My Experience of Living with Idiopathic Pulmonary Fibrosis (IPF)

I was first diagnosed with IPF in 2002 by chance. I had had a couple of doses of pneumonia type symptoms and feeling unusually unfit at my workplace requested a visit to the company doctor. On checking me out there were again signs of pneumonia and he sent me immediately to his local hospital, which happened to be Royal Brompton, and within the day a specialist there had diagnosed a lung condition that was to preoccupy me and my wife for the years ahead. The diagnosis and explanation behind the illness were of course stressful but I was told that my condition was dormant but that it might flair up in the future. There was no medical solution but the use of steroids would be helpful. The doctor actually described the condition as ‘bird fanciers disease’. I am hoping that these notes are of interest and support to any newly diagnosed sufferers.

The reason for writing a record of what I have encountered is that it quickly became clear and has remained so that little was known of this lung condition, its causes, and what as a patient, you might encounter. Doctors have been supportive but know little and therefore guidance towards the future is limited. There is a keenness to sign up a patient for trials, which it is difficult to resist, but patient care has always seemed low on the list of understanding. IPF isn’t as sexy as cancer for instance and therefore there is little to no publicity or financial backing for research. Only recently have drug companies stepped in to sign up hospitals and patients as they search for new drugs to extend their business model. 

By way of background I stated that my condition was first diagnosed in 2002 but where and when did it begin. Retrospectively I recall being unusually short of breath when playing squash around 1997 at the age of 53. I hadn’t played for a couple of years and so put this down to not being fit but it was an unusual form of breathlessness. I joined a David Lloyd Centre about that time to improve my fitness and found difficulty in swimming any distance beyond a couple of lengths, while my wife was cruising up and down length after length. But if I try to get a fix on a specific happening then that would relate to a business trip to Malaysia and Singapore. Shortly after my return I went through a severe pneumonia and was hospitalised for two weeks. This single event undoubtedly leaves a thought in my mind as to whether I contracted something that I have never been able to shake off.

If the condition isn’t contracted could it be in family genes? My father died suddenly from a bout of flu, something that he suffered from periodically but it was of a severe flu he was about 68 and this was 1968! An older cousin of mine died almost certainly from IPF after a year or so of fluctuating illness at the age of 73. I should mention that my father was from India as was my cousin. The point about these last two paragraphs is that there has been little interrogation by doctors treating me into my history….how do they expect to identify the cause if there is little other than cursory time spent on investigation

So what has happened since 2002 and how might my experience help you! Following 2002 my condition remained stable for about three years and then I noticed severe breathing difficulty on any exercise. I attended a private doctor for a check and continued to be monitored via x-ray, breathing tests and the usual finger nail review for clubbing and general questions. Late 2008 I was referred to Papworth Hospital checked over and pronounced as having active IPF. Average life expectancy 2.9 years, no need for a biopsy it is clear cut. Prescribed low level steroids, Mucodyne and keep your fingers crossed.

It is important to stress here that the 2.9 years expected life span is an average i.e. a mathematical calculation. I have heard of people that have died within a year and others like me whose progression has been a lot slower. It does appear to me that the disease is observed in older people as a rule. However, clearly the cause has been in place for some years but is unnoticed until severe health restrictions are encountered. Because of oncoming age many people accept a natural slowing down and if they are not otherwise active ignore any apparent symptoms’ of IPF as an inconvenience of their advancing age.

To continue, I was to attend three monthly reviews to be checked by way of the cursed breathing tests, six minutes walks, occasional x-rays and echo-cardiogram. At that stage I was getting about quite well but I undertook a test for flying……realism dawned, I could only fly with oxygen! At least a holiday to Egypt could go ahead and I found the airlines most helpful but you must check out their policy on the matter and some charge quite substantially for the provision of cylinders.  Treatment continued much along these lines for eighteen months but all along my breathing was tightening up, the six minute walks slightly shortening. It was suggested that I should have the use of therapeutic oxygen to help when exercising i.e. walking etc. and this I took to quite readily and it appeared to be helpful. At this stage the oxygen was provided in lightweight small cylinders which could be carried by way of a shoulder strap or wheeled. Either way it was not a problem. My wife and I holidayed as before except that I gave up flying. The hassle of the departure hall and security all while using oxygen cylinders was too much for me to be bothered with. To my way of thinking to put everything into the car and drive off seemed much the preferred option. We did however manage one holiday by Eurostar to Cannes. The train service was excellent with staff on hand to handle bags on and off trains. This was subject to prior notice but all worked well. By that time I had purchased a portable oxygen concentrator and this could be plugged into sockets conveniently positioned by the seats in the train. The battery within the concentrator plus an additional Velcro strapped battery gave a useable period of about four hours and is sufficient for most exercise. This machine works on the basis of pulse flow which of course is fine in the early days of disability. But for sleeping a continuous flow machine is essential. I managed to afford one and this gave greater flexibility for holidays and short stays away. Both of these machines were purchased from Smile of Newbury and their support and service has been friendly with competitive prices.
On a previous occasion, via Papworth, oxygen had been delivered directly to a hotel in Cornwall. This included a machine for overnight use plus cylinders for day use. Again I must say that the delivery was on time and I am grateful to the NHS for providing such a service.

