Late-stage IPF Patients Have Progressively Worse Quality of Life, Real-world Study Reports  

Late-stage IPF Patients Have Progressively Worse Quality of Life, Real-world Study Reports  

Idiopathic pulmonary fibrosis (IPF) patients experience a significant decline in quality of life and worse symptom burden during the last months of life, a real-world study shows.

The Finland-based study, “Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life,” which evaluated IPF patients for two years, highlights the need for early palliative care and improved symptom management for late-stage patients. It was published in the journal BMC Pulmonary Medicine.

Previous reports have shown that a reduced quality of life is an independent prognostic feature in IPF. According to these studies, IPF patients have a lower quality of life than the general population. Physical activity of patients is especially affected.

Nevertheless, the quality of life and symptom burden of IPF patients in the end-of-life phase have not been studied in detail. Knowing more about the quality of life and symptoms in the late stages of the disease could help improve the palliative care offered to IPF patients who have reached this point.

To address this, researchers in Finland investigated health-related quality of life and symptom burden in a group of IPF patients in their last two years of life. All IPF patients analyzed were registered in the FinnishIPF study, which is a national prospective registry available in Finland since 2012.

Three separate questionnaires were sent to 300 IPF patients evaluating shortness of breath, other disease symptoms, and health-related quality of life. Of these, 247 patients responded. Changes in outcomes were then measured through follow-up questionnaires that were sent every six months for two years (five follow-up occasions in total).

Quality of life was assessed by scoring eight different health concepts related to physical, mental, and social parameters.

Results showed that IPF patients scored lower than the general population on all health concepts analyzed at the first evaluation. All health concept scores, except for “physical role,” which was already exceptionally low at the first evaluation, decreased significantly during the two-year period.

By the end of the study, a total of 92 IPF patients died, experiencing a significant increase in symptom burden during the last months of life. Except for pain at rest and insomnia, all symptoms worsened during the evaluation period.

Measurement of physical activity showed a reduced ability to perform moderate intensity exercises over time. While 34% of IPF patients were unable to perform the activities 18 to 24 months before death, this proportion increased to 62% during the last six months of life.

Results also showed that shortness of breath, coughing, and fatigue were the most severe symptoms in IPF patients, in agreement with previous studies. The follow-up evaluations showed that the intensity of shortness of breath increased over time, becoming one of the most severe symptoms in the late stages of the disease.

“In this study, we demonstrate a rapidly increasing impairment in HRQOL [health-related quality of life] and escalating symptom burden in IPF patients approaching death,” the researchers wrote.

“Low HRQOL together with severe breathlessness and fatigue were detected as early as two years before death. In addition, several dimensions of HRQOL declined further and the severity of many symptoms other than dyspnea [breathlessness] increased during the last two years of life,” they added.

Based on the results, the team highlighted the need for symptom management along with existing disease-modifying therapies and palliative care to improve the condition of patients in the late stages of the disease. They suggested that early palliative care can improve IPF patient management and end-of-life planning.

“Our results provide insight into the most important needs of end-stage IPF patients and support the use of early-integrated palliative care, which should include symptom control beyond treatment for dyspnea and psychosocial support,” the researchers concluded.


  1. Patrick Cramblet says:

    I was diagnosed with PF in Jan 2016. My latest catscan showed only minor progression. I just started occasionally ciughing. I am at 60+ in breathing test. I am 77 years old. I am not looking forward to the last year or so. I already have reduced physical abilities.

    • Lorena McManus says:

      Please see the above article and the help that early referral to Palliative Care can offer. Lorena ( feelow patient and retired Palliative Nurse Administrator)

  2. David Collard says:

    I have IPF since 2013 and up until this year have managed on esbreiet and have been relatively active. This year I was put on supplementary oxygen starting at 2.5 for activity. that lasted about a month, I am now on oxygen 24 7 and require to up the volume to 4 for any activity. the 2.3 level has increased to 3 for most of the time. the most difficult times are to move from a startinary position to go the the bathroom. Even when I start at Oxygen saturation levels set at 4 to get to a %SpO2 level of 97 by the time I get to the washroomm my 5Sp)02 levels drop to 82 and sometimes I am at the 72 level…… more to come – just got interrupted.

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