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What is the treatment and how will I be affected?
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. Perfenidon 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.
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