 It was now into the spring of 2010 and between then and the autumn I trundled along at my own pace now used to carrying the oxygen. While I had adapted to my new state one of the problems of the earlier years and that time was regular bouts of infection which manifested itself in the form of a mild pneumonia causing a slowing down together with light headiness and depression. At this point I was carrying an emergency supply of antibiotics and after a week or so my condition started to clear but it might take up to three weeks to fully clear. The depression was the most negative experience and it certainly gets one down. There is an over whelming  feeling of inadequacy and insecurity which manifests itself in tears, frustration and of course taking it out on those nearest to you.  My wife and I set off for a holiday to Tuscany, by road of course. From the west of France, Alsace, I began to feel run down and after arriving in Lucca I began to feel really below par. I became very high headed and could hardly walk more than a few yards without the need to steady myself. Depression was again setting in and although I was taking the antibiotics there was no respite. I spoke by phone with a doctor at Papworth who was most comforting but nevertheless I began to panic and the only thought was to get home for security, with two days drive ahead this was no easy decision. We left Lucca and while the drive was through wonderful scenery I was glad to be heading home. However the point that I want to make here is of some importance to anyone going through IPF. Due to increased vulnerability there are regular bouts of infection and all that goes with it. There is little to be achieved by panicking. If you are self-medicating then being at home simply means that you can rest, which of course is comforting but you can also do this while you are away.   I am saying that rest patiently while on holiday and give your body and mind chance to recover, retrospectively you will better for this and for being more in control.

On a couple of occasions we travelled to northern Spain by Brittany Ferries from Portsmouth to Santander. The service is excellent; on arrival, as a disabled person you are quickly identified and directed to park near to the service doors/lift. We were met by a staff member who helped us to our cabin. Again I must say that the cabins for the disabled are excellent and the service on the boat more than adequate. Santander, San Sebastian and the whole coast and inland to the Rioja area is a delightful place to visit and drive through. The ferry makes it such an easy destination to reach and I recommend it to anyone who finds their journeys become restricted. However on the last occasion I had set off after a couple of days of being quite busy and feeling a little below par. The following morning at the hotel my oxygen levels were struggling and I was feeling depressed and light headed. I panicked a little as usual and the hotel called for assistance from the local hospital. The medics were excellent and I was taken by ambulance to hospital where I spent the day under observation. The conclusion, having checked my medical file which I always take with me, was that there was nothing that they could do. I should return home or stay quietly at my hotel. We elected the latter and limped home after a few days. But the fact is I remained in control and I was pleased about that.   

As part of my initial appraisal or medical checks at Papworth I was advised that a lung transplant could be considered and I had the obligatory chat with the doctor at the transplant unit. By the end of 2010 my condition was getting worse and so I was asked to undertake medical checks in order that my suitability could be assessed. I was sent off for an angiogram to a local hospital and identified as having a minor impairment to the artery feeding the right side of the heart. The doctor at the hospital thought that the problem could be dealt with by a regular dose of aspirin but in fact Papworth said no, any impairment was a negative and I was not a suitable recipient of a new organ. I was not greatly disappointed. Yes, it suggested that there was no way out of the IPF dilemma but from all that I had read a transplant is no easy alternative. However I deeply aggrieved by the offhand way that the Papworth transplant unit advised me or should I say not tell me. It took from Monday until the Friday to get any feedback to the result of the angiogram and then only after I pressed the unit for some news. I was told that I was not the only patient that they were dealing with but even accepting this simple statement there was a complete lack of sympathy shown towards a patient or anyone whose emotions are running high while awaiting such a decision. I did complain through the hospital PALS unit but frankly this was a waste of time and it is no more than a procedure to offset patient’s grievances and defuse an otherwise uncomfortable situation, in other words a whitewash! 

I had been under Papworth for just two years and following my negative transplant assessment I was told by the consultant doctor that there was nothing else that could be done. I was a little unsettled at this statement and believing that I owed it to myself and family to do all that I could to manage the rest of my days,  in conjunction with my GP  I elected to transfer to Royal Brompton Hospital, one of the centres of IPF research. The patient experience at RBH was quite mixed. Over a two year period I saw the consultant doctor once as part of a routine check. In order to see him I had to go privately to an evening appointment, not ideal but I came to understand that at such a busy hospital a patient is not more than an NHS statistic. The nursing was excellent but the over-riding care was totally lacking. On the plus side the doctor did suggest an intensive course of the steroid Prednisolone however this was not successful. But he also suggested that I use a regular medication; the antibiotic Septrin, a drug that I had trialled at Papworth. This has proven to be most successful and has helped considerably alleviate the intensity of infection.

At Papworth the care was always in evidence and I decided to return there, with the prior approval of their doctors. It is now almost four years since I was first diagnosed as being in an advanced stage of IPF. Life is now becoming difficult, I can walk a few yards and with great support from my wife I get about. I have the use of a wheelchair and a small scooter which I find invaluable. We have fitted a chairlift which was supplied by Handicare of Stourbridge and modified our shower to provide easy access. I have just taken possession of a perching stool courtesy of NHS. I am now slowly coming under increasing care of the NHS system via my GP. The need for additional care at home is being assessed but I feel reassured under the care of the District Matron and Macmillan Nurses.
There is one overriding comment to make which became evident to me while at a recent visit to RBH where I was chatting to a like IPF sufferer. You must have help and support at home and at work of course if you are to continue at a workplace. You may be able to manage for a few months but the going will get tough both mentally and physically. You cannot go it alone; the person I was talking to was alone and was clearly suffering lack of support. If you do live on your own then seek to secure all the support that you can.