How IPF has changed our lives

Abstract

April 11, 2014, is the day that changed our lives completely. The day Ron was diagnosed with idiopathic pulmonary fibrosis (IPF) at the age of 53 years, and told that he had 3–5 years to live.Where do you go from there? It was like a bomb exploding in our lives. We had only recently got together in 2012, believing we had years stretched out ahead of us. But now, the love we had found was going to be cruelly snatched away from us in a relatively short space of time. Ron was a triathlete, swimming and cycling every day, he looked so well. How could he be that ill?For me, those first days and weeks were extremely emotional, full of tears, sleepless nights, fear of losing the man I loved. We wanted to grow old together. How was our life going to change? How quickly was it going to happen? What was the end going to be like?We had nowhere to turn at that time, no leaflets, no-one to guide us or be there to stop us feeling so alone. That year was full of hurdles; first the diagnosis, then the passing of Ron's mum, as well being made redundant from his job. We were at an all-time low; we did not believe Ron would get to 60 years, and certainly not to retirement age, so what was the point of planning a future? We started to just live, waiting and watching for something to happen—the inevitable change.Ron's confidence took a dive. He was always the life and soul of a party, and he loves to make people laugh. We loved to dance, and as time went on, if we were on a cruise and Ron wanted to go to the disco in the evening, he had to rest for 3–4 h in the afternoon for 30 mins of enjoyment and trying to feel normal for his age.Everything we do has to be planned. Are there any hills, inclines, or steps? If so, we don’t go to those places, which can make life quite restricting. A busy day is always followed by a day of rest. Lifting heavy things, carrying the shopping, mowing the lawn, or exerting himself causes Ron to have a coughing fit, so I have do all of these jobs. Mentally, at his age, watching me do all this has been hard.We were foster carers at the time of diagnosis and had to give up because the chance of catching infections became too much of a risk, much to our regret. Our lives were changing very quickly, and this was challenging physically and mentally.One day, we were talking about our future, and we decided we had been dealt this hand and we couldn’t change it, but we could choose whether to let it beat us or take charge of our situation and find the positives this new challenge could bring. Yes, we had expected to carry on working, fostering, seeing all our family and friends, enjoying our home, and planning for our retirement, when after all the years of hard work we would retire and we could enjoy travelling together and enjoying life to the full. However, with IPF snatching that from us, we took the decision to get married. It was a very emotionally charged day, as we both knew that the line “’Till death do us part” was a prominent part of the service, there were many tears from all attending, but we made the day full of laughter despite Ron being exhausted at the end of it.We then decided to fulfil Ron's bucket list. We sold our house and downsized to a bungalow to prepare for the future and release capital that we set aside for our retirement. We immediately started planning trips to all the countries we had dreamed of visiting. We have visited 78 countries in 4 years, and have ticked off a lot on our to do list. We know we were lucky to have this opportunity, but Ron sees it as getting what he thought would be 20 years or more condensed into 5.During our journey, we were introduced to the Royal Papworth PF Support Group. We attended our first meeting with trepidation, but soon realised the relief we felt meeting people with the same disease, laughing, living, and getting the best out of life while supporting each other. Ron was soon asked to become the chairperson of the group, and this gave him great satisfaction and renewed enthusiasm to raise awareness of this disease. This led to several opportunities coming along for Ron, which included giving talks to Members of European Parliament, to pharmaceutical companies, support groups, and other medical arenas about how the disease has impacted his life.In his bid to help and support others, he was asked to become a trustee of the Pulmonary Fibrosis Trust, who provide practical aides to people with IPF or PF in their time of need. He has received many letters of gratitude from patients who have been given the freedom to get out after being given a mobility scooter. This is extremely rewarding for him. Ron has become an advocate for patients raising awareness wherever and however the opportunity arises.At one of Ron's patient advisory board meetings in Europe, I was asked if I would like to join. I was only there as his carer so I was somewhat surprised when I was encouraged to participate fully. This opened my eyes to the idea that carers’ points of view did matter, and the concerns and issues we feel and have to deal with are just as valid as the patients themselves. Without carers, a patient's world is extremely limited. I have since become a passionate advocate for carers, as on this disease journey it has shown me how little support and understanding a carer receives. I, like Ron, have taken every opportunity to raise awareness of a carer's plight and joined The Taskforce for Lung Health as a