Management of diuretics in infants with bronchopulmonary dysplasia discharged on home oxygen.

BACKGROUND: Infants with bronchopulmonary dysplasia (BPD) are often prescribed diuretics before the neonatal intensive care unit (NICU) discharge. It is unknown whether outpatient medication weaning strategies affect the duration of home oxygen therapy. METHODS: This was a secondary cohort analysis of infants born <32 weeks gestational age with BPD from 2015 to 2018 discharged from our NICU or regional NICUs, referred to our pulmonary clinic for home oxygen management. We compared three groups: those discharged with no diuretics, diuretics actively weaned (dose decreased), and diuretics passively weaned (dose not adjusted). RESULTS: Out of 125 infants, 116 were included in the analysis. Forty-five infants were discharged without diuretics, 52 infants were discharged with diuretics that were actively weaned, and 19 infants were discharged with diuretics that were passively weaned. Infants who were passively weaned spent the most time on home oxygen (median 28 weeks, interquartile range [IQR] 16-52; p = 0.011); there were no differences in home oxygen duration in infants actively weaned (median 13 weeks, IQR 10-26) versus not on diuretics (median 22 weeks, IQR 12-30, p = 0.285). Multivariable adjustment for other illness characteristics associated with the duration of home oxygen did not change this finding. CONCLUSIONS: Active weaning of diuretics did not prolong the duration of home oxygen, in the setting of a standardized clinical guideline for weaning home oxygen in infants with BPD. These data can serve as baseline information to implement and test standardized strategies for outpatient medication management.

Using a home oxygen weaning protocol and pCO2 to evaluate outcomes for infants with bronchopulmonary dysplasia discharged on home oxygen.

BACKGROUND: Predischarge capillary blood gas partial pressure of carbon dioxide (pCO2 ) has been associated with increased adverse events including readmission. This study aimed to determine if predischarge pCO2 or 36-week pCO2 was associated with increased respiratory readmissions or other pulmonary healthcare utilization in the year after neonatal intensive care unit (NICU) discharge for infants with bronchopulmonary dysplasia (BPD) discharged with home oxygen, using a standardized outpatient oxygen weaning protocol. METHODS: This was a secondary cohort analysis of infants born <32 weeks gestational age with BPD, referred to our clinic for home oxygen therapy from either from our level IV NICU or local level III NICUs between 2015 and 2017. Infants with major nonrespiratory comorbidities were excluded. Subject information was obtained from electronic health records. RESULTS: Of 125 infants, 120 had complete 1-year follow-up. Twenty-three percent of infants experienced a respiratory readmission after NICU discharge. There was no significant association between predischarge or 36-week pCO2 and respiratory readmissions, emergency room visits, new or increased bronchodilators, or diuretics. Higher 36-week pCO2 was associated with a later corrected age when oxygen was discontinued (<6 months; median, 54 mmHg; interquartile range [IQR], 51-61; 6-11 months; median, 62 mmHg; IQR, 57-65; ≥12 months, median, 66 mmHg; IQR, 58-73; p = .006). CONCLUSIONS: Neither predischarge pCO2 nor 36-week pCO2 was associated with 1-year respiratory readmissions. However higher pCO2 at 36 weeks was associated with a longer duration of home oxygen. Neonatal illness measures like 36-week pCO2 may be useful in communicating expectations for home oxygen therapy to families.

Impaired lung function following e-cigarette or vaping product use associated lung injury in the first cohort of hospitalized adolescents.

