IPF - Acute Exacerbation in UK

[Salmo]

Can anyone from UK help me out with following question about acute exacerbations in IPF?.

I live in an area where there are no IPF specialists/support groups so was wondering if anyone with IPF has had an acute exacerbation and knows how it presented itself, or was detected, what types of test can be done to confirm it and if they were given any treatments. Particularly interested in hearing from folk who are under UK centres that specialise in IPF/ILD.

As background, I've had IPF for 3 years and my lung function has gradually declined over that time. Up till now this could not be detected on a weekly/monthly basis but was obvious over a 6 month to a year time scale. For example, I would find that I was out of breath climbing a hill that I used to have no problem climbing a year or 6 months ago. However, in last 6 weeks the declines can be detected on a weekly basis. For example, in 1 week I went from walking 6 miles without oxygen (SpO2 levels fell to 92) to requiring oxygen just to walk up the stairs or else my SpO2 levels fell to low 80s (previously no fall in SpO2 without oxygen). There was no obvious illness/respiratory infection at the time. Over last 2 weeks a second biggish decline has occured.

My main concern is that I could be having an acute exacerbation but I will not see my respiratory consultant (excellent, but not an IPF specialist) for at least a month so any information would be useful to have.

[hufferpuffer]

Hello Salmo, I'm so sorry to hear you are without any local groups or people who specialize in IPF . I'm sure someone will be along with some advice and you could try phoning the BLF nurses for a chat and advice , I wish I could help but I don't know a lot about it I'm afraid just that there doesn't seem to be very much information available.

Let us know how you get on with your quest , wishing you wellness , huff x

[Salmo in reply to hufferpuffer]

Hello Huff - Many thanks for the reply - will let you know if I get any info. Salmo

[HuwieHex]

I was first diagnosed with IPF in late 2008 and had an acute exacerbation in 2013. I am fortunate that I have a Community Matron who was able to arrange an early appointment with my Chest Consultant. I am at the stage now where life is rather difficult and there wasn't a lot that could be done for me medically.

[Salmo in reply to HuwieHex]

Thanks for the response and sorry to hear about your situation. I get impression that there is not a lot that can be done for acute exacerbation and its very much a case of hoping the decline slow down naturally. Salmo

[Tenter]

Hi Salmo,

I can only sympathise with what is happening to you.

I think you are right, about acute exacerbation, it is just a term to say it has got worse quickly.

Here is my background and a few random thoughts.

I have IPF which was diagnosed in 2015 and has been getting steadily worse since. Then, it was later confirmed that I have a pulmonary shunt where the blood goes to the IPF bits of lung gets no oxygen, sothe body grows more blood vessels to the IPF bit of the lung to help to get more oxygen . Of course this makes things worse. Which of these 2 problems is causing the worsening of my low oxygen I dont know. I do find that I am fine and stable for a short while, then, something happens and I have to increase the amount of oxygen. I am now almost bed bound and permantly on 15 lpm.

The blunt truth is that IPF will get worse over time.

If you are on oxygen, then, you should be able to contact the managers of your oxygen supplier. In my case it is the the community respiratory team, even though the original prescription (HOOF) was done by the hospital. If your not sure, I guess the oxygen supplier will know. My community respiratory team got me in to hospital in less than a day, to see the respiratory consultant after a sudden drastic change were I ended up lying on the bathroom floor trying to get my oxygen levels up.

There are drugs that will slow down the IPF, they are Perfenidone and Nintedanib. If you are not on one of these yet ask about them. I am not sure that your GP will be able to prescribe them, it may need an IPF respiratory specialist with ILD nurse support.

There is a chance of getting on the lung transplant list, as I have done, but, that is dependent on age, fitness and your current condition. Getting refered from a Respiratory Centre, took several months. You will need to be fit, try exercising gently every day.

The mind plays a big part in handling this condition, for me I try to do what I can today, because, I might not be able to do it tomorrow. Then, accepting there are things that I cannot do anymore.

The positives of knowing the probable outcome, are that I have had time to get my affairs in order, I probably won't get dementia. Okay, so I may have now returned to a child state, see post earlier today about transplant update with free Unicorn picture.

I hope my ramblings help and may spark a few ideas.

Best wishes, tenter

[Salmo in reply to Tenter]

Hi Tenter - That was a really useful reply and many thanks for taking the time to share your thoughts which provided a lot of useful information and suggestions.

At this stage I only have the option to follow this up with my consultant but he covers a huge geographical area (and every respiratory disease !) and must be worked off his feet coping with that workload. At the moment I feel the only thing I can d is to try and get as much information as I can about AEs from other IPF patients so that I can get the best out of the next meeting with him. Once again thanks for your reply. Salmo

[Billybob10]

I'm 72.I have IPF. Breathing in only through the nose is so beneficial it is almost miraculous.Take control!

[Salmo in reply to Billybob10]

Thanks Billybob - Spot on with that advice. As a former mouth only breather (more of a rapid panter!) I can confirm what a remarkable difference nose and pursed lip breathing can make. The only problem I have is that I have to focus so much on it that I end up making ridiculous nose tune noises which attract more than a few strange looks - Salmo

[Billybob10 in reply to Salmo]

Don't force it.No need to.

Slow down your lifestyle activities.

Breathe gently and slowly.Too much O2 is not good,Co2 is needed in our bodies to be healthy.I've only recently learned this.Good luck.

[Patriciapotts]

If DISTANCE is no problem to you

get your consultant to refer you to The Brompton Hospital, Chelsea, London.

IPF/ILD Centre of excellence

[Salmo in reply to Patriciapotts]

Thanks for suggestion - longer term that sounds like one of best centres in UK. Have certainly heard good things about it on this and other websites and consultant was going to check this centre out regarding lung transplant assessment. Distance may be an issue but then living in Scotland distance is always going to be an issue whichever centre I'm assessed in.

[Patriciapotts in reply to Salmo]

Since being under the care of the Brompton coping with my IPF has improved enormously Unfortunately I am to old for transplant but the care is second to none.

The Brompton have overnight rooms very cheaply for those travelling a distance so no expensive hotel bills

Time is of the essence as no cure for IPF/ILD, so push for whatever is best for you

Good luck

[winfar]

Hi salmo. I have just recently put posts on regarding my ipf diagnosis and if you read them it will give you an idea of treatment if any for this disease.

[Salmo in reply to winfar]

Thanks winfar, much appreciated. Will check it out. Salmo

[Halbmond]

Just out of a matter of interest, where are you exactly? The BLF have a telephone support system which may be able to answer your questions

[Salmo in reply to Halbmond]

Another good suggestion as I live in a very remote part of Scotland. Appears they do have an IPF/ILD nurse I can speak to.