IPF

[Tina_B]

I was wondering if anyone could help, my mum is 77 and has IPF she was diagnoised September 2014 she left hospital with steroids and a course of cyclophosphamide, which wasn't completed due to a chest infection She just about manages with light day to day chores and tries desperately to remain active. But of late there has been a noticeable deteriororation in her legs, they are very jelly like. I know that the steroids aren't helping and her consultant has advised that a new drug pirfenidone, might be the answer, but mum being older and sometimes confused is reluctant to change drug, due to the long list of possible side effects the doctor told her was possible. No amount of persuasion on my part is changing her mind. I was wondering if anyone had tried this drug and what the benefits if any where. This is such a scary illness and one i know very little about other that what i read on the internet. It seems there are support groups but they ar quite a distant from mum. Many thanks in advance

[Azure_Sky]

Welcome Tina,

I am sure you will find lots of support and advice here. You can also ring the BLF nurses, or email them.

Your mother is understandably nervous about taking new medication. However so many medications have long lists of terrifying side effects.

Some of them are extremely rare. It is worth looking on the internet to find out what they are and how likely they are to happen.

One of our members spoke about black hairy tongue as a side effect of a particular medication. Guess what? I got it with an antibiotic I was taking.

It is still there, although I finished them, the beginning of last week. I am seeing the dentist tomorrow, who prescribed the pills.

[jenss]

Hi Tina, I am sorry to hear about your mum. With IPF it is very important to see an ILD specialist at an ILD centre. A "normal" pulmologist often does not have up to date knowledge and experience. Steroids are contraindicated in IPF and according to the guidelines should not be prescribed to treat IPF long term as they do more harm than good. There are two new medications pirfenidone and OFEV. There can be side effects when taking them, but many people do not get any. There are steps that can be taken to reduce side effects such as taking the drugs with food etc..... your dr should be able to help.... provided of course that they are a specialist with relevant experience. Good luck convincing your mum to see a specialist and be open to take the new meds. Ringing the BLF helpline as Azure suggested is also great idea. Best wishesxxx

[imelda16]

Hello Tina the drugs you talk about are Pirfenidone and Ofev is another new one and I have been on Pirfenidone for 6mths but that didn't suit me. I have IPF and was diagnosed in 2011 I'm 74 now and have been lucky enough to stay out of hospital since being diagnosed with the desease although my condition has got worse. I had the jelly legs and was shaking but they put me on oxygen for 15hrs and it helps a lot even though it's made changes to my life. I am starting the Ofev in October the specialist consultant says that may help. There is no treatment to cure the fibrosis but it could stop it going worst unfortunately your mothers GP will not know much as most consultant s don't and they can only suggest lungs transplant. It does help to catch up on forums for just IPF because it's difficult to meet anyone and that's why the BLF forum is helpful. I have picked loads of information from this site even though it's mainly COPD. Must warn that the Perfenidone treatment ends up with 9 capsules a day 3 with each meal so your mum will need help keeping up. Good luck x

[Tina_B in reply to imelda16]

Many thanks for your reply in a strange way it is comforting knowing my mum is not alone in this terrible illness. Are you mobile and able to care for yourself. Mum is currently living in her own home and although very slow she can do the basics of caring for herself. I know there are times she is very scared but is getting to grips with knowing she isn't able to do what she used to be able Can you tell me Ofev is? what medication do you currently take Do you have oxygen very often? Mum does have a consultant but takes comfort by not having to see him until September, she gets herself into a terrible state of anxiety in the run up to seeing him, fearing he will admit her to hospital Do you have frequent tests i.e. lung capacity test. Sorry to ask all the questions but i have never spoken to anyone who has this condition. Finally how has your condition got worse? Very best wishes Tina

[imelda16]

Hi Tina it is nice to know your not alone and someone can identify with your problems my family felt lost at first. I am fairly strong at times so I kept the first year to myself probably in disbelief but as time passed and symptoms began to develop such as the breathlessness and low oxygen levels when out and about went low I had to decide on using oxygen portable cylinder. But since January I have to have it 15 to24 hrs daily. I'm not on my own my Husband is here but not much help as he has problems of illness and is 83 but the family are always around but I like to stay independent for as long as possible. Ofev is a similar drug but you only take 2aday but these drugs are new so there are no real promises more wait and see. I only got monitoring visit s with consultant and lung function tests.Facebook has a forum under pulmonary fibrosis they are good for family as well. Good luck IMELDA x

[Sooki]

Hi,I have IPF and so did my mum,she was diagnosed at age 79, she was also on steroids we are going back 5 years and now they are not prescribed for IPF.My mum lived alone and it was difficult to cope,reluctantly she went into sheltered housing,her own flat,it made such a big difference to her,she loved it,I hadn't been diagnosed then,I was her carer.The long use of steroids weakens the bones,she fell and broke her hip,never came out of hospital.i was diagnosed nearly 3 years ago,I am on pirfenidone,it's not that scary,you build up to 9 tablets a day,yes there can be side effects,but your body does get used to them,or it can be you can take 6 a day,I have just built back up to 8 as I had difficulty to start,I am fine on them now.Your mum should be seen at a specialist ILD centre,I don't know where you live,although I live in Birmingham,I travel to Papworth in Cambridge ,usually seen every 3 months for lung function tests, etc and always seen by consultant,.Your mum may need to be tested for oxygen,I need ambulatory oxygen.You are entitled to be referred where you like,The BLF have a good page about IPF,there are several support groups around the country ,the Facebook page for Pulmonsry Fibrosis UK has a thousand plus members,it's a closed group,you just need to join,it's the best group we all support each other and you can ask what you like.Hope this helps,you can also message me if you need any advice.Sooki.