Hello,
I am a 25 year old male with pulmonary fiborsis. I have approx. 3-5 years left to live. I am handling it ok and haven't needed anti-depressants yet. I have been a white rights activist for some time and the pain I've endured watching my race die out outweighs this illness, I think I'm conditioned enough to deal with it calmly and realise we all die and it is what we did in life that counts. My family isn't handling it so well. I have 2 questions:
1. How can I convince my family that I am happy with my life and this isn't the end of the world. They seem to worry 24/7 that I'm miserable, yet I only worry about their sadness, not me dying, how can I convince them I honestly feel that way?
2. Is all lung scarring considered to be pulmonary fibrosis? In other words, in pulmonary fibrosis once the lung is scarred the scars grow until you can no longer process oxygen. Is there such a thing as a permnanent lung scar which is NOT pulmonary fibrosis (doesn't grow)?
Thank you Cyril, I am sorry to hear you have pulmonary fibrosis. I have it as well. I was diagnosed in 2003 and I am now 54 years old. It is unusual for someone your age to get it. Unfortunately, not much is known about the mechanism of the disease, but recent studies indicate the disease may be genetic in nature. The other responders have given you chapter and verse on the disease, so maybe I can address your questions from a sufferer's point of view.
First your family issues - it sounds like you have gotten to the acceptance phase of things in fairly good shape (mentally). It will probably take your family a lot longer to accept your fate. I have had this over 4 years and many of my family and friends are convinced I am going to beat this thing. I know I am not, but they seem to need to believe that, so I let them. I have a wife and we have been madly in love for 31 years. I worry about her sadness as well. She has finally accepted my fate, but I still see the sadness in her eyes. I don't think anything can be done to erase it. She will be sad until the day I die for the suffering I go through, and she will be sad after I die for more personal reasons. The best thing you can do is simply continue to live your life like each day is a gift. After a while, they will stop thinking about it all the time and things may get back to normal, or at least as normal as they can get. Hopefully they will be able to draw strength from you - the way you cope has a lot to do with the way they will cope.
As far as the scarring goes, if you have a definitive diagnosis of IPF (biopsy is the only true way), then your only hope for survival rests in research or a lung transplant. The scarring will continue. The rate of scarring varies from person to person, but it keeps going until it gets you. I have to tell you, this is a really lousy way to die. I live in Oregon and we have physician assisted suicide available to any terminally ill person who has less than 6 months left to live. I am not saying I will use it, but it is nice to know it is available if things get too bad. For now, I still walk 2 miles every day (with supplemental oxygen) and I really enjoy my life. It is not the same life I used to have, but it is a great one just the same.
You sound like a mature person who is facing a difficult reality with grace and courage. Most people could only hope to have a son with those character traits. Good luck to you. Who knows, maybe they will discover a treatment in the next year or two. You never know. In the meantime, just enjoy each day like it is your last. Check these sites out for more information.
http://www.pulmonaryfibrosis.org
http://www.coalitionforpf.org i don't know how to help you but i just want to say i'm really sorry about your disease/illness and i hope you know that you are loved quite a bit. god bless you xx What is pulmonary fibrosis?
"Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes (sarcoidosis, Wegener鈥檚 granulomatosis ), infections, environmental agents (asbestos, silica, exposure to certain gases), exposure to ionizing radiation (such as radiation therapy to treat tumors of the chest), chronic conditions (lupus, rheumatoid arthritis), and certain medications.
In a condition known as hypersensitivity pneumonitis, fibrosis of the lung can develop following a heightened immune reaction to inhaled organic dusts or occupational chemicals. This condition most often results from inhaling dust contaminated with bacterial, fungal, or animal products.
In some people, chronic pulmonary inflammation and fibrosis develop without an identifiable cause. Most of these people have a condition called idiopathic pulmonary fibrosis (IPF) that does not respond to medical therapy, while some of the other types of fibrosis, such as nonspecific interstitial pneumonitis (NSIP), may respond to immune suppressive therapy.
Synonyms (other names) for various types of pulmonary fibrosis that have been used in the past include chronic interstitial pneumonitis, Hamman-Rich Syndrome, and diffuse fibrosing alveolitis.
