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>
Parent-Reported Outcomes of Prognostic Communication in Hospitalized Children with Advanced Heart Disease (S762)
adult; cardiology; Caucasian; child; cohort analysis; conference abstract; controlled study; conversation; decision making; expectation; female; heart single ventricle; high school; hospitalization; hospitalized child; human; infant; intubation; length of stay; major clinical study; male; married person; medical record review; mother; prognosis; prospective study; pulmonary hypertension; pulmonary vein stenosis; recall; young adult
Objectives: * Describe a prospective study to evaluate how parents of children with advanced heart disease (AHD) perceive communication about prognosis with their child's physicians. * Describe results from this analysis that found no correlation between length of stay of a child with advanced heart disease and parent reports of conflicting information from physicians. Original Research Background and Objectives: Communication between parents and physicians is essential to high-quality care. For families of patients with AHD, understanding prognosis is critical to decision making and is associated with less perceived suffering at end of life. This is the first study to prospectively evaluate how parents of children with AHD perceive prognostic communication with their child's physicians.
Miller M K; Morell E; Feraco A; Goldberg S; Reichman J; Sahakian L; Sleeper L; Blume E
Journal of Pain and Symptom Management
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/10.1016/j.jpainsymman.2019.12.323" target="_blank" rel="noreferrer noopener">10.1016/j.jpainsymman.2019.12.323</a>
Sleep abnormalities in untreated patients with mucopolysaccharidosis type VI
clinical feature; priority journal; preschool child; cross-sectional study; prospective study; human; article; child; female; male; controlled study; clinical article; prevalence; disease severity; pulmonary hypertension; polysomnography; disease association; anamnesis; apnea; Doppler echocardiography; Glycosaminoglycans; Lysosomal storage diseases; lysosome storage disease; macroglossia; Maroteaux Lamy syndrome; Morquio syndrome; Mucopolysaccharidosis; oxygen saturation; oxygen/ec [Endogenous Compound]; physical examination; pigeon thorax; Sleep apnea; sleep apnea syndrome/di [Diagnosis]; sleep disorder; snoring; breathing difficulties; MPSVI; trajectory; characteristics; witnessed apnea
Mucopolysaccharidosis type VI (MPS VI) is a lysosomal storage disease that affects an enzyme responsible for the degradation of glycosaminoglycans (GAGs). Partially degraded GAGs accumulate in several tissues, such as the upper airways (UA), which leads to the development of obstructive sleep apnea (OSA). Our objective was to determine the prevalence of OSA in a group of untreated patients with MPS VI and the association of OSA with clinical and echocardiographic findings. Patients aged 4 years or older with a biochemical diagnosis of MPS VI were included. Data about clinical history, physical examination, Doppler echocardiogram, and overnight polysomnography (PSG) were collected. Our results showed that of the 28 participants, 14 were boys; mean age was 98.5 months, and mean age at MPS VI diagnosis was 48.4 months. Snoring, witnessed apnea, pectus carinatum, and macroglossia were the main clinical findings. PSG results showed that 23:27 patients (85.1%) had OSA which was mild in 4, moderate in 5, and severe in 14 patients. Echocardiograms showed evidence of pulmonary hypertension (PH) in 14 patients. Lower (P=0.037) and nadir SpO<inf>2</inf> (P=0.007) were positively associated with PH. Clinical signs suggestive of respiratory abnormalities during sleep were not significantly correlated with the results of PSG. We conclude that the prevalence of OSA in patients with MPS VI was high, and the level of desaturation was positively correlated with PH. Symptoms during sleep were not associated with PSG findings, which suggests that this population should undergo routine PSG as earlier as possible. This study provides baseline data to estimate the potential impact of specific treatments in the sleep abnormalities presented by patients with MPS VI. © 2011 Wiley-Liss, Inc.