carer representative to make sure our voices are heard.If you had told us both 6 years ago what we would be doing now, raising awareness, neither of us would have believed it, as we did not know we had it in us. It is amazing when you have a passion for helping others what you can achieve, despite your own circumstances.While we are still constantly aware of Ron's illness and his deterioration, we are fortunate to have access to the antifibrotic drugs—pirfenidone, which he took for the first 4·5 years and after a steep decline changed to nintedanib, which he is currently on. Despite both drugs causing Ron to have side effects, he feels that they have taken him passed the original 5-year prognosis given. However, he is now on ambulatory oxygen. This in itself causes obstacles, making sure he has enough if we go out and coping with the looks you get from strangers. He is down to 50% lung capacity, so our own personal future is changing, and reality does hit home. We have noticed friends have backed away and our world is becoming a smaller place due to Ron's limitations now. We have to manage our own mental and physical wellbeing and this past year has been even more challenging.Ron has received phone calls from the local hospice regarding his mental wellbeing because at times it is really hard to deal with a terminal illness and all the implications that holds on your own. I know he tries not to worry me, so having a neutral person to talk to is a massive help to him.We both find that the satisfaction we get from helping others and seeing the changes happening in the pathways of this disease, knowing that one phone call to a patient or carer can change their outlook and make them feel less alone and able to fight this illness, keeps us both positive. We feel that although IPF has taken the future we had planned away from us; we have fought back. The idea that we may have made even one person's life easier or better is comforting. As strange as it sounds, this disease has made us better people and brought us a lot of happiness, and some amazing friends who we would never have otherwise met.IPF will not define Ron. It has made him a complete person. Before, with life, work, and family, we didn’t have the time to reflect and see how much acts of kindness can make such a difference. We were busy living, getting on with life, rushing around—tomorrow was expected, life had no end. IPF has taught us to enjoy the here and now, and the people who are there for us and support us on our journey.Ron turned 60 years old in December, 2020; a huge milestone for him that we never thought he would see. The next goal is to reach retirement age, and with the hope of a possible lung transplant, we remain positive he will get there. April 11, 2014, is the day that changed our lives completely. The day Ron was diagnosed with idiopathic pulmonary fibrosis (IPF) at the age of 53 years, and told that he had 3–5 years to live. Where do you go from there? It was like a bomb exploding in our lives. We had only recently got together in 2012, believing we had years stretched out ahead of us. But now, the love we had found was going to be cruelly snatched away from us in a relatively short space of time. Ron was a triathlete, swimming and cycling every day, he looked so well. How could he be that ill? For me, those first days and weeks were extremely emotional, full of tears, sleepless nights, fear of losing the man I loved. We wanted to grow old together. How was our life going to change? How quickly was it going to happen? What was the end going to be like? We had nowhere to turn at that time, no leaflets, no-one to guide us or be there to stop us feeling so alone. That year was full of hurdles; first the diagnosis, then the passing of Ron's mum, as well being made redundant from his job. We were at an all-time low; we did not believe Ron would get to 60 years, and certainly not to retirement age, so what was the point of planning a future? We started to just live, waiting and watching for something to happen—the inevitable change. Ron's confidence took a dive. He was always the life and soul of a party, and he loves to make people laugh. We loved to dance, and as time went on, if we were on a cruise and Ron wanted to go to the disco in the evening, he had to rest for 3–4 h in the afternoon for 30 mins of enjoyment and trying to feel normal for his age. Everything we do has to be planned. Are there any hills, inclines, or steps? If so, we don’t go to those places, which can make life quite restricting. A busy day is always followed by a day of rest. Lifting heavy things, carrying the shopping, mowing the lawn, or exerting himself causes Ron to have a coughing fit, so I have do all of these jobs. Mentally, at his age, watching me do all this has been hard. We were foster carers at the time of diagnosis and had to give up because the chance of catching infections became too much of a risk, much to our regret. Our lives were changing very quickly, and this was challenging physically and mentally. One day, we were talking about our future, and we decided we had been dealt this hand and we couldn’t change it, but we could choose whether to let it beat us or take charge of our situation and find the positives this new challenge could bring. Yes, we had expected to carry on working, fostering, seeing all our family and friends, enjoying our home, and planning for our retirement, when after all the years of hard work we would retire