Still shielding

 There is so much unknown about Pulmonary Fibrosis, and a general lack of knowledge about the nature of the disease and how it relates to other diseases.

In medicine not everyone reacts to a disease in the same way. Not everyone gets the same symptoms, and a treatment which helps one person doesn’t necessarily help everyone else.

Currently there are no treatments which stop fibrosis or reverse fibrosis. 

With pulmonary fibrosis, whilst the fibrosis can appear to reduce the volume of the lungs, it doesn’t always do so significantly, and can also sometimes only reduce the volume in the very last stages of the illness. Fibrosis makes the wall of the lungs - the interstitium - less flexible and thicker, thus making it harder to breathe, and making the person more breathless when exerting themselves. 

From observation it would seem that getting the right nutrition is extremely important. Rapid weight loss is not good and can result in a very poor prognosis. I have known several people on Nintedanib who have suffered with stomach upsets, including severe diarrhoea, and not lasted long, I suspect because they have persevered with the medication for too long and their organs have suffered as a result.

I believe that when a patient is being seen by a specialist centre, then that specialist centre should look after the whole health of the patient. Currently there is a game of symptom ‘ping-pong’ with some aspects of a patients health being looked after by a GP, and the fibrosis being monitored by a specialist. The problem being that the GP, who will be asked to advise on nutrition, blood pressure, weight management, cough and other things generally has little or no knowledge of PF and its treatment.

The patient is left with a dilemma in that he doesn’t really know who he should approach when symptoms worsen suddenly. 

Time moves on

There is a new report that shows that if you have IPF and catch the COVID-19 virus you only have a 50-50 chance of surviving, worse if your FVC is less than 80% or if you’re obese.

Sobering thought.

What is annoying is that there are people who strongly believe that there is no virus and it is all a scam to convince people to have a vaccination, have a ‘controller’ implanted, and your mind and beliefs will be controlled from then on. Such utter rubbish, but people are spreading these theories.

It is extremely worrying that there are people like this around and frustrating that there is no point in arguing with them.

I’m still isolating. Reading a lot now. Managing pretty well.

Trying to work out how we can reach more patients with IPF to give them support - if they need it.

Moving on

20th April. Lockdown extended for another 3 weeks, and I would guess that I’m not going to be going out for the rest of the year. If there is a vaccination which is effective it could be earlier - but these take at least 18 months.

My little WhatsApp support group is about 17 members now, from Poole to Falmouth. Messages every day.

I’m just about coping with things, but it’s getting harder constantly worrying about whether I’ve got symptoms. My friends little boy Karies has had a temperature for 2 or 3 days now. Not excessively high, but worrying all the same.

Saving money, the wildlife are exploring new places which are now suddenly quiet.

COVID-19

Sudden unexpected change of lifestyle. Have been in isolation for five weeks now. Have now been getting groceries delivered to home. Medicines too. Don’t see anyone.

The country, like half the world is in lockdown as a result of the spread of a virus which originated in China, back in January. The Corona virus or CoVid-19.

Currently over 100,000 deaths. Over 10000 in the UK. The US has over 500,000 people infected.

Neighbours are spying on each other and informing the police when they think they are breaking the social distancing rules. Anyone who criticises the government is vilified, even though their reaction to the spread of the virus, and their initial strategy for controlling it were badly flawed - basically allowing the virus to spread unchecked, on the basis that the more people that were infected the better - building up a ‘herd immunity’.

There are no easy answers however.

Many conspiracy theories exist.

I’m fine for the minute, but not sure how I will ever be able to mix normally again until such time as a vaccination is found.

Published in the Lancet

In November last year I was asked if I could write an article for the Lancet about my experiences as a patient with IPF.

It went through a couple of iterations, but this week it was published, and I've had some very positive feedback from it.

https://www.thelancet.com/journals/lanres/article/PIIS2213-2600(20)30067-9/fulltext#.XkjnsBZUqO8.twitter

I hope it brings some inspiration and help to some other patients, but also helps the medical professionals understand the patient side of things a little better.

I'm feeling a lot better now - but I still have an occasional cough, and at night I feel I'm not getting enough air, so am constantly using the CPAP machine, which helps me get off to sleep.

Yesterday I had my dental check up. Everything fine, so no work needed at all, but I need to floss my teeth more.

The storms have quietened down, actually quite sunny at the moment, but there is a lot of water around. If it rains again there will be instant flooding.

I've had some issues with the lights at home, constantly blowing the fuse. It's been sorted now thank goodness. All the visible connections have been changed, so now I need a load of new lampshades - currently have Russian lampshades in all the rooms.

Possibly overdoing it..

Choral Diversity, a choir in the Hereford area which rehearses at Leominster Golf Club had a concert in Hereford, and raised £5745 for Action for Pulmonary Fibrosis.

I had the pleasure and honour to meet them to pick up the cheque. It was a long drive but well worth it.

Listened to part of their choir practice.

My granddaughter Molly is home from University ill. Fever and cough. Quite poorly.

Yesterday, before I drove up to Hereford, I waited in for my Oxygen equipment to be serviced. Today, I waited in for my Pirfenidone delivery. I was glad of the chance to sit and doze for a few hours, although I could have done with staying in bed.

I really must slow down.

I feel a lot better, but when I start coughing I get sick, and giddy. Really not good when I'm driving. It's a really weird feeling, feels like my vision does a 'freeze frame', and I sort of lose consciousness. I always stop when I feel this coming on.

There's a paper with my name on being submitted for publication in the Lancet. That'll make two in the Lancet - Yay. All about how short Telomeres can be a cause of PF - which could lead to a test at an early stage, before symptoms become apparent.

Added to this there is a second study just published which indicates that first degree relatives of IPF patients have a 30% chance of getting IPF themselves - and if this could be detected through the test for short Telomeres - could be a game changer.

Looking forward now to another weekend chilling out, doing nothing.

Hopefully now getting better

At the Arena Session at the Medical school, one of the students asked the question - why do I keep going, why don't I just give up.

The context being that this IPF is incurable and there is no sign of a cure, and as any new drug needs several years of tests, trials, and decision making before it can be used - what hope is there for me, as I've already been diagnosed for over 5 years and the median life expectancy after diagnosis is 3 to 5 years.

 This was further compounded by a conversation I had with the lead researcher at the medical school and my consultant on Tuesday, We were talking about the fact that some therapies give short term benefit - make you feel better and give a better quality of life, but have in fact been shown to actually speed up the progression of the disease.

Most notably steroids in the case of IPF, although they are still widely used long term for PF, but also Pulmonary Rehab, this is one I've been worried about for some time, and also possibly supplementary Oxygen.

My own theory is that using supplementary oxygen encourages people to do more physical exercise than they should and consequently overstretches the lungs, causing more scarring - in the same way that pulmonary rehab does.

Back to the original question - why don't I just give up? I guess it's just in my nature to just keep going, it's possibly being in a perpetual state of denial. It's easier to sit in my office behind a desk keeping myself occupied, than sit and home worrying, and you never know, I may help someone find the elusive cure.

I admit though, I do learn a lot about the disease every time I meet with others who have knowledge of the disease.