Thursday, July 28, 2022

Long COVID in Children - What is it?

 COVID 19 infections in children and adolescent or rarely associated with a severe respiratory illness.  However, persistent respiratory symptoms have been reported in children after a COVID 19 infection.  With increased awareness about "long COVID" in adults, the question could be asked does such a disease exist in children.  This study attempted to answer this question by studying adolescents after COVID infection who had persistent respiratory symptoms like cough and shortness of breath.   The most common conditions identified in this small cohort of children included asthma features, paradoxical vocal fold motion, deconditioning and dysautonomia.



Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes long-term pulmonary sequelae in adults, but little is known about pulmonary outcomes in pediatrics.


The aim of this study was to describe long-term subjective and objective pulmonary abnormalities after SARS-CoV-2 infection in pediatric populations.


Single-center, retrospective cohort of patients seen in post-coronavirus disease 2019 (COVID-19) pulmonary clinic in 2021. Subjects evaluated had persistent pulmonary symptoms 4 weeks or more after initial infection. Clinical testing included a 6-min walk test (6MWT), chest X-ray, pre- and postbronchodilator spirometry, plethysmography, and diffusion capacity. Patients were followed 2-to-3-months after the initial visit with repeat testing. The primary outcome was the presence of abnormal pulmonary function testing. Secondary measures included variables associated with pulmonary outcomes.


Eighty-two adolescents were seen at a median of 3.5 months postinfection, with approximately 80% reporting two or more symptoms at clinic presentation (cough, chest pain, dyspnea at rest, and exertional dyspnea). At follow-up (~6.5 months) exertional dyspnea persisted for most (67%). Spirometry was normal in 77% of patients, but 31% had a positive bronchodilator response. No abnormalities were noted on plethysmography or diffusion capacity. Clinical phenotypes identified included inhaled corticosteroid responsiveness, paradoxical vocal fold motion disorder, deconditioning, and dysautonomia. Multivariable modeling demonstrated that obesity, anxiety, and resting dyspnea were associated with reduced 6MWT, while female sex and resting dyspnea were associated with higher Borg Dyspnea and Fatigues scores.


This is the largest study to date of pediatric patients with long-term pulmonary sequelae post-COVID-19. Identified clinical phenotypes and risk factors warrant further study and treatment.

Read article here.

Monday, April 4, 2022

New Narcolepsy Therapy?

Narcolepsy is a rare disorder associated with excessive sleepiness despite adequate sleep time.  A potentially highly efficacious therapy for narcolepsy, Xywav, is complicated by a difficult dosing regimen requiring twice nightly administration for optimal effect.  A new therapy in development by Avadel may provide a similar benefit in a once nightly dose.

 Avadel Pharmaceuticals plc, whose marketing application for narcolepsy drug candidate FT218 is under review by the US Food and Drug Administration (FDA), presented in eight posters at World Sleep 2022. FT218 is an investigational formulation of sodium oxybate designed to be taken once at bedtime for the treatment of excessive daytime sleepiness (EDS) or cataplexy in adults with narcolepsy.

Data presented at World Sleep 2022 include results from a discrete choice experiment designed to characterize and quantify drivers of preferences for attributes associated with oxybate treatments for narcolepsy, with a background survey providing patient and clinician perspectives on narcolepsy disease burden, treatment approaches, and satisfaction with current narcolepsy treatment options. New post-hoc data from the completed pivotal Phase 3 REST-ON clinical trial of FT218 and interim data from the ongoing RESTORE open-label extension/switch study of FT218 was also presented.

“We know that untreated narcolepsy can have a devastating effect on patients’ quality of life and ability to function and that additional treatment options would benefit patients and clinicians. Results from the discrete choice experiment identified that the overall driver of treatment choice for oxybate therapy is once-nightly dosing,” says Anne Marie Morse, DO, director of child neurology and pediatric sleep medicine at Geisinger Medical Center at Janet Weis Children’s Hospital, in a release. “Taken together with the robust clinical data already published, along with new post-hoc analyses from the pivotal trial and interim long-term data from the ongoing open-label study….FT218 shows great potential for patient care.”

