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.

Discuss the latest research in the Pulmonary Fibrosis News forums!

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)

    • Patti says:

      Hi Patrick, Obviously IPF is one of the most challenging diseases to cope with. My husband is 77 and is in his 5th year of IPF. He amazingly proceeds enthusiastically with his life as if it were a temporary problem. He is now on 10L flow from a large oxygen concentrator and when out in the car he is on an E size oxygen tank. I bought him a little stair climbing cart for his tank. He sets the flow at 6, when just sitting, but elevates it, with sudden activity, to 10. You are working hard to breathe and you need to be sure you are eating sufficient protein and adequate intake of calories. You may need to eat four small meals each day. Of course sleep is very important to your hard working body.

      God bless you.

  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.

    • Susan Imig says:

      Hi, Mary Lou!
      My husband was just diagnosed with IPF. I’m trying to figure out how this communication system works seeing as how replays are not posted.
      Im not even sure if I can hear back from you!
      Thank you, Mary Lou!

  3. Tommagic1 says:

    I suspect some will say I am being “negative”. some will say, I am being rudely “blunt”. The study never included and impressions from any participants after they died. One would wonder, or at least I do, what they could add to the study. The end of this journey is pretty much a coma…or drug induced state of a coma…not a drug induced “euphoria” regarding the passage of life.

    The study ignores the impact of a patient in end of life…the impact on the caregiver, spouse, children and friends and family.

    I was diagnosed with IPF 2 years ago. My brother diagnosed this past April (2018). My mother passed from this disease about 20 years ago. I’ve lived through it, believe me. My mother was an RN, she new illness, pain and death. She raised 5 children.One being me, which would singularly be a handful for any mother. Her last few months in Hospice (they were wonderful) and the lack of her…well to any normal dignity we would all have…I’m not going there any further.

    My wife and I were at her bedside, she asked me to help her find a better life in heaven….take my pillow. We both left the room, stunned, I asked my wife…did she REALLY say that? Fast forward about a month, my wife again at her bedside, she asked me why I continued to “fight her”. I responded and said “How am I fighting you?” She basically told me I should make her a strong cocktail (that was her dose of morphine and orange juice) and help her to heaven. Of course that did not happen. another month passed when I alone arrived…my Dad said she had been “sleeping a lot”. Fact was, he was in denial, she was in a coma. When he left the room, I tried to close her mouth, but it was rigid. I took my mothers hand, I remember it was so very cold. I told her I was here, she fought the good fight, that I’d be here with Dad, so if she wanted to “go”, it was ok and I’d take care of him. Five minutes later she passed.

    These studies are pretty “medical”. Until you hear the “real deal”..that is the world we live in and the world we are going to leave.

    • Judith says:

      Thank you for sharing your story and experience with your mother’s passing. I am sorry you and your brother have been diagnosed with PF. Words can’t begin to explain the feelings that go along with last days of a loved one’s life here on earth. Your story touched my heart. Suffering, illness and death were not in God’s original plan for his people. But sin got in the way. I am glad your mother, who helped care for many people through her life, knew Jesus and had hope in heaven. It is so difficult to see your loved one suffer, and be asked to help end their suffering. From the caregiver side, sitting alongside my husband in his last months, days, making him comfortable was the main goal. I realized I had the ‘tools’ to prolong life or hasten it and that was scary for me for a moment. As Christians, we ask God to have mercy on each of us, and that in His timing He knows what is best, both in life and death. So we wait on the Lord. For those who believe and trust in the Lord, we have confidence that we will one day be reunited with those who have gone before us. I pray you and your brother’s journey in life with IPF will be one of growing closer to our Savior and you will have the confidence of being with your mother in heaven one day. Hopefully with the new treatments of OFEV and ESBRIET you will live a long life, building relationships and memories, enjoying what this world offers, keeping your body strong through exercise and eating healthy. Especially through the hardest part of my husband’s last 6 months, at the end of each day he would reflect on how God still had a purpose for him even in his suffering. He encouraged others and was given opportunities to share his faith. His life on earth was a beautiful journey and I have been blessed to have been married to him for 51 years.

  4. Dennis R Felt says:

    Hello my name is Dennis I have had IPF since 09. My father died from it back in 98 after having it for 7 yrs.He was only 66 when he died. Iam 64 and I have been using Esbriet for the past 2yrs. and I feel it has been keeping it at bay. The most inportant thing you can do is keep a positive attitude at all times and keep doing the things you always loved to do.
    I still ride my motorcycle every summer I have rode to key west,Portland Maine,San Fran.and Mont.For some reason I don’t need my oxygen while i’m riding
    I have my oxygen set at 3 when doing nothing then set to 4 when washing my bike but it will still drop into the low 80’s I have to take a lot of breaks but that’s OK !Other than my lungs i’m in good shape.I live between Detroit and Ann Arbor,Michigan

  5. Deepankar says:

    My mother is suffering from this disease and totally depends on me. for past 7-8 months she is totally confined to bed.i just want to know how to take away her suffering.its very hard to see such wasting of a loved one.Guide me.

Leave a Comment

Your email address will not be published. Required fields are marked *