Pulmonary Fibrosis Online Q and A

A special thank you to Dr. Martin Kolb and Professor David Taylor for taking the time to share their expertise and provide answers to these important questions. Thank you as well to the many individuals from across the globe who took the time to ask a question. Pulmonary fibrosis can be an isolating disease and therefore CPFF is extremely grateful to Ward Health for providing the opportunity to engage with a global community of people affected by PF. It is events such as this online Q&A, programs such as our CPFF patient support program and other awareness activities that help bring people together and to let them know that they are not alone and that together we can drive awareness to this little known, yet fatal disease. Increased awareness leads to more funding for research that will one day lead to a cure.

Even a little exercise will help.  Try walking a little, even just a few houses down the street will help and you will soon be able to do more.  The Canadian  Pulmonary Fibrosis Foundation has a video that can help you exercise.  It was produced with the help of respirologists (pulmonolgists) and physiotherapist who are experts in the treatment of PF and is available free of charge on our web site at www.cpff.ca

It is correct that some studies report presence of Herpes virus (HPV and others) in autopsy tissue. However, these findings are difficult to validate and even normal lung tissue is often positive for viral particles. It requires special molecular techniques (called PCR or hybridization) to look for minimal amounts of particles in the samples. The scientific and clinical value of these reports is not yet strong enough to warrant anti-viral therapies. Of course, special situations may cause the treating physician to consider anti-virals.

Cough is one of the typical and sometimes first signs of IPF. However, not all patients get cough. It often starts as dry cough and, with more advanced disease, can also be productive of some phlegm. The treatment of cough can be quite challenging and different madications may be needed. Initially, over-the-counter cough medication should be tried.

There is some evidence that the incidence of the condition is increasing in countries like the US and the UK, even allowing for factors like population ageing. One reason could be better diagnosis. Another might be increased survival with other serious lung disorders. Pulmonary Fibrosis is classified as a rare disease in that the annual rate of new cases is in the order of 1 per 10,000 population. But with an average life expectancy from diagnosis of around four years this means that at any one time there are 100-150 thousand people living with pulmonary fibrosis in North America, and about 200 thousand in the European Union. In total there could be as many as two million with PF across the world, albeit that on average populations are younger in regions like Asia and South America.

Most people diagnosed with pulmonary fibrosis are aged 50 or above. But its origins can be linked to their earlier lives, and the consequences of PF can impose serious distress on family members of all ages.

While most patients are between the ages of 50 to 70 at diagnosis there a number of younger patients although theirs tends to be as a result of other factors such as scleraderma

Being enrolled in a clinical trial always offers benefits for patients with IPF. It is well know that even patients who are on placebo in the setting of a trial do much better than patients who are not in a trial. One of the reasons is the close follow-up and attachment to an expert center that is provided by the clinical trial unit. Obviously, drugs at the earlier stage of development may have unexpected side effects. One of the goals of a clinical trial is to learn more about new drugs, both about their good and also bad effects. If you are enrolled in a clinical trial, the study center will closely monitor your health and blood tests and you will have access to the center 24/7.

There is no doubt in my mind that the answer is yes, especially if their prices differentially reflect each community’s ability to pay. Seen rationally, what we cannot afford to do is to stop innovating. There will of course always be arguments about whether or not particular companies are making too much or too little profit, or whether or not University and hospital based researchers are giving good value for the public and private money they receive. But if we in the long term want humanity to survive, and in the shorter term to relieve the suffering caused to individuals by disorders like Pulmonary Fibrosis and all the many other currently untreatable diseases, we need continuing bio-scientific progress.

All the medicines ever discovered currently cost the world between 1 and 2 per cent of our gross annual income. This proportion has been roughly stable for decades, in large part because once new medicines become established products they can be made and distributed as low cost generics. Human labour, by contrast, is always relatively expensive. Few people say they want unchecked competition to drive down the wages of doctors, nurses and other health and social care workers to poverty levels. At the same time it is in patient and wider public interest terms right to try to ensure that enough is paid for new medicines like better treatments for IPF to encourage continuing high risk investment in ongoing innovation.

While crackles and velcro sounds are not entirely unique to PF they would indicate that there is probably a 90% to 95% chance that PF is present and an urgent referral to a respirologist is called for.  They do not appear in COPD or other lung diseases

Any well trained respirologist will be aware and can make a diagnosis of IPF. Of course, ILD clinics usually have a broader expertise in this field and have more experts who have seen many of the more than 100 different disorders that are listed under the term “ILD”. These special clinics are particularly helpful for patients who don’t have a typical presentation, for example who have a non-typical CT image or who are younger than we expect for IPF (anyone younger than 55-60). Many respirologists would seek a second opinion from an ILD center to advise them in the diagnosis and management of IPF.