Highlights from the Poster Presentations

  • The discreet choice experiment confirmed that once-nightly dosing, when compared to twice-nightly dosing, was the most important attribute driving both patient and clinician preference for overall oxybate product choice, as well as patient quality of life and reduction of patient anxiety/stress.
  • Dosing frequency was also viewed as a more important attribute as compared to other attributes assessed, including sodium content.
  • In the REST-ON post-hoc analyses, FT218 demonstrated improvement in subjective measures of daytime sleepiness, sleep quality, and refreshing nature of sleep as early as week 1 with the 4.5 g starting dose, with even greater improvement at week 2 soon after starting the 6 g dose compared to placebo.
  • Additional post-hoc analyses, stratified by narcolepsy type, as well as concomitant stimulant use, or without stimulants, demonstrated positive results that are generally consistent with previously reported positive endpoints from REST-ON and add to the existing body of evidence for FT218.
  • In the first presentation of an interim safety analysis from the ongoing RESTORE study, FT218 has generally been well tolerated, with some participants receiving therapy for more than 18 months; no new safety signals have emerged.

“In our first look at data from the interim analysis of the ongoing RESTORE study, we have affirmed that the tolerability of FT218 is in line with the well-established profile of sodium oxybate. We’re also pleased to share new results from our post-hoc analyses of the REST-ON trial that demonstrated clinically meaningful improvements with FT218 versus placebo in both subjective and objective measurements of narcolepsy symptoms, including EDS and disrupted nighttime sleep, with a dosing regimen preferred by patients and clinicians alike, consistent with our previous observations,” says Douglas Williamson, MD, chief medical officer of Avadel, in a release. “At Avadel, we believe in listening to patients to develop solutions that will have a meaningful impact on their symptoms. Our goal is to do just that for people living with narcolepsy, and we are working to bring FT218 to patients as a once-at-bedtime treatment option as quickly as possible.”

Read article here.

Wednesday, March 16, 2022

Sleep Awareness Week

This is Sleep Awareness Week.  Organizations such as the National Sleep Foundation routinely survey adults and find that sleep disorders are extraordinarily common and can significantly impact quality of life.  Sleep disorders in children and adolescents, though, are vastly underrecognized with between 20-30% of young children affected by disrupted sleep and 60-70% of adolescents experiencing reduced sleep time.  The effects can be numerous, including reduced cognitive function, mental health concerns, and increased metabolic and cardiovascular health risks.  A pediatric sleep specialist is NOT the sleep lab doctor.  A pediatric sleep clinic provides a comprehensive evaluation and treatment for all manners of sleep disorders to provide evidence based treatment recommendations for your entire health care team.

 To kick off Sleep Awareness Week, the National Sleep Foundation released the results from the 2022 Sleep in America Poll. The annual survey is one of the longest running records capturing Americans’ perceptions, attitudes, and behaviors around sleep. 

The 2022 Sleep in America Poll documents significant opportunities to improve daily activities that are associated with a range of positive sleep and health outcomes. Results show that many Americans are not getting bright light exposure during the day and are looking at screens around bedtime. In addition, many fall short of recommendations for exercise and take meals at inconsistent times.

Poll results show:

  • Nearly half of Americans say they aren’t exposed to the recommended levels of bright light when indoors in the morning and afternoon.
  • More than a third of Americans fall short of CDC’s recommendations for moderate or vigorous activity, another key factor in ensuring a sound sleep.
  • Four in 10 Americans eat meals at inconsistent times, making it more difficult for their bodies to regulate the sleep/wake process.
  • More than half of Americans indulge in screen time within an hour before bed or even while in bed.

“Over the last 31 years, the National Sleep Foundation has conducted the Sleep in America Poll to gain a better understanding of Americans’ sleep health and habits. These latest results highlight how people are doing with key daytime behaviors, knowing that what we do during the day and at night is important for sleep,” says John Lopos, CEO, National Sleep Foundation, in a release. “The best practices we continue to share can help you become your ‘Best Slept Self’— and by that we mean the radiant, positive feeling reflected in the way you look, feel, and navigate the day.”

Things you can do to help be your Best Slept Self:

  • Increase exposure to bright light upon waking and throughout the afternoon.
  • Help regulate your body’s sleep/wake process by eating meals at consistent times during the day and avoiding heavy meals 2-3 hours before bed.
  • Avoid screentime at least one hour before bed.
  • Make your bedroom a sleep-friendly space by keeping it cool, dark, and quiet.
  • Follow recommended guidelines on proper exercise to improve overall health and your sleep. Aim for at least 20 minutes of exercise a day.
  • Get the recommended hours of sleep per night. The National Sleep Foundation recommends 7-9 hours for most adults.

