IT TAKES YOUR BREATH AWAY

My journey with Idiopathic Pulmonary Fibrosis (IPF)

WHO AM I?

Hello! my name is Anne Sharman and this about my journey with IPF or Idiopathic Pulmonary Fibrosis as a wife and carer for my husband Andy who has this horrible life limiting disease.  An IPF Warrior of the highest order. When you care for someone you are in the journey together. I do not have any medical qualifications only experience living with someone with this disease. 

HAT IS IPF?

It is a journey of stress ups and downs of shock fear and distress and total disbelief.  It is a journey of fighting for recognition of a disease that is terminal but is not cancer.  A disease that kills more people than Leukaemia and yet hardly anyone has ever heard of it.  We certainly hadn’t. When Andy was admitted to hospital very ill recently with Pneumonia most of the nurses hadn’t a clue.  The medical profession need serious education about IPF and quick! When Andy was hospitalised with severe Pnuemonia the conversation went something like this..  

(Nurses)  COPD?

(Andy gasping and coughing through his oxygen mask)  No IPF 

A blank look would follow.

(Andy)                  Keith Chegwin died of it 

Another blank look

(Me whispering)   Try Katie Price's mum Amy they are too young to know who Keith Chegwin is! 

(Andy)                  Katie Price's mum Amy has it?

(Nurses)               Oh yes I know! 

I met Amy at a meeting not long after he came out of hospital and told her that she was helping spread the word about this disease although she said it was hard to get some people to listen. If nursing staff are not aware of it then what hope do we have?  We need to educate and spread the word. 

IPF is disease of scarring of the lungs.  The fibrosis causes the lungs to harden and make them less pliable than they once were and therefore making breathing more and more difficult a the disease progresses.  There is no cure it is terminal and the prognosis is 2 to 5 years although there are 2 drugs that are available to try and slow down the inevitable.  These drugs are not available to the patient unless their lung capacity is between 80 and 50% in England.  This is stipulated by NICE (National Institute for Health and Care Excellence) and in other areas in Great Britain and other countries patients are given these drugs immediately they are diagnosed if necessary.  Imagine a cancer patient being told that they cannot have a drug to extend their life as they are not ill enough! This is something people in our situation feel very strongly about.  They need to be prescribed as soon as someone has had the diagnosis.  My husband is on Nintenadib which has horrible side effects mainly causing the bowels to react. Some people cannot tolerate it but luckily with an Immodium a day Andy is able to take it.  We have to have 3 monthly checks with the specialised pharmacist at the hospital to ensure that his blood levels are okay to continue. We have had to install a stairlift to help him as we have no downstairs toilet and although at first he tried not to use it he is now using it to suit him when necessary.

This is a terrible and heart breaking journey to travel.  Seeing your loved one gradually diminishing before your eyes, being terrified that they will pick up a cold which will set them back for weeks and weeks so all the time avoiding anyone who suffers from one. Practically hibernating in the winter as cold air can cause all sorts of problems. It is heart breaking to watch a loved one go through this illness as a once healthy and active person they gradually have to give up their independence and rely on oxygen more. My husband would enjoy fishing gardening bowling and boating amongst other things. He still tries to live life to the full as much as he can.  He goes to the gym and uses the treadmill to strengthen his legs (very important to have strong legs) and uses his oxygen doing so.

Why do people get this disease?  No one knows as Idiopathic means unknown cause so it is not attributed to anything like smoking.  It can run in families to but not every time. There are also parts of this country where it is more prevalent. Andy took part in a survey that asked about peoples jobs and careers to see if there is a link.  I think hobbies also should be investigated as they could be contributory. 

