Idiopathic Pulmonary Fibrosis
Management of the rapidly progressive disease known as idiopathic pulmonary fibrosis (IPF) requires a confident diagnosis, access to clinical trials for promising new treatments, and precise timing for lung transplantation. This uncommon disease causes inflammation and scarring deep in the alveoli and their supporting interstitium. Unlike other idiopathic interstitial pneumonia's idiopathic pulmonary fibrosis is resistant to known medical therapy such as corticosteroids, and cytotoxic agents.
Early referral of IPF patients is critical
Early referral of patients with potential IPF to a specialty center such as the Temple Lung Center is critical. While survival rates after diagnosis vary widely, in general the rates are highest when the disease is diagnosed at a young age, in an early stage, and when the initial response to medications may be good. Only prompt referral and diagnosis will give patients with IPF access to the appropriate clinical trials or lung transplantation.
Temple pulmonologists have expertise in making a prompt and accurate diagnosis of IPF and in managing difficult-to-treat cases of fibrotic lung disease. Specifically, our pulmonologists are highly skilled in assisting with the crucial radiological and histological evaluations; the differential diagnosis; the management of concomitant IPF and treatment of pulmonary hypertension; the initiation of immunosuppressive therapy, experimental anti-fibrotic agents, and pulmonary rehabilitation; as well as the critical timing of the transplantation referrals for deteriorating, oxygen-dependent patients with severe functional impairment.
Exploring the best surgical and medical options for IPF patients
Although there is still no cure for IPF, a wider range of surgical and medical alternatives (eg, novel pharmaceuticals and biologicals) are now available. Temple currently has one of the largest lung transplant programs in the Mid-Atlantic region. Fully half of our newly waitlisted patients (July 2006 to June 2007) for lung transplantation had IPF as their primary disease1. See the separate section on Lung Transplantation for a description of the growing Temple transplantation program. Temple is also currently enrolling IPF patients in two clinical trials involving anti-fibrotics and in one nationwide registry.
Linking IPF patients into large referral programs with active transplantation and research programs is essential. Not only does this referral potentially allow patients to benefit from new therapies, it also offers them education, social support, exhaustive examinations by experts, and highly personalized assistance from a large team of professionals in maintaining a high standard of care. Please contact us for information on any of our ongoing IPF clinical trials and for specifics on patient eligibility criteria.
Temple is Asking:
What works best to slow IPF progression?
The Temple Lung Center is pleased to be participating as one of five Pennsylvania institutions in the Pennsylvania Idiopathic Pulmonary Fibrosis Registry (PA-IPF Registry). The registry will not only help clinicians and researchers learn more about IPF natural history, prevalence, and best methods of medical and surgical care but, equally important, will help your patients learn more about their condition and improve their access to advanced specialty care, including transplantation and clinical trials. Following the model of the cancer registries that now operate in almost every state, the new PA-IPF Registry is aimed at generating information that will be useful in understanding this disease and its burden and, ultimately, in improving treatment. In the short term, the statewide effort will also be extremely useful in expanding the number of IPF patients involved in clinical trials with novel combination regimens and experimental antifibrotic agents. Such trials are critical because most patients with IPF will not respond to standard steroid or cytotoxic therapies and the transplantation option is limited by the shortage of donated organs.
Click here for information on current IPF trials.
References
- Scientific Registry of Transplant Recipients, Program Specific and OPO Reports January 2008: www.ustransplant.org
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