What are pulmonary fibrosis symptoms?
Symptoms of pulmonary fibrosis include shortness of breath, coughing and diminished exercise tolerance. The severity of symptoms and the progression (worsening) of symptoms over time can vary and are at least partially dependent upon the cause of the fibrosis.
How is pulmonary fibrosis diagnosed?
Pulmonary fibrosis is suggested by a history of progressive (worsening over time) shortness of breath with exertion. Sometimes, during examination of the lungs with a stethoscope, the doctor can hear crackling sounds in the chest. The chest x-ray may or may not be abnormal, but a special x-ray test called a high resolution CAT scan will frequently demonstrate abnormalities. Lung function testing is distinctly abnormal.
The diagnosis can be confirmed by lung biopsy. An open surgical biopsy, meaning that the chest wall must be surgically opened under general anesthesia to remove a portion of lung tissue, may be necessary to obtain enough tissue to make an accurate diagnosis. The removed tissue is examined microscopically by a pathologist to confirm the presence of fibrosis.
How is the pulmonary fibrosis treated?
The treatment options for idiopathic pulmonary fibrosis are very limited. There is no evidence that any medications can help this condition, since scarring is permanent once it has developed. Lung transplantation is the only therapeutic option available. At times, this diagnosis can be difficult to make even with tissue biopsy reviewed by pathologists with specific experience in this field. Research trials using different drugs that may reduce fibrous scarring are ongoing. Since some types of lung fibrosis can respond to corticosteroids (such as Prednisone) and/or other medications that suppress the body's immune system, these types of drugs are sometimes prescribed in an attempt to decrease the processes that lead to fibrosis.
The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Once scarring has developed, it is permanent. Those whose conditions improve with immune suppressive treatment probably do not have idiopathic pulmonary fibrosis.
The toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced in this condition. The lung specialist will determine the need for treatment, the duration of treatment, and will monitor the response to therapy along with any side effects. Only a minority of patients respond to corticosteroids alone, so other immune-suppressing medications are used in addition to corticosteroids. These include gamma-interferon, cyclophosphamide, azathioprine, methotrexate, penicillamine, and cyclosporine. The anti-inflammatory medication colchicine has also been used with limited success. Ongoing trials are underway using newer drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone.
Pulmonary fibrosis can cause decreased oxygen levels in the blood. A decrease in blood oxygen level (hypoxia) can lead to elevated pressure in the pulmonary artery (the vessel that carries blood from the heart to the lungs to receive oxygen), a condition known as pulmonary hypertension, which can in turn lead to failure of the right ventricle of the heart. Therefore, patients with pulmonary fibrosis are frequently treated with supplemental oxygen to prevent pulmonary hypertension.
There is also evidence that patients suffering from pulmonary fibrosis may be at increased risk for blood clots that travel to the lung (pulmonary emboli), and therefore anticoagulation (blood thinning) therapy may be indicated.
Other things to look at/consider:
Screening and diagnosis
Diagnosing pulmonary fibrosis can be extremely challenging. The difference between idiopathic and nonidiopathic forms of the disease isn't always clear, and the naming and classification systems for both have historically been confusing and controversial. In addition, many medical conditions, including chronic obstructive pulmonary disease (COPD), asthma and even heart failure, can mimic pulmonary fibrosis, so doctors must rule these out before making a definitive diagnosis.
A complete medical history, physical exam and even a chest X-ray aren't enough to diagnose pulmonary fibrosis. For that reason, you may have tests such as a high-resolution computerized tomography (CT) scan, which provides sharper and more detailed images than conventional CT scans do; an exercise test on a treadmill or stationary bike to monitor your lung function when you're active; and pulmonary function tests to measure how well your lungs work overall.
Often, though, pulmonary fibrosis can be definitively diagnosed only by examining a small amount of lung tissue (biopsy) in a laboratory. The tissue sample may be obtained in one of these ways:
Bronchoscopy (transbronchial biopsy). In this procedure, your doctor removes very small tissue samples 鈥?generally no larger than the head of a pin 鈥?using a small, flexible tube (bronchoscope) that's passed through your mouth or nose into your lungs. The risks of bronchoscopy are generally minor 鈥?most often a sore throat and temporary hoarseness from swallowing the bronchoscope 鈥?but the tissue samples are sometimes too small for an accurate diagnosis.
