Patient-reported symptoms and functioning as indicators of mortality in advanced cystic fibrosis: A new tool for referral and selection for lung transplantation

详细信息    查看全文

• 作者：Amparo Solé ; MD ; PhD^a ; ^b ; ^{sole_amp@gva.es" class="auth_mail" title="E-mail the corresponding author} ; Iné ; s Pé ; rez ; BSc^a ; ^b ; Isabel V&aacute ; zquez ; MD ; PhD^a ; ^c ; Amparo Pastor ; MD ; PhD^a ; Juan Escriv&aacute ; MD^a ; Gabriel Sales ; MD ; PhD^a ; David Herv&aacute ; s ; MSc^d ; Allan R. Glanville ; MBBS ; MD ; FRACP^e ; Alexandra L. Quittner ; PhD^f
• 关键词：cystic fibrosis ; lung transplantation ; waiting list ; health-related quality of life ; survival ; patient reported outcomes
• 刊名：Journal of Heart and Lung Transplantation
• 出版年：2016
• 出版时间：June 2016
• 年：2016
• 卷：35
• 期：6
• 页码：789-794
• 全文大小：452 K

文摘

Despite well-known risk factors and predictive survival models, many patients with cystic fibrosis (CF) die while on the waiting list for lung transplant. We evaluated whether specific Cystic Fibrosis Questionnaire (CFQ-R) scales provide additional benefit to conventional tools in identifying referral timing and waitlist mortality.

Methods

From January 2010 to January 2015, 152 patients (34% on the waitlist) were evaluated with the CFQ-R and standard protocol quarterly. Data were used to explore the prognostic association of health-related quality of life.

Results

The Physical Functioning domain (PFD) of the CFQ-R predicted mortality in advanced CF disease better than habitual parameters (p = 0.005). For patients with the same forced expiratory volume in 1 sec (FEV₁), a low score categorized patients with an increased risk of death. For patients with CF and FEV₁ <30% predicted and a low Physical score, mortality rate was ~35% at 2 years. The best model for probability of inclusion on the waitlist was FEV₁ % (p < 0.001, hazard ratio [HR] = 0.94; 95% confidence interval [CI] [0.90, 0.97]) and Physical Functioning (p = 0.013, HR = 0.96; 95% CI [0.95, 0.99]). The best model for probability of death similarly included FEV₁ % (p = 0.09, HR = 0.97; 95% CI [0.94, 1.00]) and CFQ-R Physical Functioning score (p = 0.005, HR = 0.97; 95% CI [0.95, 0.99]). The Health Perception score showed similar results. A low Health Perception score combined with a high resting heart rate showed a trend for mortality.

Conclusions

The CFQ-R may be an additional tool for guiding decisions to place a patient with CF on the waiting list for lung transplantation. The CFQ-R Physical Functioning and Health Perception scales were more accurate than conventional tools in predicting death before transplant.