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Site Admin Joined: 21 Dec 2002 Posts: 214 Location: WA

(As published on the original Huff n Puff website, written by Mike, the original site administrator.)

What are the other names for IPF?

Idiopathic Pulmonary Fibrosis, Farmers lung, Interstitial Pneumonitis, Stiff Lung Disease, Honeycomb Lung, Hammond-Rich, Cryptogenic Fibrosing Alveolitis, and many many others

Background

I do not present the following as unquestionable data. In fact from what I have been able to ascertain, there is no unquestionable information other than this is a serious terminal illness. If you have been recently diagnosed with any of the interstitial lung diseases you will find very little in the way of explanations for causes and treatment. However, there are some! I will provide you with as many links as possible to what information there is and let you decide as to whether or not you wish to become more knowledgeable of it.

The information supplied here comes in majority from the internet via research centres, forums and home pages like this one. Some of what is here is verbatim while the rest is my interpretation of what I have either read or been told. I caution you to apply the same degree of accuracy to not only this but all pages on the internet dealing with any topic. Do not accept what is written as being above reproach and undeniably correct and accurate. You can, I believe, put more stock in what is submitted by professional establishments such as hospitals etc. than you can from any personal home page.

I do not believe that anyone would intentionally mislead, especially not with respect to the type of content contained at this site. However, misinterpretation and misunderstanding can often promote inaccuracies either in what is written or what is perceived to have been written. It is very difficult, when one is convinced that his or her interpretation is the correct one, to put forth a collection of ideas and opinions in such a manner as not to lean heavily in favour of the authors views on the subject. Hopefully this and other sites will serve to enable us to unite in a concerted effort to do what we can to defeat this deadly enemy through an exchange of information and better understanding, or at the very least smooth our path as we wander aimlessly along the IPF highway.

What is CLD?

CLD stands for Chronic Lung Disease. Chronic simply means something that lasts a long time, constantly returns or is never ending

What is ILD?

ILD stands for Interstitial Lung Disease. This is any disease that attacks the interstitium or lining of the lung and generally affecting the exchange of gases to and from the blood supply by impairing or destroying the alveoli. These are the tiny sacs that transfer gases.

What is Interstitial Pulmonary Fibrosis?

Interstitial Pulmonary Fibrosis sometimes referred to as Idiopathic Pulmonary Fibrosis, is not a disease but rather the result of a disease. It is a term used to indicate that the interstitium of the lung has suffered irreparable damage in the form of fibrosing or hardening of the tissue. This hardening or fibrosis is often described as honeycombing as it resembles that type of pattern when viewed through a microscope.

What is interstitial pneumonitus? Interstitial pneumonitus is one of over 130 different types of Interstitial Lung Disease (ILD). A disease which by majority strikes the middle aged (40-60 yrs old) male and female equally. It is most often described as idiopathic; however the "I" in ILD or IPF may indicate either idiopathic or interstitial. Idiopathic means unknown, Interstitial is a lining around the lung which in this disease becomes inflamed and without treatment leads to Fibrosing.

The most common symptoms are shortness of breath when exercising and a non-productive cough. Some experience fever, weight loss, fatigue, muscle and joint pain, as well as abnormal chest noises depending on the cause of their particular infection. During the later stages of the progression of the disease clubbing or rounding of the fingers and toes is common with still more abnormal noises. Still later the stress imposed on the other body organs often result in failure or diminutive efficiency of such things as the heart. Make no mistake about it, this is a very serious disease. It is a terminal disease with no known cure. Life expectancy from time of diagnosis is usually accepted by the medical community as an average of 2 to 10 years depending on which type of ILD you have. The disease is also unpredictable. Quite often it will appear stable for as many as 2 years or more. It may progress rapidly, then without explanation slow or cease to progress. You are sitting on a bomb whose timing mechanism is faulty and unpredictable. The only thing that is a surety is that it will eventually go off.

How Does This happen?

ILD is a condition where as the interstitium initially becomes inflamed. This is the part of the lung which includes a portion of the connective tissue of the blood vessels and air sacs that make up the membrane where gas exchange takes place. This inflammation then invades the air sacs (alveoli) resulting in alveolitis. The alveoli are very much akin to little bellows. They fill with air on inhale and then expel the oxygen into the blood stream by contracting. Then they absorb Carbon Monoxide from the blood when they inflate. This is then carried out of the body by exhaling. A rather simplified explanation but one I have come to view as sufficient at least for my needs.

