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My Dad just diagnosed with IPF

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Vonnie21
Vonnie21

Hello, New here and would like some advise please, 

My Dad was just recently diangosed with IPF although we suspected hes had it for approx a year already, diagnosis got delayed as to the type of fibrosis he had due to him having a heart attack, - which became a priority has he had to have stents and then recover, then he developed a chest infection & was in hospital all over xmas, they couldnt do a CT scan until his chest infection had cleared completely, so they could see exactly what was going on. He has had a persistent cough for a long time, and very severe breathlessness, very low iron - which contributed to the breathlessness.  He is home now on Oxygen 16 hours a day, they plan to ween him down off this gradually so see how he can cope, as he was on it due to the infection and low iron making his oxgyen lower than the fibrosis itself. 

He is 73 with diabetis, high blood pressure, moderate kidney disease, they have advised us he will be sent to Aintree, Liverpool, where they will decide if my Dad will eligible for the new drug Pirifidene (spelling), does anyone know what they use as a qualifer? I am concerned they will dis-qualify my Dad as he is 73 with other ailments.  He is in shock at the  moment as he is worried he will die suffering soon, and he doesnt want to suffocate, this makes me sad, as I know this feeling myself after nearly dying from heart failure through pregnancy, choking and gasping for air (my life was saved and I am fine now, my heart recovered) and I dont want my Dad to go through this ordeal.

They have told him in July his fibrosis was mild, and just recently when they confirmed diagnosis last week, they said it had gone worse (probably through the infection) but only slightly.   I just hope he will get this new drug - does anyone know how long it can extend life expectancy? I understand everyone is different.   I am getting Married in August, and I know he is worried about walking me down the Aisle (but it has also been his dream) and I know hes very nervous about doing a speech and I worrying this would make him even more breathless.  His breathing was so bad before Xmas, he was out of breathe just resting sitting down , and his cough was awful to watch.   Hes improved since his antibiotics and iron tablets,  I also worry about my Mum in all of this, she isnt well either, and they both live a hour away from me, I dont think they always understand what the doctor is telling the or what questions to ask.  I try to go with them when I can

I am going to go with my Parents when Dad goes to Aintree to see if they will fund this drug for my Dad, but if they dont, can we go elsewhere to get this drug?  Ive read and heard people pass away within months with this disease others live with it for years and years, can it stay mild for a good while then all of a sudden quickly progess?  Does anyone have experience with this new drug?

Thank you in advance for reading.

Yvonne

 

1 like, 7 replies

7 Replies

  • Qva
    Qva Vonnie21

    Posted

    Vonnie--I'm a newby to IPF too, and like you, have been asking questions. Seems that along with prayer, it's the best tool we have. Thanks to people on this site who helped me, I was able to put together a list of questions to give to my husband's doctor when we met with him today and, along with getting the doctor's complete attention, we got a lot of answers I think might not have come up othewise.  I wish I could help you, but the only advice I know now it to encourage you to keep asking questions and maybe consider writing them down and even presenting a copy of them to the doctor so they aren't overlooked. 
  • john15065
    john15065 Vonnie21

    Posted

    Hi Vonnie. Bad luck. BUT keep your chin up. I suffered the same as your Dad in regard to breathlessness, tiredness, cough but whilst I have a heart condition its probably not as bad as your Dads - so I feel my comments can be made on a similar platform.

    My diagnosis took over a year too. It's thought I've had it for about 4 years now - so many "tomorroes" have come :-).

    Firstly, help your Dad to be proactive. Whatever excercise he can take, with ambulatory Oxygen or whatever - do it - as often as he's able. Even sitting down or walking around the room at whateverv pace he can manage.

    I know this sounds difficult - but it's a difficult situation.

    As for him feeling nervous or frightened -    me too !! BUt he's just gone through a really bad patch and hopefully he'll feel a better sence of well-being as he rcovers

    I attendeded a Pulminary "Workou" which, although it wasn't very pleasant, it helped in two way - firstly it did make me stronger and secondly it had the additional benefit that I was able to met other people in the same or similar condition.

    Strong muscles require less oxygen and it's a comfort to share your experience with people who understand.

    His condition and well being WILL have been worsened by a lung infection. Even mild infections can have a dramatic effect on people with IPF. Hope fully antibiotics will aid that.

