- Email: [email protected]
Better Understanding Quality of Life for Those Living With Marfan∗
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
VOL. 69, NO. 23, 2017
CROWN COPYRIGHT ª 2017 PUBLISHED BY ELSEVIER ON BEHALF OF THE
ISSN 0735-1097/$36.00
AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION. ALL RIGHTS RESERVED.
http://dx.doi.org/10.1016/j.jacc.2017.04.022
EDITORIAL COMMENT
Better Understanding of Quality of Life for Those Living With Marfan Syndrome* John R. Pepper, MD
I
n this issue of the Journal, Goldfinger et al. (1)
stenosis provides an example. Patients with aortic
should be congratulated for examining this
stenosis who are asymptomatic may be referred for
under-researched area of investigation with
intervention. They have attributed their limitation to
respect to Marfan syndrome. They also should be
“anno domini” until the aortic stenosis is relieved by
commended for having the foresight to set up a regis-
valve replacement. On the other hand, people may
try. Individuals with a genetic disease and a family
recalibrate. Cancer patients who graded their quality
history studded with cardiovascular events are usu-
of life as poor at baseline before treatment may want
ally well informed about their illness, but they are
to go back when they are receiving chemotherapy and
also extremely worried.
regrade downward when they know what bad quality of life feels like (4).
SEE PAGE 2821
We should remember that quality-of-life measures
It is interesting and reflects well on current medical
were derived by health economists to find some
care that the elements in the lives of these Marfan
equivalence in quality-of-life change associated with
patients—which are significant—are those that apply
treatment and relative treatment costs. Thus, a study
to all people who live in the United States. If you are
confined to Marfan syndrome means that you cannot
uninsured and do not have a job, life is precarious and
learn much about generic loss of quality of life due to
disconcerting. In trying to analyze this, the authors
Marfan. Whatever patients’ Marfan-related problems
recognize that it is very difficult and probably
are, they will tend to find some sort of common level,
impossible to separate “can’t work” from “very sick.”
a bit below par. The external effects are what remain
Patients with chronic conditions tend to adjust their
after adjustment.
quality-of-life perception upward, as they survive and interpret their position in life optimistically (2,3).
ADDRESS FOR CORRESPONDENCE:
However, things may change during treatment.
Pepper, Royal Brompton Hospital, Sydney Street,
Patients often grade their quality of life as good and
London
then have some chronic condition relieved. Aortic
[email protected].
SW3
6NP,
United
Dr. John R.
Kingdom.
E-mail:
REFERENCES 1. Goldfinger JZ, Preiss LR, Devereux RB, et al., for the GenTAC Registry Consortium.
3. Fosbraey J. The psychosocial impact of personalised external aortic root support surgery in
4. Jansen SJT, Stiggelbout AM, Nooij M, Noordijk E, Kievit. Response shift in quality of life measure-
Marfan syndrome and quality of life in the GenTAC registry. J Am Coll Cardiol 2017;69: 2821–30.
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.
ment in early-stage breast cancer patients undergoing radiotherapy. Qual Life Res 2000;9:603–15.
2. Treasure T. Living with a univentricular heart. Heart 2001;86:5–6.
*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. From the Royal Brompton Hospital, London, United Kingdom. Dr. Pepper has reported that he has no relationships relevant to the contents of this paper to disclose.
KEY WORDS GenTAC, Marfan syndrome, quality of life, SF-36
VOL. 69, NO. 23, 2017
CROWN COPYRIGHT ª 2017 PUBLISHED BY ELSEVIER ON BEHALF OF THE
ISSN 0735-1097/$36.00
AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION. ALL RIGHTS RESERVED.
http://dx.doi.org/10.1016/j.jacc.2017.04.022
EDITORIAL COMMENT
Better Understanding of Quality of Life for Those Living With Marfan Syndrome* John R. Pepper, MD
I
n this issue of the Journal, Goldfinger et al. (1)
stenosis provides an example. Patients with aortic
should be congratulated for examining this
stenosis who are asymptomatic may be referred for
under-researched area of investigation with
intervention. They have attributed their limitation to
respect to Marfan syndrome. They also should be
“anno domini” until the aortic stenosis is relieved by
commended for having the foresight to set up a regis-
valve replacement. On the other hand, people may
try. Individuals with a genetic disease and a family
recalibrate. Cancer patients who graded their quality
history studded with cardiovascular events are usu-
of life as poor at baseline before treatment may want
ally well informed about their illness, but they are
to go back when they are receiving chemotherapy and
also extremely worried.
regrade downward when they know what bad quality of life feels like (4).
SEE PAGE 2821
We should remember that quality-of-life measures
It is interesting and reflects well on current medical
were derived by health economists to find some
care that the elements in the lives of these Marfan
equivalence in quality-of-life change associated with
patients—which are significant—are those that apply
treatment and relative treatment costs. Thus, a study
to all people who live in the United States. If you are
confined to Marfan syndrome means that you cannot
uninsured and do not have a job, life is precarious and
learn much about generic loss of quality of life due to
disconcerting. In trying to analyze this, the authors
Marfan. Whatever patients’ Marfan-related problems
recognize that it is very difficult and probably
are, they will tend to find some sort of common level,
impossible to separate “can’t work” from “very sick.”
a bit below par. The external effects are what remain
Patients with chronic conditions tend to adjust their
after adjustment.
quality-of-life perception upward, as they survive and interpret their position in life optimistically (2,3).
ADDRESS FOR CORRESPONDENCE:
However, things may change during treatment.
Pepper, Royal Brompton Hospital, Sydney Street,
Patients often grade their quality of life as good and
London
then have some chronic condition relieved. Aortic
[email protected].
SW3
6NP,
United
Dr. John R.
Kingdom.
E-mail:
REFERENCES 1. Goldfinger JZ, Preiss LR, Devereux RB, et al., for the GenTAC Registry Consortium.
3. Fosbraey J. The psychosocial impact of personalised external aortic root support surgery in
4. Jansen SJT, Stiggelbout AM, Nooij M, Noordijk E, Kievit. Response shift in quality of life measure-
Marfan syndrome and quality of life in the GenTAC registry. J Am Coll Cardiol 2017;69: 2821–30.
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.
ment in early-stage breast cancer patients undergoing radiotherapy. Qual Life Res 2000;9:603–15.
2. Treasure T. Living with a univentricular heart. Heart 2001;86:5–6.
*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. From the Royal Brompton Hospital, London, United Kingdom. Dr. Pepper has reported that he has no relationships relevant to the contents of this paper to disclose.
KEY WORDS GenTAC, Marfan syndrome, quality of life, SF-36