Should I be concerned?

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Grovee01
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by Grovee01 on Tue Apr 14, 2015 5:01 pm

Should I be concerned?

Hello, my mother died in 2001 of Cryptogenic Fibrosing Alveolitis just 9 months after diagnosis, aged 58. My brother has recently passed away (feb 15) aged 45. I had not seen him for a few years (last time he was in reasonable health with no signs of lung disease). He died of aortic and mitral stenosis but the coroner also added 'chronic lung disease' to the death certificate. I queried this with his doctor who has confirmed a diagnosis of Interstitial Lung Disease, although the exact one he is not sure. I am now trying to get information from his respiratory consultant and a copy of the postmortem report. Am I completely over reacting here? I am concerned that two immediate members of my family could have had IPF and just feel I need to know. I appreciate that there are other forms of ILS, but does seem a co-incidence but trying not to jump to conclusions. I have two sons of my own. Thank you.

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Dr Helen Webberley
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by Dr Helen Webberley on Wed Apr 15, 2015 8:35 am

Re: Should I be concerned?

Hello, it is very natural to be concerned, lung disease is a scary thing.

There may be a very small hereditary component to idiopathic pulmonary fibrosis but we are still not very clear about it, and by far the majority of cases are not genetic as far as we know.

Lung disease is often cited on PM results, because the lungs are often the thing that lets us down in the end, and causes the final demise, but it sounds like your brother had heart problems rather than lung problems really doesn't it?

For yours and your sons own lung health, it is very important that you don't smoke, and that you keep a healthy lifestyle. Have you had your blood presure and cholesterol checked?

It is always a worry, but I don't think you have a lot to worry about here. I hope this helps, Dr Webberley
Dr Helen Webberley
NHS GP and Director of www.MyWebDoctor.co.uk
MBChB MRCGP MFSRH DipGUM DipIPM
http://www.talkhealthpartnership.com/on ... berley.php

Grovee01
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by Grovee01 on Wed Apr 15, 2015 9:11 am

Re: Should I be concerned?

Hello, yes, it does help, thank you.

I am fit and healthy as far as I know, and yes I have had those checks.

My brother did have heart problems but I know enough about IPF to know that he would have died fairly soon anyway, my mum died after just 9 months.

I will talk to my children about not smoking, one of my children has an acid reflux problem which is a concern to me, I will talk to him about managing that too. It is a shame more money is not put into research for this awful disease. I researched it back in 2000 when my mother was diagnosed and I am amazed about how much information is 'out there' now 14 years later. It also surprises me the statistics around familial statistics but I appreciate an external trigger is required.

On a positive note, this has made me want to raise awareness wherever I can.

Thanks again.

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Vicky Barber
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by Vicky Barber on Wed Apr 15, 2015 9:30 am

Re: Should I be concerned?

Good morning,

I completely agree with all Dr Webberley's points.

I would suggest though that most specialist Interstitial Lung disease nurses, would recommend full lung function testing with gas transfers (not spirometry done at your GP's surgery), as a precaution if you have two or more family members with this particular type of lung condition.

Clearly this would need to be arranged via your GP.

Idiopathic pulmonary Fibrosis is one of the British Lung Foundations primary focus areas for research.

If you would like to speak to a nurse about the research for this disease area, you can contact us on

03000 030 555
Vicky Barber
Nurse Manager- British Lung Foundation
http://www.talkhealthpartnership.com/on ... barber.php

Grovee01
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by Grovee01 on Wed Apr 15, 2015 9:38 am

Re: Should I be concerned?

Thank you so much Vicky. I will wait to see what the post-mortem tells me. At the moment I have a letter from his G.P confirming Interstitial Lung Disease. I would be more than happy to take part in any research. More needs to be done and I am happy to do whatever I can, even if just raising awareness. My mother having it was awful in itself.

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Vicky Barber
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by Vicky Barber on Wed Apr 15, 2015 9:59 am

Re: Should I be concerned?

Hi,

Please feel free to give the British Lung Foundation a call anytime on 03000 030 555, if you want to have a chat, or need anything explaining.

Best wishes

Vicky
Vicky Barber
Nurse Manager- British Lung Foundation
http://www.talkhealthpartnership.com/on ... barber.php

malcolmw
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by malcolmw on Wed Apr 15, 2015 4:55 pm

Re: Should I be concerned?

Not all people with IPF go downhill as fast as your mother. I was diagnosed in 2006 and I am sure that I had the condition for many years before that. There are new drugs coming available which are designed to slow down the progression of the illness. You are right to be concerned and you should ask your GP to refer you to a specialist centre for ILD.

Grovee01
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by Grovee01 on Wed Apr 15, 2015 6:16 pm

Re: Should I be concerned?

