivers ("caregivers") rated the ability of CF care teams to provide various aspects of PC using a 5-point scale from "poor" to "excellent." Median ratings were compared between and among groups using Mann-Whitney and Kruskal-Wallis tests. Results: A total of 520 participants, including 70 patients, 100 caregivers, and 350 providers, completed surveys. CF care teams consistently rated their PC skills higher than patients or caregivers rated providers' skills. Providers rated their teams "very good" at pain and depression assessments, discussing lung transplant, and discussing prognosis, and "good" at discussing advance care planning (ACP), code status, end of life, and hospice. Patients and caregivers agreed that teams provide "very good" pain assessment, but rated teams "good" at assessing depression (P<0.001) and discussing prognosis (P=0.006), and "poor" at discussing lung transplant (P<0.001), ACP (P<0.001), code status (P<0.001), end of life (P<0.001), and hospice (P<0.001). Providers, patients, and caregivers affiliated with adult CF care teams rated teams more highly then providers, patients, and caregivers affiliated with pediatric teams at discussing lung transplant (P<0.001), end of life (P=0.006), ACP (P<0.001), code status (P=0.012), and hospice (P=0.016). A majority of patients (69%) and caregivers (60%) felt CF care teams should definitely receive more PC training. Conclusions: Discrepancies exist among patient/caregiver and provider perceptions of PC skills in CF, and skills of adult and pediatric teams may differ. While patients, caregivers, and providers agree that CF care teams are skilled in some "primary" PC skills like pain and depression assessment and discussing prognosis, patients and caregivers feel providers' skills are lacking in discussing lung transplant, ACP, code status, end of life, and hospice. Education for all groups could promote awareness of PC, and CF care teams may benefit from specific PC training to enhance "primary" PC skills as well as understanding when and how to utilize specialty PC services.
Dellon EP; Basile M; Hobler M R; Georgiopoulos A; Goggin JL; Chen E; Goss CH; Hempstead SE; Faro A; Kavalieratos D
Pediatric Pulmonology
2018
<a href="http://doi.org/10.1002/ppul.24152" target="_blank" rel="noreferrer noopener">10.1002/ppul.24152</a>
Treating dyspnea with morphine sulfate in nonverbal children with neurological impairment
Dyspnea; Children; Morphine; neurological impairment; respiratory distress
Children with severe neurological impairment (NI) are at risk for recurrent respiratory illness with risk for associated distressing respiratory symptoms as respiratory exacerbations become more frequent. Evidence for treating dyspnea in adults with severe pulmonary disease offers interventions for consideration, including morphine sulfate. This case series of four individuals with severe NI reviews the benefit from morphine for respiratory distress. Information includes descriptors of distress, starting dose, and dose increases. This is the first report to review the use of morphine for the treatment of dyspnea in nonverbal children with severe impairment of the central nervous system. Pediatr Pulmonol. © 2014 Wiley Periodicals, Inc.
2014
Hauer JM
Pediatric Pulmonology
2014
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.23140" target="_blank" rel="noreferrer">10.1002/ppul.23140</a>
The challenge of asthma in adolescence
Humans; adolescent; Adolescent Transitions; Asthma/diagnosis/mortality/therapy
The adolescents with asthma are a distinct group of patients with different problems and needs compared to children and adults. Specific issues of asthma in adolescence are the variability of the clinical spectrum, the presence of particular risk factors for the persistence of symptoms, underdiagnosis and undertreatment of the disease. Refusal of the sick role, denial of symptoms, carelessness about dangerous inhalation exposure, erratic self-medication, overexertion without taking precautions against exercise-induced asthma, and a poor relationship between patients, their families, and often doctors are the main obstacles to successful management of asthma in this critical age. There are also major problems of compliance for these patients. The goal of optimal quality of life will be achieved only if the physician thoroughly understands the adolescent's needs and provides optimal care.
