Did the Fat Boy Snore?

Abstract

It is assumed by many clinicians that Joe, the rotund, hypersomnolent messenger boy from the Pickwick Papers by Charles Dickens, had obstructive sleep apnea. His signs and symptoms were popularized by Burwell in 1956 as the “Pickwickian syndrome.”1Burwell CS Robin ED Whaley RD Bickelmann AG Extreme obesity associated with alveolar hypoventilation—a Pickwickian syndrome.Am J Med. 1956; 21: 811-818Abstract Full Text PDF PubMed Scopus (529) Google Scholar With the first descriptions by Gastaut et al2Gastaut H Tassinari CA Duron B Polygraphic study of the episodic diurnal and nocturnal (hypnic and respiratory) manifestations of the Pickwick syndrome.Brain Res. 1966; 2: 167-186Crossref Scopus (300) Google Scholar of obstructive sleep apnea, many sleep investigators have concluded that the Pickwickian syndrome or obesity-hypoventilation syndrome, as it is often called, invariably is caused by recurrent obstructive apneas occurring during sleep.3Sharp JT Barrocas M Chokroverty S The cardiorespiratory effects of obesity.Clin Chest Med. 1980; 1: 103-118Abstract Full Text PDF PubMed Google Scholar In this issue of Chest (see page 627) Rapaport and colleagues provide data that question this concept. They studied eight patients with obstructive sleep apnea and the obesity-hypoventilation syndrome. In four of their patients, treatment with either tracheostomy or a nasal CPAP device not only abolished the apneas, but also normalized arterial blood gases. The remaining four, despite correction of the sleep apnea, continued to hypoventilate. The authors postulated that these latter four are the “true Pickwickians” with chronic alveolar hypoventilation of unknown etiology. The additional mechanical stress of recurrent obstructive apneas may have tipped them over and led to worsening pulmonary hypertension and right-sided heart failure. The authors could only speculate whether the alveolar hypoventilation was acquired or instead, resulted from a congenital abnormality in ventilatory control. Over the last five years, I have had the opportunity to observe at least four patients who presented with all the classic symptoms of the obesity-hypoventilation syndrome (morbid obesity, hypersomnolence, polycythemia, pulmonary hypertension, cor pulmonale, daytime hypoxemia and chronic hypercapnia), yet demonstrated no episodes of apnea on repetitive nocturnal polysomnographic testing. It thus appears that the obesity-hypoventilation syndrome may occur as a result of recurrent obstructive apneas, as a result of a primary defect in alveolar ventilation, or secondary to a combination of the two factors. This evidence that the obesity-hypoventilation syndrome is not a distinct entity may explain seemingly discrepant results from previous studies. A population of obesity-hypoventilators studied by one sleep researcher may not be equivalent to a group of subjects evaluated by another. For example, Sampson et al4Sampson MG Grassino A Neuromechanical properties in obese patients during carbon dioxide rebreathing.Am J Med. 1983; 75: 81-90Abstract Full Text PDF PubMed Scopus (112) Google Scholar studied a group of patients who formerly had the obesity-hypoventilation syndrome, but were currently eucapnic. They concluded that their patients had inherent blunted central hypercapnic responsiveness and that hypoventilation and cardiac decompensation occurred when they were exposed to an acute respiratory load. Since these authors did not perform sleep studies on their subjects, we do not know whether or not they had coexistent obstructive apnea. Lopata and Onal,5Lopata M Onal E Mass loading, sleep apnea, and the pathogenesis of obesity hypoventilation.Am Rev Respir Dis. 1982; 126: 640-645PubMed Google Scholar on the other hand, found that central respiratory drive was actually not reduced in their group of subjects with the obesity-hypoventilation syndrome. They hypothesized that the obesity-hypoventilation syndrome resulted instead from impairment in both neuromuscular coupling and in respiratory muscle inefficiency.5Lopata M Onal E Mass loading, sleep apnea, and the pathogenesis of obesity hypoventilation.Am Rev Respir Dis. 1982; 126: 640-645PubMed Google Scholar Their subjects, however, did have obstructive sleep apnea and may not be comparable to Sampson's subjects. How can we tell whether the fat boy Joe had obstructive sleep apnea or was a “true Pickwickian?” One clue would be to see if he snored. Since snoring is a sound produced by inspiring through a partially obstructed upper airway, the presence of snoring would suggest, though not confirm, the presence of obstructive apnea. Careful perusal of Dickens’ text has indeed revealed that the fat boy did snore: He “goes on errands fast asleep, and snores as he waits at table.”6Dickens C The posthumous papers of the Pickwick Club. Grosset & Dunlap, New York1930: 48Google Scholar Based on the study of Rapoport and colleagues, one wonders whether Joe's hypoventilation could have been corrected if our current treatment modalities were available in Dickens’ time.