Vinicio De Jesus Perez, MD
Dr. Vinicio de Jesus Perez received his MD from the University of Puerto Rico Medical School and completed an internal medicine residency at Massachusetts General Hospital. He completed a fellowship in pulmonary and critical care medicine in Denver, followed by postdoctoral research training at Stanford University. He focused on researching genetic and molecular mechanisms of pulmonary hypertension (PH) and idiopathic pulmonary fibrosis (IPF) and has devoted his clinical practice to diagnosis and management of these conditions. Dr. de Jesus Perez is presently assistant professor of medicine and staff physician of the Stanford Adult PH Clinic, where he trains fellows pursuing careers in PH and IPF. He is principal investigator of a research program with the ultimate goal of identifying new therapeutic targets to treat PH and IPF. As a medical professional belonging to a minority group, Dr. de Jesus Perez is involved in academic endeavors seeking to improve access of care for patients with disadvantageous ethnic backgrounds and promote diversity in medicine.
Q: I’ve developed a heavy, dry cough. How do I know if it’s something minor like allergies or something more serious like IPF?
Idiopathic pulmonary fibrosis (IPF) is a rare condition that affects mostly men between the ages of 60 and 70 years. It has an estimated incidence of three to nine cases per 100,000 each year in Europe and the United States. People with IPF present with progressive shortness of breath and dry cough over several months. While cough is one of the major symptoms associated with IPF, your doctor should first rule out more common causes of chronic cough such as allergies, chronic sinusitis, reflux, and asthma before initiating a workup for IPF. However, suspicion for IPF is heightened if cough is accompanied with persistent shortness of breath, decreased exercise tolerance, and fatigue.
Q: Are COPD and IPF different from each other?
Chronic obstructive pulmonary disorder (COPD) is a lung disease associated with persistent airway obstruction and airflow limitation. In contrast to IPF, COPD is a fairly common disorder that affects approximately 5 percent of the population and represents the third most common cause of death in the United States. Usually, people with COPD present with a history of progressive shortness of breath and chronic productive cough associated with heavy tobacco use. While tobacco use is also considered a risk factor for IPF, the association is not as strong as that with COPD. Common diagnostic studies such as pulmonary function tests (PFTs) and chest imaging (for example, chest X-ray or CT scan) can help clinicians differentiate between COPD and IPF. Those with COPD exhibit reduced expiratory airflows and abnormally large lung volumes (obstructive pattern). In contrast, those with IPF demonstrate elevated expiratory airflow and reduced lung volumes (restrictive pattern). Imaging studies in COPD typically show extensive lung tissue destruction, inflamed airways, and presence of large air pockets (bullae). In contrast, the lungs in IPF demonstrated a heavy scar burden as evidenced by peripheral, bibasilar reticular opacities associated with tissue destruction, honeycomb changes, and accompanying dilated airways (traction bronchiectasis).
Q: I was just diagnosed with IPF and don’t know how to tell my family. Do you have any tips?
Education is the key to understanding the implications of the disease and planning subsequent care. Your medical team can help organize a meeting with family members and caregivers to educate and answer questions regarding the disease and what their roles should be in your care, and discuss plans for end-of-life care. Also, I would recommend joining national organizations and patient support groups dedicated to raising awareness and bringing together people with IPF and their caregivers across the nation.
Q: Is there a specific diet I should be eating?
Obesity should be avoided as it can worsen IPF symptoms by reducing mobility and exacerbating shortness of breath. Therefore, weight loss should be a priority for people with IPF. In addition to exercise, you should avoid foods with high salt, fat, and carbohydrate content and substitute with a diet rich in fruits, vegetables, and protein. A nutritionist can help you develop a dietary plan and identify which specific foods to avoid.
Q: What kinds of treatments are available for IPF?
At present, there is no curative therapy for IPF. The cornerstone of IPF management is oxygen therapy. Most with IPF require supplemental oxygen to correct low oxygen levels at rest and/or with physical activity. Low oxygen directly contributes to exercise intolerance from muscle fatigue and can also lead to other complications of IPF such as depression, pulmonary hypertension, and heart failure. Pirfenidone (Esbriet) and nintedanib (Ofev) are two novel medications that can slow disease progression in people with mild to moderate IPF. At present, there is no evidence to suggest that one medication is superior to the other. The decision to start either agent rests on its availability, your preference, and awareness of potential adverse effects, such as diarrhea and liver function test abnormalities (Ofev) vs. nausea and rash (Esbriet). Medical management is usually complemented with supportive interventions such as pulmonary rehabilitation, avoidance of air pollutants (for example, tobacco smoke), respiratory vaccines (such as the yearly flu vaccine), and palliative care. For those with end-stage IPF, lung transplantation remains the only treatment option.
Q: Are there any complications that could arise if I don’t treat IPF?
IPF can be complicated with other conditions such as pulmonary hypertension, major depression, sleep apnea, and venous thromboembolism, among others. These comorbidities can further impact functional status, limit response to therapies, and reduce survival. In addition, people with IPF can develop acute exacerbations characterized by worsening shortness of breath, new lung infiltrates on chest imaging, persistent cough, and severe oxygen deficiency of the blood (hypoxemia). For these cases, admission to a hospital for respiratory support and aggressive medical management is mandatory to reduce the risk of respiratory failure. In advanced cases, intuition of mechanical ventilation may be required to support oxygenation.
Q: I was officially diagnosed with IPF a few years ago. How will I know if my condition is getting worse or if I’m just aging?
Regular follow-up is key to assessing disease progression and presence of comorbidities. You should see your clinical care team every three to six months and undergo assessment with PFTs, pulse oximetry, and exercise testing. If these studies show evidence of worsening, your physician will likely recommend further studies, adjust dosage of medications, and refer you to pulmonary rehabilitation.
Q: I’m having trouble sleeping through the night because of my coughing. Is there something I can do or take to help with this?
Chronic coughing is one of the most persistent and irritating symptoms of IPF. The mainstay of management is to treat and remove any contributing factors such as gastroesophageal reflux (GERD), smoking, sleep apnea, ACE inhibitors, allergies, asthma, and infections. Medication options include non-opiate antitussive medications such as oral guaifenesin (Mucinex), dextromethorphan (Delsym), or benzonatate (Tessalon Perles). If these strategies fail to alleviate your cough, a short trial of oral prednisone (Deltasone, Rayos) can be considered. Narcotic agents such as guaifenesin/dextromethorphan with codeine can be effective. But given the risk of dependence, this mediation should only be used briefly or as part of a palliative care program.
Q: What’s the best way for me to live a full life with IPF?
Remain active and avoid a sedentary lifestyle! Most with IPF are under the false belief that they should avoid exercise, but nothing could be further from the truth. Lack of physical activity not only contributes to deconditioning, but also adversely affects mood, nutritional status, and quality of life. If you’re unsure about the level of activity you can safely undertake, participation in a pulmonary rehabilitation program can provide guidance and boost confidence in you and your caregivers.
Q: What kinds of questions should I be asking my doctor about IPF?
No question is trivial when it comes to your condition. Be candid with your doctor at all times regarding your concerns and those of your caregivers. The most relevant questions to ask include the status of your lung function, the side effects of medications and possible need of lung transplantation. Also, you should discuss in advance any travel plans and potential physical activities to review necessary precautions and plan accordingly. Finally, be sure to discuss advance directives and end-of-life care options.