2007
de Benedictis D; Bush A
Pediatric Pulmonology
2007
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.20650" target="_blank" rel="noreferrer">10.1002/ppul.20650</a>
Lung disease in Niemann-Pick disease
Child; Female; Humans; Male; Prognosis; Severity of Illness Index; Biopsy; Preschool; infant; Q3 Literature Search; Diagnosis; Differential; Lung Diseases; Respiratory Function Tests; Bronchoalveolar Lavage; Bronchoalveolar Lavage Fluid/cytology; Interstitial/diagnosis/etiology/physiopathology; Niemann-Pick Diseases/complications; Radiography; Thoracic
BACKGROUND: Lung involvement in children with Niemann-Pick disease has rarely been studied systematically. OBJECTIVE: To assess the involvement of the lung and the value of bronchoalveolar lavage in children with Niemann-Pick diseases. DESIGN: Retrospective analysis of patient records. PATIENTS: Thirteen patients, with type A (n = 1), type B (n = 10), and type C (n = 2) Niemann-Pick disease, aged 2 months to 9 years at diagnosis, were included in the study. INTERVENTIONS: Lung involvement was assessed by clinical evaluation, chest radiograph, lung computed tomography (CT) scan, pulmonary function tests, and bronchoalveolar lavage fluid analysis. RESULTS: Respiratory symptoms were present at diagnosis in 10 patients and developed during follow up in the three other patients. All patients showed signs of interstitial lung disease on chest X-ray and lung CT scan. Bronchoalveolar lavage fluid analysis (n = 7) revealed a marked accumulation of foamy macrophages (Niemann-Pick cells) in all patients. At follow up, one patient died of respiratory failure, five patients required long term oxygen therapy and seven other patients presented a chronic obstructive pulmonary disease (n = 6) or chronic cough (n = 1). CONCLUSION: Lung disease was observed in all the patients included in the present study. Bronchoalveolar lavage may be useful in Niemann-Pick diseases by showing the presence of characteristic Niemann-Pick cells.
2007
Guillemot N; Troadec C; de Villemeur TB; Clement A; Fauroux B
Pediatric Pulmonology
2007
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.20725" target="_blank" rel="noreferrer">10.1002/ppul.20725</a>
Noninvasive ventilation during gastrostomy tube placement in patients with severe duchenne muscular dystrophy: case reports and review of the literature
PedPal Lit; Adult Follow-Up StudiesGastrostomy Humans Laryngeal Masks Male Muscular Dystrophy; Duchenne/therapy Positive-Pressure Respiration/methods Retrospective Studies Severity of Illness Index
Individuals with Duchenne muscular dystrophy may benefit from gastrostomy tube feeding due to progressive dysphagia and malnutrition. However, due to their severely impaired pulmonary function, these individuals are at risk of severe complications when they are sedated or undergo anesthesia for the procedure. We previously described a technique of noninvasive positive pressure ventilation to provide respiratory support during gastrostomy tube placement in such patients, but this technique had risks and limitations. In this case report, we examine two alternative techniques we used to provide respiratory support successfully to patients with severe muscular dystrophy and malnutrition who underwent percutaneous endoscopic gastrostomy tube placement. We then review the literature and discuss the potential benefits, risks, and limitations of the above techniques and of other options for gastrostomy placement in people with severe muscular dystrophy.
2006
Birnkrant DJ; Ferguson RD; Martin JE; Gordon GJ
Pediatric Pulmonology
2006
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Journal Article
<a href="http://doi.org/10.1002/ppul.20356" target="_blank" rel="noreferrer">10.1002/ppul.20356</a>
Perspectives of patients with cystic fibrosis on preventive counseling and transition to adult care
Female; Humans; Male; Adult; Health Care Surveys; Prospective Studies; Massachusetts; Boston; Needs Assessment; Practice; adolescent; Attitudes; Adolescent Transitions; Health Knowledge; Aging/psychology; Continuity of Patient Care/organization & administration; Counseling/organization & administration; Cystic Fibrosis/psychology/therapy; Day Care/organization & administration; Preventive Medicine/organization & administration; Pulmonary Disease (Specialty)/statistics & numerical data
The purpose of this study was to investigate how adolescents and adults with cystic fibrosis (CF) view preventive counseling and their transition to adult-centered care within a children's hospital. Thirty-two patients >/=16 years old diagnosed with CF were recruited from a pediatric tertiary care setting. During face-to-face interviews, patients were asked 27 structured questions and completed a 30-item self-administered questionnaire on preventive counseling by healthcare providers and on transition issues. The median age of patients was 25.5 years (range, 16-43 years); 69% of patients identified a pulmonologist as their "main doctor," even though 78% had a primary care provider. Participants felt that 13-16 years of age was the best time for them to begin spending time alone with their main doctor. Less than half of the participants recalled receiving preventive counseling during the previous 12 months, and more patients wanted to discuss issues than actually did. Qualitative data emphasized the importance of independence in making decisions in healthcare and establishing relationships with providers, and many patients did not desire to transfer care to an adult hospital. Participants identified adult-focused services such as inpatient rooms, discussion groups, work options, and social service support that would enhance care. In conclusion, the majority of adolescent/young adult patients with CF receiving care in a pediatric institution reported satisfaction with their healthcare. However, patients identified preventive issues that they desired to be more regularly addressed, starting in early adolescence, and changes in the delivery of services to enhance transition to adult-oriented care. This study underscored the understanding of the integration of transition planning into the facilitation of healthcare decision-making by the adolescent in issues of self-care, sexuality, education, and finances. Future initiatives to enhance the care of patients with CF should provide training of pulmonologists in preventive care and increased attention to helping patients utilize appropriate primary-care services during the adult years. In addition, prospective studies are needed to compare outcomes of CF patients who have transitioned and transferred to adult hospitals and those transitioning to adult-oriented services in a pediatric institution.
