a Daughton

[Guest guest]

a,

Just wanted to welcome you to the board! I'm sorry you had reason to join us but am glad you are here nonetheless. Please feel free to share your story with us and we will share ours with you. This is a great group of people who over the last two years have become more like family to me.

My name is Beth and I am co-moderator of this board with Joyce. I live in Durham NC, I'm divorced and have a 22 year old son. I was diagnosed with NSIP ( one of the many forms of pulmonary fibrosis) almost exactly 2 years ago. I've been extremely fortunate in that my disease has remained stable since that time. I am on oxygen 24/7 but have learned to live with it and have found that my life while different is still good.

The first thing I'd tell you is not to panic, take a deep breath (or as deep as you can) and know that here you are among friends, people who understand. Tell us something about yourself and how you came to be diagnosed. I know I speak for all of us when I say we will help in any way we can!

Beth

Age 48 Fibrotic NSIP 06/06

Change everything. Love and Forgive

recently dx : Diagnosing Pulmonary Fibrosis

Thank you for this information. I have just been dx with ipf and am very confused about what to do next. Can you give me the name of someone on the support group that I could share my experence with and perhaps get some help. My Dr. has not been helpful at all.

Thanks for getting back to me. a <kpoooh9yahoo (DOT) com> wrote:

Read this article that I thought was very good so passing it on to you guys P:

>> Diagnosing Pulmonary Fibrosis > Environmental factors and genetics increase the risk of developing > this fatal disease > By Bronzell-Wynder, NP > > > Mr. is a 60-year-old man referred to a pulmonologist after > preadmission testing for a total knee replacement. His chest > radiograph proves abnormal, showing bilateral reticular opacities. > His knee surgery is cancelled, and he is referred to a lung > practitioner. > > A review of systems during the lung practitioner' s assessment reveals > Mr. ' progressive shortness of breath the past year. On visual > assessment, he has digital clubbing with cyanosis of his fingertips > and lips. On auscultation, he has bibasilar crackles. The lung >

practitioner orders several tests, which reveal pulmonary fibrosis.> > Definition> > Pulmonary fibrosis is one of many interstitial lung diseases that > scar lung tissue. An incurable, restrictive lung disease, it causes > scarring and fibrosis between the alveoli (chronic interstitial > inflammation) . Scarring and stiffening of the alveoli decreases > transport of oxygen across the alveolar membrane. > > One clear sign of pulmonary fibrosis on CT scan is honeycombing and > scarring, which irreversibly lessen elasticity and lung compliance.1> > Pulmonary fibrosis is known by different names, including idiopathic > pulmonary fibrosis (IPF), defined by progressive dyspnea and > declining pulmonary function test.2 Chest radiographs display > interstitial infiltrations and biopsy shows fibrosis, inflammation or > both. > >

Idiopathic interstitial pneumonias fall into eight categories. > > Desquamative interstitial pneumonia (DIP): A rare form of fibrosis. > Average age at onset is 42. Of these patients, 90 percent have a > smoking history, and 75 percent respond to steroids. > > Bronchiolitis obliterans > > Organizing pneumonia > > Cryptogenic organizing pneumonia (CFA) > > Respiratory bronchiolitis interstitial lung disease (RBILD): Takes on > the same process of DIP. Average age at onset is 36.1,2 > > Acute interstitial pneumonia (AIP): Acute onset with rapid > progression of shortness of breath and respiratory failure. Life > expectancy is 6 months. > > Nonspecific interstitial pneumonia (NSIP): More common in women. > Average age at onset is 49. Inflammation is more common than > fibrosis. > >

