Dynamic patient counseling: a novel concept in idiopathic pulmonary fibrosis.
BACKGROUND
The characteristics of long-term survivors with idiopathic pulmonary fibrosis (IPF) have never been fully elucidated. We sought to illustrate the attenuated mortality and describe the characteristics of patients with IPF who survived at least 5 years beyond their initial presentation.


METHODS
Patients with IPF evaluated between 1997 and 2006 were identified through the clinic database. Patients who survived beyond 5 years from the time of their evaluation were compared with those who died or underwent lung transplantation within 5 years. Survival analyses were performed from the time of initial evaluation and contingent on annualized survival thereafter.


RESULTS
Eighty-seven patients who survived at least 5 years formed the comparator group to whom other patients were contrasted. These patients had a higher BMI, FVC % predicted, FEV1 % predicted, total lung capacity % predicted, and diffusing capacity of lung for carbon monoxide % predicted, but a lower FEV1/FVC ratio and lower mean pulmonary artery pressures. More than one-half of these patients had moderate or severe disease at the time of presentation. Our annualized contingent survival analyses revealed a progressively increasing median survival dependent on the duration of the disease.


CONCLUSIONS
Although we were able to demonstrate differences in our 5-year survivors, rather than being a distinct group, these patients appear to exist within a continuum of improving survival dependent on prior disease duration. This progressively improving time-dependent prognosis mandates the serial reevaluation of an individual patient’s projected outcomes. The implementation of dynamic counseling is an important concept in more accurately predicting life expectancy for patients with IPF who are frequently haunted by the prospects of a dismal survival.
Advanced Lung Disease and Transplant Program, Falls Church, VA; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA.
A Whitney
AW
Brown
Advanced Lung Disease and Transplant Program, Falls Church, VA; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA.
Oksana A
OA
Shlobin
Advanced Lung Disease and Transplant Program, Falls Church, VA; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA.
Nargues
N
Weir
Fairfax Radiological Consultants, Fairfax, VA; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA.
Maria C
MC
Albano
Advanced Lung Disease and Transplant Program, Falls Church, VA; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA.
Shahzad
S
Ahmad
Advanced Lung Disease and Transplant Program, Falls Church, VA; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA.
Mary
M
Smith
Mayo Clinic, Scottsdale, AZ; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA.
Kevin
K
Leslie
Advanced Lung Disease and Transplant Program, Falls Church, VA; Department of Medicine, Inova Fairfax Hospital, Falls Church, VA. Electronic address: steven.nathan@inova.org.
Steven D
SD
Nathan
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003376:Counseling / Q000379:methods*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D014109:Total Lung Capacity; D014481:United States / Q000453:epidemiology