2003
Zack J; Jacobs CP; Keenan PM; Harney K; Woods ER; Colin AA; Emans SJ
Pediatric Pulmonology
2003
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.10342" target="_blank" rel="noreferrer">10.1002/ppul.10342</a>
Developmentally appropriate healthcare for young people with chronic illness: questions of philosophy, policy, and practice
Humans; United States; Adult; Practice Guidelines as Topic; adolescent; Adolescent Transitions; Continuity of Patient Care/organization & administration; Adolescent Development; Chronic Disease/therapy; Health Policy/trends; Health Promotion/trends; Preventive Health Services/trends; Quality of Health Care/trends
2003
Sawyer SM
Pediatric Pulmonology
2003
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.10369" target="_blank" rel="noreferrer">10.1002/ppul.10369</a>
Transition programs in cystic fibrosis centers
Humans; Great Britain; Adult; Continuity of Patient Care; adolescent; Adolescent Transitions; Cystic Fibrosis/therapy; Patient Transfer
2004
Conway SP
Pediatric Pulmonology
2004
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.10419" target="_blank" rel="noreferrer">10.1002/ppul.10419</a>
Myopathy with mitochondrial changes presenting as respiratory failure in two brothers
Child; Humans; Male; Biopsy; Fatal Outcome; Q3 Literature Search; Diagnosis; Differential; Interstitial/diagnosis/pathology; Lung Diseases; Mitochondrial Myopathies/diagnosis/genetics/pathology; Needle; Nuclear Family; Respiratory Function Tests; Respiratory Insufficiency/etiology
Lemos AB; Mosquera J; Mate A; Sirvent J
Pediatric Pulmonology
1999
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/(sici)1099-0496(199903)27:3%3C213::aid-ppul11%3E3.0.co" target="_blank" rel="noreferrer">10.1002/(sici)1099-0496(199903)27:3%3C213::aid-ppul11%3E3.0.co</a>
Strategies for improving transition to adult cystic fibrosis care, based on patient and parent views
Female; Humans; Male; Adult; Health Care Surveys; Interviews as Topic; Questionnaires; Middle Aged; Continuity of Patient Care; adolescent; Adolescent Transitions; Cystic Fibrosis/therapy; Adolescent Health Services/organization & administration; Patient Satisfaction/statistics & numerical data
Although general principles guiding the transition of individuals with chronic illness from pediatric to adult care have been established, guidelines specific for the transition of individuals with cystic fibrosis (CF) have not. To aid in the development of CF-specific transition guidelines, an anonymous pretransition questionnaire and posttransition interview were used to assess the concerns and expectations of 60 CF patients and their parents as they went through the transition from pediatric to adult care. Along with demographic and clinical information, respondents were asked to rate on a scale of 1-5 their general attitude toward, or level of concern on 22 questions involving transition concerns, adult program expectations, and general view of transition. The two most important concerns identified by patients prior to transition to adult care were potential exposure to infection (3.4 +/- 1.3) and having to leave their previous caregivers (3.4 +/- 1.0). Introduction to the adult CF team prior to transition was associated with significantly lower levels of concern in all areas, particularly about having to leave previous caregivers (3.9 +/- 0.7 vs. 2.5 +/- 0.6, P < 0.004). Age, gender, severity of lung disease, and age at diagnosis were not predictive of level of concern for any area. Parents' most significant concern was ability of their child to care for their CF independently, a concern their children did not share (4.0 +/- 1.1 vs. 1.5 +/- 0.5, P < 0.0001). As their most important expectations for the adult program, patients identified ready phone access to a nurse (4.9 +/- 0.6) and education about adult CF issues (4.6 +/- 0.7). The overall attitude toward the development of an adult CF program was overwhelmingly positive for both patients (4.9 +/- 0.7) and parents (4.9 +/- 0.3). By allowing patients to interact with the adult team prior to transition and developing transition protocols which address CF-specific issues like infection control and fertility, successful transition from pediatric to adult cystic fibrosis care can be accomplished.
