IPF Suffers Have Legal Claim for Compensation

Aylstock, Witkin, Kreis and Overholtz has spent over five years of practice representing victims of diet drug induced primary pulmonary hypertension/PPH. After investigating the recently publicized matter of Intermune's off-label promotion of Actimmune to the medical community and patients suffering through the struggles of IPF, we believe Intermune's actions in promoting Actimmune for IPF were based on financial considerations; particularly after Intermune became aware that Actimmune lacked efficacy i.e. that there was no statistically significant relationship between Actimmune and the treatment of IPF.

This negligent action resulted in (among other things) pulmonologists being misled (and therefore misleading their patients), the worsening of IPF patients' condition (caused by lapse of time while under ineffectual drug therapy), and Intermune's delay in rolling out an aggressive pre-market approval launch of Pirfenidone. 

We are hopeful that the CAPACITY trial's initial results — which appear quite positive – hold firm, and that it will be an effective IPF therapy on all formularies and covered by all - Medicare and private insurers soon (Tracleer/bosentan with which I have familiarity as used by PPH sufferers has apparently shown promise as well).  However, we believe it is important that Intermune pay for the cost of Actimmune as well as the costs of ongoing medical monitoring and care (lung transplants) for sufferers and their families. 

If you or a loved one has been injured as a result of using Actimmune, you may be entitled to receive compensation for your pain and suffering. For more information on Actimmune, Intermune and the right to compensation, please call our offices today at 888-255-2956.

Off-Label Use of Actimmune Found Ineffectual

Patients with interstitial lung disease (ILD) suffer terribly with the incurable illness and its symptoms. With very little hope, such patients became easy prey for drug maker Intermune, Inc. which pushed its drug Actimmune as a cure for a branch of ILD called idiopathic pulmonary fibrosis (IPF). These desperate IPF patients spent countless dollars chasing an “off-label” dream promoted by Intermune's spokespeople.

Actimmune was never approved by the FDA for use with IPF. Moreover, it became obvious early on that it was ineffective and that it could not alleviate IPF symptoms.  This was determined through clinical trial results proving that the drug fails to provide effective treatment to pulmonary fibrosis patients.  Specifically, Intermune cancelled an 826-patient trial after it was determined that 14.5 percent of IPF patients taking Actimmune die in relation to those taking a placebo. 

Intermune made approximately $90 million each year from Actimmune (interferon gamma) and a majority of the sales were for the off-label treatment of IPF. Although there are approximately 100,000 IPF patients nationwide, it is believed that there are only 800 people in the US
with chronic granulomatous disease or severe malignant osteopetrosis.

Once the scandal broke in large part to the efforts of one of Intermune's former sales persons—who was forced into selling the product off label—the government stepped in and fined Intermune approximately $37 million to resolve a lawsuit.  Additionally, Intermune was fined another $30 million which went to pay Medicare, Medicaid, the VA and other insurers that lost money paying for what amounted to snake oil.

As assistant U.S. attorney general Peter Keisler put it: "It is vital to public health and safety that pharmaceutical companies are deterred from improperly marketing their drugs to doctors and patients to treat illnesses that these drugs are not approved to treat".

Aylstock, Witkin, Kreis and Overholtz has spent over five years of practice representing victims of diet drug induced primary pulmonary hypertension/PPH. After investigating the recently publicized matter of Intermune's off-label promotion of Actimmune to the medical community and patients suffering through the struggles of IPF, we believe Intermune's actions in promoting Actimmune for IPF were based on financial considerations; particularly after Intermune became aware that Actimmune lacked efficacy i.e. that there was no statistically significant relationship between Actimmune and the treatment of IPF.

This negligent action resulted in (among other things) pulmonologists being misled (and therefore misleading their patients), the worsening of IPF patients' condition (caused by lapse of time while under ineffectual drug therapy), and Intermune's delay in rolling out an aggressive pre-market approval launch of Pirfenidone. 

We are hopeful that the CAPACITY trial's initial results — which appear quite positive – hold firm, and that it will be an effective IPF therapy on all formularies and covered by all - Medicare and private insurers soon (Tracleer/bosentan with which I have familiarity as used by PPH sufferers has apparently shown promise as well).  However, we believe it is important that Intermune pay for the cost of Actimmune as well as the costs of ongoing medical monitoring and care (lung transplants) for sufferers and their families. 

If you or a loved one has been injured as a result of using Actimmune, you may be entitled to receive compensation for your pain and suffering. For more information on Actimmune, Intermune and the right to compensation, please call our offices today at 888-255-2956.

Additionally, if you would like to speak to other IPF sufferers, we invite you to visit the Internet blog: "Huff-'n-Puff".

Respiratory Disease

When you breathe, your lungs take in oxygen from the air and deliver it to the bloodstream. The cells in your body need oxygen to work and grow. During a normal day, you breathe nearly 25,000 times. People with lung disease have difficulty breathing. Millions of people in the U.S. have lung disease. If all types of lung disease are lumped together, it is the number three killer in the United States.

