The Gasping Syndrome

By Dr. Bastian M.D.

A few times a year, distressed patients present from their internists or pulmonologists to see if I can explain their shortness of breath. The patient has already undergone numerous tests: e.g. chest x-ray, pulmonary function tests, EKG, stress test, echocardiogram, CT, blood tests, and even bronchoscopy. Everything has come back normal, or anything even slightly abnormal (the reader can fill in the blank) has been treated maximally without any reduction of the patient’s symptoms.

In short, the patient has complied with every test and treatment recommendation; yet there is still no apparent explanation for this difficulty breathing, and no relief provided by numerous treatment trials.

The patient seems weary of the problem. My “antennae” do not pick up any signals of la belle indifference (a peculiar lack of concern about symptoms that when present suggests that the problem may be non-organic). She tells me that her breathing is never noisy, and that her shortness of breath may or may not be associated with exercise. I apply my subjective but extremely useful “flow-volume loop” by asking her to exhale to “empty” and then rapidly breathe in until completely full, and there is no unusual noise, and no prolongation of the time required to fill.

My initial thought in this scenario? The gasping syndromeWhat is that? A recognizable but to my knowledge previously undescribed disorderHere is my composite description, meaning that individual patients may not fit every element of what follows:

  1. Either with exertion or at rest, the patient has an abrupt* sense of “smothering,” or air hunger. It happens almost from one second to the next. Like the abrupt shooting pain of neuralgia, the abrupt tickle of sensory neuropathic cough / throat clearing, or the abrupt laryngeal closure of laryngospasm. The patient says she can be engrossed in a movie or a book strolling with the dog when suddenly her consciousness is invaded by a need to “get more air.”
  2. She responds by taking a deep breath, but there is no relief and the sensation remains. So, she takes a series of deep breaths, all of them to no avail. The feeling is oppressive, anxiety-provoking, gaspingThis sensation may last for a few seconds, to several hours.* Unfortunately, it can happen again some time later, or maybe just a time or two per day or even per week.

Having seen many people who fit the gasping syndrome across the decades, though not usually more than a handful per year, I have speculated that this is a sensory disturbance. After all, I ponder, if it represents more than a primary neuropathic sensation, why is oxygen saturation measured by oximetry, including during exercise, always normal? Why are all the tests of heart and lung function normal? Why no mitral valve prolapse, or something to explain this? Why nothing on imaging? Why no noisy breathing?

My mind goes to patients who describe a similar sensation at the moment IV contrast is injected for a CT scan. In addition to “warmth,” “a sensation of needing to urinate,” etc., they can have a sensation of abrupt, quite compelling, and thankfully transient smothering.  Or to someone I know who in the middle of an IV infusion of a biologic modifier for cancer, (with prior warning) experienced profound air hunger that started abruptly and was completely gone 20 seconds later. In each of these cases, it seems like a primary sensory phenomenon more than an alerting and protective sensation in response to low oxygen or high carbon dioxide levels.

How would one conceptualize a mechanism? Here is one thought experiment: consider that we are all supplied with pulmonary stretch receptors. They send messages “in the background” several times across the day and night to the respiratory center: Excuse me… Take a deep breath; expand those alveoli; your surfactant is giving you some atelectasis. And so, without being aware of it, we sigh now and then. We roll over in our sleep, mutter, grind our teeth, and take a deep breath. While reading a book we subconsciously shift in our seat and inspire deeply as we turn a page. Again, we are unaware of this, but an observer watching us read or, equipped with night vision goggles and watching us sleep, will see it.

What if the pulmonary stretch receptors send a signal Hey! Time to take a deep breath! and despite our taking a deeper breath outside of conscious awareness, the respiratory center does not receive the return communication, Action completed. Even with normal O2 and CO2 levels, if the brain thinks that deep breath did not happen, would it not intrude on conscious thought to re-command more urgently: Hey! Deep breath please!

Where does one go to help the patient with this thought experiment diagnosis? Just give her this explanation and leave it there? Or, maybe punt back to the primary care or pulmonary or cardiology physician…? But what if the person has already seen 3 of each specialty—one who is local, another at a nearby university center, and a third at a national referral center? What if “every conceivable test” has already been done 3 times?

A follow-on thought experiment: to the patient, and via a summary letter to her doctors, explain the concept of dysesthesia or sensory disturbance. Give her analogies: at the dentist, after anesthesia is in place to allow that root canal or filling, your tongue/lip can feel swollen, but a look in the mirror shows them to be normal. When a leg goes to sleep, it can feel fatter and heavier than the other one, but it is not. When you suffer nerve damage from diabetes, it can feel like bees are stinging your feet, but none are present in the room.