If the inflammation is not brought under control it results in scarring or fibrosis, a stiffening of the lung tissue. This, when it occurs, is a permanent condition. Often it is likened to a honeycomb appearance and so is also referred to as honeycomb or honey lung. You do not have to have injured the lung or caused a traumatic situation to occur in order to contract ILD. It may have been nothing more serious than inhaling when you should have exhaled or perhaps just plain held your breath. Maybe you should not have helped your neighbour with the haying last year. Who knows?

How Will I Know if I Have it?

Few of you will have much interest in this. I mean you are here right? You most likely already know you have it! However, on the off chance some of you are just surfing and misspelled a word to find yourself here this is for you! I won't bother with the obvious things such as short of breath cyanosis etc etc. I will relate to you the symptoms I had before I was diagnosed that should have tipped me off. It's the old "If I knew then what I know now!" thing.

There are lots of "look for this and look for that's" out there. Most of them are pretty much common sense and if anyone noticed that happening few if any would NOT go to a doctor. What I will tell you is how I knew. No I didn't know at first and in fact didn't know until I was diagnosed that there was even anything seriously wrong with me. Once I was diagnosed however, thinking back I SHOULD have known a long time before I did! Even so I was as lucky as the Turkey who got through Thanksgiving! They still managed to find my disease very early in it's development. What really ticks me is that I ignored or covered up the tell tail signs for a long time before that. We fools seem to be right up there with the suckers - "There's one born every minute!" What I want to tell you is how you can load the dice in your favour for a change. There is some soul searching required and some honest self evaluation but coupled with what is on the net for symptoms you could have a jump on it. I sure as H - - - hope so anyway!

The way I see it if you can read, and I assume since you are here you can do that, then if you are gasping for air, turning blue, your fingers and toes are clubbing and your chest sounds like a babies rattle you are more than likely to have seen a doctor long ago and this isn't going to do you a load of good! So here goes. These diseases are not the type that present themselves in a flamboyant and obvious manner. They generally can be quite devious. You may get a little cough that is nothing more than annoying. Next you find that perhaps on occasion taking a shower get you a little bit out of puff, or maybe changing that tire or even your daily walk seems to suck the life right out of you. At least more than it has in the past.

A lot of people notice this happening around the time they are middle aged. If a child gets winded or wears down easily it is noticeable, not so with older people. Generally most will pass it off as the aging process. Hey I'm headed for 50 what do you expect? Sadly it doesn't become an issue until at 60 or 65 they realize they can't keep up with those who are 80 and older. Now there is something not quite right! By then it is also all too late. If you want to know how late check the transplant lists and see how many over 60 are on it!

So if you find that you just don't seem to be able to keep up with those your own age give or take ten years; If you feel completely worn out, even if only occasionally, when performing some menial task that shouldn't really slow you down at all; or you find that the temperature bothers you more than it used to, then perhaps it is time for you to take an assessment of your state of health.

How do I go about assessing my health?

You should do this as only you can really know what has been going on in your own body with respect to it's ability to perform.

First be honest with yourself. Maybe you are a smoker. Is that cough really JUST a smokers cough? When did you first notice it? Do you ever bring up anything or is it simply a dry hack? Maybe it is just that - a smokers cough - but maybe it isn't? Are you active physically? Maybe you were until recently and then gave it up. Why did you quit? Was it really just too much of a bother or did you find that subconsciously you were telling yourself - Hey this is too much I can't do it any more! Last year or last month you had no problem, now you can't do it anymore? Was this something that's been coming on for a while or is it a surprise even to you?

Maybe you loved to camp in the mountains, fish, walk the trails photograph the wild life. Now you don't even want to think about it. Why? Just tired of doing it or deep down do you know you can't? Not without it being a chore and a completely uncomfortable experience. Do you find yourself telling others that you can't play ball or do whatever because you have something else that just HAS to get done this weekend. Then that something else turns out to be the couch potato Olympics?

Do you get a cold and just never seem to be able to get rid of it? That sucker just hangs in there forever? You can't stand the heat in the summer and you hate the cold in the winter when you use to love both?

