Remembering the Classics: Chronic Respiratory Failure in Infants

Mark Schreiner MD and John J. (“Jack”) Downes MD

Today’s PAAD by Drs. Mark Schreiner and Jack Downes is a special treat for me and hopefully for all of you too. For the “yutes” reading this, Jack was the legendary chief of Pediatric Anesthesia and Critical Care Medicine at the Children’s Hospital of Philadelphia at the dawn of the modern era of our practice. He and his CHOP colleagues trained and mentored an entire generation of pediatric anesthesiologists and intensivists, many of whom went on to become the leaders in the field.  One of Jack’s mantras was “if you can’t accept the failures you can’t accept the successes”.  In the PICU there were many successes and failures. Amongst the failures of greatest concern were neonates and infants who developed chronic respiratory failure and became ventilator dependent. I asked Mark Schreiner, one of Jack’s fellows and colleagues, to interview Jack for today’s PAAD and to give us the back story of how this paper came about.  Myron Yaster MD

Original Article

Schreiner MS, Downes JJ, Kettrick RG, Ise C, Voit R. Chronic respiratory failure in infants with prolonged ventilator dependency. JAMA 1987 18;258(23). PMID: 3682138

In 1983, when I (MS) was a pediatric anesthesia and critical care fellow, I became interested in how long an orotracheal or nasotracheal tube could be maintained before performing a tracheostomy (See comment below MY). Jack told me that he thought that he had data that could answer that question. He had been following a cohort of patients who as neonates required prolonged mechanical ventilation (> 1 month).  At the time, he had followed over 80 infants with “chronic” respiratory failure. I spent the next 3 years examining the data and reviewing the medical records of this cohort which eventually included 101 infants.

Despite 3 years of effort, there was no answer to my original question. Instead, I found myself enmeshed in a project that had started in 1967 when Jack became involved in the care of an infant with chronic respiratory failure. In 1984, I presented the abstract of our work as the last paper at the last session on the last day of the annual meeting of the Society of Critical Care Medicine. As I sat in the audience waiting my turn, George Gregory who was in the audience stood up and asked piercing questions after every presentation. I was so unnerved, I had to leave the room. Dennis Fisher and Bill Gild were lounging in the bar and decided that what I needed was a stiff drink. Thus, fortified I ventured back to the meeting room prepared to take my lumps. After my presentation, George stood up and instead of tearing our paper to shreds, tried to make the data even better than it was (See comments below). And, Jack handled all the questions from the audience.

Eventually, our paper (my first as a faculty member) representing 20 years of Jack and Bob Kettrick’s work and devotion, was published as the lead article in JAMA.

The paper was a case series presenting the clinical course of 101 infants with chronic respiratory failure, all cared for in the pediatric intensive care unit at The Children's Hospital of Philadelphia between January 1967 and December 1984. Thirty-six children had severe BPD, 50 had congenital anomalies, and 15 had neuromuscular disorders. On average the infants required about a year of mechanical ventilation, about 70% were still alive, ¾ of whom had been weaned from mechanical ventilation. Pulmonary insufficiency and cardiac failure were the predominant causes of death in 17 of 22 infants in the first two years after the onset of CRF. Given that pulse oximetry and end-tidal CO2 monitoring were not available, it was not surprising that 4 of 8 deaths that occurred beyond two years were caused by airway- and ventilator-related accidents. Interestingly, deaths related to ventilator disconnects happened in the hospital as well as at home.

Jack’s interest continued and he helped lead the formation of the Pennsylvania Ventilator Assisted Home Program funded by the state of Pennsylvania. Bob Kettrick was the first head of the program (1979-1987). Jack headed the program from 1987 until 2016.

Comments from MY

The management of patients, really neonates, who require prolonged mechanical ventilation was recently reviewed with surprisingly similar results to the Schreiner paper. In this review, Sauthier et al.1 state that these chronic ventilator dependent patients require “complex multidisciplinary care and have a significant economic and logistical burden with a high mortality rate. Moreover, most survivors developed a significant neurological impairment, even if most of them are successfully weaned of any respiratory or nutritional support. Several causes can contribute to the neurological impairment in this heterogenous population (for example, intra-ventricular hemorrhage, hypoxic ischemic encephalopathy, genetic susceptibility, low cardiac output, etc.). The problem was already described more than three decades ago (today’s original article),2 but very few recent studies presented an updated description of the situation, including the increased use of modern ventilation modes, such as high flow nasal cannula”.  One major difference between the 2 studies: in the current study, “17% of the patients still needed a support at 18 months and most of them were at home with a standard nasal cannula. Only 2% of the patients still required invasive ventilation at this point. However, in the Schreiner et al. study, 25% of the survivors still required a ventilation support even with the longest duration having been over 7 years. The differences are probably due to the evolution in ventilatory modalities and support. The review was of 18 years of followup, but the longest duration of mechanical ventilation in Schreiner’s population was 2533 days (7+ years).

Mark also highlighted his terror of George Gregory’s ability to eviscerate bad science and presentations at national meetings.  Although it is true that George did not suffer fools gladly, he was extremely gentle and generous with first time presenters.  His comments were almost always spot on and his suggestions often turned abstracts into published papers.

Mark and Jack’s reflections on the origin of this paper and the intense work it took to bring it to publication illustrate for us all the role of the importance of curiosity in the advancement of clinical knowledge and practice: asking a question, discussing it with a mentor and availing oneself of clinical resources available to answer the question (although current data repositories may make this effort much less time consuming). Also highlighted is the importance of presenting such data analysis to colleagues at meetings and getting input from colleagues near and far, who themselves may become mentors. These are valuable lessons for current trainees and junior faculty.

Finally, as I think most of you know or suspected, I was a Jack Downes fellow too.  You should note that in Mark’s opening statement he said he wondered “how long an orotracheal or nasotracheal tube could be maintained”.  He did not say an oral endotracheal tube could be in place.  Precision of language was a hallmark of the CHOP training.  “It’s a tracheal tube not an endotracheal tube” was their mantra. I once asked one of Jack’s colleagues, Russ Raphaely, if he was “free at 3:00 for a meeting?”  His response: “I’m never free…indeed, I am actually quite expensive. On the other hand I can be available…”  As a visiting medical student at CHOP in 1975, like Dorothy in the Wizard of Oz, I realized immediately “I wasn’t in Kansas anymore!” My experience there changed my entire life and I am eternally grateful to Jack and all of his colleagues for helping make me who I am today.  Myron Yaster MD

References

1.         Sauthier M, Sauthier N, Bergeron Gallant K, Lodygensky GA, Kawaguchi A, Emeriaud G, Jouvet P: Long-Term Mechanical Ventilation in Neonates: A 10-Year Overview and Predictive Model. Front Pediatr 2021; 9: 689190

2.         Schreiner MS, Downes JJ, Kettrick RG, Ise C, Voit R: Chronic respiratory failure in infants with prolonged ventilator dependency. Jama 1987; 258: 3398-404