“Idiopathic pulmonary fibrosis is a specific form of progressive, fibrosis interstitial pneumonia of unknown etiology.”1 More common in older males, IPF has not given up any of its secrets for over 50 years. Despite all the data and research a lot is still unknown, causing little progression in knowledge and patient mortality un-changing. An estimated 30,000 cases of IPF are found in the United States per year with a median survival rate of 3.2 years (which rivals many forms of cancer). The most common clinical complaint is breathlessness, especially on exertion. Other clinical findings that suggest IPF include but not limited to; a nonproductive cough, PFTs revealing restrictive physiology, and a decrease in total lung capacity, forced vital capacity and diffusing capacity for CO. Pulmonary function declines steadily over the course of the illness and may be episodic and unpredictable. Patients can experience stabilization over the course of IPF but never any ...view middle of the document...
The most prevalent way to diagnose IPF is through lung biopsy. Lung biopsy is extremely unsafe for IPF patients and most of the time cannot be performed due to the progression of the disease. Due to this problem researchers have found that searching for specific biomarkers is much more effective and safe for the patient. SP-A (surfactant protein-A) is the first biomarker found to have clinical significance on IPF. One study founded that elevated SP-A serum levels help to better distinguish between IPF and other ILDs (interstitial lung diseases).3
Many treatments that have been used in past IPF patients have been proven ineffective and harmful. Studies have shown that patients taking anti-inflammatory mediations, versus those patients taking a placebo, had a significant increase in progression of the disease over a 32 week period. With this new knowledge there is little that clinicians can do for patients as far as giving medications. The only treatment that has been noted to extend life for IPF patients is a lung transplant. Even this treatment is hard to come by due to the fact that most IPF patients are over the age of 65. In order to be considered for a possible transplant the patient would need to be under 65 years of age, with no contraindications, and to maintain SPO2 >89%.3 Because of the lack of treatment certain care measures can be taken by the therapist and/or clinician in order to provide a comfortable end of life setting.
1) Disease-centered management
2) Symptom-centered management
3) Education and self-management
These are the 3-pillars of supportive care for patients with IPF according to Debbie Lewis and Jane Scullion. With effective care and knowledge IPF can progress slowly rather than quickly.4
With more awareness and research IPF can be better diagnosed, and treated. Researching and studying the etiology can be a pivotal turning point in the study of this disease and for all other interstitial lung diseases. With more and more research the medical community has found that most treatments have not worked, the previously thought etiology is incorrect, and biomarkers are becoming more significant. In the medical research setting, being able to prove something wrong is the first and most imperative step.