So if all of or even some of these are you, do you have IPF? Of course not. All this tells you is that things have changed in your life.

What matters is how quickly, in what way and how did these changes take place. Most if not all of these things happen to everyone at some point. But if you are honest with yourself you will know if they are really just part of the process of aging or perhaps they should be given a closer look. Some people really are old at 50 you know! But are you?

IPF is the result of a lung disease. Most people just assume that the only impairment will be a breathing problem. This is not true.

The body is made up of greedy parts and pieces and just so much oxygen rich blood is provided by the lungs. It follows a very definite course through the body. Seldom if ever are the routes of travel changed.

Each part will extract what it needs. Not a percentage of what is available but 100% of what it needs. If the total is less than 100% of what the sum of the whole requires then something has to suffer somewhere.

So the last on the delivery line starts to suffer undue stress and insufficient fuel to operate which will lead to diminished capacity and eventually earlier than expected failure. So do not expect to only see a decrease in your ability to breathe.

By doing less, you can cover this up for quite some time, and avoid that uncomfortable feeling of being out of breath. Even for years! You can fool yourself about getting older with the natural slow down theory for some time.

However, if you find that maybe you go to extra effort to avoid certain tasks that were not a problem in the past you might be well advised to take a good look at why this is so. Maybe you over stock the fridge and cupboards to avoid a few extra trips to the basement larder and freezer? So much so that things are falling out onto the counter and you have difficulty keeping the refrigerator door closed?

You think all this is ridiculous? I'll give you ridiculous.

A fellow has been getting his hair cut by a lady who lives 5 houses up the hill from where he lives. She has been cutting his hair for 20 years! All of a sudden she doesn't cut his hair right any more and he now has to go 15 miles down the road to the hair dressers. At first glance nothing unusual right? Well think about this! 5 doors UP THE HILL. Would you drive the car?? Not likely! Now that would give the neighbours something to talk about wouldn't it?

So what do you do? Become a hold over from the 60's? Nope - you get a hair dresser far enough away that you have to drive there! Problem solved!

Tell me that isn't ridiculous!

If you find some, or many of these, are familiar then run (that is if you can) don't walk to your doctor and have it all checked out.

Make sure you explain everything to him/her so they will know what area should be investigated.

A quick once over in the back office is NOT going to find IPF even if it's there big time! So it is very important that your doctor understand the severity of change you have either made happen yourself of has just come about a little at a time but far too quickly and far too early in life.

Doctors are not mind readers or physics! They can only react to the information that is supplied to them. If you don't sound the warning horn, they can't manoeuvre you to avoid a collision! Result? Road Kill !

Is IPF contagious? Will my offspring get it?

NO! and not likely. But then the results of that investigation are not entirely over as yet.

The odds are not great that any individual will contract this disease. Most physicians will categorize this as a very rare disease. In fact it is generally accepted that only 10% of the population is a candidate for infection. However, if you are one of the 10% the odds won't really mean a lot will they?

There are several theories as to the source of such infliction. Recently scientists have discovered the gene thought responsible for an individual being a candidate for contracting ILD.

The first one that comes to mind of course is "Smoking". ILD is very similar in a lot of ways to emphysema, which except in exceptionally rare cases (less than 1%) is solely and entirely caused by smoking. Even in those (Alpha1) types of emphysema many physicians dispute the theory that you can get emphysema without smoking. They maintain that if you are Alpha 1 type you will definitely get emphysema if you smoke unlike most other people who will not necessarily get emphysema if they smoke.

Now there's a debate that will go on for years.

Overall there are two types of lung disease categories. Restrictive meaning something has happened to restrict the efficiency of the lung operations. The other is obstructive, which is something has happened to obstruct the operation of the lung. Though they initially seem to be identical they are in fact very different. Emphysema is an obstructive lung disease while ILD is a restrictive lung disease.

The explanations I have been able to obtain for these categories have a somewhat grey area to them. Restrictive was explained as likened to a shrinking of the air ways there by restricting the volume of air which could be utilized by the interstitium. Obstructive was described as likened to having a blockage of sorts. Though the lung and airways were capable of utilizing more air the air was blocked by an obstruction which prevented its passing either to or through the lung properly.