Lymphangioleiomyoma tosis (LAM): A rare form of pulmonary fibrosis > affecting only women. Average age at onset is 34. > Other disease processes can cause pulmonary fibrosis, including > lupus, rheumatoid arthritis, scleroderma and sarcoidosis.> > Epidemiology > > Studies show that 13 to 20 people per 100,000 develop interstitial > lung diseases, including an IPF prevalence of 7.4 women per 100,000 > and 10.7 men per 100,000.1> > Pulmonary fibrosis typically affects adults 40 to 70 years of age. > About two-thirds are over age 60. The mean age at diagnosis is 66. > Incidence increases after age 75, to 175 per 100,000. Overall death > increases at age 75, as well, with the mean length of survival > following diagnosis at 3 to 5 years.1 > > Unfortunately, pulmonary fibrosis patients, not knowing their > condition for years,

attribute shortness of breath to other causes, > such as weight gain, aging and lack of exercise. As a result, the > condition is often diagnosed in the late stages after a comparatively > minor medical event, for example, flu requiring hospitalization. > After diagnosis, life expectancy for most patients is 2 to 4 years, > with the 5-year survival rate at 30 percent to 50 percent.1> > Risk factors > > There are six known risk factors for pulmonary fibrosis: > > cigarette smoking > > environmental factors> > genetic predisposition> > chronic aspiration > > infectious agents > > some prescription medications. 1 > While cigarette smoking plays a role in the development of pulmonary > fibrosis, it has actually extended survival in some patients, > compared with nonsmokers or former smokers.3

> > A likely explanation is that these patients may seek medical > attention earlier for smoking-related symptoms.3 These patients also > can have more digital clubbing and less crackling on examination, > increased incidence of pulmonary hypertension on chest radiograph and > decreased spirometry and diffusion capacities on pulmonary function > tests.3 > > Environmental factors that increase the risk of developing pulmonary > fibrosis include exposure to solvents and to dust from metal and > wood, and atopy.4> > Genetic predisposition also may be a factor. No specific genetic > markers have been linked to IPF, but there have been cases of two > immediate family members having IPF, though this is currently being > researched.> > Chronic aspiration is another risk factor for IPF. These patients > tend to aspirate secondary

to gastric reflux disease. However, it is > still not clear how chronic aspiration leads to IPF. > > Several infectious diseases can lead to IPF, including Legionnaires > disease, herpes virus, parainfluenza 3 virus, HIV-1, hepatitis C, > cytomegalovirus, measles and the Epstein Barr virus.1,5 Some studies > evaluated DNA markers of CMV, EBV, HHV-7 and eight and have shown > that patients with these viruses were at an increased risk for > pulmonary fibrosis.5> > Antidepressants and tricyclic medications may lead to pulmonary > fibrosis.6 Dothiepin and imipramine have shown to increase incidence > of IPF, but how these medications affect the disease process is not > known.6 > > Other medications may cause pulmonary fibrosis using different > mechanisms. They include amiodarone, amphotericin B, nitrofurantoin, > methotrexate, bleomycin,

vinca and alkaloids. Prolonged use of > amiodarone leads to pulmonary toxicity. Chemotherapeutic medications > such as bleomycin and vinca alkaloids, can cause pulmonary fibrosis > in 10 percent of the population. Methotrexate, standard dosaging in > patients with arthritis and nitrofurantoin, also can cause pulmonary > fibrosis.7 Other medications can lead to pulmonary fibrosis, with > high doses or chronic use related to the disease process. > > IPF does not have a known etiology unless the causative agent is > biopsied from the lung.> > Pathophysiology > > Pulmonary fibrosis is caused by stimuli, or exposure to a substance > that triggers a triad of responses.2 First, the stimulus is > introduced causing chronic inflammation within the lung tissue, which > releases cytokines that eventually lead to lung injury. This is > caused by