2001
Boyle MP; Farukhi Z; Nosky ML
Pediatric Pulmonology
2001
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/ppul.1154" target="_blank" rel="noreferrer">10.1002/ppul.1154</a>
A report of four cases of acute, severe pulmonary hemorrhage in infancy and support with extracorporeal membrane oxygenation
Female; Humans; Male; Extracorporeal Membrane Oxygenation; Respiration; Severity of Illness Index; Acute Disease; infant; Artificial; Hemorrhage/complications/therapy; Lung Diseases/complications/therapy; Respiratory Insufficiency/etiology/therapy
Introduction
Pulmonary hemorrhage is an uncommon event in infants. It has been described most commonly in the sick premature neonate, older child, or adolescent with chronic cardiopulmonary disease. Acute idiopathic pulmonary hemorrhage in previously healthy infants has, to our knowledge, been reported only rarely. During the past 5 years we have successfully treated 4 infants with sever respiratory failure secondary to acute idiopathic pulmonary hemorrhage. Two of these patients were managed with the conventional therapy of mechanical ventilation, while the other two were successfully managed with extracorporeal membrane oxygenation (ECMO) after failure of conventional mechanical ventilation. In this report we review the current literature on this unusual pediatric problem and describe the use of ECMO as a modality in supporting patients after an acute pulmonary hemorrhage.
Siden HB; Sanders GM; Moler FW
Pediatric Pulmonology
1994
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Journal Article
<a href="http://doi.org/10.1002/ppul.1950180512" target="_blank" rel="noreferrer noopener">10.1002/ppul.1950180512</a>
Early involvement of palliative care in cystic fibrosis in inpatient setting: A quality improvement initiative
cystic fibrosis; hospital patient; palliative therapy; Total Quality Management; Child; disease exacerbation; e-mail; Human; lung; outcome assessment; pulmonologist; social worker
Background: Cystic fibrosis (CF) is a life-limiting disease with multiple comorbidities. It has been recommended that palliative care discussions for psychological adjustment and optimal symptom control should be initiated early. However, the palliative care service in this population remains underutilized. Objective: To increase utilization of palliative care services for patients admitted for CF exacerbations. Methods: A SMART aim statement was created with the goal of increasing consults by the palliative care service for patients admitted to the hospital with CF exacerbation. We measured the number of palliative care consult orders placed over a 2-week period. These charts were then reviewed for the presence of corresponding consult orders and notes. We conducted two PDSA (Plan-Do-Study-Act) cycles. The first intervention was sending informational emails to the pulmonary team covering the inpatient service. The second intervention was an educational session for pediatric pulmonologists and fellows to learn from social workers and palliative care specialists about their training and indication for consults. Social workers also helped educate the patients and their families on the additional benefit of palliative care services. Results: Baseline data over a 2-week period suggested that none of the CF inpatients had received involvement from the palliative care team during the corresponding admission. The percentage of CF patients who received palliative care consults increased to 33% from baseline in 5 weeks at the end of the second intervention. Conclusion: The inter-professional quality improvement model was effective in increasing palliative care consults for CF patients admitted to pulmonary service. Educational initiatives regarding palliative care services are an effective intervention to facilitate this process. Further data are required to measure outcomes in the form of feedback from patients, families and providers.