Thank you.

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Dr Joanna Porter
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by Dr Joanna Porter on Thu Apr 16, 2015 10:37 am

Re: Should I be concerned?

Dear Grovee01

I was so sorry to hear that your mother died so quickly and so young from what sounds like Idiopathic Pulmonary Fibrosis (CFA) and of your brother's death at an even younger age. I think that it is very sensible to try and get the information from his respiratory consultant and, if possible, the post mortem diagnosis. It may even be helpful to have the lung histology reviewed by an ILD specialist centre.

I don't think that you are over-reacting at all. It is becoming increasingly apparent that having a first degree relative with IPF is a risk factor for developing the disease. It is also apparent that more cases of IPF are actually familial (2 or more first degree affected relatives) than previously realised, and that many relatives of patients diagnosed with famililal PF, thought to be disease-free, do have underlying respiratory abnormalities.

Unfortunately, it is really unclear what individuals in your situation should do. Obviously, not smoke and keep healthy, but further specifics are less clear. I am of the opinion that early diagnosis of PF is beneficial, although this has never been proven. Screening methods have not yet been employed as no treatments have YET been shown to be effective in PF with normal lung function. But, I suspect that this will change as new drug treatments reach the market.

We, at Breathing Matters/UCLH, are planning a large scale study of asymptomatic first degree relatives of patients with IPF to see if screening will alter mortality many years down the line. At the moment, though I am not aware of such studies.

If I was in your situation, I would ask my GP to refer me to a local specialist ILD centre for a CXR and lung function testing. I would hope that the centre would agree to this and to repeat your lung function at 12 months (or sooner if you developed chest symptoms) and continue to monitor you annually. If at any stage you had a decline in your lung function (>8% fall in FVC or >10% fall in TLco), or new symptoms, I would repeat lung function at a shorter interval (3 months for example) and if the fall was maintained or increased, then I would arrange a high resolution CT scan and take it from there. I am sure that your GP can arrange this and you are very welcome to be referred to UCLH if you are near London.

Another important aspect is the genetics. It would be very useful, but may not be possible, to have genetic samples from your mother, your brother, yourself and any of your unaffected siblings if you have any. It is becoming cheaper and easier to sequence entire genomes. There are many different genes that can cause familial PF and your own family may have a unique genetic mutation that has caused this. By having samples from various family members, we can work out which gene may be involved which makes it much easier to screen other family members.

I do hope that this is helpful. I do not wish to alarm you, but I think it is important to watch you closely so that early intervention can be initiated.

Very best wishes, Jo Porter
Dr Joanna Porter
Medical Director and Clinical Lead of Lung Fibrosis
http://www.talkhealthpartnership.com/on ... porter.php

Grovee01
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Joined: Tue Apr 14, 2015 6:38 am
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by Grovee01 on Thu Apr 16, 2015 1:14 pm

Re: Should I be concerned?

Thank you so much, that is really helpful.

The coroners office is chasing the post-mortem report for me but it seems to be taking some time as does a response from the consultant (I know they are all busy people). So, I am making the most of this forum but keeping in mind that I do not have all of the information just yet.

My brothers doctor has confirmed by letter that he was taking Oxygen Therapy for a presumed diagnosis of Insterstitial Lung Disease since May 2013. He had undergone a CT scan which confirmed a restrictive pattern. However, there were questions as he had a scoliotic spine and hiatus hernia. After discussion at a Multi Disciplinary Team Meeting they felt another CT scan was required with good inspiratory scan. I am not sure if this was ever done as I had not been in contact with my brother for about 4 years. My brother had severe Aspergers and other underlying health problems (although was in stable health when I last saw him). He would not have been able to give his consultant his family history as he would not have known, I suspect he would have advised that my mother died from pneumonia, which of course she did due to CFA. She underwent steroid and immune-suppressant treatment at the time for her disease.

My brother did die from heart disease but his GP concludes his letter by confirming underlying lung disease along the lines of interstitial lung disease.

So, my question is - does interstitial lung disease mean fibrosis whatever the particular disease?

My brother had treatment for scoliosis as a teenager but I had never witnessed any other problems in the years that I cared for him following the death of my parents. (father died from COPD just before my mother). My brother and I have never smoked.

Interestingly my mother worked with metal dust and my brother kept budgies. We always had budgies as pets as children. I think that both are thought triggers for fibrosis. I cared for my brother for 9 years, all of the time he had these budgies and I never witnessed an allergic reaction.

Sorry, I know there is a lot here, just trying to get as much information as I can before this forum closes.

What does interstitial disease mean - a form of fibrosis. Even if my brother did not have IPF, would I still need the same checks?

Thank you again.

Elaine.

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