Bronchoalveolar lavage. In this procedure, your doctor injects saltwater (saline) through a bronchoscope into a section of your lung, and then immediately suctions it out. The withdrawn solution contains cells from your air sacs. Although bronchoalveolar lavage samples a larger area of the lung than other procedures do, it may not provide enough information to diagnose pulmonary fibrosis.
Surgical biopsy (video-assisted thoracoscopic surgery). In some cases, your doctor may recommend a surgical biopsy. This is a more invasive procedure in which surgical instruments and a small camera are inserted through two or three small incisions between your ribs. The camera allows your surgeon to view your lungs on a video monitor while removing tissue samples from your lungs. Because video-assisted thoracoscopic surgery doesn't require cutting through a rib, you're likely to have less pain and to heal more quickly than you are with traditional open-lung surgery. Still, because this procedure has a number of risks, it's usually used only when other methods have failed to provide a diagnosis.
Complications
Complications of pulmonary fibrosis may include:
Low blood-oxygen levels (hypoxemia). Because pulmonary fibrosis reduces the amount of oxygen you take in and the amount that enters your bloodstream, you're likely to develop lower than normal blood-oxygen levels. Lack of oxygen can disrupt your body's basic functioning, and severely low levels can be life-threatening.
High blood pressure in your lungs (pulmonary hypertension). Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. It begins when the smallest arteries and capillaries are compressed by scar tissue, causing increased resistance to blood flow in your lungs. This in turn raises pressure within the pulmonary arteries. Pulmonary hypertension is a serious illness that becomes progressively worse and may eventually prove fatal.
Right-sided heart failure (cor pulmonale). This serious condition occurs when your heart's lower right chamber (ventricle) has to pump harder than usual to move blood through blocked pulmonary arteries.
Respiratory failure. This is often the last stage of chronic lung disease. It occurs when blood-oxygen levels fall dangerously low and carbon dioxide levels become too high. Low blood-oxygen levels can lead to heart arrhythmias and unconsciousness; high carbon dioxide levels cause sleepiness and confusion. In either case, respiratory failure may prove fatal.
Treatment
The lung scarring that occurs in pulmonary fibrosis can't be reversed, and no current treatment has proved effective in halting the progress of the disease or improving quality of life. Still, many people diagnosed with pulmonary fibrosis are initially treated with a corticosteriod (prednisone), sometimes in combination with other drugs that suppress the immune system.
These medications can cause severe side effects, including diabetes, glaucoma, reduced production of red blood cells, skin cancer and lymphoma. For that reason, treatment is usually discontinued if there's no improvement after six months. About one in three people improves temporarily on immunosuppressant drugs, though it's not clear why some people respond and others don't.
Adding high doses 鈥?600 milligrams three times a day 鈥?of the natural enzyme N-acetylcysteine to a standard regimen of prednisone and azathioprine helps improve lung function, but it's not clear whether this is because the acetylcysteine is beneficial or because it reduces the toxicity of the other drugs.
Lung transplantation
Lung transplantation may be an option for younger people with severe pulmonary fibrosis who aren't likely to benefit from other treatment options. In order to be considered for a transplant, you must agree to quit smoking if you smoke, be healthy enough to undergo surgery and post-transplant treatments, be willing and able to follow the medical program outlined by therehabilitation and transplant team, and have the patience and emotional strength and support to undergo the wait for a donor organ. The last requirement is particularly important because donor organs are in short supply.
Other treatment approaches
Other pulmonary fibrosis treatments focus on improving quality of life. They include:
Oxygen therapy. Using oxygen can't stop lung damage, but it can make breathing and exercise easier, prevent or lessencomplications from low blood-oxygen levels, and improve your sleep and sense of well-being. It can also reduce blood pressure in the right side of your heart. You're most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.
Pulmonary rehabilitation. This is a formal program for people with chronic lung disease that includes, but goes far beyond, medical management. The aim of pulmonary rehabilitation is not only to treat a disease or even improve daily functioning, but also to help people with pulmonary fibrosis live full, satisfying lives. To that end, pulmonary rehabilitation programs focus on exercise, on teaching you how to breathe more efficiently, on education, and on emotional support and nutritional counseling.