Kotwal N; Kilaikode S; Koumbourlis A; Perez G
Pediatric Pulmonology
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.23840" target="_blank" rel="noreferrer">10.1002/ppul.23840</a>
Advanced care planning in cystic fibrosis
cystic fibrosis; 7782-44-7 (oxygen); chest tube; Child; Clinical Article; comfort; durable power of attorney; Female; Forced Expiratory Volume; hemoptysis; Hospitalization; Human; lifespan; living will; Male; outpatient; oxygen; Palliative therapy; Pilot study; pneumothorax; practice guideline; school child; thinking
Background: Advanced care planning (ACP) is recommended for people with cystic fibrosis (CF). To date, a CF-specific consensus statement that would help CF care teams incorporate ACP into clinical practice has not been developed. Additional research on ACP topics and preferences for optimal implementation is needed to support evidence-based incorporation into routine CF care. Objective: To assess ACP experiences and preferences among individuals with CF in order to inform future interventions aimed at improving ACP in CF. Methods: We surveyed 41 patients with CF aged >=12 years participating in a pilot study of a primary palliative care intervention (Coping, goal Assessment, and Relief from Evolving CF Symptoms [CF-CARES]). We assessed 4 domains of ACP: prior thoughts about ACP, comfort with ACP, preferences for ACP, and prior completion of ACP. We also evaluated the impact of disease severity on certain measures. Severe disease was defined as: Forced Expiratory Volume in 1 second (FEV<inf>1</inf>) <30%, >=4 CF hospitalizations in past year, ever had pneumothorax requiring chest tube placement, ever had massive hemoptysis/hemoptysis requiring hospitalization, or current home oxygen use. Results: We found that most participants worry about ACP topics: 92% worry about the impact of CF on their lifespan and 84% worry about what living with CF would be like if they were to get sicker. The majority (52%) had thought at least "somewhat" about what their important goals and wishes would be if their health situation were to worsen. Only 37% had specific wishes about the types of medical treatment they would or wouldn't want at end of life. The vast majority of participants reported feeling very comfortable talking with CF providers about ACP topics. However, only 5% reported previously talking to a CF team member about the care they would want if they became too ill to make decisions on their own. Few participants (11%) had completed a durable power of attorney for health care or living will. Participants overall preferred to have ACP discussions initiated by any member of the CF team who knows them well, during a period of stability when generally healthy but meeting a certain threshold (e.g. >=4 hospitalizations per year or FEV<inf>1</inf> <40%), and in the outpatient setting. Severe disease was not statistically associated with subjects' worry about getting sicker, comfort talking to CF providers about ACP, or setting preferences for ACP. Conclusions: Our results support the need for a consistent approach to ACP for CF patients earlier in the illness course when patients are still generally healthy. People with CF appear to worry about ACP topics, but need more support from the CF team to understand and document their ACP choices. Future guidelines on ACP in CF, as well as CF-specific ACP materials for patients, may help ensure that all individuals with CF benefit from ACP..
Linnemann RW; Friedman D; Altstein L; Georgiopoulos A; Islam S; Bach K; St John A; Moskowitz SM; Yonker LM
Pediatric Pulmonology
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.23840" target="_blank" rel="noreferrer">10.1002/ppul.23840</a>
Attitudes on pain and opioid prescription practices in cystic fibrosis centers
chronic pain; cystic fibrosis; opiate; prescription; 8002-76-4 (opiate); 8008-60-4 (opiate); 53663-61-9 (opiate); Adult; Analgesia; Anxiety; Child; cyst; drug combination; Female; Human; Life Expectancy; Male; Mood; nurse; opiate addiction; Palliative therapy; Prevalence; quality of life; Questionnaire; sinus headache; thorax wall
Introduction: The high incidence and prevalence of chronic pain in patients with CF is well documented. In patient surveys, reports of intense pain range from 33% - 82% of respondents. Commonly reported sources of pain include headache, sinuses, chest pain, and arthralgias. Furthermore, pain interferes with general activities, mood and occupational performance in 41-57% of patients. These studies concluded that the prevalence of pain in CF patients may be underestimated and can greatly affect quality of life (Loganes C, et al. J Cyst Fibros. 2004;3:51-7; Lechtzin N, et al. Chest 2011;140:1598-603). While opioids can be effective for palliation of pain in an end-of-life setting, there are no data on the use of opioids for chronic pain management in the general CF population. Methods: A questionnaire concerning the prevalence and characteristics of pain in patients with CF was distributed to accredited pediatric and adult CF programs throughout the US. Additional questions addressing provider attitudes on pain management and opioid use were also included. Those respondents that do not utilize opioids at their center were able to skip 13 of the 30 questions. Results: Survey responses from 100 programs were analyzed. Responders who completed the survey included CF providers (67%), clinic coordinators (21%) and nurses (6%). Responses came from 51 adult core or affiliated centers (Adult Responders - AR), 36 from pediatric core or affiliated centers (Pediatric Responders - PR), and 13 were from combined programs. While most of the PR (71%) indicated that 0-10% of their patients experience chronic pain, 48% of AR reported that 11-25% of their patients have chronic pain. Furthermore, 43 of the AR said that >50% of those with chronic pain also have comorbid depression or anxiety. When asked to rank types of chronic pain, 56% of PR ranked sinus/headache symptoms as the most common, with GI pain as the second most common. AR ranked chest wall as the most frequent site (57%), with headache/sinus ranked next. Chest wall pain was the most common reason for prescribing opioids in adults with chronic pain. While most centers (83%) report that pain management in patients with CF is a very important or important issue, 48% of AR feel uncomfortable in prescribing opioids. A majority (66%) would prefer a pain specialist to be responsible for prescribing opioids, yet 49% of the CF providers are currently responsible for this task. Only 32% of AR use pain specialists. 88% of PR and 83% of AR would find guidelines on pain management helpful or very helpful. Conclusion: Chronic pain is common in adult CF patients, and management presents a formidable challenge to providers. Most providers would prefer not to prescribe opioids but are often doing so despite inadequate training and a lack of guidelines to follow. Providers have multiple concerns regarding potential drug side effects, most notably, the possibility of opioid addiction. As life expectancy increases for adult patients with CF, chronic pain will likely be more prevalent and problematic. The development of a guideline and/or collaboration with pain specialists will likely be beneficial to both patients and providers..