And then explain scenarios where persons have to accommodate to or neglect the feeling of contact lenses. The sound of new tinnitus. The tickling of an indwelling tracheotomy tube. The dramatic sensation a sword swallower must ignore during the show. Or the sensation of air hunger the pearl diver must overcome to stay submerged for 2 minutes while swimming vigorously.

Perhaps point out that it appears there is no danger from this ominous-feeling sensation.  Remind her of the multiplicity of prior “normal” tests. Maybe suggest that she experiment with assuming “control” of her response to the sensation by saying to herself: It is just a feeling! Or, Stupid pulmonary stretch receptors! Maybe gently alter behavior to see if it has any impact. Introduce inspiratory resistance via “straw-breathing.” Or a gentle Valsalva maneuver. Or, exhale slowly through pursed lips whose opening is the size of a coffee-stirrer.

And if nothing else works, suggest trying to mentally shrug and “throw the sensation over the shoulder.” And then keep going. The very worried individual can purchase an oximeter. Or even a stethoscope as a “crutch” to allay anxiety.

All of this can be offered with a physician’s apologies that he or she has nothing better to offer. And also with encouragement that these ideas have “liberated” other patients struggling with the same problem.

A final thought: just as neuromodulators can help persons suffering from neuralgia or sensory neuropathic cough/throat clearing, and laryngospasm, consider a “sensory neuropathic cough” strategy of working one-by-one, from medication to medication such as amitriptyline, gabapentin, etc., hoping to find one that helps.

*Some describe a more continuous, lower-grade background sensation of difficulty breathing with abrupt “peaks”

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Granulomatosis with polyangiitis (GPA)

A newer term for the auto-immune disorder previously called Wegener’s Granulomatosis.

Idiopathic subglottic stenosis

A subtype of subglottic stenosis that is inflammatory.  One view is that this entity is actually a limited expression of Wegener’s Granulomatosis (aka Granulomatosis with polyangiitis).

Podcast:


Photos:



Subglottic stenosis

Subglottic stenosis is narrowing just below the vocal cords, in the lowest part of the larynx and immediately above the first tracheal ring. Examples of causes include scarring from a breathing tube used during a long ICU stay, Wegener’s Granulomatosis (aka Granulomatosis with polyangiitis), and idiopathic subglottic stenosis (aka limited Wegener’s Granulomatosis).


Photos of subglottic stenosis:








Vocal cord synechia

Vocal cord synechia is a strand of scar tissue that tethers the vocal cords to each other. It can prevent the vocal cords from opening fully for breathing.

A synechia can also form in other parts of the body. (Note the subglottic synechia shown below.)


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Videos:

Vocal Cord Synechia
This video provides a clear example — using laryngeal videostroboscopy — of a vocal cord synechia.

Stenosis

Abnormal narrowing of a passageway in the body. At our practice, stenosis typically refers to narrowing in the breathing passage, such as for narrowing in the glottic, subglottic, or tracheal areas.

Stenosis in the airway can be the result of prolonged endotracheal intubation, external trauma such as gunshot wound, crush injury, or tracheotomy, an inflammatory or auto-immune process, surgical resection of part of the airway for tumor, or other causes. Persons with airway stenosis will note a reduced capacity for exercise. Often the clinician hears noisy breathing on inhalation, especially when the patient is asked to fill the lungs quickly. Esophageal stenosis gives symptoms of difficulty swallowing solids more so than liquids.


Photos of stenosis:































Videos:

Tracheal Stenosis: Before and After
In this video, trachea (windpipe) blockage causes shortness of breath until the narrowed segment is removed. You will see views of the trachea before and after surgical repair.
Post-Radiation Hypopharyngeal Stenosis
People with larynx or pharynx (voice box or throat) cancer often undergo radiation therapy as part of their treatment regimen. An uncommon complication is stenosis (narrowing, scarring) of the entrance to the upper esophagus at the junction of the throat and esophagus. This video provides an example of this disorder.

Pharyngocele

A dilated outpouching from the normal contour of the pharynx.


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Redundant supraglottic mucosa

An excess of mucosa overlying one or more structures in the larynx above the vocal cords. Mucosa in this area should “fit” snugly, like leggings, but in the case of redundant supraglottic mucosa, the fit becomes more like baggy pants.