development of increased fibroblast activity, which leads > to fibrosis of the lung parenchyma.2, 8 > > A new hypothesis outlines how pulmonary fibrosis developes: First, > the patient is exposed to a stimulus, which initiates lung injury > that can cause inflammation depending on the patient's dominant > inflammatory phenotype. Eventually, th lung tissue begins to heal > causing fibrosis.2 Some of the factors modifying the > patient's "fibrotic" response are a history of smoking, environmental > exposures and viral infections.> > Clinical presentation > > Patients with pulmonary fibrosis will complain of a cough with or > without mucus, and about 25 percent to 44 percent will have digital > clubbing. The most common and insidious side effect is progressive > shortness of breath. On auscultation, bibasilar inspiratory crackles > can be

heard. > In more advanced cases, right-sided heart failure and increased > pressure can be seen.8 Other symptoms include decreased appetite, > chest tightness and achy joints or muscles.> > Diagnosis > > The patient should be referred to a pulmonologist, who will perform a > history and physical examination and order a series of tests. A > pulmonary function test allows the pulmonologist to see the severity > of disease, measured by low residual volume and total lung capacity, > as well as a decreased diffusion capacity. > > A 6-minute walk test and cardiopulmonary exercise test show hypoxemia > related to a "widened" alveolar-arterial oxygen gradient. To watch > the progression of the disease, serial pulmonary function testing is > recommended. 8 > > Blood work is ordered next, including a complete blood count, >

antinuclear antibodies, rheumatoid factor and immunoglobulins. The > blood work may show increased inflammatory markers, mild anemia and > unspecified rises in the ANA and rheumatoid factor levels. These > levels can be high in the absence of any collagen vascular disease, > including the rise in autoantibodies. 2 > > A chest radiograph will show bilateral reticular opacities > infiltrated with a lower lobe predominance seen in the periphery. > These patients also should have a high-resolution CT scan. With the > progression of the disease, cystic dilatation leads to honeycombing, > traction bronchiectasis and ground glass.2 > > A bronchoavelolar lavage (BAL) is not the best way to confirm > diagnosis of pulmonary fibrosis because the sample is normally too > small.2 BAL is useful in ruling out other diseases such as > infection, malignancy and

sarcoidosis. > > The gold standard of testing is a surgical lung biopsy, which will > show usual interstitial pneumonitis in patients with idiopathic > pulmonary fibrosis. The pattern is usually one of a heterogeneity of > scarring with cysts demonstrating a honeycombing form along with > destruction of the alveoli. This pattern is normally seen in patients > with pulmonary fibrosis from drug toxicity, collagen vascular > diseases, asbestosis, chronic hypersensitive pneumonitis and familial > pulmonary fibrosis.8 > > Biopsy results allow the pulmonologist to rule out any other possible > causes of pulmonary fibrosis. The following types of patients should > have a biopsy: recurrent pneumothorax, general systemic complaints, > over age 50, rapid disease progression, CT scan showing changes and > unclear diagnosis.8 If a patient presents with four of

the major > criteria or three of the minor criteria, surgical biopsy is > unnecessary.> > Treatment > > No immunosuppressive medications improve the overall survival of > patients with pulmonary fibrosis. Anti-inflammatory drugs are > mainstays of treatment, even though some studies have shown they > offer little improvment.9 Typically 20 mg/day of prednisone is > prescribed in conjunction with azathioprine at 3 mg/kg/day, not to > exceed 200 mg/day.8 > > It is imperative that health care providers know the side effects of > prednisone and azathioprine. With prednisone, monitor for > hyperglycemia, osteoporosis, myopathy and exaggerated hypertension. > The patient should be placed on calcium with vitamin D for > osteoporosis prevention. For long-term use, a dexa scan should be > performed at least yearly. > > Before

initiating any prophylactic steroid therapy, a purified > protein derivative test should be performed to ensure the patient > does not have tuberculosis. With azathioprine, monitor for bone > marrow suppression and hepatotoxicity. 8> > Another group of medications is the antifibrotic agents, which slow > down the fibrosis in the lungs. Some of these drugs interfere with > the cellular matrix to help slow the progression of pulmonary > fibrosis. Pirfenidone and interferon gamma-1b are currently under > investigation. Cholchicine has been found to stop the fibroblast > proliferation by inhibiting collagen formation.10> > Oxygen > > Another important treatment for patients with pulmonary fibrosis is > oxygen. With increased scarring within the lung parenchyma, the > patient will have decreased oxygen perfusion. This supplement helps > to