The first thing that patients usually experience is shortness of breath (SOB).  In our case it was whilst on the way to a meal out with our Cruising Club (the boating kind not the naughty kind)!  We were rushing to get there and it was up a hill.  Andy was gasping for breath and said could we stop as he was having a problem breathing.  He made a GP appointment the next week and was promptly sent for an X ray.  About 18 months prior to this he had also been sent for an X ray as he had a cough after a cold that lasted for weeks.  Nothing was reported back to us so we just imagined that all was okay and forgot about it.  We were about to go to a family reunion with lots of cousins arriving from all over the place and off he went off to the Drs to get the result of his X ray.  He came back and without saying a word he immediately Googled "scarring of the lungs".  It gave one answer IPF.  The prognosis 2 to 5 years.  No cure.  Nothing could be done.  Nada.  We drove to the reunion in silence and shock as staying at home would have been worse

START OF THE JOURNEY

Our GP referred us to a Consultant at a London hospital.  We did not know at the time but the GP's father had IPF and he recognised the 'Velcro' sound in Andy's lungs which signifies IPF .  Then the wait and the anguish began.  4 months to wait for an appointment whilst knowing all the time what the outcome would be.  I cannot tell you what those 4 months were like.  I don't think we slept a single night through.  It was awful. We woke each morning hoping that it would all be a dreadful dream.

Eventually the appointment with the Consultant arrived.  He listened to Andy's lungs looked at X rays and informed us that whoever had looked at the X rays 18 months previously had missed the signs of IPF.  It was there for the world to see.  He was almost 100% certain that this was the diagnosis but he wanted Andy to have a scan and further tests before we returned in 2 weeks for the final diagnosis. 

DIAGNOSIS 

That was a day to I will always remember.  We were torn in two as a lovely neighbour of many years funeral was that day and we felt awful not attending but we were so stressed by then about the illness and had had to wait for so long to get an appointment we decided the appointment was our priority.  No messing about we were told that Andy did indeed have IPF and that he had probably had it for 2 years already. There it was in the cold light of day.  It changed our lives for ever.  He had IPF and that would progress and eventually kill him. Andy asked the Consultant how long he had but the Consultant would not say as he said the medication he was recommending would hopefully stabilise it for a bit. Without that it would be 2 to 5 years and he had definitely had it for at least 2 by that point. There was no cure  apart from a lung transplant which is not available in the UK for over 65's usually and then only 50% successful (see below) and basically he was on his own.  There was no nice nurse sitting there to help with the fall out after the diagnosis no support only a card with the telephone number of the clinic and the specialised Nurse to call if you need any information.  The next appointment given was for a years time.  He was spoken to about the medication available and as I have already mentioned Andy opted to try Nintenadib. Our hopes for the future and our retirement died that day. Luckily our specialised nurse was walking past and we were introduced to her.  She is an absolute star and works so hard as she has all the patients in the clinic to look after on her own.  We arranged to see her about any support we could find.  We were in shock we were strangely relieved to get the diagnosis but at the same time the world had been whipped away from under our feet.  We found out that she was trying to set up a patients IPF group to help patients who like us felt lost through Action for Pulmonary Fibrosis who are a charity that helps people with IPF.  Since then several groups up and down the country have been started to support people with the disease.  It so very important to belong and to meet people in the same situation. 

What did we do after we had been given the diagnosis?  We did a strange thing looking back - it was a weird relief in some ways we were in shock and yet it felt like a weight had been lifted.  I know it was just the beginning for worse to come but at that point we were full of shock and relief and we found the nearest wine bar, ordered a bottle of wine and drank it toasting our lives together that we had left.  We then went to our favourite restaurant had a slap up meal and more wine.  We wanted to celebrate the rest of our lives. 

 

Our life savers were the two Support Groups we joined through Action for Pulmonary Fibrosis. One at Guys Hospital rung by the fantastic ILD nurse and the other a local group run by a husband and wife team who have been wonderful.  The wife now my friend also set up a carers group for us carers.  It is a small group as unfortunately due the nature of the disease the group gets smaller.  Like the Support Groups people are sometimes unable to attend due to health reasons or pass away through IPF.  

Action for Pulmonary Fibrosis  

The British Lung Foundation  

Pulmonary Fibrosis Trust       

are all wonderful organisations the latter one run by patients for patients.   You feel so alone and desperate for help and support. They help support others who like us are living in the twilight world of reality and shock. 

