We did our best to make sure that mum lived well with IPF

Karen lives in London with her husband. Her mum Joyce, lived with idiopathic pulmonary fibrosis (IPF), for 11 years until she sadly passed away in July 2020 at the age of 88. During this time, Karen helped to organise care for her long distance, with the help of her sister. Karen describes her mum’s journey and the impact of being involved with her care.

At diagnosis, we didn’t dwell on the terminal aspect of IPF

My mum, Joyce, started experiencing some shortness of breath and a cough. Initially, we thought nothing of it, but it did not go away. Mum went to the GP, who referred her to a consultant at the local hospital. She had lung function tests and a scan and was diagnosed with IPF in 2009. It was a shock. I remember the consultant telling us there wasn’t much they could do, and he gave my mum some medication for her cough. My mum accepted this.

Karen with her mum, Joyce

We never really talked about the terminal aspect of the diagnosis, because it seemed like that would be a long way off or maybe would never happen. At this stage, mum was still going out with friends, driving and ballroom dancing. She managed her breathlessness at this point by knowing her limitations.

I convinced mum to go and see a specialist in IPF

Over the next few years, mum’s symptoms got worse and her cough became bothersome, impacting her quality of life. One evening, I attended an event organised by the Royal College of Physicians in London where a consultant was talking about the latest treatments for IPF. I researched this further and realised there were more treatment options that my mum could receive.

I asked mum if she would allow me to make an appointment with an expert in IPF, at the Royal Brompton. At first, she said no quite adamantly, but I managed to convince her. I sold it to her on the basis that she visited me all the time and I lived near the hospital. She didn’t have anything to lose.

When we went to the first appointment and sat in the clinic waiting room, mum wasn’t feeling very hopeful, as she had been told her IPF was untreatable. The consultant, however, presented several options for treatment and he started mum on Pirfenidone, which is an antifibrotic and has been shown to slow the progression of the disease. He later changed her medication to Nintedanib.

I will never forget when we came out of the consultation.  Mum sat down in the waiting area and cried. She said, “I never thought that anyone would offer anything else to help me, I thought he would just tell me there was nothing to try and to go home.” 

From then on, mum was under the care of the Royal Brompton, who communicated with her local respiratory consultant in Cornwall. Her medication was delivered to her home. Within a couple of weeks, she had started the medication and was trying the first treatment option to help relieve her cough. The treatment helped relieve mum’s cough for some time and she was able to start going for lunch or morning coffee with her friends and visit the local village church.  She had stopped all this when the cough became disruptive. I think IPF can be a lonely disease, as people don’t understand why you cough or why you’re breathless. It’s not a commonly recognised problem that others can relate to.

Mum was a very independent woman, but she was also stubborn!  When she had to start using oxygen she was reluctant to do so. She thought she would be better without it, but once she got used to living with oxygen, it helped her manage things that she couldn’t do before and her daily life felt a little easier. She enjoyed being around other people, and when she was referred to pulmonary rehabilitation (PR) by her local Respiratory Nurse Specialist, she found it helpful to be around people who understood breathlessness. It was her support network. The group sessions, the exercise and the advice given by the specialists who ran the group was invaluable to mum.

I realised there was support out there to provide ways for mum to have some quality of life

Over the years, mum required more oxygen and for the last six months of her life she became housebound. She did struggle for a couple of years but we were lucky enough to be supported well by a local team of carers and healthcare professionals and her local GP practice.

Mum got the best possible care and quality of life which relieved my anxiety as living a long way away made me worry about mum. There was always something to research or plan. It felt like a full time job just keeping up to date with it all and making sure things were in place.

During the later stages of her illness, mum was supported by the local palliative nurse specialist, district nurse team and respiratory clinical nurse specialists.  They visited and phoned regularly and organised community support from a hospice team. The oxygen company were also knowledgeable and supportive, their regular visits were more than just a delivery. Mum had a foot care specialist, physios, equipment loan to aid mobility and an electric mobility bed to ensure her safety. All of the teams involved pulled together to organise all this and as a family, we are forever grateful.

We eventually had 24-hour carers in place and they looked after mum’s daily needs and were incredibly kind to her. When I was there, we would have time to ourselves and I would help as well. Sometimes, we just had quiet family time.  Mum enjoyed just having me and my sister there.

My advice to carers is to look for support everywhere and give things a try

I miss mum a lot. There is a big void now. We did everything we could to ensure that she had the best quality of life possible throughout her illness. My advice to patients and carers is to be proactive and look for support. Not just because it is there, but because it really helps. The British Lung Foundation (BLF) provided a lot of information on the condition and where to go for help.

I’d also say research treatment options and ask questions. I remember mum being proud and not wanting to use a wheelchair, have oxygen or any help from carers. Having a healthcare professional as a third party to encourage mum to try these things and realise the benefits was essential to mum having a quality of life.  Portable oxygen and a wheelchair became positive things, which meant that we could still go out for a drive with a picnic or go to the supermarket.  These things may sound trivial, but it helped mum function and be a part of life.

Finally, be proactive, be positive, seek help and share your experiences. One day, the things that you’ve been through will help others in their journey too.

The Taskforce recommends that every person with lung disease has a personalised care and support plan. Patients, families and carers should have access to relevant information about their condition, treatment and management.

Every person with lung disease and their carer(s) should have access to a shared decision-making programme where they can identify areas where they need additional support.

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14 May 2021