The term lung disease refers to many disorders affecting the lungs, such as asthma, chronic obstructive pulmonary disease, infections like influenza, pneumonia and tuberculosis, lung cancer, and many other breathing problems.

Numerous conditions make up the group of disorders called interstitial lung disease. Most cause progressive scarring of lung tissue that eventually affects your ability to breathe and get enough oxygen into your bloodstream, but beyond this, the disorders vary greatly.

Although most cases of interstitial lung disease develop gradually, a few come on suddenly. Doctors can pinpoint why some cases of interstitial lung disease occur, but many have no known cause.

In all cases, lung scarring, once it occurs, is generally irreversible. Medications occasionally can slow the damage of interstitial lung disease, but many people never regain full use of their lungs. Researchers hope that newer drugs, many of them still experimental, may eventually prove more effective in treating interstitial lung disease.

Symptoms of Respiratory Disease

Despite the wide variety of disorders classified as interstitial lung disease, the signs and symptoms are often similar:

• A feeling of breathlessness (dyspnea), especially during or after physical activities
• A dry cough

Because these problems are vague and tend to develop gradually — often long after you have irreversible lung damage — you may attribute them to aging, to being overweight or out of shape, or to the residual effects of an upper respiratory infection.

Symptoms tend to become progressively worse, however, and eventually you may notice you're getting out of breath during routine activities — getting dressed, talking on the phone, even eating. At this point, breathing problems become impossible to ignore.

Other, far less common signs and symptoms of some types of interstitial lung disease include wheezing, chest pain and clubbing of the fingers, a physical sign that occurs when your fingertips painlessly enlarge and the nails curve over the tops of your fingertips.

Causes of Respiratory Disease

Each time you inhale, air travels to your lungs through two major airways called bronchi. Inside your lungs, the bronchi subdivide like the roots of a tree into smaller airways (bronchioles) that finally end in clusters of tiny air sacs (alveoli). Within the walls of the air sacs are small blood vessels (capillaries) where oxygen is added to your blood and carbon dioxide — a waste product of metabolism — is removed.

In interstitial lung disease, the walls of the air sacs may become inflamed, and the tissue (interstitium) that lines and supports the sacs becomes increasingly thickened and scarred. Normally, the air sacs are highly elastic, expanding and contracting like small balloons with each breath. But scarring (fibrosis) causes the thin, interstitial tissue to become stiffer and thicker, making the air sacs less flexible. Instead of being soft and elastic, scarred air sacs have the texture of a stiff sponge, which makes it more difficult to breathe and harder for oxygen to enter your bloodstream.

Scarring in interstitial lung disease seems to occur when an injury to your lungs triggers an abnormal healing response. Ordinarily, your body generates the right amount of tissue to repair damage. But in interstitial lung disease, the repair process goes awry, producing excess scar tissue that increasingly interferes with lung function.

Because interstitial lung disease has a wide range of causes, determining the reason for an initial injury to lung tissue can be difficult. Some of the many possible precipitating factors include:

• Occupation and environmental factors. Long-term exposure to a number of toxins or pollutants can lead to serious lung damage. Workers who routinely inhale silica dust (silicosis), asbestos fibers (asbestosis) or hard metal dust are especially at risk of debilitating lung disease. So are people exposed to certain chemical fumes and ammonia or chlorine gases.

But chronic exposure to a wide range of substances, many of them organic, also can damage your lungs. Among these are grain dust, sugar cane, and dust from bird and animal droppings. Other substances, such as moldy hay, can be a problem when they cause a hypersensitivity reaction in the lungs (hypersensitivity pneumonitis). Even bacterial or fungal overgrowth in poorly maintained humidifiers and hot tubs can cause lung damage.

• Infections. These include viral infections such as cytomegalovirus, a particular problem for people with compromised immune systems; some bacterial infections, including pneumonia; fungal infections such as histoplasmosis; and parasitic infections.
  
• Radiation. Some people who receive radiation therapy for lung or breast cancer show signs of lung damage long after their initial treatment. The severity of the damage may depend on how much of the lung is exposed to radiation, the total amount of radiation administered, whether chemotherapy also is used and the presence of underlying lung disease.
  
  
• Drugs. Some drugs can damage the interstitium of the lungs, especially chemotherapy drugs, medications used to treat heart arrhythmias and other cardiovascular problems, certain psychiatric medications, and some antibiotics.
  
  
• Other medical conditions. Interstitial lung disease can occur with other disorders. Often, those conditions don't directly attack the lungs, but instead involve systemic processes that affect tissue throughout the body. Among these are connective tissue disorders and hematological diseases, including lupus, scleroderma, rheumatoid arthritis, dermatomyositis, polymyositis, Sjogren's syndrome and sarcoidosis.

Idiopathic pulmonary fibrosis: When the cause isn't known

Although doctors can determine why some people develop interstitial lung disease, in many cases the cause isn't known. Disorders without a known cause are considered a subset of interstitial lung disease and are grouped together under the label idiopathic pulmonary fibrosis or idiopathicinterstitial lung disease. Although the idiopathic diseases have certain features in common, each also has unique characteristics.