Most often, this multifaceted approach requires a team of health care professionals that may include a doctor, nurse, rehabilitation specialist, dietitian and social worker.Programs can vary widely, however. Your doctor can usually tell you about pulmonary rehabilitation programs in your area. Or contact the American Lung Association for more information.
Treatments under investigation
A number of treatments for pulmonary fibrosis are being developed or are in clinical trials. You can find an extensive listing of clinical trials in the National Institutes of Health Clinical Trial Database on the Web. You can also contact the National Heart, Lung, and Blood Institute for more information. If you think you might be interested in participating in a clinical trial, your doctor can help you find an appropriate program.
Self-care
Being actively involved in your own treatment and staying as healthy as possible are essential to living with pulmonary fibrosis. For that reason, it's important to:
Stop smoking. If you have lung disease, the best thing you can do for yourself is to stop smoking. Talk to your doctor about options for quitting, including smoking cessation programs, which use a variety of proven techniques to help people quit. And because secondhand smoke can be just as harmful to your lungs, don't allow other people to smoke around you.
Exercise regularly. Exercise is a double-edged sword for people with lung disease. It can make symptoms worse, yet it's essential for maintaining lung function, reducing stress and improving overall health and well-being. What's more, strong muscles work more efficiently than weak muscles do, so they require less oxygen to accomplish ordinary tasks.
If you're already exercising, don't stop. And if you're not currently physically active, consider starting with a moderate workout, such as riding a stationary bike or walking. For instance, you might begin walking at a comfortable pace for just 10 minutes a day. Once you can walk the entire time without stopping to rest, increase the length of your walk by a minute or two each week. Many people with severe lung disease eventually can walk at least 30 minutes nonstop. If you've been prescribed oxygen for regular use, be sure to use it when you exercise.
You might also ask your doctor for a referral to an exercise physiologist, who can design an exercise program specifically for you. Most of all, don't allow friends and family to talk you out of getting the exercise you need.
Eat well. People with lung disease may lose weight both because it's uncomfortable to eat and because of the extra energy it takes to breathe. Yet a nutritionally rich diet that contains adequate calories is essential. The type of food you eat, the time of day and the size of portions can all play a role in getting the nourishment you need. Because it's easier to breathe when your stomach isn't completely full, you may want to eat smaller meals throughout the day rather than two or three large ones. You might also try choosing lighter fare, such as fruit and salads, rather than rich or fatty foods, which take more energy to digest. A dietitian can give you further guidelines for healthy eating.
Get plenty of rest. Getting at least eight hours of good-quality rest every night can boost your immune system and sense of well-being.
Control GERD. If you've been diagnosed with GERD, it's essential to keep it under control. You can help reduce acid reflux by waiting at least three to four hours after eating before lying down, eating smaller meals, avoiding any foods that trigger your symptoms and maintaining a healthy weight.
WHAT IS INTERSTITIAL LUNG DISEASE?
Interstitial Lung Disease (ILD) is a general term that includes a variety of chronic lung disorders. When a person has ILD, the lung is affected in three ways. First, the lung tissue is damaged in some known or unknown way. Second, the walls of the air sacs in the lung become inflamed. Finally, scarring (or fibrosis) begins in the interstitium (or tissue between the air sacs), and the lung becomes stiff.
Breathlessness during exercise can be one of the first symptoms of these diseases. A dry cough also may be present. These are common symptoms that many people ignore. Someone with these symptoms may wait until they feel quite ill before going to the doctor.
People with different types of ILD may have the same kind of symptoms but their symptoms may vary in severity. Their chest X-rays may look alike. Further testing is usually recommended to identify the specific type of ILD a person has. Some ILDs have known causes and some (idiopathic) have unknown causes.
WHY IS IT CALLED INTERSTITIAL LUNG DISEASE?
The tissue between the air sacs of the lungs is called the interstitium. Interstitial lung disease is named after this tissue because this is the tissue affected by fibrosis (scarring). Interstitial lung disease is sometimes also known as "interstitial pulmonary fibrosis." The terms interstitial lung disease, pulmonary fibrosis and interstitial pulmonary fibrosis are often used to describe the same condition.
