Help Steve Kivlen Fight Idiopathic Pulmonary Fibrosis
Organized by: Steve Kivlen and Friends of IPFCure
Steve Kivlen has been diagnosed with Idiopathic Pulmonary Fibrosis and the Friends and Family of Steve wish to raise funds for him for Medical Treatment and Research.
- Steve's Story? (Click for Watch)
- What is IPF? (Click for Watch)
- Treatment Options (Click for Watch)
- Additional Stories
About the Disease
Pulmonary fibrosis is a chronic disease that occurs when scar tissue and, at times, inflammatory cells build up in the lungs, making it difficult to breathe. This process of scarring and inflammation, the former of which is referred to as fibrosis, may be caused by inhaling a toxin (like asbestos or cigarette smoke), or as the result of taking certain medications, radiation therapy, or an autoimmune disease. Often, however, the cause of fibrosis is never determined, in which case the patient’s condition is known as idiopathic pulmonary fibrosis (IPF).
Over time, progressive fibrosis prevents the lungs from transmitting oxygen into the bloodstream effectively. This causes patients to cough and experience shortness of breath, and can lead to other medical problems, such as lung infections, blood clots in the lung, lung cancer, and coronary artery disease. Over time, patients lose the ability to breathe and may experience lung failure.
Idiopathic pulmonary fibrosis is one of several disorders known together as interstitial lung disease (ILD), because they affect the lining of the lungs’ air sacs, which is known as the “interstitium.” There are more than 100 forms of ILD, including various types of idiopathic interstitial pneumonia, hypersensitivity pneumonitis, and sarcoidosis. IPF is one of the most common forms of ILD.
How is pulmonary fibrosis typically treated?
Every patient is unique. For some, fibrosis occurs quickly. For others, the scarring happens more gradually and may level off. Treating an interstitial lung disease varies from patient to patient, according to the degree of fibrosis and the amount of inflammation in the lungs. Inflammation may be reversible or, potentially, controlled with medical therapies such as steroids (like prednisone) or stronger medications, called immunosuppressive agents (such as azathioprine, mycophenolate mofetil, or cytoxan). The decision to use steroids or immunosuppressive medications depends on each patient’s unique situation. Notably, fibrosis is not responsive to anti-inflammatory therapies.
Other treatments include oxygen therapy to raise the level of oxygen in the blood and pulmonary rehabilitation—that is, rehab for the lungs through exercise, nutrition, and energy-conserving and breathing techniques. In severe cases of pulmonary fibrosis, a patient may require a lung transplant. Researchers at CUMC and other institutions are currently exploring new treatment methods through clinical trails.
What causes pulmonary fibrosis?
Pulmonary fibrosis can be caused by an injury to the lungs that results in scarring as the lungs heal. This lung injury may be caused by inhaling a toxin such as coal dust, asbestos, or silica dust; smoking; or as a result of radiation exposure, certain medications, or an autoimmune disease. Most of the time, however, the exact cause of pulmonary fibrosis is never determined, in which case the condition is known as idiopathic. The word “idiopathic” indicates that a disease arises spontaneously or for unknown reasons. Genetics may be a cause of IPF in some patients, as a number of genes and genetic variants have been associated with IPF. Research conducted by physician-scientists at Columbia University Medical Center is looking into the causes of interstitial lung disease, and examining the role of genetics in the rise of ILD. [link to Research page]
Is there a cure?
Currently, there is no known cure for pulmonary fibrosis. Once the lungs have been scarred, there is no way to reverse the damage. Care for patients focuses on slowing down the scarring process, and on managing and lessening symptoms. Patients with severe fibrosis may ultimately require a lung transplant