The Sleep in America Poll is fielded annually and results are shared during National Sleep Foundation’s Sleep Awareness Week. Sleep Awareness Week runs Sunday, March 13 through March 19. NSF independently produces Sleep Awareness Week, the Sleep in America Poll, and all related official educational content.

Sleep Awareness Week 2022 is supported by unrestricted funds from Jazz Pharmaceuticals, Eisai, Inc., Avadel Pharmaceuticals, Graber, Harmony Biosciences, Huckleberry, Idorsia, and PureCare. 

Read article here.

Monday, March 7, 2022

Asthma Education Reduces Hospitalization and Emergency Room Risk


Asthma is a complex respiratory disorder that significantly raises risk for ER visits and hospitalizations.  Despite it's complexity, there is a consistent knowledge gap that can put patients and parents at a significant disadvantage.  Following a consistent strategy grounded in evidenced based practice can make a difference in reducing chaos and preventing frequent sick visits.  This study demonstrated that a focused asthma education program consistently reduced these risks.

Background It remains unknown whether child-oriented asthma education is associated with better health outcomes. This meta-analysis investigated the effects of asthma education on hospitalisation and emergency department and clinic visits.

Methods We searched the Cochrane Library, PubMed and EMBASE for relevant studies from inception to 4 July 2021, and selected studies that reported hospitalisation or emergency department or clinic visits as outcomes. The participants were only children. Two authors independently selected the studies, assessed the quality of the included studies and retrieved the data. A third senior author was engaged to resolve disagreements. Fifteen longitudinal studies were included for the systematic review and meta-analysis. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 was used as the standard of reporting (PRISMA registration ID is 284509).

Findings Compared with the control group, the asthma education group had 54% lower hospitalisation risk (95% CI 0.32 to 0.66), and 31% lower emergency department visit risk (95% CI 0.59 to 0.81). Sensitivity analysis showed that the asthma education group had a reduced clinic visit risk (risk ratio (RR)=0.80, 95% CI 0.67 to 0.97). Subgroup analysis showed that asthma education involving both children and parents/guardians was associated with fewer hospitalisations (RR=0.38, 95% CI 0.24 to 0.59) and emergency department visits (RR=0.69, 95% CI 0.57 to 0.83). Asthma education in hospitals or non-hospitals can reduce the risk of hospitalisation and emergency department visits. However, only education in the hospitals was associated with the reduction of clinical visits (RR=0.45, 95% CI 0.22 to 0.92).

Interpretation Education is effective for controlling asthma, especially for reducing hospitalisation and emergency department and clinic visits. Education involving both children and parents/guardians is more effective than that involving only children. The setting of asthma education does not impact its effect to a large extent.

Read article here.

Monday, February 7, 2022

Hypoxemia and Risk for Bronchopulmonary Dysplasia In Preterm Infants

Bronchopulmonary dysplasia (BPD) is a chronic lung disease in infants associated with extreme prematurity.  Understanding contributing factors is important since there is a strong link between this condition and neurodevelopmental impairment and other childhood disease.  This study demonstrated a strong association between frequency and severity of oxygen desaturations in the development of severe BPD.  Importantly, oxygen desaturations of 1 minute or longer were significantly correlated.  


Rationale: Bronchopulmonary dysplasia increases the risk of disability in extremely preterm infants. Although the pathophysiology remains uncertain, prior exposure to intermittent hypoxemia may play a role in this relationship. Objectives: To determine the association between prolonged episodes of intermittent hypoxemia and severe bronchopulmonary dysplasia. Methods: A post hoc analysis of extremely preterm infants in the Canadian Oxygen Trial who survived to 36 weeks' postmenstrual age was performed. Oxygen saturations <80% for ⩾1 minute and the proportion of time per day with hypoxemia were quantified using continuous pulse oximetry data that had been sampled every 10 seconds from within 24 hours of birth until 36 weeks' postmenstrual age. The study outcome was severe bronchopulmonary dysplasia as defined in the 2001 NIH Workshop Summary. Measurements and Main Results: Of 1,018 infants, 332 (32.6%) developed severe bronchopulmonary dysplasia. The median number of hypoxemic episodes ranged from 0.8/day (interquartile range, 0.2-1.1) to 60.2/day (interquartile range, 51.4-70.3) among the least and most affected 10% of infants. Compared with the lowest decile of exposure to hypoxemic episodes, the adjusted relative risk of severe bronchopulmonary dysplasia increased progressively from 1.72 (95% confidence interval, 1.55-1.90) at the 2nd decile to 20.40 (95% confidence interval, 12.88-32.32) at the 10th decile. Similar risk gradients were observed for time in hypoxemia. Significant differences in the rates of hypoxemia between infants with and without severe bronchopulmonary dysplasia emerged within the first week after birth. Conclusions: Prolonged intermittent hypoxemia beginning in the first week after birth was associated with an increased risk of developing severe bronchopulmonary dysplasia among extremely preterm infants. Clinical trial registered with (ISRCTN62491227) and 

Read abstract here.

