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.