Symptoms and treatment:

This redundant supraglottic mucosa most commonly develops on the apex and posterior surface of the arytenoid cartilage. Such mucosa sometimes draws inward during breathing and fills the laryngeal vestibule. In a severe case, inspiration can become noisy (stridor) or even effortful. When symptoms like these become troublesome, the excess mucosal tissue can be removed with endoscopic laser surgery.

Redundant supraglottic mucosa vs. laryngomalacia:

Redundant supraglottic mucosa is similar to the disorder laryngomalacia. In both disorders, supraglottic tissue is pulled into the laryngeal vestibule during breathing and can cause stridor. However, the causes of these symptoms are different. In the case of redundant supraglottic mucosa, the main problem is an excess of overlying mucosa, but in the case of laryngomalacia, the main problem is that the underlying structural tissue, such as that which comprises the aryepiglottic cord and epiglottic cartilage, is abnormally weak or soft.


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Nonorganic breathing disorder, laryngeal

A nonorganic disorder in which a person’s vocal cords partially or fully close during breathing, which causes noisy breathing. Also called vocal cord dysfunction (VFD) or vocal cord dysfunction (VCD).

The fundamental disorder is not in the mechanism itself, but rather in the patient’s “use” of the mechanism. Consciously or sub-consciously, the patient inappropriately narrows the space between the vocal cords, usually for secondary gain. Unlike with asthma or nonorganic breathing disorder of the trachea, the noisy breathing is mostly heard when the person breathes in (inspiration). Still, on occasion, individuals with this disorder are treated for years as having asthma before this diagnosis is finally made.


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Videos:

Nonorganic Breathing
This video portrays a breathing abnormality that is non-organic / functional.

Nonorganic breathing disorder, tracheal

A breathing disorder, often mistaken for asthma, in which a person induces his or her trachea to narrow, causing wheezing or apparent shortness of breath. The person’s oxygen saturation remains normal, but his or her description of the problem and apparent breathing distress may be quite dramatic; in severe instances, the person may have been intubated and placed in intensive care, based purely upon the “drama”—that is, the person’s distressed appearance and audible noises. The extent of medical intervention prompted by this pseudo-asthma can be remarkable.

How it happens:

The person’s trachea momentarily narrows or collapses, but unlike with tracheomalacia, this narrowing or collapse is not due to any anatomical or physiological disorder of the person’s trachea. Instead, it can be seen as an added capability of the trachea: an unusual, heightened capability of the membranous tracheal wall to flex inward and decrease the caliber of the “pipe.” A person whose trachea has this added capability may figure out how, with inconspicuous excessive expiratory effort—a sort of semi-Valsalva maneuver that isn’t evident to observers—to induce this tracheal or tracheobronchial collapse that creates the wheezing heard by family, friends, or co-workers.

The nonorganic element:

Often, this upper airway wheezing ability can be just a personal quirk (like double-jointedness) that has no particular significance to the person’s life; think of a “wheezy laugh.” Such a case would not be a nonorganic breathing disorder case. In extreme cases, however, a person may begin to use this wheezing ability to masquerade (perhaps sub-consciously) as having asthma, in order to achieve some kind of “secondary gain”; this added element puts a case into the category of a nonorganic breathing disorder. Sometimes, the person does have asthma, but is able to markedly amplify the asthma’s apparent severity by overlaying on it this dramatic nonorganic upper airway wheezing ability.

How it is diagnosed:

If a clinician listens to this person’s breathing with a stethoscope placed over the lung fields, the wheezing can indeed sound exactly like asthma. However, there are some key diagnostic criteria that help the discerning clinician to recognize a case of nonorganic breathing:

  • The wheezing is louder over the manubrium (uppermost part of the sternum) than over the peripheral lung fields.
  • There is a surprising incongruity between, on the one hand, the person’s apparent distress and, on the other hand, his or her objective findings, such as oxygen saturation, pulmonary function tests, blood gas measurement, and so forth.
  • Potent treatments for asthma do not seem to diminish or abolish the wheezing.
  • There appears to be some kind of “secondary gain” (mentioned above). Examples of secondary gain might be simple increased attention from family, healthcare workers, and so forth, or else avoidance of school or work, or an enhancement of the chance of winning a lawsuit, or release from responsibility for losing a competitive race, or the ability to manipulate others who have high levels of empathy combined with low levels of discernment of others’ motivations.