and we could enjoy travelling together and enjoying life to the full. However, with IPF snatching that from us, we took the decision to get married. It was a very emotionally charged day, as we both knew that the line “’Till death do us part” was a prominent part of the service, there were many tears from all attending, but we made the day full of laughter despite Ron being exhausted at the end of it. We then decided to fulfil Ron's bucket list. We sold our house and downsized to a bungalow to prepare for the future and release capital that we set aside for our retirement. We immediately started planning trips to all the countries we had dreamed of visiting. We have visited 78 countries in 4 years, and have ticked off a lot on our to do list. We know we were lucky to have this opportunity, but Ron sees it as getting what he thought would be 20 years or more condensed into 5. During our journey, we were introduced to the Royal Papworth PF Support Group. We attended our first meeting with trepidation, but soon realised the relief we felt meeting people with the same disease, laughing, living, and getting the best out of life while supporting each other. Ron was soon asked to become the chairperson of the group, and this gave him great satisfaction and renewed enthusiasm to raise awareness of this disease. This led to several opportunities coming along for Ron, which included giving talks to Members of European Parliament, to pharmaceutical companies, support groups, and other medical arenas about how the disease has impacted his life. In his bid to help and support others, he was asked to become a trustee of the Pulmonary Fibrosis Trust, who provide practical aides to people with IPF or PF in their time of need. He has received many letters of gratitude from patients who have been given the freedom to get out after being given a mobility scooter. This is extremely rewarding for him. Ron has become an advocate for patients raising awareness wherever and however the opportunity arises. At one of Ron's patient advisory board meetings in Europe, I was asked if I would like to join. I was only there as his carer so I was somewhat surprised when I was encouraged to participate fully. This opened my eyes to the idea that carers’ points of view did matter, and the concerns and issues we feel and have to deal with are just as valid as the patients themselves. Without carers, a patient's world is extremely limited. I have since become a passionate advocate for carers, as on this disease journey it has shown me how little support and understanding a carer receives. I, like Ron, have taken every opportunity to raise awareness of a carer's plight and joined The Taskforce for Lung Health as a carer representative to make sure our voices are heard. If you had told us both 6 years ago what we would be doing now, raising awareness, neither of us would have believed it, as we did not know we had it in us. It is amazing when you have a passion for helping others what you can achieve, despite your own circumstances. While we are still constantly aware of Ron's illness and his deterioration, we are fortunate to have access to the antifibrotic drugs—pirfenidone, which he took for the first 4·5 years and after a steep decline changed to nintedanib, which he is currently on. Despite both drugs causing Ron to have side effects, he feels that they have taken him passed the original 5-year prognosis given. However, he is now on ambulatory oxygen. This in itself causes obstacles, making sure he has enough if we go out and coping with the looks you get from strangers. He is down to 50% lung capacity, so our own personal future is changing, and reality does hit home. We have noticed friends have backed away and our world is becoming a smaller place due to Ron's limitations now. We have to manage our own mental and physical wellbeing and this past year has been even more challenging. Ron has received phone calls from the local hospice regarding his mental wellbeing because at times it is really hard to deal with a terminal illness and all the implications that holds on your own. I know he tries not to worry me, so having a neutral person to talk to is a massive help to him. We both find that the satisfaction we get from helping others and seeing the changes happening in the pathways of this disease, knowing that one phone call to a patient or carer can change their outlook and make them feel less alone and able to fight this illness, keeps us both positive. We feel that although IPF has taken the future we had planned away from us; we have fought back. The idea that we may have made even one person's life easier or better is comforting. As strange as it sounds, this disease has made us better people and brought us a lot of happiness, and some amazing friends who we would never have otherwise met. IPF will not define Ron. It has made him a complete person. Before, with life, work, and family, we didn’t have the time to reflect and see how much acts of kindness can make such a difference. We were busy living, getting on with life, rushing around—tomorrow was expected, life had no end. IPF has taught us to enjoy the here and now, and the people who are there for us and support us on our journey. Ron turned 60 years old in December, 2020; a huge milestone for him that we never thought he would see. The next goal is to reach retirement age, and with the hope of a possible lung transplant, we remain positive he will get there.