Idiopathic Pulmonary Fibrosis is a progressive disease that results in respiratory failure. For many years treatment focused on suppression of the immune system. Unfortunately, this strategy was not just ineffective but as we recently learned, harmful to patients. Until the approval of Esbriet and Ofev, there were no medicines that were proven to slow disease progression or improve survival.
With the approval of Esbriet and Ofev, we have embarked on a new era in IPF care. Treatments may now be divided into general supportive measures and disease specific therapies.
General Supportive Measures
Oxygen for Idiopathic Pulmonary Fibrosis
As the normal lung is replaced by scar tissue, the lung’s ability to exchange gas and deliver oxygen into the blood is impaired. If enough of the lung is involved, this can result in low oxygen levels in the blood. This is referred to as hypoxemia or hypoxia. We measure your blood oxygen level in two ways.
Noninvasive oxygen measurements are made with a pulse oximeter (these cost about $40-$100 at various stores and on-line). The pulse oximeter reads a saturation that measures the percentage of hemoglobin that is carrying oxygen. Normal values are between 96-100%.
A more accurate way to measure the amount of oxygen in your blood is with an arterial blood gas. This requires sticking a needle into the artery in your wrist and removing a few milliliters of blood. The oxygen tension is then directly measured. For most patients the non-invasive technique is adequate.
Oxygen is prescribed when your saturations are less than 88-89% either at rest, with activity or when sleeping. Resting oxygen saturations are generally higher than exercise oxygen saturations. Sleeping oxygen saturations are usually in between. Medicare has agreed to pay for oxygen when the saturations are less than 88-89%. There is nothing magical about that particular saturation. It is just convention that Medicare and now all the other insurers have agreed to pay for oxygen at that threshold. Medicare will require recertification, which entails your doctor documenting a low oxygen saturation and submitting the information to your payer.
Oxygen is delivered from tanks or concentrators via nasal cannula. Usual flow rates start at 2 liters per minute but may be increased as needed. Advanced delivery systems such as oximizer pendants can improve oxygen delivery for patients that require high flow rates.
A growing body of data supports a role for Gastro-Esophageal Reflux Disease (GERD) in the development and progression of Idiopathic Pulmonary Fibrosis. We routinely prescribe acid suppressing therapy such as H2 Blockers, including Famotidine (Pepcid) or Ranitadine (Zantac) or proton pump inhibitors such as Omeprazole (Prilosec). We also recommend elevation of the head of the bed and avoiding late meals.
Vaccinations for Idiopathic Pulmonary Fibrosis
All patients with lung disease irrespective of age (and all adults over 65) should get vaccinated against pneumonia. There are now 2 different vaccines (PneumoVax and Prevnar). PneumoVax is given as 2 doses separated by at least 5 years and separated from Prevnar by at least one year. Prevnar is given as a one-time dose.
Influenza vaccination should be given yearly. There is no risk of getting pneumonia or flu from these vaccines. They are inactivated. You can expect to have a low grade fever and feel achy. This results from your immune system being activated to make a response that will protect you from getting sick.
Pulmonary Rehabilitation (Rehab)
Pulmonary Rehabilitation is a structured exercise program that focuses on both aerobic and strength training. In addition, patients are educated in the proper use of their medications and oxygen. Patients also learn about their lung disease from trained respiratory therapists and exercise physiologists.
The advantages of pulmonary rehab have been well proven over the years. Patients are able to exercise more effectively, feel better and have an improved sense of well-being. The social context of a group exercise class is also important in helping patients cope with a progressive disease.
Cough can be one of the most vexing symptoms of Idiopathic Pulmonary Fibrosis. The severity of cough is not related to the severity of the fibrosis. Some patients with early/mild disease have severe cough. Treatments include over the counter lozenges, prescriptions medications such as dextromethorphan, codeine, benzonatate (Tessalon Pearls), and even nebulized lidocaine. Some small studies have reported improved cough with Thalidomide. Insurance reimbursement limits access to Thalidomide. There is no one best medication. Your physician should work with you to help find the best strategy.