The main difference I have been led to believe is that obstructive reduces the elasticity of the lung tissue allowing it to expand but causing it not to have the necessary property to return to its previous state. Thus, preventing the patient from fully expelling the air they have inhaled.

I envision it something like the elastic waste band of a pair of pants that has been stretched too far once too often. Still has some give to it but not enough return to hold up the pants.

Restrictive lung disease on the other hand results in the lung tissue becoming hardened or stiff thereby eventually preventing the patient from inhaling sufficient volumes of air necessary to sustain life. In my mind I see this as akin to attempting to blow up a tire tube by mouth!

Personally I have difficulty understanding the difference between the two in all cases. However those who are experts in this field have done so.

Therefore smoking, as such, is neither a cause nor cure in relation to ILD. One should not take this as condoning the use of cigarettes or smoking.

In emphysema the alveoli break down until what was once millions of small air sacs is now one large air sac but without the ability to transfer gases to and from the blood.

Large in this instance being smaller than the pointy end of a needle.

In ILD the alveoli are damaged in such a way that they become hard inflexible and therefore unable to transfer the gases to and from the blood.

If a healthy person were to remove all his or her alveoli and open them up flat. Then place them side by side they would cover the area of a football field. A person with ILD or Emphysema perhaps would cover a basketball court.

What causes CLD?

One of the primary causes of ILD is thought to be air-borne fungi particles. These may have been ejected into the air from such sources as hay, air conditioning vents put into use after the winter shut down without being properly cleaned and disinfected and bird droppings.

Other possible causes are inorganic dust (such as silica and hard metal dusts), organic dust like bacteria, animal proteins. Also gases, fumes, drugs, chemicals and other poisons.

Possible drugs and poisons are chemotherapeutic agents, antibiotics (rare) and radiation. Residue left by certain infections may also play a role in this disease. Two possibilities are Systemic Lupus Erythematosus and Rheumatoid Arthritis.

In making a diagnosis your pulmonary specialist will or should consider a careful and thorough medical history including environmental and occupational exposures to dusts, gases, chemicals, pets, air conditioners, and humidifiers. You probably will have a complete physical examination. A chest x-ray. X-ray results are usually abnormal but in as many as 10% of cases, may be normal.

Pulmonary Function Tests or PFT's. Results are nearly always abnormal for PFT's. The early tests will most likely be conducted before and after an inhaled bronchodilator treatment. You may or may not have an exercise test which includes an Arterial blood gas test. Usually an artery in the wrist is utilized and measurements may be normal or show decreased oxygen.

Often elusive, this disease can demand a further battery of tests. Like anything else the best proof is something you can see. To that end your doctor may perform or have performed a bronchoalveolar lavage. This is where a very thin bronchoscope is passed through the nose into the windpipe to get a good look at the airways. This instrument also has a device attached to the end which enables small tissue samples to be collected for scrutiny in a lab. If good samples are obtained positive disease identification may be arrived at. However, in a good many cases it is sometimes necessary to do open lung biopsy so that better samples can be obtained.

Though not a high risk operation by today standards, this procedure does require hospital stay and can have some discomfort associated with it. An accurate diagnosis is critical to treatment for each individual. ILD's progression can vary from person to person, and people respond differently to therapy. Therefore it is very important to have the specific diagnosis made along with the staging of the disease. Staging indicates how much of the affected tissue is inflamed and how much is scarred. Some other ILD disorders are hypersensitivity pneumonitis, eonsinophilic granuloma, sarcoidosis, chronic eosinophilic pneumonia, Wegener's granulomatosis, idiopathic pulmonary hemosiderosis, and bronchiolitis obliterans.

What are the treatments for IPF?

One thing is very important to understand. Therapy either drug or otherwise cannot and will not reverse the damage done by scarring. This is why it is extremely important to diagnose and treat the disease as early as possible. If an occupational or environmental exposure has been identified or is strongly suspected, removal from or of the problem is essential. This may mean giving up your job, hobby, or changing where you live. Patients who respond well to therapy generally report fewer symptoms, show chest x-ray improvement, decreased shortness of breath with exercise and stable breathing tests. In making your decisions with regards to these matters remember you are dealing with a terminal illness. Only you can decide.