Tuesday, January 18, 2022

Helping High Risk Infants and Parents Sleep Better: ATS Guidelines for Outpatient Care of Premature Infants

Premature infants, particularly born before 30 weeks gestation who have been given a diagnosis of chronic lung disease, bronchopulmonary dysplasia, or post-prematurity respiratory disease face significant challenges in transition from the NICU environment to life at home.  The lack of guidelines to help focus care and quality medical literature differentiating outcomes is stark.  Parents and physicians need tools to care for these high risk infants. These new ATS guidelines nicely evaluate the role of inhaled medications, swallowing assessment, airway endoscopy, and especially sleep testing and provide timely recommendations.

Outpatient Respiratory Management of Infants, Children, and Adolescents with Post-Prematurity Respiratory Disease: An Official American Thoracic Society Clinical Practice Guideline

New recommendations are available to help guide physicians who must determine when and how to treat infants, children and adolescents with post-prematurity respiratory disease (PPRD). The American Thoracic Society has published an official clinical practice guideline in which a multidisciplinary panel of experts provide 13 conditional recommendations on the diagnostic testing and clinical management of these young people. The complete guideline detailing these recommendations was posted online ahead of print in the American Journal of Respiratory and Critical Care Medicine.

Worldwide, approximately 12 million —10 percent of live births—are born prematurely and are at risk for respiratory disease, the most common of which is bronchopulmonary dysplasia (BPD). However, all , even those who do not meet the criteria for having BPD, may develop poor respiratory health later in life with signs and symptoms including cough, recurrent wheezing, exercise intolerance, low blood oxygen (hypoxemia) and reduced pulmonary function. These individuals are classified as having PPRD.

Tuesday, January 11, 2022

SARS-CoV-2 acute bronchiolitis in hospitalized children: Neither frequent nor more severe

SARS-CoV-2 associated respiratory illnesses may include pneumonia, asthma exacerbations and acute bronchiolitis among others.  Risk to children in comparison to adults is remarkably low.  However, acute bronchiolitis is the most common cause of hospitalization in infants.  This study assessed frequency of hospitalization due to SARS-CoV-2 and found frequency and severity to be low, estimated at less than 2% of all hospitalized children.

SARS-CoV-2 acute bronchiolitis in hospitalized children: Neither frequent nor more severe


Endemic coronaviruses have been found in acute bronchiolitis, mainly as a coinfecting virus. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been responsible for respiratory illness in hospitalized children. The characteristics of patients with bronchiolitis have not been extensively described.


Cross-sectional study of patients with bronchiolitis and SARS-CoV-2 infection enrolled in a prospective multicenter cohort of children hospitalized with COVID-19 in Spain from March 1, 2020 to February 28, 2021.


Twelve of 666 children infected with SARS-CoV-2 who required hospital admission met the diagnostic criteria for bronchiolitis (1.8%). Median age was 1.9 months (range: 0.4–10.1). Six cases had household contact with a confirmed or probable COVID-19 case. Main complaints were cough (11 patients), rhinorrhea (10), difficulty breathing (8), and fever (8). Eleven cases were classified as mild or moderate and one as severe. Laboratory tests performed in seven patients did not evidence anemia, lymphopenia, or high C-reactive protein levels. Chest X-rays were performed in six children, and one case showed remarkable findings. Coinfection with metapneumovirus was detected in the patient with the most severe course; Bordetella pertussis was detected in another patient. Seven patients required oxygen therapy. Albuterol was administered in four patients. One patient was admitted to the pediatric intensive care unit. Median length of admission was 4 days (range: 3–14). No patient died or showed any sequelae at discharge. Two patients developed recurrent bronchospasms.


SARS-CoV-2 infection does not seem to be a main trigger of severe bronchiolitis, and children with this condition should be managed according to clinical practice guidelines. 

Pediatric Pulmonology


January 2022

Pages 57-65

Read article here.