Author + information
- John R. Pepper, MD∗ ()
- ↵∗Address for correspondence:
Dr. John R. Pepper, Royal Brompton Hospital, Sydney Street, London SW3 6NP, United Kingdom.
In this issue of the Journal, Goldfinger et al. (1) should be congratulated for examining this under-researched area of investigation with respect to Marfan syndrome. They also should be commended for having the foresight to set up a registry. Individuals with a genetic disease and a family history studded with cardiovascular events are usually well informed about their illness, but they are also extremely worried.
It is interesting and reflects well on current medical care that the elements in the lives of these Marfan patients—which are significant—are those that apply to all people who live in the United States. If you are uninsured and do not have a job, life is precarious and disconcerting. In trying to analyze this, the authors recognize that it is very difficult and probably impossible to separate “can’t work” from “very sick.” Patients with chronic conditions tend to adjust their quality-of-life perception upward, as they survive and interpret their position in life optimistically (2,3). However, things may change during treatment. Patients often grade their quality of life as good and then have some chronic condition relieved. Aortic stenosis provides an example. Patients with aortic stenosis who are asymptomatic may be referred for intervention. They have attributed their limitation to “anno domini” until the aortic stenosis is relieved by valve replacement. On the other hand, people may recalibrate. Cancer patients who graded their quality of life as poor at baseline before treatment may want to go back when they are receiving chemotherapy and regrade downward when they know what bad quality of life feels like (4).
We should remember that quality-of-life measures were derived by health economists to find some equivalence in quality-of-life change associated with treatment and relative treatment costs. Thus, a study confined to Marfan syndrome means that you cannot learn much about generic loss of quality of life due to Marfan. Whatever patients’ Marfan-related problems are, they will tend to find some sort of common level, a bit below par. The external effects are what remain after adjustment.
↵∗ Editorials published in the Journal of the American College of Cardiology reflect the views of the authors and do not necessarily represent the views of JACC or the American College of Cardiology.
Dr. Pepper has reported that he has no relationships relevant to the contents of this paper to disclose.
- Goldfinger J.Z.,
- Preiss L.R.,
- Devereux R.B.,
- et al.,
- for the GenTAC Registry Consortium
- Treasure T.
- ↵Fosbraey J. The psychosocial impact of personalised external aortic root support surgery in Marfan syndrome [dissertation]. Available at: http://www.marfanaorticrootsupport.org/images/downloads/The_psychosocial_impact_of_personalised_external_aortic_root_support_surgery_in_Marfan_syndrome.pdf. Accessed July 1, 2001.