Immunosuppression for Pulmonary Fibrosis
For 30 years, the experts in IPF advocated for suppressing the immune system of patients with IPF using medications like prednisone and Azathioprine (Imuran). In a recently published NIH-sponsored study, these medications were found to be ineffective and increased the risk of infections. In general there is no role for immunosuppression in the routine management of stable IPF. There are exceptions for patients with acute or sub-acute exacerbations of their disease. Steroids remain the standard of care under these circumstances. Another use for prednisone is for refractory cough. Some patients experience improvement in their cough with low doses of prednisone. Your doctor will discuss the pros and cons of this approach.
NAC (N-acetyl cysteine)—Mucomyst
A recent study showed that this medication is not helpful. Unlike immunosuppression, there is probably no harm to taking this medication. However, I do not recommend it for my patients.
Disease Specific Therapy
Esbriet (Pirfenidone) to Treat Idiopathic Pulmonary Fibrosis
This is the first medicine approved to treat IPF. It’s mechanism of action remains somewhat poorly understood. It is described as an antifibrotic agent. It turns down the process of scar formation. The medicine was approved on the basis of three large studies completed over the past decade. The data shows that it slows down the decline in lung function over time.
Furthermore, when the three studies are viewed together, the medicine improved overall survival. Esbriet is taken three times a day. Patients start taking one pill (267mg) three times a day and then a week later they increase to two pills three times a day and a week later they increase to the final dose of three pills (801mg) three times a day. The most common side effects are gastrointestinal. Stomach upset was the most common. The drug makes patients very sensitive to sun exposure. Patients must use SPF 50 sun block, hats and long sleeves if they are in direct sunlight. Failure to take these precautions can lead to an impressive rash in sun-exposed areas.
Lastly, patients should have monthly blood tests to monitor their liver function for the first six months then quarterly thereafter.
OFEV (Nintedanib) to Treat Idiopathic Pulmonary Fibrosis
This is the second medicine approved to treat Idiopathic Pulmonary Fibrosis. In studies it was shown to slow the decline in lung function. The mechanism of action involves turning off signaling molecules that are overactive in IPF. The full dose is 150mg twice daily. Like Esbriet, the most common side effects are gastrointestinal. Some patients may require a dose reduction. Blood tests are required to monitor liver function monthly for the first six months then quarterly there after. There are reports of bleeding and clotting as well. Patients should report any unusual bleeding immediately.
Beware of Scams
Stem Cells Have no Role in Treating Idiopathic Pulmonary Fibrosis
As of 2015, there is no role for stem cell transplantation in the treatment of IPF. This has not been shown to be helpful. Unscrupulous practitioners are now offering this pseudo-medical intervention at a hefty price. Not only is this intervention not helpful, it carries risks.
These are marketed as nutritional supplements and the claims of their efficacy are quite extraordinary. If you read their testimonials, the same set of supplements will cure most any disease. None of the claims have been verified by the FDA, nor are there good quality scientific data supporting their claims. Testimonials are useless as a means of evaluating a drug’s efficacy. Buyer beware. You will be parting with substantial amounts of money and there is no good scientific data supporting their benefits.
Transplantation for Idiopathic Pulmonary Fibrosis Patients
For patients who have more than very mild disease, consideration and active discussion about lung transplantation should be part of the treatment landscape. Many patients will have obvious exclusions such as advanced age, cancer or complex medical comorbidities or obesity. However, for patients who are otherwise healthy and not markedly overweight or underweight, lung transplantation remains the most effective treatment option. This is especially true for patients with advanced disease or who are progressing rapidly.
Many patients with Idiopathic Pulmonary Fibrosis will eventually die from their disease. While we strive to extend the duration of life for our patients, there are circumstances where quality of life becomes the primary treatment objective. For patients with advanced disease and severe symptoms, palliative care is appropriate. Through collaboration with hospice agencies we are able to keep our patients comfortable, surrounded by family and loved ones and often in their own homes. The dying process is challenging for patients, families and even their doctors. An open discussion about the dying process helps patients and families cope.