Usual interstitial pneumonitis is the most prevalent of the idiopathic interstitial lung diseases. Because it's so common, the terms "usual interstitial pneumonitis" and "idiopathic pulmonary fibrosis" are often used interchangeably. Because usual interstitial pneumonitis develops in patches, some areas of the lungs are normal, others are inflamed and still others are marked by scar tissue. The disease affects more men than women and usually develops in people over 50.

Although the names are nearly identical, pneumonitis is not the same as pneumonia. Pneumonitis is lung inflammation without infection, whereas pneumonia is lung inflammation that results from infection. In addition, pneumonia is generally limited to one or two areas of the lungs, but pneumonitis involves all five lobes — two in the left lung and three in the right.

Other, less common types of idiopathic pulmonary fibrosis include nonspecific interstitial pneumonitis, bronchiolitis obliterans with organizing pneumonia (BOOP), respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonitis, lymphocytic interstitial pneumonitis and acute interstitial pneumonitis.

Diagnosing Respiratory Disease

Identifying and determining the cause of interstitial lung disease can be extremely challenging. An unusually large number of disorders fall into this broad category. What's more, the distinction between interstitial lung disorders with identifiable causes and those with no known cause isn't always clear, and the nomenclature and classification systems of both have historically been confusing and controversial.

In addition, the signs and symptoms of a wide range of medical conditions — among them chronic obstructive pulmonary disease (COPD), heart failure and asthma — can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.

To help cut through the confusion and rule out other possible illnesses, doctors normally begin by taking a comprehensive medical history, focusing especially on occupational exposure to lung-damaging toxins, on medications and on the presence of health problems commonly associated with lung disorders.

But although a medical history and physical exam can be useful in ruling out certain conditions, they can't accurately diagnose interstitial lung disease. Instead, doctors normally rely on tests such as:

• Chest X-ray Although this is often the first test given in cases of suspected lung problems, a chest X-ray isn't as effective as a CT scan in diagnosing interstitial lung disease. It can, however, help eliminate conditions that cause signs and symptoms similar to those of interstitial lung disease, including emphysema and a collapsed lobe of one of the lungs.
  
• High-resolution computerized tomography (HRCT) scan Whereas a traditional chest X-ray produces two-dimensional images of your lungs, a computerized tomography scan uses an X-ray-sensing unit and a large computer to create cross-sectional images that are far more detailed. A high-resolution CT scan goes even further, showing lung tissue in great detail and providing more information than conventional CT scans do.
  
  
• Pulmonary function tests (PFTs) These noninvasive tests check how well your lungs function. For the test, you're usually asked to blow into a simple instrument called a spirometer, which measures how much air your lungs can hold and the flow of air in and out of your lungs. As scarring becomes worse, you're able to take less air in and blow less out. This part of the test takes just a few minutes. Full PFTs, which give far more information and take longer, can measure the amount of gases exchanged across the membrane between your alveolar wall and capillary membrane.
  
  
• Exercise tests: Because symptoms of interstitial lung disease are worse when you're active, your doctor may assess your lung function while you exercise, usually on a stationary bike or treadmill. Although specific tests vary, your blood pressure and blood oxygen levels are usually monitored as the difficulty of the exercise increases.
  
  
• Bronchoscopy (transbronchial biopsy) In many cases, interstitial lung disease can be definitively diagnosed only by examining a small amount of lung tissue (biopsy). In a transbronchial biopsy, your doctor passes a flexible, fiber-optic tube (bronchoscope) through your mouth into your lungs and removes one or more tissue samples, each about the size of the head of a pin. These are then examined in a laboratory. Bronchoscopy is performed on an outpatient basis using local anesthetic.
  
  
• Bronchoalveolar lavage In this procedure, your doctor injects salt water (saline) through a bronchoscope into a section of your lung, and then immediately suctions it out. The withdrawn solution contains cells from the air sacs. Although bronchoalveolar lavage samples a larger area of the lung than other procedures do, it may not provide enough information to diagnose a specific interstitial lung disease. Instead, doctors often use it to check the progress of a lung disorder or to help determine the best treatment.

• Video-assisted thoracoscopic surgery: When less invasive tests don't yield a specific diagnosis, a thoracic surgeon may perform a surgical lung biopsy. In this procedure, a flexible tube with a camera (endoscope) is inserted through a small incision between your ribs, allowing the surgeon to view your lungs on a video monitor. Surgical instruments are then inserted through another incision, and the surgeon removes thumbnail-sized tissue samples from two or three sites in your lungs.

Because video-assisted thoracoscopic surgery allows a surgeon to make small incisions in your chest wall rather than a long cut between your ribs, you're likely to have less pain and to heal more quickly than you are with traditional open lung surgery. Risks of the procedure include infection, bleeding, an air leak in the lung wall and pneumonia.