INTERSTITIAL LUNG DISEASES ARE PUZZLING
The course of these diseases is unpredictable. If they progress, the lung tissue thickens and becomes stiff. The work of breathing then becomes more difficult and demanding. Some of the diseases improve with medication if treated when inflammation occurs. Some people may need oxygen therapy as part of their treatment.
The diseases may run a gradual course or a rapid course. People with ILD may notice variations in symptoms -- from very mild to moderate to very severe. Their condition may remain the same for long periods of time or it may change quickly. It's important to stay in touch with your doctor and report any changes in symptoms. You and your doctor can work together to manage ILD.
COMMON LINK IN INTERSTITIAL LUNG DISEASE
While the progress and symptoms of these diseases may vary from person to person, there is one common link between the many forms of ILD. They all begin with an inflammation. The inflammation may affect different parts of the lung, as explained below:
1. The walls of the bronchioles (small airways). When inflammation involves the bronchioles, it is called bronchiolitis.
2. The walls and air spaces of the alveoli (air sacs). When inflammation involves the alveoli, it is called alveolitis.
3. The small blood vessels (capillaries) of the lungs. When inflammation involves the small blood vessels, it is called vasculitis.
Inflammation of these parts of the lung may heal or may lead to permanent scarring of the lung tissue. When scarring of the lung tissue takes place, the condition is called pulmonary fibrosis.
Fibrosis, or scarring of the lung tissue, results in permanent loss of that tissue's ability to transport oxygen. The level of disability that a person experiences depends on the amount of scarring of the tissue. This is because the air sacs, as well as the lung tissue between and surrounding the air sacs, and the lung capillaries, are destroyed by the formation of scar tissue. If this happens, your doctor may prescribe oxygen to help you breathe easier.
IDIOPATHIC PULMONARY FIBROSIS (IPF)
When all known causes of interstitial lung disease have been ruled out, the condition is called "idiopathic" (of unknown origin) pulmonary fibrosis (IPF).
There are several theories as to what may cause IPF; including viral illness and allergic or environmental exposure (including tobacco smoke). These theories are still being researched. Bacteria and other microorganisms are not thought to be the cause of IPF.
There is also a familial form of the disease, known as familial idiopathic pulmonary fibrosis. Additional research is being done to determine whether there is a genetic tendency to develop the disease, as well as to determine other causes of IPF.
WHAT ARE THE SYMPTOMS OF IPF?
Shortness of breath is the main symptom of idiopathic pulmonary fibrosis. Since this is a symptom of many types of lung disease, making the correct diagnosis may be difficult. The shortness of breath may first appear during exercise. The condition then may progress to the point where any exertion is impossible. If the disease progresses, the person with IPF eventually may be short of breath even at rest.
Other symptoms may include a dry cough (without sputum). When the disease is severe and prolonged, heart failure with swelling of the legs may occur.
HOW IS IPF DIAGNOSED?
A very careful patient history is an important tool for diagnosis. The history will include environmental and occupational factors, hobbies, legal and illegal drug use, arthritis, and risk factors for diseases that affect the immune system. A physical examination, chest X-ray, pulmonary function tests, and blood tests are important. These tests will help your doctor rule out other lung diseases and determine the extent of disease.
Bronchoalveolar lavage (BAL) -- a test which permits removal and examination of cells from the lower respiratory tract -- may be used to diagnose IPF: this test helps a doctor identify inflammation in lung tissue, and also helps exclude infections and malignancies (cancer) as a cause of a patient's symptoms. The test is done during bronchoscopy, a special examination of the lung.
Lung biopsy --Either done during bronchoscopy or as a surgical procedure that removes a sample of lung tissue for your doctor to study -- this proceedure is usually required for for diagnosis of IPF.
Diagnostic Tests That May Be Used to Identify Pulmonary Fibrosis or Interstitial Lung Disease
1. Blood Tests
2. Pulmonary Function Tests
3. Chest X-ray
4. CT Scan
5. Bronchoscopy
6. Bronchoalveolar Lavage
7. Lung Biopsy
8. CT Scan
WHAT IS THE TREATMENT FOR IPF?