Undoubtedly you, like the rest of us, will find more to contend with than just an illness. You should be aware of all the possibilities and probabilities that will surface in dealing with the overall affair. The disease itself and the drug treatment may not be your worst experience in dealing with IPF. IE: Get a full and complete copy of any insurance policies which cover your medical or disability and study it until you can repeat it verbatim! Do not let anyone tell you it does not exist and do not let them tell you that you are not entitled to it. Do not read into it something that is not there and do not take anything for granted! If you think you are covered most likely you are but you can also be rest assured they are not going to follow through on that coverage without a fight. Don't be surprised if they even try to get out of the contract by claiming you got this disease deliberately and therefore committed suicide! You are dealing with people (and I use the term loosely) whose collective IQ does not appear to be more than that of your average garden variety cabbage and whose morals, integrity and scruples are even less. Add to this the probability that they are employing a business practice that picked up where Alphonse Capone's protection scheme left off and you may appreciate what you will be up against! You see TV shows which depict how less than honourable people are defrauding the insurance industry on every claim from a sore toe to mass murder. If you have ILD or any other disabling disease you are about to find out where those people got their training!

What are the medications used? Not everyone reacts to treatment in the same manner. This is one topic that swings from the low to high end of a scale from patient to patient. It can be from nothing to it, all the way to where it seems worse than the disease itself. Some of those you come into contact with in different professions will tell you "you're crazy to take such medication". However, this is easy to say when you're not the one with a terminal illness. The most basic instinct of all living creatures is survival. It's more deeply rooted than reproduction, love, or any other instinct. Since this is a requirement for the fulfillment of all other instincts I suppose it is only natural that it enjoy the highest level of the pecking order. With that said I shall venture into the realm of medications used in the treatment of ILD.

Surprisingly there are generally only three main drugs of choice. Prednisone a corticosteroid, cytoxan a cyclophosfamide and azathioprine commonly known as imuran. Quite often the latter two are used in conjunction with the first.

PREDNISONE seems to be the primary drug used to aggressively fight this disorder. It is a seemingly innocent looking little white powdery like pill about the size of the "O" on your keyboard. Not all that much to look at really. This size is 5mgs. It also comes in 50mgs tablets. Dosage varies but generally is from 20 to 80 mgs per day. For this reason the smaller tablets are more common to ILD patients. The adrenal gland produces cortisol naturally in the body. When on a corticosteroid the body ceases to produce. With long term steroid treatment it is imperative that gradual withdrawal be under taken with the strict supervision of a physician. DO NOT attempt to do this yourself. Withdrawal periods can exceed a year.

Should that be all there is to it then it wouldn't be so bad right. Well that's not quite all. Even aspirin can have side effects. Here is a list of just some of the common side effects of long term corticosteroid use. Not all people experience all of them. But from my personal experience and those whom I've been in contact with a large percentage seem to suffer from most of them. The most obvious is the swelling or bloating. The visual distortion is most evident in the face and abdomen. Visualize an 11 month pregnant Pillsbury dough-boy. This can occur rather quickly. When a person of average weight for their build suddenly expands and increases their body weight by as much as 25 to 30 % in a short period of time the discomfort cannot be described in words. None of your clothes fit, you can't see your feet, even your glasses if you wear them dig into your face. If you try to bend down the oversized stomach forces what little air you have in your lungs out and you must stand immediately. Just tying your shoes becomes a major chore. Putting on your socks in the morning becomes a rendition of the courting dance of some exotic wetland bird.

Other less immediately noticed side effects are such things as mood swings. You can go from passive devil may care to patients my butt I'm going to kill something in a blink of an eye. This is most often referred to as "roid rage". Then there's high blood pressure, salt and fluid retention, tendency to bruise easily, depression psychosis, or hyperexcitability, tendency to develop diabetes, peptic ulcers, infections, cataracts, glaucoma, osteoporosis and insomnia. You will find that the summer heat is unbearable causing you to sweat profusely and require a change of clothes every two to three hours. One point I should make here is that if you are traveling or out of your area of normal care you should take precautions to identify yourself, in case of accident, as a long term corticosteroid user. This will be very important to paramedics or hospital staff in charge of your care should something happen. Better safe than sorry.

CYTOXAN may be used by itself in cases where corticosteroids are not possible for whatever reason. It is my understanding that this drug is not as effective as the steroids but has lesser side effects. It is also slower to produce results and requires most generally 6 months or longer to be effective. The side effects of this medication include gastrointestinal irritation, bladder inflammation, bone marrow suppression, infection, irregular menstruation, and blood disorders. Tests indicate that if used together with prednisone better results may be realized.

