Of the 247 patients included in the study 92 (37%) died by August 2017 and were included in our follow-up cohort (Figure 1).
The cumulative mortality of the patient cohort (n = 92) is presented in Figure 2. The median overall survival was 4.4 years (IQR 3.1–5.7). Patient characteristics are shown in Table 1. A majority of the patients had comorbidities, of which cardiovascular diseases were the most common ones. None of the patients had lung cancer when entering the study, but two patients were diagnosed with lung cancer during the follow-up. Lung function measurements within the last six months of life were available in only 28 (30%) of the patients (mean FVC 2.2 l (SD 0.6), 57%). Antifibrotic medication was used by 33 (35%) of the patients.
Cumulative mortality after the diagnosis of IPF. Time-point of the diagnosis is marked with 0 and 95% confidence intervals with the grey area. Kaplan-Meier method was used to estimate the cumulative mortality
The proportion of the patients who could not perform continuous moderate intensity physical exercise for at least 30 min during the previous six months increased from 34% 18–24 months before death to 62% during the last six months of life. Six months before death, 67% of the patients reported needing assistance with their daily activities, while 18–24 months before death this proportion was 56%.
Health-related Quality of Life
Figure 3 shows the changes in the different dimensions of HRQOL measured with RAND-36 during two years before death. The decline in HRQOL was highly significant in all dimensions except in physical role, which showed very low scores by 24 months before death.
Relationships between Health-related quality of life domains (RAND-36) two years before death. The curves were derived from a 5-knot restricted cubic splines regression models. The models were adjusted for age and gender. The grey area represents 95% confidence intervals. Dashed lines mark Finnish general population levels
The intensity change of the symptoms measured by the ESAS during the last two years of life is presented in Figure 4. The intensity of all symptoms except pain at rest and insomnia increased significantly near death. During the last six months of life, the mean NRS scores were as follows: 7.1 (SD 2.8) for breathlessness, 7.0 (SD 2.3) for tiredness, 6.0 (SD 2.5) for wellbeing, 6.0 (SD 3.0) for dry mouth, 5.8 (SD 2.9) for cough, 5.0 (SD 3.5) for pain with movement, 3.9 (SD 3.1) for insomnia, 3.9 (SD 2.9) for anxiety, 3.8 (SD 2.9) for depression, 3.6 (SD 3.1) for constipation, 3.4 (SD 3.3) for loss of appetite, 3.1 (SD 2.8) for pain at rest and 1.8 (SD 2.5) for nausea.
Relationships of symptom severity measured by ESAS two years before death. The curves were derived from a 5-knot restricted cubic splines regression models. The models were adjusted for age and gender. Grey area represents 95% confidence intervals. ESAS, Edmonton symptom assessment scale
The steep change in the proportion of patients with MMRC scores ≥3 (needing to stop walking after approximately 100 m or a few minutes because of breathlessness) during the last two years of life is shown in Figure 5.
BMC Pulm Med. 2018;18(172) © 2018 BioMed Central, Ltd.