John A; Fagondes S; Schwartz I; Azevedo A C; Barrios P; Dalcin P; Menna-Barreto S; Giugliani R
American Journal of Medical Genetics Part A
2011
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/ajmg.a.33902" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.33902</a>
Talking to families when death becomes a likely outcome-a pilot simulation programme to improve end of life conversations during and around PICU admission
conversation;death;simulation;advanced cancer;bone marrow transplantation;cancer staging;cerebrovascular accident;Child;conference abstract;controlled study;craniofacial surgery;experimental therapy;human;immunology;life sustaining treatment;multidisciplinary team;nursing role;oncology;palliative therapy;pulmonary hypertension;treatment failure
Aims & Objectives: End of life conversations form the basis of many complex communication scenarios in paediatric intensive care (PICU). These conversations are sometimes initiated late in the disease process. Anecdotal evidence is that many clinicians shy away from the subject matter for a wide variety of reasons. A multidisciplinary, one-day pilot simulation course to address this unmet need was designed by the PICU and palliative care teams for members of the multidisciplinary team (MDT) who admit to our PICU. Methods The course was oversubscribed and candidates were evenly drawn from medical and senior nursing roles; specialties represented included PICU, immunology, bone marrow transplant, oncology, pulmonary hypertension and craniofacial surgery. A range of clinically complex and personally challenging clinical scenarios were developed for use with experienced educational actors. These included: Previously fit and well child post cerebrovascular accident with a deeply religious family Child with end stage cancer whose parents had the option of an experimental treatment Child with advanced pulmonary hypertension whose parents are angry about failure of treatment Child with incurable malignancy with parents reluctant to ask for withdrawal of life sustaining treatment but who secretly wish it to be offered A demonstration scenario was run by the faculty for all candidates and four simulations were run in groups of four. Simulation safety and debrief tools were utilised throughout Results Feedback from the day was universally positive. Conclusions There is significant need for this course. Plans are now under way to extend it to a wider cohort of the PICU MDT and run it on a regular basis.
Sidgwick P;Du Pre P;Skellett S
Pediatric Critical Care Medicine
2018
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/10.1097/01.pcc.0000537439.06687.80" target="_blank" rel="noreferrer noopener">10.1097/01.pcc.0000537439.06687.80</a>
Timing of Pediatric Palliative Care Consults in Hospitalized Patients with Heart Disease
intensive care unit; Palliative Care; child; article; female; human; major clinical study; male; newborn; retrospective study; advance care planning; palliative therapy; medical decision making; pediatrics; artificial ventilation; terminal care; hospitalization; information technology; hospital patient; patient referral; life sustaining treatment; congenital heart disease; consultation; mortality; pulmonary hypertension; infant; hospitalized child; length of stay; pediatric patient; extracorporeal oxygenation; disease course; cardiomyopathy; heart disease; catheterization; myocarditis; myopericarditis; pericarditis
Pediatric palliative care (PPC) provides an extra layer of support for families caring for a child with complex heart disease as these patients often experience lifelong morbidities with frequent hospitalizations and risk of early mortality. PPC referral at the time of heart disease diagnosis provides early involvement in the disease trajectory, allowing PPC teams to longitudinally support patients and families with symptom management, complex medical decision-making, and advanced care planning. We analyzed 113 hospitalized pediatric patients with a primary diagnosis of heart disease and a PPC consult to identify timing of first PPC consultation in relation to diagnosis, complex chronic conditions (CCC), and death. The median age of heart disease diagnosis was 0 days with a median of two CCCs while PPC consultation did not occur until a median age of 77 days with a median of four CCCs. Median time between PPC consult and death was 33 days (interquartile range: 7-128). Death often occurred in the intensive care unit (n = 36, 67%), and the most common mode was withdrawal of life-sustaining therapies (n = 31, 57%). PPC referral often occurred in the context of medical complexity and prolonged hospitalization. Referral close to the time of heart disease diagnosis would allow patients and families to fully utilize PPC benefits that exist outside of end-of-life care and may influence the mode and location of death. PPC consultation should be considered at the time of heart disease diagnosis, especially in neonates and infants with CCCs.Copyright © 2023 Georg Thieme Verlag. All rights reserved.
Green DJ; Bennett E; Olson LM; Wawrzynski S; Bodily S; Moore D; Mansfield KJ; Wilkins V; Cook L; Delgado-Corcoran C
Journal of Pediatric Intensive Care
2023
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/10.1055/s-0041-1730916" target="_blank" rel="noreferrer noopener">10.1055/s-0041-1730916</a>