From what I read the drugs will only be given to those in the low to medium category.....that is between 50-80% of normal. Lower than 50% of normal is deemed to be high and in effect not worth the effort/cost for benefit gained. Higher than 80% they say you are not affected much - my last score before this was 109%, I do not yet know the last score!

As usual the accountants are in the loop so it has to be 'cost effective' and does not take into account any other benefits!

David has had IPF since 2008 and is now facing the final stages of life. His blog post deals with the subject of dying in an honest and open way.


David - IPF

Although I had a cough and minor problems with my breathing early in 2008 and some ‘crackling’ sounds in my lungs, it was not until later that year it was identified as idiopathic pulmonary fibrosis following a CT scan.

My consultant explained the sad reality facing me: there is very little that can be done for this disease and there is no known cure.

While the BLF now has a whole section of its website dedicated to the latest IPF developments, back in the days of my diagnosis, there was very little information available online.

But I read everything I could find, sometimes not understanding half of it. I felt very alone knowing there was there was so little help and support of value available to me.

Exercise makes a difference

The following year, I was offered a place on an exercise class run by the local respiratory team. I went for it! The physiotherapists and nurses were really good and the class of a dozen loved it, even the more strenuous bits!

Pulmonary rehab exercise class for lung health

And it also gave us a chance to spend time talking, getting to know about each other and sharing our daily challenges. It was quite enlightening. I was upset at how the people with COPD struggle and they were taken aback to find that the IPF prognosis was death just a few years after diagnosis.

It was a very positive experience, but the exercise course only lasted 9 weeks and any following courses would only last 8 weeks. After that, there was nothing!

When we ended the course, we all agreed that more could be done. So one day, I went to the local authority sports centre to enquire about the possibilities. It turned out that the manager of the gym and the lead physiotherapist at the pulmonary rehabilitation unit knew each other, and everything slotted into place. They worked to make sure the group could continue their exercise and others could join too.

Not only were we moving again, we were also talking and looking after each other when the going got tough.

Sadly it only lasted a little over a year. Access to this class was by referral only, from a doctor or respiratory nurse, and the facility was withdrawn because there were no new referrals – and of course sick people drop out over time! Can you believe it?

In my experience, if you have a lung disease, make sure you exercise, even though it’s probably the last thing you want to do when you have difficulty in breathing. Don’t just rely on drugs, particularly in the early to mid-stages.

Progression

Well here I am now, five years on. My condition worsened badly in February, like falling off a cliff. I found difficulty doing anything at all. General panic all round. I developed secondary conditions like mucus creation and coughing. Regular bodily functions were disrupted.

I went through a series of drugs. But after a while, I had had enough and I was tired of living with a cloudy head, so it was goodbye to the morphine.

Live, laugh, love

I have found the best possible way to deal with this extremely unpleasant disease is to laugh at it. Clint Eastwood famously said: ”a man’s gotta know his limitations”. Too true with IPF.

So now, in the words of A. A. Milne I largely ‘sits and thinks and sometimes I just sits.’ I have been outside to the garden once this year. I use a commode. I never walk more than five paces and that is to get into and out of bed. My wonderful wife dresses me and washes me and generally holds me physically and mentally together when it all seems too much and perhaps it is.

End of life

I know that my medical notes say this is the ‘end stage’ and that I am a dying patient. Well, not yet matey, I’m not – try again next week.

But while I am excited and curious about what comes next I have no fear about it. I am also curious about what comes after, especially as I face the end! I find that few people want to talk or contemplate dying, death and after death. Yesterday I told the plumber with a big smile I was moving towards the end and doing just fine but the body was a bit of a bind. We ended up laughing.

But I am interested in how I will die. Will it be painful, traumatic or gentle and quiet? I have wondered and felt distinctly wobbly when I have heard doctors say bluntly I will die of suffocation. The most I know is that somewhere in our house there is a sealed bag that contains drugs that can only be administered by a qualified person. These drugs that sit quietly waiting their turn are not to speed the end, they are to carry me through the inevitable end process of this disease in ‘comfort’ rather than in deep distress.

But there remains the question: not when it will be but how it will be?

Coming to terms

We all know idiopathic pulmonary fibrosis is a pretty rare disease. So it is rare to know anyone near you who has it. It just so happens that the father of one of my carers died about five years ago and rather than have him taken into hospital, she nursed him through it to the end with her mum. Until she came here and we talked, she wasn’t sure exactly what he died of. But the more she talked, the more it became clear the symptoms and events matched mine. He had died of IPF.

He had struggled through all the problems mentioned above attended constantly by a nurse in the last week until close to the end he said he didn’t think he could stand it anymore. The package was opened and the nurse injected the drug.

She said the effect on her dad was immediate. Where he had been struggling desperately to breathe he just relaxed on the bed and appeared to be in a gentle sleep, breathing lightly. He died peacefully the next day.

My relief at hearing this was palpable. My carer was so pleased she had been able to help in this way. Also, unwittingly, I had given her the opportunity and a safe place to let it all out which was of some therapeutic value for her. So we have gone on talking about it and I continue to learn. I hope something in all this may now be of help to others.

Meanwhile, keep on truckin…

Inhaled perfenidone gets orphan drug status

Potential new immunotherapy drug supported by NIH.

I'm sorry to hear that Alan. See if you can get access to the inhaled version of the drug. It seems to have better absorption with lower side effects and has been granted orphan drug status in the US.

http://www.empr.com/inhaled-pirfenidone-desig...g-for-ipf/article/364552/

You could also ask about trying the experimental drug PTL-202 on your own. It's just a combination of an existing drug you could get off-label or on your own from overseas pharmacies and an amino acid. See this post . You would just need to clear it with your specialist. You want to avoid any drug interactions, known side effects, etc. Also you would need to get the correct dosage and correct proportion of the Pentoxifylline to NAC.