To the editor
Dr. Reich appears to have 3 quibbles with two of my editorials. I
appreciate the opportunity to address each of these.
[1] Dr. Reich is bothered by an exchange of letters related to an
important article regarding chronic cough in children by Marchant and
colleagues (1), recently published in Chest with an accompanying editorial
(2). It is not clear why he is unhappy with this editorial or how this is
related to the relationship of atypical Mycobacteria infection and
bronchiectasis to an unrecognized diagnosis of cystic fibrosis (CF). I
stand by my decision recommending the publication of the manuscript by
Marchant and I am pleased that this manuscript and editorial have
generated discussion.
[2] Dr. Reich is also bothered by my recent editorial (3) regarding
the informative manuscript by Ziedalski and colleagues (4) where I discuss
the meaning of "the Lady Windermere syndrome" (5). Reich gallantly steps
to the defense of the besieged Lady Windermere claiming that medical
eponyms are no more than tropes. However most medical eponyms are named
for the physician who either first described a specific sign or syndrome
or more often, the physician whose recognition of this syndrome was
broadly popularized. Thus we have Kartagener syndrome (6) rather than
Siewert syndrome (7) although the latter's paper was published 29 years
before Kartagener's description in 1933. Occasionally an eponym is named
for the patient or the family who have the syndrome and in this case, the
eponym is written in the possessive. I am even aware of one instance in
pulmonary medicine where the patient with the sign and the describing
physician are one and the same - that is the Schamroth digital clubbing
sign described by Leo Schamroth of South Africa in 1976 (8). However
medical eponyms are not tropes. The Pickwickian syndrome was meant as a
description of daytime somulance in the fat boy Joe (I suspect that the
name "Joe's syndrome" would be too pedestrian), and even Ondine's curse
was meant to describe the fatal curse placed on Ondine's lover so that he
lost his autonomic physiologic regulation. These literary eponyms have
fallen into disuse with the terms "obesity hypoventilation syndrome", and
"congential central hypoventilation syndrome" more descriptively taking
their place.
Scholars have pointed out that Wilde's Lady Windermere was certainly
not an inhibited Victoran woman (9). Her refusal to shake hands with the
Lord Darlington could be an attempt to avoid close contact with this cad
but more probably just it was just common politelness not to shake his
hand when her hands were wet. Dr. Reich could have chosen to name this
syndrome after Cecily Cardew from Wilde's, Importance of Being Earnest:
Act 2 Scene 3: Cecily is taking dictation of a marriage proposal from
Algernon.
"Oh, don't cough, Ernest.When one is dictating one should speak
fluently and not cough. Besides, I don't know how to spell a cough."
However this is irrelevant given my response to Dr. Reich's third
protest.
[3] The crux of this matter is that patients who have bronchiectasis
–“isolated” or otherwise - and atypical Mycobacteria do not develop this
because of an ineffective cough. Furthermore, it is now clear that many of
them either have CF or are a CF heterozygote. What is the evidence for
this?
1. There are no published data demonstrating that voluntary cough
supression leads to disease; much less to middle lobe or lingular
infection with aytpical Mycobacteria.
2. Patients with neuromuscular problems and weak cough are clearly at risk
for pneumonia (10) but they do not develop bronchiectasis unless they are
immunosupressed or have frequent aspiration of oral or gastric contents.
When these patients do get bronchiectasis it is almost never due to
atypical Mycobacteria. Thus cough supression does not cause this syndrome.
3. Patients with the "middle lobe syndrome" and persistent collapase of
the middle lobe or lingula do indeed get bronchiectasis (more than 20% in
most series) but this is almost never due to atypical Mycobacteria (11).
Thus poor collateral ventilation does not predispose to Mycobacteria.
4. Ziedalski and colleagues have now clear shown that otherwise healthy
adults with bronchiectasis that is not diffuse, and with infection by
atypical Mycobacteria, frequently have CF or are CF carriers (4).
The implications of this are great. CF is now a fairly controllable
diease with most patients living into adulthood with preserved lung
function and good quality of life. The earlier CF is recognized and
infections treated, the better the outcome. Diagnosis thus has great
implications for treatment and for counseling. This is not to say that all
otherwise healthy patients with pulmonary infection caused by atypical
Mycobacteria and/or mild bronchiectasis have CF; however this diagnosis
appears to be considreably more likely than the suggestion that this is
due to an ineffective cough.
The principle of lex parsimoniae suggests that entities should not be
multiplied beyond necessity (entia non sunt multiplicanda praeter
necessitatem). Reduction to a most likley etiology does not identify CF as
the sole cause of Lady Windermere syndrome, however reduction which leads
to the assumption that this syndrome is caused by ineffective cough is
better characterized as reductio ad absurdum.
As a student of medical history, I find it sad to write a eulogy over
a literary eponym that has outlived its usefulness. Reich is to be
congratulated for directing our attention to the association of atypical
Mycobacteria and isolated bronchiectasis. His explanation for this
association presented an innovative and testable hypothesis, which has
been shown to be incorrect. I invite him to drop his ideology, accept the
etiology, and celebrate with me the identification of the most important
established cause for this disease association that he brought to our
attention nearly 15 years ago.
References
1. Marchant JM, Masters IB, Taylor SM, Cox NC, Seymour GJ, Chang AB.
Evaluation and outcome of young children with chronic cough. Chest.
2006;129:1132-41
2. Rubin BK. Pediatricians are not just small internists. Chest.
2006;129:1118-21.
3. Rubin BK. Did Lady Windermere have cystic fibrosis? Chest.
2006;130:937-8.
4. Ziedalski, T, Kao, P, Henig, N, et al Prospective analysis of
cystic fibrosis transmembrane regulator mutations in adults with
bronchiectasis or pulmonary nontuberculous mycobacterial infection. Chest
2006;130,995-1002.
5. Reich, JM, Johnson, RE. Mycobacterium avium complex pulmonary
disease presenting as an isolated lingular or middle lobe pattern: the
Lady Windermere syndrome. Chest 1992; 101,1605-1609.
6. Siewert AK. Uber einem Fall von Bronchiectasie bei einem Patienten
mit Situs inversus viscerum. Berliner klinische Wochenschrift.
1904;41:139–141.
7. Kartagener M. Zur Pathogenese der Bronchiektasien: Bronchiektasien
bei Situs viscerum inversus. Beiträge zum Klinik der Tuberkulose, 1933;83:
489-501.
8. Schamroth L. Personal experience. S Afr Med J 1976;50:297-300.
9. Drew EW. Have you read Lady Windermere’s fan by Oscar Wilde? Chest
e-letter December 11, 2002. Available at:
http://www.chestjournal.org/cgi/eletters/101/6/1605. Accessed August 30,
2006
10. van der Schans CP, Bach JR, Rubin BK. Chest Physical Therapy:
Mucus Mobilizing Techniques. in Noninvasive Mechanical Ventilation. Ed.
John R. Bach. Hanley & Belfus, Inc. Philadelphia, PA. 2002 pp 259-284.
11. Rubin BK. Respecting the middle lobe syndrome. Pediatr Pulmonol.
2006;41:803-4.