IMURAN is a drug widely used in transplant patients. I look at this as the last line of defence. Perhaps this is not really the case but for me it is the last to be used and only because I cannot safely use the cytoxan any longer. The action of imuran is similar to cytoxan but early studies have shown that it is not as effective. The side effects are generally more manageable and include fever, skin rash, gastrointestinal irritation and blood disorders. The cost is also considerably higher for this drug than the others. If you do not have a drug plan you should be aware of this.

There are other treatments being used and still others are being evaluated. Perfenidone and Interferon Gamma 1B are but two. There are also other drugs used at times such as colchicine but by majority the three above are the main weapons of choice in the battle against IPF. Just remember your pulmonologist is your most effective weapon. If you do not ask questions they seldom offer information, but when asked they should respond in kind and in such a way you are able to understand what it is they are saying. This is not a competition between specialists and it is not something that you should drop in the lap of a doctor with the attitude that they will take care of it while you go on your merry way. This requires a team effort in order to achieve maximum results.

After all this you are probably wondering why anyone would even take such drugs? Well perhaps you may start to appreciate the seriousness of this disease when you realize that the benefits of this therapy usually outweigh the risk of the medications side effects. Other treatments include oxygen therapy. This to my knowledge does nothing to attack the disease but is solely used as a method of increasing the gas exchange rate within the lungs thereby lessening the effects of the damage done by scarring or in reality giving the remaining tissue more oxygen to work with. In short it just makes you feel better. Recently there seems to be more and more use being made of the lung transplant programs. As with all transplants you must meet certain criteria to qualify for a transplant. Should you want to investigate this option contact your nearest transplant centre and/or discuss it with your pulmonary specialist. It is also important to note that not all side effects disappear when and if the drug therapy is stopped. Some side effects result in permanent damage to joints, liver, kidneys, heart etc. This does not mean you will have these problems only that they are a possibility.

Joined: 15 Mar 2007 Posts: 166 Location: Alberta, Canada

Greg, this is the best information that I have ever read in layman's terms!
Thank you so much for taking the time to do this.
Much of what I have read on the disease might as well be written in Greek! Your writing is clear, concise and easily absorbed...a must read for all those newly diagnosed.
Thank you again.
Granny

Posted: Tue Sep 01, 2009 10:13 am

i'm fairly new to this site. THANK YOU 4 this info. u put it in language that i can understand.

Joined: 06 Oct 2008 Posts: 206 Location: Middle of Texas

Greg,
Thank you!
I just re-read this information on all the different names that we have heard so many times in the past few years. Medical terms that we'd never heard of before and then they sounded like some foreign word to us until we(or myself) found HnP. The little info I got while I was hospitalized for the first time with IPF was a printout from WebMD, which was very informative, but scared me half to death.(I couldn't have something like this. They have made a mistake!) The doctors didn't know for sure either so why not think they'd made the wrong diagnoses. I kept that information by my bedside after going home looking up different terms on the internet. I was in the hospital over 6 weeks so I should have known it was something serious. Then rehab hospital for several more weeks.
I really didn't believe that I had this IPF. I had to use oxygen 24/7, and then I wouldnt use it until I couldn't go. I would almost pass out from lack of oxygen and did not know that I was doing more damage to my body by depriving it of the needed oxygen.Today, after 5 years I still get mad because I am tied to this machine, altho I thank God everyday that I have it to use.
Now, to the newly diagnosed HnP's....you see why we always say read all the older post. There is so much to learn about this disease, even after 5 years of reading, it is still new to me.I know I read it sometimes in the beginning, but my memory isn't too good now and I forget things really fast. So read the older post to renew your memory.
Sue

Posted: Sun May 09, 2010 9:25 am

Just reread this and found a cause for IPF that I didn't notice before. Holding your breath. As kids all of us at the swiming hole would dive off the rock and see who could go the farthest underwater. Guess who always won? Now, they are all still swimming and I am sitting on the couch with my feet in a dishpan of water. Just a little exageration there. We still go to the beach and hubby puts my chair at the edge of the water. Point being, these old poats are a wealth of information.