    Being frightened isn't a failure! I 'm not afraid of dying - I just don't want to be there when it happens!

    Whilst morbid - I'm told it's more likely that "you're going" will be caused by a side issue through not being able to transfer enough oxygen into the blood, it's the affect this has on the vital organs so - whilst suffocation is of course a natural feeling to be afraid of, try try try to be positive.

    Perferidone is a drug which is is not widely avaiable but, along with others, can be obtained through the NHS. To qualify, one must be "bad enough" and this is assessed by the tests which show the ability to pull air in and then distribute the oxygen through the lungs into the blood and thereon to the vital organs.

    I was put on a waiting list but, in the meantime, another drug was partially released and approved by N.I.C.E for limited release. It's called Nintedanib - I've been on it for just under a year.

    Neither of these drugs (and many new ones are becoming available) will cure the IPF. They may however slow down the progression which is exactly what Nintedanib appears to be doing for me so far ---- again I'm feeling positive that yet another "tomorrow" will come !!

    I obtained my medication through the consultant at my hospital who referred me to the Royal Brompton and Harefiled Hospital - even though the're not in my area.

    Perfenidone and Nintedanib are apparently hugely expensive which is one reason they're not widely available, secondly they are so relatively new, results are not available in sufficient feedback to enable NICE (National Institution for Clinical Excellence) to approve the drugs for General Distribution.

    Perfnidone and Nintedanib are not without side affects. As with all things, people react to different things in different ways.

    I met a man of my age (70) at Hospital who had been on Pepirfenidone for 6 months and was taken off it. He was about to start Nintedanib. I wonder how he's fairing.

    I don't suffer from noticebale side affects other than being prone to - horrible word - diahorea. Thsi can be controlled with medication too and it's not proving to be too bad at all.

    There are so many questions you'll seek answers too, as you say - write them down.

    I'm sure Aintree will have be able to help - don't just wait though - be proactive - you just have to be.

    I'll think of you from hereon and hope August will be a great day for you all. Good luck to Mum too.

    I'll follow your thread.

    Best wishes.

    John

     

    • Vonnie21
      Vonnie21 john15065

      Posted

      Dear John,  ( My Dad is also Called John )

      Thank you so much for your informative response, Dad is doing a little better now his infection has going and his iron levels are increasing, I know there is no cure but I just want hope that these new drugs will work, and that his life will be extended.  I am going to ring my Mum and Dad and read these responses out to them, and I hope it wil help my Dad feel better mentally.   I really do appreciate your response to my Thread, thank you so much and I hope you continue to do well on the drug 

      Yvonne

  • jazztrain
    jazztrain Vonnie21

    Posted

    Hi Vonnie21

    It seems as if your dad has quite a few medical problems without this?

    However I was diagnosed with IPF in 2012 having had a bad and persistent  cough for 12 months. Last year they put me on Pirfenidone and it has completely changed my life. But the proviso is - one has to get more than 50% of oxygen in the lungs or they won't put you on it! That's all I can say BUT they do this through measuring capacity via a Lung Function Test!

    Hope this answers your question

    Jazztrain

    • Vonnie21
      Vonnie21 jazztrain

      Posted

      Hi JazzTrain, 

      Thank your for your reply, much appreicated, 

      My Dad does have a few other health problems - so its not all simple, I guess we will know soon whe he goes to aintree if he will get this drug! , Thank you for your information, I am glad the medicine has changed your life, has your IPF stayed stable?  

      Regards

      Yvonne

    • jazztrain
      jazztrain Vonnie21

      Posted

      Hi Vonnie21

      When I was first measured for Pirfenidone my lung capacity was 56%; three months later it had increased to 60% and three months later again to 62%. I am no longer on Oxygen but keep it as no doubt one day I will need to use it.

      The only medication I take on bad days is Ventolin and because I have a sliding Hiatus Hernia (not that it troubles me) Lansoprazole 15mg daily.

      The one thing you have to remember about Pirfenidone is that it is a 'Drug Trial' so constant monitoring at hospitals is necessary.

      Kind regards

      Jazztrain

  • Vonnie21
    Vonnie21

    Posted

    Dear Everyone, 

    Thank you for all your replies and information - im so grateful, Dad is doing okay at the moment and just waiting for his appt at Aintree to see if he will qualify for the drug, fingers crossed.   I will keep you all updated.

    Yvonne smile

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