PEDIATRICS Vol. 104 No. 4 October 1999, p. e48
ELECTRONIC ARTICLE:
Managing Disagreements in the Management of Short Bowel and
Hypoplastic Left Heart Syndrome
Received Dec 23, 1998; accepted Apr 8, 1999.
,, and
From the * Department of Pediatrics, Baylor College of Medicine;
and the Center for Medical Ethics and Health Policy, Baylor College
of Medicine, Houston, Texas.
Background and Objectives. When innovative, not yet fully proven therapies are introduced, physicians may have neither experience nor sufficient data in the medical literature to assist in their decision to discuss them with and/or recommend them to patients. Little is known about how physicians deal with this uncertainty. Moreover, when multiple physicians caring for a single patient have reached different conclusions regarding this new therapy, the potential for disagreement exists that could give rise to ethical issues as well as cause confusion to the patient. To explore these topics, we investigated the attitudes of specialists to therapies for two life-threatening diseases: hypoplastic left heart syndrome (HLHS) and short bowel syndrome.
Methods. A forced choice questionnaire was distributed to
the heads of neonatology, pediatric cardiology, and pediatric
gastroenterology training programs asking about their outcome
impressions and treatment recommendations and about the local
availability of treatments. In addition, responses from specialists
from the same institution were linked in a confidential manner to
evaluate the frequency of disagreement within the same institution.
Responses were analyzed using 
2 and Wilcoxon matched
pair analysis as appropriate.
Results. The overall rate of response was 79%. In institutions that had both neonatology and pediatric gastroenterology training programs, there was a 59% response rate compared with a 73% response rate from institutions that had both neonatology and cardiology programs. Significant differences were noted among specialists as to who would be involved in discussions of therapeutic options with patients in both HLHS and short bowel syndrome. Differences also were noted in the willingness of specialists to discuss and recommend therapies, in the perceived survival and quality of life by various specialists after transplant and palliative surgery, and in the local availability of various options. The neonatologists and gastroenterologists at the same institution disagreed on responses in 34% of the questions with only 1 of the 25 pairs in full agreement. In contrast, the neonatologists and pediatric cardiologists at the same institution disagreed in only 14% of the questions with 7 of the 28 pairs in full agreement.
Conclusions. Substantial disagreement among specialists about new interventions was found. There seem to be fewer differences among specialists when dealing with the more mature therapy, HLHS. Two major ethical issues arise. First, there seems to be no accepted professional standard to which individuals can appeal when determining whether to discuss or recommend new, not-yet-fully-proven technologies. Second, there is the potential for much patient confusion when counseling physicians recommend different options. Colleagues as individuals and specialists as groups should talk to each other before individual discussions with families to ensure that there is a clear understanding of differing beliefs. Key words: forecasting, prognosis, survival rate, quality of life, counseling, decision making, policy making, questionnaires, short bowel syndrome, parenteral nutrition, intestine, small transplantation, surgical stapling, hypoplastic left heart syndrome, heart transplantation, palliative care/methods.