BACKGROUND: Beginning June 2019, Children's Wisconsin was the first hospital to identify a cohort of adolescent patients hospitalized with symptoms likely associated with e-cigarette use. Our report adds to the growing literature describing the radiographic, gross and cytopathologic bronchoscopic findings, and short-term lung function outcomes in this cohort of adolescents with e-cigarette or vaping product use associated lung injury (EVALI). METHODS: We present 15 adolescents hospitalized from June to September, 2019 with confirmed EVALI. We abstracted data from inpatient hospitalization and first outpatient pulmonary clinic visit. RESULTS: There were 15 patients (11 male, 12 White) with a mean age of 17.1 years. All patients presented with subacute pulmonary, gastrointestinal and constitutional complaints. Diagnostic workup was guided by the Centers for Disease Control criteria for confirmed EVALI case surveillance. Flexible bronchoscopy was performed in 13/15 patients with 10/13 demonstrating gross pathologic abnormalities. Seven of 15 patients required intensive care and 2 met criteria for pediatric Acute Respiratory Distress Syndrome. Patients had dramatic improvement with systemic glucocorticoid therapy and 14/15 were discharged on room air. Eleven patients were seen as outpatients. Despite 11/11 patients reporting resolved or improved symptoms, 7/11 had abnormalities on pulmonary function testing. We initiated inhaled corticosteroids for 5/11 patients and 4/11 patients remained on their corticosteroid wean. CONCLUSIONS AND RELEVANCE: We report short-term outcomes of the first cohort of adolescent patients hospitalized with EVALI. An association is observed between clinical improvement and treatment with systemic corticosteroids. However, residual airway reactivity or diffusion abnormalities persisted when patients were re-evaluated in the short-term period (mean 4.5 weeks).

Polysomnography Provides Useful Clinical Information in the Liberation from Respiratory Technology: A Retrospective Review.

Background The prevalence of respiratory-technology dependent children is increasing although for most children the goal is liberation from technology. Liberation from home mechanical ventilation (HMV) and decannulation strategies vary due to the lack of clinical practice standards. The primary objective of this study was to describe our practice utilizing a polysomnography (PSG) in the liberation from respiratory-technology process. Methods Retrospective study of tracheostomized children with and without HMV who underwent an evaluation for decannulation between January 2006 and June 2016. Patient demographics, indication for tracheostomy, indication for PSG, PSG results and interventions performed after the PSG were collected. RESULTS: We identified 153 decannulation attempts in 148 children. Ninety-nine children had a tracheostomy only and 49 children had a tracheostomy with HMV. There were 190 PSGs performed. Almost two-thirds of the children (N = 92) had at least one PSG, 37 children (25%) had two and 19 children (13%) had more than 2 PSGs. Children with tracheostomy and HMV had more PSGs compared to children with tracheostomy only. PSGs were performed at four points: (1) prior to tracheostomy placement (N = 23); (2) to titrate HMV (N = 19); (3) off-HMV support (N = 43); and with a capped tracheostomy (N = 101). Most of the off-HMV PSGs (N = 39) were favorable for discontinuing HMV. About two-thirds of the capped PSGs (N = 73) were favorable for decannulation; of the unfavorable capped PSGs (N = 28), thirteen required airway surgeries following the unfavorable PSG. CONCLUSION: : Overnight PSG provides useful information to the liberation process, particularly when determining readiness for discontinuing HMV and decannulation.

Liberation from home mechanical ventilation and decannulation in children.

BACKGROUND: The prevalence of children requiring outpatient invasive long-term mechanical ventilation is increasing. For some children, liberation from home mechanical ventilation (HMV) and decannulation is the desired outcome. This study describes our experience liberating tracheostomy and HMV (T-HMV) dependent children from respiratory technologies. METHODS: We reviewed charts of T-HMV dependent children who were cared for at our institution and decannulated between July 1999 and December 2011. Patient characteristics, diagnoses, and important steps leading to decannulation were recorded. RESULTS: Forty-six children achieved HMV independence and decannulation. The most common indications for T-HMV were lower airway and parenchymal lung disease. The median ages at tracheotomy, initiation of HMV, initiation of tracheostomy collar (TC) trials, HMV independence, and decannulation were 3.5, 6.0, 12.0, 25.5, and 40.5 months, respectively. Twenty-five children (54%) skipped either using a speaking valve, tracheostomy capping, or both without increased likelihood of recannulation. (P = 0.03). Common procedures prior to decannulation were airway surgery, bronchoscopy, and polysomnography (n = 30, 46, and 46 children, respectively). A median of 9.5 clinic visits and 5 hospitalizations occurred from initial hospital discharge to just prior to decannulation. HMV was primarily weaned as an outpatient. CONCLUSION: Liberation from respiratory technology is a complex, multi-step process that can be accomplished in medically complex children with varying underlying disease processes at relatively young ages. Five major steps (tracheotomy, initiation of HMV, initiation of TC trials, HMV independence, and decannulation) performed in conjunction with clinic visits, procedures, and home nursing support were integral in the successful decannulation process. Pediatr Pulmonol. 2016;51:838-849. © 2016 Wiley Periodicals, Inc.