maintain the patient's oxygen pressure at the level of the > alveoli. > > Oxygen saturation should be maintained at 90 percent or more. A 6-> minute walk test, a cardiopulmonary exercise test, or arterial blood > gas should be performed to determine if a patient requires oxygen.11 > These tests will give the practitioner information on how much oxygen > to order for the patient at rest and on exertion.> > Lung transplant > > Patients under age 65 with few conflicting medical problems are > normally good candidates for lung transplant and undergo vigorous > testing to evaluate their overall pulmonary and cardiac status. > > Of course, lung transplantation carries risks. According to the > United Network of Organ Sharing, 77 percent to 78.6 percent of > patients are alive one year after transplant, while 40 percent to > 46.2

percent live 5 years or longer. Research has shown lung > transplantation improves pulmonary fibrosis patients' overall > survival.12 > > Hence, it is imperative that physician assistants recognize signs and > symptoms of pulmonary fibrosis and provide early referral for > treatment with medication and placement on the lung transplant list.> > References> > 1. American Thoracic Society. Idiopathic pulmonary fibrosis: > diagnosis and treatment: international consensus statement. American > Journal of Respiratory Critical Care Medicine. 2000; 161:646-664.> > 2. Gross T, Hunninghake G. Idiopathic pulmonary fibrosis. New England > Journal of Medicine. 2001; 345:517-525.> > 3. King T, et al. Predicting survival in idiopathic pulmonary > fibrosis: scoring system and survival model. American Journal of > Respiratory and Critical

Care Medicine. 2001; 164:1171-1181.> > 4. Miyake O, et al. Occupational and environmental factors and > idiopathic pulmonary fibrosis. British Occupational Hygiene Society. > 2005; (49)3:259-265.> > 5. Tang Y, et al. Increased detection of herpesvirus DNA in > idiopathic pulmonary fibrosis. Chest. 2001; 120(1 Suppl):74s-75s.> > 6. Hubbard R, et al. Exposre to commonly prescribed drugs and the > etiology of crytogenic fibrosing alveolitis. American Journal of > Respiratory and Critical Care Medicine. 1998; 157:743-747.> > 7. Ozkan M, et al. Drug-induced lung disease. Cleveland Clinic > Journal of Medicine. 2001; 68(9):782-795.> > 8. Khalil N, O'Connor R. Idiopathic pulmonary fibrosis: current > understanding of the pathogenesis and the status of treatment. > Canadian Medical Association Journal. 2004; 171(2):153-160.>

> 9. Richeldi L, et al. Corticosteroids for idiopathic pulmonary > fibrosis. The Cochrane Database of Systematic Reviews. 2003; > 3:CD002880.> > 10. Selman M, King T, Pardo A. Idiopathic pulmonary fibrosis: > prevailing and evolving hypothesis about its pathogenesis and > implications for therapy. ls of Internal Medicine. 2001; 134:136-> 151.> > 11. Lindell K, s S. Pulomonary fibrosis: new guidelines for > diagnosing and managing the disease demand a fresh approach to > nursing care. American Journal of Nursing. 2003; 103(4):33-42.> > 12. Thabut G, et al. Survival benefit of lung transplantation for > patients with idiopathic pulmonary fibrosis. The Journal of Thoracic > and Cardiovascular Surgery. 2003; 126:469-475.> > > > Bronzell-Wynder, NP, works in the transplant surgery department > at

Jefferson University Hospital in Philadelphia.> P PM (Polymositis) 12/98, UIP 8/00, o2 24/7 8/04, PH 3/06, ILL yo 59