Yang Y; Mukadam Z; Laxova A; Meyer KC; Hollatz T
Pediatric Pulmonology
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.23840" target="_blank" rel="noreferrer">10.1002/ppul.23840</a>
Advance care planning in adolescents with cystic fibrosis: A quality improvement project
Advance Care Planning; Attitude To Health; Cystic Fibrosis/th [therapy]; Adolescent; Advance Directives; Cystic Fibrosis/pp [physiopathology]; Female; Forced Expiratory Volume; Humans; Male; Noninvasive Ventilation; Oxygen Inhalation Therapy; Patient Care Planning; Proxy; Quality Improvement; Severity Of Illness Index; Surveys And Questionnaires; Terminal Care; Young Adult
INTRODUCTION: Advance care planning (ACP), though recommended, has not been studied in adolescents with cystic fibrosis (CF). This quality improvement project engaged adolescents with advanced CF disease in ACP and assessed patient and CF provider attitudes and preferences regarding ACP discussions and tools. MATERIALS AND METHODS: Patients <=22 years with advanced CF (FEV<sub>1</sub> <=40% predicted, >2 pulmonary exacerbations requiring IV antibiotics in 1 year, and/or use of home oxygen or non-invasive ventilation) were referred to the pediatric palliative care team (PC). After establishing rapport, ACP was discussed using Voicing My CHOiCESTM: An Advanced Care Planning Guide (VMC). Patients completed a survey assessing attitudes and preferences around ACP. PC also led a training session for CF providers around ACP and VMC and provider attitudes were assessed via a pre- and post-training survey. RESULTS: Twelve patients (mean age 17.9+/-2.2 years) reviewed VMC and completed the ACP survey. The majority (83%) found ACP helpful. None felt it was harmful. All found VMC easy to understand and 90% felt it was appropriate for patients with CF. Of participating CF providers (pre-training, n=6; post-training, n=7), 83% found ACP worthwhile, but desired more training in this area. All found the training session useful and felt VMC was appropriate for patients with CF. DISCUSSION: Adolescents with advanced CF disease felt ACP was a positive experience and not harmful. CF providers valued ACP, but desired more training. Both patients and providers felt that VMC was a useful, disease-appropriate tool. Pediatr Pulmonol. 2016;51:1304-1310. � 2016 Wiley Periodicals, Inc.
Kazmerski TM; Weiner DJ; Matisko J; Schachner D; Lerch W; May C; Maurer SH
Pediatric Pulmonology
2016
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ppul.23559" target="_blank" rel="noreferrer">10.1002/ppul.23559</a>
Pediatric Lung Transplantation And End Of Life Care In Cystic Fibrosis: Barriers And Successful Strategies
Cystic Fibrosis (cf); Lung Transplantation
Pediatric lung transplantation has advanced over the years, providing a potential life-prolonging therapy to patients with cystic fibrosis. Despite this, many challenges in lung transplantation remain and result in worse outcomes than other solid organ transplants. As CF lung disease progresses, children and their caregivers are often simultaneously preparing for lung transplantation and end of life. In this article, we will discuss the current barriers to success in pediatric CF lung transplantation as well as approaches to end of life care in this population.
Dellon E; Goldfarb S B; Hayes D Jr; Sawicki G; Wolfe J; Boyer D
Pediatric Pulmonology
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
10.1002/ppul.23748