Treatment of pediatric restless legs syndrome.

OBJECTIVE: The primary aim was to determine if iron supplementation effectively treats children with restless legs syndrome (RLS), the time to improvement or resolution of symptoms, and patient characteristics (family history of RLS, secondary sleep disorders, medical diagnoses, and/or mental health diagnoses) that may affect outcome. METHODS.: This was a retrospective chart review of children between 5 and 18 years old who were diagnosed with RLS at the pediatric sleep disorders clinic at Children's Hospital of Wisconsin in Milwaukee, Wisconsin. Documented RLS treatment approaches included supplemental iron, nonpharmacologic interventions, melatonin, gabapentin, clonidine, and dopamine agonists (pramipexole and ropinirole). RESULTS: Ninety-seven children were diagnosed with RLS; 60.8% of children were between 5 and 11 years old. Most children (65%) received iron either as monotherapy or in combination with other treatments. Approximately 80% of the children who received iron and had follow-up had improvement or resolution of their symptoms. The median baseline ferritin level was 22.7 ng/mL, and 71% of children had a ferritin level less than 30 ng/mL. The median time to improvement or resolution of symptoms was 3.8 months. CONCLUSIONS: Supplemental iron as monotherapy or in combination with other treatments is effective in treating pediatric RLS. A prospective study could help determine if the initial ferritin level and degree of change in the ferritin level impact response to iron treatment. It is also important to study the long-term outcomes in these patients.

Severe central sleep apnea in a child with leukemia on chronic methadone therapy.

We describe a child with acute myeloid leukemia (AML) who developed severe central sleep apnea (CSA) on methadone therapy for chronic pain management. His chemotherapy-related cerebral atrophy and renal insufficiency with impaired methadone clearance may have also contributed to the severity of his sleep-disordered breathing. Maintenance methadone treatment is not a common pediatric practice; therefore, the adverse effects of methadone therapy, including CSA, are rarely reported in children.

Non-respiratory indications for polysomnography and related procedures in children: an evidence-based review.

OBJECTIVE: This evidence-based review provides a systematic and comprehensive review of the literature regarding the utility of polysomnography for the evaluation of non-respiratory sleep disorders in children including hypersomnias, parasomnias, sleep-related movement disorders, and sleep in other special populations. METHODS: A task force of pediatric sleep medicine experts performed a systematic review of the literature regarding the use of polysomnography for non-respiratory sleep disorders in children. They identified and graded 76 papers as evidence. RESULTS: The main results include (1) polysomnography combined with the multiple sleep latency test is useful for evaluating disorders of excessive somnolence to objectively quantify sleepiness. The results have to be interpreted with consideration of the pubertal stage and regularity of the sleep patterns of the child; (2) polysomnography is indicated in children with parasomnias or sleep related movement disorders who have a high likelihood of having obstructive sleep apnea (OSA); (3) polysomnography is not routinely indicated in children with enuresis unless there is a high likelihood of OSA; (4) polysomnography can be helpful in evaluating children with restless legs syndrome (RLS) and when periodic limb movement disorder (PLMD) is suspected. CONCLUSIONS: These findings suggest that, in children with non-respiratory sleep disorders, polysomnography should be a part of a comprehensive sleep evaluation in selected circumstances to determine the nature of the events in more detail or when the suspicion of OSA is relatively high.

