Complications of IPF can be divided into several categories.
Complications related to Pulmonary Fibrosis and disease progression
- Worsening shortness of breath is a predictable feature of Idiopathic Pulmonary Fibrosis as the disease progresses. Strategies to help include pulmonary rehabilitation, use of oxygen, and even low doses of narcotic medications as the disease becomes very advanced.
- Hypoxia (low oxygen levels) is the rule not the exception as IPF advances. Your doctor will assess your oxygen saturation at rest and with activity at each visit. Medicare has rigid criteria for when oxygen will be reimbursed. Saturations less than 89% are generally required. Your physician may also check your oxygen saturations while you sleep using an overnight oximetry test. You will wear a small probe on your finger that is connected to a recording device while you sleep. The results are sent to your physician. Reduced oxygen levels during sleep are common and easily dealt with through nighttime oxygen. Patients often feel much better when this problem is identified and treated.
- Pulmonary hypertension refers to increased pressure within the blood vessels of the lungs. Mild pulmonary hypertension is common in advanced Idiopathic Pulmonary Fibrosis. In some patients there is an exuberant increase in pulmonary artery pressures relative to the severity of the lung disease. Special medications may be used to help treat this complication. It is wise to see an expert in pulmonary hypertension if you are told that you have more than mild pulmonary hypertension, as the medications are complex and expensive. Furthermore, there are a variety of clinical trials that are ongoing to address the best approach to treating this problem.
- Cough is a vexing and common symptom of IPF. Cough severity is not clearly related to severity of Pulmonary Fibrosis. Most patients with IPF will experience cough as part of their disease. I have devoted an entire page to the approach to cough.
- Flares or exacerbations are discreet episodes of increased respiratory symptoms that evolve over a few days to a couple of weeks. Patients note increased shortness of breath and often cough. Worsening oxygenation and declining pulmonary function tests are also seen. These episodes should be taken very seriously. An evaluation for infection and possibly blood clots may be undertaken. Milder exacerbations are often treated with a brief course of steroids with or without antibiotics. More severe exacerbations require hospitalization and aggressive broad treatment that includes steroids, antibiotics and often blood thinners if a blood clot in the lung can’t be excluded. Increased oxygen is also often required.
Complications related to having structurally abnormal lungs
- Pneumothorax occurs when a part of the lung pops or tears resulting in air leaving the lung but remaining within the thorax or chest. This lung collapse can be a life-threatening problem. Patients will have sudden onset increased shortness of breath with or without chest pain. This is an emergency and generally requires a chest tube be inserted into the pleural space (space around the lung inside the chest but not within the lung) to re-expand the collapsed lung.
- Pneumonia is a common problem especially as patients get older. It is unclear if patients with IPF are at increased risk for pneumonia or if they just tolerate pneumonia much less well. All patients with IPF should receive age appropriate vaccinations to prevent bacterial pneumonia and influenza. Current guidelines recommend Pneumovax and Prevnar (2 different types of bacterial pneumonia vaccines) and annual influenza vaccination. It is impossible to get pneumonia or influenza from the vaccines. However, many patients will feel achy or even develop a low-grade fever for a day or two. These symptoms are your body building an immune response that will help prevent infection.
Separate diseases that occur more commonly in patients with Pulmonary Fibrosis
- Lung cancer may be more common in patients with Idiopathic Pulmonary Fibrosis. Although the data is not entirely clear, there does appear to be an increased risk for developing lung cancer even when accounting for prior tobacco exposure. Should we screen patients with IPF for lung cancer by doing CT scans? At present the answer is no. However, areas of fibrosis that do not appear typical for IPF should raise concern and not be ignored. How best to biopsy and treat IPF patients is a complex problem and requires taking into account IPF-related prognosis, patient age and comorbidities and patient preferences. Rarely are patients with IPF candidates for surgery to remove a large part of their lungs. Increasingly, specialized radiation therapy is being used to treat early lung cancer in this group of patients.
- Depression and anxiety are common in all patients with advanced lung disease. Declining capabilities is terribly frustrating and symptoms of cough and shortness of breath can lead to increased anxiety symptoms. The best approach to dealing with these symptoms is to discuss them with your doctor and your family. There are a variety of approaches that are effective including talk therapy and medications. Pulmonary rehabilitation programs are also helpful. Not only does pulmonary rehab provide education about breathing techniques, exercises that improve aerobic and muscle condition but there is also an important social component to participating.
- Gastro-Esophageal Reflux (GER) is very common in patients with Idiopathic Pulmonary Fibrosis and aggressive treatment is warranted. Use of acid suppressing medications, elevation of the head of the bed, and avoiding late meals are simple measures that are helpful.
- Pulmonary embolism occurs when a blood clot that usually started in the veins of the legs breaks loose and travels to the lungs. Patients with IPF are probably at increased risk of this problem. What is certain is that IPF patients don’t have the pulmonary reserve (extra lung capacity) to tolerate pulmonary embolism very well. As a result, even small clots can cause big problems. To prevent these blood clots, you should avoid prolonged periods of immobility—if you travel, be sure to stretch your legs and walk frequently. If you require hospitalization, your doctor will use “leg squeezers” or medications to prevent the development of blood clots. Once formed, blood clots are treated with blood thinners.
Diseases that are common but not clearly related to IPF
- Coronary artery disease (CAD) is a very common problem as we age. Unfortunately, IPF is not protective. New chest discomfort or shortness of breath that does not follow the usual pattern should prompt consideration for CAD. Medications are now very effective in treating CAD and preventing heart attacks.
- Upper respiratory viral infections are the most common infection in America. Though not usually life threatening, they can causes severe symptoms in patients with pre-existing lung disease. Patients that use oxygen often find that the nasal congestion that accompanies a cold interferes with effectively using their nasal cannula. Over the counter treatments such as antihistamines are generally safe and provide modest symptoms benefit. Decongestants are often more effective but you should check with your physician before using medicines like Sudafed or Phenylephrine.