• Does it pay to call or write to the editor?
  • You bet it does. Tom, from Seattle, wondered why we had so little information about Restrictive disease in the Second Wind. That is a good question and something that had been of concern to your editor for some time. The truth is, there is just not that much information available about this complicated condition. Tom’s plea spurred us to contact many University Centers that work with patients with different types of restrictive disease. We wanted to know what was new out there and what written information that might be suitable for the Second Wind. Dr. Cecilia Smith at the University of California San Diego promised to help us out. Dr. Julie Woods, who works with Dr. Smith, wrote the following introductory article for us.

  • Pulmonary Fibrosis
  • It is a chronic lung disease due to excessive lung scarring that usually affects individuals of middle age or older. It is an uncommon disease affecting approximately 70 people of every 100,000 as compared with asthma, which affects approximately 280 people of every 100,000. Often the first symptom noticed is a gradual onset of shortness of breath with exertion, accompanied by a dry cough. As the disease progresses, the scarring and inflammation within the lung causes increasing shortness of breath eventually leading to the need for oxygen. The disease progression can be rapid or very gradual, and varies greatly from person to person. Lung biopsies can sometimes help with the diagnosis, but the cause in some cases cannot be determined.
  • It is known to be associated with exposure to certain agents such as asbestos, but can also be associated with diseases like rheumatoid arthritis, or exposure to certain drugs. When a cause cannot be determined, the disease is called “idiopathic” pulmonary fibrosis (meaning no known cause).
  • There is no known cure. Treatment includes steroids, which help decrease the inflammation and cytotoxic drugs (similar to drugs used for cancer) such as Imuran or Cytoxan (both taken orally), which help restructure the lung. Some patients respond to these drugs and are able to carry on a normal lifestyle, others, however, do not and the disease continues to destroy normal lung tissue. Transplantation may also be an option.
  • As with any chronic illnesses, it is extremely important to become well educated about the disease; knowledge, emotional support and psychological counseling are the cornerstones of coping. For this reason Dr Cecilia Smith and Dr Julie Wood, who run the Interstitial Lung Disease Clinic at UCSD, have started a regional support group in San Diego. These meetings are very interactive and focus on the sharing of information, feelings and solutions to problems. The meetings usually start with a coordinated short lecture on a specific topic with time for questions afterward.

  • For more information about these meetings please contact Julie Wood, M.D. at her E-mail address, jwmcclure@mindspring.com. In addition to being a transplant center, and having a support group, Dr. Smith has long had a list through which she tracks those with IPF (idiopathic pulmonary fibrosis) and hopes to learn more about this condition.
  • We hope to have other articles on restrictive disease written for us by Dr. Wood in future issues of the Second Wind.
  • Another resource, for those of you wishing more detailed information about restrictive disease, is The Coalition for Pulmonary Fibrosis in San Francisco. Their informative web site is www.coalitionforpf.org. Mark Shreve is the Executive Director and can be reached at the toll free number of 888-222-8541.