Treatments

How is IPF treated?

The clinical course of idiopathic pulmonary fibrosis (IPF) is highly variable and may be difficult to predict. As a result, strategies to treat IPF are highly individualized, based upon the specific patients’ medical history and other conditions (comorbitities). While there are currently no effective treatments or a cure for IPF, there are a variety of therapeutic options to help patients manage their condition and maintain their quality of life and activities of daily living (ADL’s).

Since there are currently no FDA approved therapies to treat IPF, many patients choose to participate in clinical trials. New, experimental therapies are tested for their effectiveness through clinical trials. It is very important that patients discuss this possibility of participating in a clinical trial with their physician upon diagnosis. It is through clinical trials that a cure for the disease will be found. Please visit the Research section of this Web page to learn more about active clinical trials in the United States.

Typical standards of care may include prescription therapies, supplemental oxygen, pulmonary rehabilitation, and lung transplantation. Lung transplantation remains the most viable course of treatment to extend the lives of those with IPF, and this option should be discussed with your physician as soon as you are diagnosed.

Therapeutic Options: For some patients depending on their diagnosis and biopsy, medication will stabilize their disease and some patients may benefit from their continuing usage. While there remains no consistent standard of care in the IPF community, the following medications are commonly prescribed in an attempt to treat your symptoms:

Corticosteroids: (Prednisone) Prednisone is used for suppressing the immune system and inflammation. It mimics the action of cortisol which is produced by the adrenal glands. Depending on the dose, prolonged therapy can cause the adrenal glands to stop producing its own cortisol. For this reason when prednisone is discontinued, it may be necessary to gradually lower or taper the dose to allow time for the adrenal glands to recover. Since Prednisone suppresses the immune system, it can potentially increase the frequency and severity of infections. Prednisone has many side effects including sugar intolerance (can worsen diabetes), weight gain, swelling, depression, anxiety, fatigue, and peptic ulcer (to name just a few). Individuals receiving prolonged treatment or higher doses need to be carefully monitored.

Cyclophosphamide (Cytoxan): Cytoxan is an anticancer drug and is used for its immune suppression properties. Cytoxan is frequently given in conjunction with Prednisone or may be given alone. While it is usually taken daily by mouth, in some instances it may also be administered intravenously, usually monthly for six months.

Azathioprine (Imuran): Although there have been some successful reports in a small number of individuals, its effectiveness has not been confirmed in a randomized clinical trial to-date.

N-acetylcysteine (NAC): NAC is a naturally occurring antioxidant. It can be taken orally and theoretically could prevent some of the oxidative injury that precedes fibroproliferation. A small non-randomized study demonstrated some improvement in lung function in patients with IPF. There are number of ongoing studies investigating the efficacy of NAC in combination with other drugs.

As with any medicine for any condition, patients should discuss specific treatment options directly with their physician to determine the best approach for your case.

Supplemental Oxygen Therapy: All the body’s functions depend upon delivery of a steady supply of oxygen. Because IPF inhibits an adequate transfer of oxygen into the blood stream, some patients may require supplemental oxygen. This helps to reduce breathlessness, enabling the patient to be more active. Some may need oxygen therapy all the time while others may only need it during sleep and exercise. By testing the saturation level of oxygen in your blood, your physician can tell if you require supplemental oxygen. If your doctor has prescribed oxygen, use it. Many patients are fearful that they will become “addicted” to oxygen. This just is not true.

Pulmonary Rehabilitation: Pulmonary rehabilitation has become the standard of care for people with chronic lung disease, and recent studies have demonstrated improvements in both exercise capacity and health-related quality of life in patients with IPF. (12). The goal of pulmonary rehab is to restore the patient’s ability to function without extreme breathlessness. These programs offer a variety of services and can be inpatient, outpatient or home/community based. The programs are “multidisciplinary,” meaning that the team includes nurses, respiratory therapists, physical therapists, social workers, dieticians, etc. The range of services includes: exercise training; breathing exercises and retraining; anxiety, stress and depression management; and nutritional counseling, to name a few. Another recent study recommended that pulmonary rehabilitation be considered as a standard of care for those with ILDs like IPF because of its potential to improve functional status and dyspnea. (13)

Lung Transplantation: IPF is now the leading indication for lung transplantation in most large centers. In 2009, at the Cleveland Clinic, University of Pittsburgh Medical Center, and number of other large transplant centers, over 50% of the lung transplants performed were for IPF. Transplantation can improve both longevity and the quality of life in properly selected patients who have no other significant health problems. Previously it was uncommon for individuals over the age of 70 to receive transplants, however as surgical techniques and outcomes have improved; more individuals over 70 are receiving transplants, and many medical centers have updated their age requirements to now include those over the age of 70.

Until recently, because of long pre-transplant wait times, early referrals were essential so that patients could begin accruing time on the transplant waiting list. Fortunately with a new lung allocation system (LAS) used by the United Network for Organ Sharing, or UNOS (www.unos.org), candidates are evaluated based on the severity of their disease, and as a result wait times for those with IPF have been dramatically reduced. Similarly, in many institutions, the one-year survival rate is over 90%. Transplantation is not without risk, and patients should discuss all the possible complications with their physician.