Executive summary of respiratory indications for polysomnography in children: an evidence-based review.

OBJECTIVE: This comprehensive, evidence-based review provides a systematic analysis of the literature regarding the validity, reliability, and clinical utility of polysomnography for characterizing breathing during sleep in children. Findings serve as the foundation of practice parameters regarding respiratory indications for polysomnography in children. METHODS: A task force of content experts performed a systematic review of the relevant literature and graded the evidence using a standardized grading system. Two hundred forty-three evidentiary papers were reviewed, summarized, and graded. The analysis addressed the operating characteristics of polysomnography as a diagnostic procedure in children and identified strengths and limitations of polysomnography for evaluation of respiratory function during sleep. RESULTS: The analysis documents strong face validity and content validity, moderately strong convergent validity when comparing respiratory findings with a variety of relevant independent measures, moderate-to-strong test-retest validity, and limited data supporting discriminant validity for characterizing breathing during sleep in children. The analysis documents moderate-to-strong test-retest reliability and interscorer reliability based on limited data. The data indicate particularly strong clinical utility in children with suspected sleep related breathing disorders and obesity, evolving metabolic syndrome, neurological, neurodevelopmental, or genetic disorders, and children with craniofacial syndromes. Specific consideration was given to clinical utility of polysomnography prior to adenotonsillectomy (AT) for confirmation of obstructive sleep apnea syndrome. The most relevant findings include: (1) recognition that clinical history and examination are often poor predictors of respiratory polygraphic findings, (2) preoperative polysomnography is helpful in predicting risk for perioperative complications, and (3) preoperative polysomnography is often helpful in predicting persistence of obstructive sleep apnea syndrome in patients after AT. No prospective studies were identified that address whether clinical outcome following AT for treatment of obstructive sleep apnea is improved in association with routine performance of polysomnography before surgery in otherwise healthy children. A small group of papers confirm the clinical utility of polysomnography for initiation and titration of positive airway pressure support. CONCLUSIONS: Pediatric polysomnography shows validity, reliability, and clinical utility that is commensurate with most other routinely employed diagnostic clinical tools or procedures. Findings indicate that the "gold standard" for diagnosis of sleep related breathing disorders in children is not polysomnography alone, but rather the skillful integration of clinical and polygraphic findings by a knowledgeable sleep specialist. Future developments will provide more sophisticated methods for data collection and analysis, but integration of polysomnographic findings with the clinical evaluation will represent the fundamental diagnostic challenge for the sleep specialist.

Emerging medicolegal issues in the practice of pediatric sleep medicine.

With the rapid growth in the field of pediatric sleep medicine, health care providers need to be aware of several emerging legal issues that have the potential of impacting their clinical practice. This article provides an overview of emerging legal areas that might impact the practice of pediatric sleep medicine, and discusses civil liability emerging from medical malpractice, issues that health care providers must be aware of including issues related to providing care for minors, and newer areas that relate to legal prosecution for health care fraud as it may relate to violations of quality of care.

Diagnostic studies in the assessment of pediatric sleep-disordered breathing: techniques and indications.

In summary, SDB is a common condition in children that can have serious complications if left undiagnosed and untreated. OSA is the most commonly recognized form of SDB in children. The history and physical have limitations in their capacity to determine which children have SDB. Abbreviated or screening techniques, such as audio- or videotaping, oximetry studies, nap studies, or home studies, tend to be helpful if the results are positive but have a poor predictive value if the results are negative. Overnight polysomnography is the gold standard for the diagnosis of SDB in children. It is important that children are studied in laboratories that have expertise with children. Often, children with SDB have associated nonrespiratory or behavioral sleep disorders that also must be evaluated and addressed.