LUNG TRANSPLANTS IN THE UK

After meeting and talking to Steve Jones the Chair of Action for Pulmonary Fibrosis at our local groups fundraising event we decided to find out more.  At the time of speaking with Steve Andy was in hospital with  severe pneumonia . Steve asked me if Andy had any other health problems and I said no only the IPF he invited us to a talk the following week if Andy could make it or me if he couldn't attend.  The meeting was in Cambridge and was to talk about Lung Transplants with a transplant surgeon from Papworth Hospital.  Steve himself had a lung transplant in his late 60's and was a walking testimonial that it can work.  Andy was discharged and managed to make the meeting.  It was very interesting. There were people there that had also had the transplant and those that had been accepted and were on the waiting list as well as those contemplating it.  The meeting was very informative and I was so impressed by the surgeon who gave the information and spoke about all the tests and criteria that are needed before you are accepted on the list. It also depends on your build and what size lungs would be available.  Unfortunately many people pass away before a match can be made but the chance is there to prolong your life if you have a transplant and it is possible to do that.  It was all sounding very positive to be honest and we saw a chink of light in the distance.  However that was soon put out when the Surgeon showed us a chart of mortality rates. 50% of people over the age of 65 do not survive a year beyond transplant.  In the UK this is the percentage although I know in the US the percentage is not so bad. It is always a risk and one that we quite honestly decided we did not want to take. 

SO WHAT DO YOU DO NEXT? 

So what do you do once you know what is about to happen?  You put on your big girl knickers and pull them up as far as they will go and just get on with it!  You either put up or shut up and we were definitely not prepared to shut up  Andy is the most  determined person I have ever met.  He does not moan and groan about his circumstances he reaches for the stars but if he misses at least he tried! We read everything we could find on the subject we joined groups online and threw ourselves at the mercy of IPF!  

STRUGGLES WITH OTHER THINGS 

There have been other struggles we have had to face as well as this hitting us.  We had a massive flood in our house when we were away on our last holiday abroad.  We lost nearly everything and the stress was off the Richter Scale.  We were homeless at a time when we didnt need that extra stress.  We lost a lot of our personal possessions and at one point the insurance company didnt tell us whether we would get our claim honoured.  All the damp and mould at the house would be absolutely a no no for Andy.  We had to live in B & B for 7 weeks then got a rented house.  All the time the worry and stress made things worse for Andy.  His breathing was getting worse but instead of curling up in a ball in the corner of the room we fought back.  It gave us something else to focus on even though it was stressful beyond belief. We insisted that everything should be bone dry and every bit of mould gone. 11 months later after a lot of hassle we got back home. We found real and true friends in this time who we are so grateful to. 

Oxygen – that’s another battle.  Being told you can only have one sort of oxygen at a time when that is impossible to live an active life.  They want to take away and put other oxygen methods in place. You have to fight and argue and insist and it is all very wearing when you are ill.  Everyone wants the dignity of living a normal life as they can with any disease so why make it so difficult for them?

A Blue Badge - it took 7 months to obtain.  I applied online and heard nothing and my continual emails pursuing the application went unopened and unanswered. It went on and on until I asked my friends husband a local Councilor at that point for help.  One phone call and it was done. Still when we struggled to the venue to see the person that would assess us they had failed to tell us that he was not going to be there that day.  Not good enough.  Not good enough to spend months trying to obtain something like that. There are so many battles that you have to fight and people who are on their own and ill simply do not have the energy to do so.  Eventually we got one without an assessment. I mean who needs an assessment when they are plainly ill and on oxygen? 

We cannot look too far ahead – cancelling hotels social events meals and trips out have all happened this year due to Andy’s health and I do not like to book anything too far in the future as we never know what will happen nearer the time.  I book everything with a cancellation policy in order if I do book to go anywhere.  But we carry on the best we can.  

People not accepting that you have a serious illness and asking you if you are better or telling you not to worry. - This is very difficult. Without upsetting people here I know that people mean well but need to realise and accept that if someone has an incurable progressive illness that they are not getting better and never will and they will most definitely worry! If not them their carer will.  It is human nature that people do not know what to say but maybe "how are you doing today?" is a suggestion. 

MY MISSION

My mission on this site and in my blog is to make people aware of this dreadful disease and to educate more people including the medical profession about IPF.  Hopefully a cure will be found if not in our lifetime but in the future to stop others having to suffer. This site is for me to try and help and give a bit of information to those who suffer or someone in their family or circle of friends suffers with IPF

Above all if you are a Carer for a loved one or friend with this disease I hope that you will find a little solace and comfort in joining my on my journey. Caring is a very difficult lonely emotional tiring and overwhelming place to be.  You are Amazing remember that!!  

 Anne x