Corticosteroids may be administered to treat the inflammation present in some people with IPF. The success of this treatment for many forms of pulmonary fibrosis is variable and is still being researched. Other drugs are occasionally added when it is clear that the steroids are not effective in reversing the disease.
Some doctors may use corticosteroids in combination with other drugs when the diagnosis is first established. Which drug treatment plan is effective, and how long to use the drugs is the focus of current research.
Oxygen therapy may be prescribed for some people with IPF. The need for oxygen will be determined by your doctor and may depend on the severity of disease, as well as your activity level. Talk with you doctor if you think you may need oxygen or if you have concerns about oxygen.
Influenza vaccine and pneumococcal pneumonia vaccine are both recommended for people with IPF or any lung disease. These two shots may help prevent infection and keep you healthy.
Lung transplantation may offer hope for selected people with severe IPF and other lung diseases. Speak with your doctor about eligibility criteria for lung transplant recipients.
Rehabilitation and education programs may help some people with IPF. Local support groups have been of benefit for people with IPF and their family members and friends
I hope that this information helps you out or sheds light on your questions in some fashion or another. You will be in my prayers. =] Hi Hon.. I'm only going to start this here in hopes we can maybe chat or email..
I lost my sister January 27th of this year to Pulmonary Fibrosis.. I know this disease and how it effects the infected as well as the family members and loved ones..
You have a rough road ahead.. all of you.. My sister was 46 when she crossed over.. I was right there holding her hand..
Pulmonary fibrosis is not just scarring.. basicly the lungs turn to like concrete from the bottom to the top.. my sis was told she had 2-5 years.. she made it 4 years.. basicly what happens is when the lungs fill.. they do what is called honeycombing.. looks like a bee honeycomb.. those tiny holes are all the space for your oxygen..
Are you on oxygene 24/7 now? are they giving you prednisone? advair? what type of breathing treatments? have they started giving you morphine tabs for pain?... these things do help ease the pain as it gets worse..
I know the pain your feeling and sadly it will get a lot worse..
My sister was able to get disability .. a power scooter, meds etc.. and you will need them..
She went through phases.. positive one day, screaming and crying the next.. sometimes in a matter of minutes.. its hard for everyone..
As a family member it is just so damn hard to watch someone you love suffer and know there is nothing you can do for them.. towards the end though as it gets so horrid to see.. and you hear the person you love saying they are ready, they can't handle the pain anymore.. they can't walk to the bathroom anymore.. when they become basicly invalid.. and the time finaly comes.. it is painful yes.. tearful yes.. but in many ways the family feels a relief..
Search for a support group in your area.. we found one here and we went together, my sister, mother and I until my sis could no longer get out..
I would like to talk more with you and would gladly email or snail mail your Mom and Dad.. I so understand and I am so sorry this hit you..
It sounds like you were just diagnosed so you have years to go still. enjoy them.. spend time doing things you want to do with family because the time will come when you can't..
Let them know you love them and when you have the anger strike let them know its not them its just the disease.. you as well as they will go through many phases of mourning even while you are living..
The scarring in your lungs is what causes IPF... the biopsy my sis had done said.. well they "think" it was caused from scarring due to her extreme acid reflux.. the burning liquid scarred the lungs causing the fibroids.
It is also suprisingly not the lungs that end the life ... you have a stroke or heart attack because your brain and your heart try to take over breathing when the lungs can't do it anymore..
My sisters breathing reps were so rapid the day she crossed that the hospice nurse could not count them.. her heart was trying to breath for her lungs untl it finaly just couldn't do it and stopped..
but honey the moment she passed away..I could see the pain was gone.. I could see relief in her.. her skin color was normal.. she looked like my ole sister.. not the grey , ashen suffering woman she had become.. she looked beautiful again and that helped soooo much to see..
The oxygen will give you this ashen , greyish skin tone and it gets very dry..
Be sure to find the BEST Pulmonologist you can...
Make sure to apply for disability and stick with it.. the money and medical benefits are a great help..
I hope to be friends..
Blessed Be
Moon
email me please if you'de like to talk more..
whitemoon1111@yahoo |