NSIP (Non-specific interstitial pneumonia) describes a pattern of scar distribution on a CT scan or a pattern of microscopic changes on a lung biopsy. It can occur without any detectable cause and then is caused idiopathic NSIP. It can also occur secondary to causative agents (e.g. drugs etc) or connective tissue diseases (e.g. rheumatoid arthritis or scleroderma). Idiopathic NSIP is typically less progressive than IPF, but it can sometimes also behave in a similar way. Regular follow-up visits and breathing tests will help to monitor the disease and adjust the management plan. Tissue biopsy is not necessary, but can be helpful to distinguish from IPF. To date, there are no trials done at a larger scale for NSIP.

No. Their full potential is not yet known in either the early or later stage disease contexts, alone or in combinations with other agents. The best that can be presently expected is that they will slow the development of Pulmonary Fibrosis, and perhaps that of other fibrotic diseases. They are not without unwanted side effects, and should not be thought of as ‘magic bullets’. But is good news that advances are being made. If we continue to invest in treating rare disorders like PF progressively more effective curative and/or preventive interventions will become available. As we ‘unpack’ both the genetics of given illnesses and the biology of normal immune, inflammatory and other fundamental biological processes we will be increasingly able to treat distressing disorders of all types, rare and common alike.

Different types of Pulmonary Fibrosis may respond to different types of treatment. Some patients – resources permitting – might be suitable for lung transplantation and some respond to anti-inflammatory and immuno-suppressant therapies. But in general the options for people with Idiopathic Pulmonary Fibrosis have been disappointingly limited. That is why it is exciting that in the past year two new medicines have become available. Pirfenidone acts on fibroblasts, the cells that make connective collagen. Nintedanib also acts as an antifibrotic, via different inhibitory mechanisms. Other innovative therapeutic approaches are either in trial or in earlier stages of development.

There are treatments that can help with some of the symptoms of the disease.  These would include exercise, prednisone and perhaps NAC.  There are also  two treatments approved in many countries that slow down the progression of the disease.  One is esbriet (pirfenidone) manufactured and distributed by Hoffman La Roche and Ofev (nintedanib) manufactured by Boehringer Ingelheim.  Both these drugs are prescribed to patients with mild to moderate forms of IPF.  Ofev is not yet approved by Health Canada but that is expected this year.

Lung biopsies are nowadays only done if there are doubts about the correct diagnosis. No medical procedure is without risk, and the potential risk has to be less than the expected benefit of getting a tissue diagnosis. This is particularly important when other forms of lung fibrosis that could improve with steroids are considered. In general, the risk of a complication after biopsy increases with more advanced disease. The most dangerous complication is a flaring of fibrosis which would require hospital admission.

IPF is a relatively rare disease compared to other lung diseases (such as asthma, OPD or cancer) and awareness of IPF has therefore been low. Organizations like the CPFF together with expert physicians are working hard to change this and always need the help of affected patients and their families. With more awareness comes more research funding, which is instrumental in learning more about the complex biological process underlying IPF. Knowing the biology is key to developing new drugs that hopefully will stop the progression of IPF once and forever.

The processes that cause fibrotic diseases are related to the scarring that occurs when a wound heals. Such disorders involve the excessive and damaging build-up of connective tissue in organs like, for example, the heart and the liver. In the case of Pulmonary Fibrosis (literally, lung scarring) the disease starts in the walls of the alveoli, the tiny air sacs deep in the lung, which thicken and stiffen. It causes symptoms like chronic coughing and problems with breathing.

People with diagnosed Pulmonary Fibrosis have relatively short life expectancies. Some forms of the condition are associated with other lung disorders such as COPD (chronic bronchitis) and appear to be triggered by smoking or breathing in other known lung irritants. But the most common type, Idiopathic Pulmonary Fibrosis IPF), is of unknown cause, albeit biomedical advances are now throwing light on the fundamental mechanisms underlying its occurrence.

PF is a disease characterized by progressive and irreversible scarring of the lungs.  As the lungs become more scarred it becomes more difficult for patients to properly exchange gases (Oxygen/CO and CO2) and the patient suffers from shortage of breath and the oxygen saturation declines affecting other organs.