Family Health Pulmonary Hypertension Q&A By Pulmonary Hypertension, June 5, 2014 Ask the Expert: M. Patricia George, MD Question: What are the different types of lung disease? Answer: Lung disease is a huge category that includes many diseases. One simplified way to think about the different types of diseases is by where in the lung they target. For example, there are diseases that attack the airways (the tubes in the lungs) such as COPD, asthma and bronchiectasis. Then there are diseases that affect the lung tissue such as pulmonary fibrosis, coal miner’s lung. There are also diseases that affect the blood vessels such as pulmonary hypertension or pulmonary embolism (blood clot in the lungs). And then there are diseases that can affect multiple parts of the lung, such as sarcoidosis which can affect the tissue or the airways or blood vessels. And lung cancers can come from different areas of the lung depending on the type of cancer. Question: What do healthy lungs do that those with lung disease don’t? Answer: Healthy lungs are responsible for respiration – the process of getting oxygen into our bodies and also getting rid of carbon dioxide. Our bodies need oxygen for cells to be happy and function properly. We breathe nearly 25,000 times per day, and normally this all happens in a very efficient manner – so efficient and automatic that we are not usually aware of it. That is until we have lung disease. Lungs can become diseases in different ways that affect how this process happens, but in general when lungs become impaired in their function, this process requires more energy and effort. Examples would be an asthmatic who becomes short of breath and very uncomfortable from narrowed and inflamed airways, or a patient with a serious pneumonia who has an infection that is filling the air spaces and not allowing this process to happen efficiently. Question: How can I decrease my chances of lung disease? Answer: Don’t smoke, or if you smoke cigarettes, quit. This is the number one step to decreasing chances of lung disease – especially COPD and lung cancer. Avoid second hand smoke. Test your home for radon (while cigarette smoking is by far the greatest risk factor for developing lung cancer, radon is the second leading cause of lung cancer in the United States). Avoid working with asbestos (and if your job requires it wear protective equipment) and protect yourself from dust and chemical fumes. Talk to your doctor about whether influenza and pneumococcal vaccinations are appropriate for you. Question: If someone has pulmonary arterial hypertension (PAH) because of a Congenital Heart Defect and Bi-directional Shunting, is it possible they could also have PH because of Lung Agenesis/Hypoplasia or Restrictive Lung Disease? Answer: Thank you for your question. Pulmonary hypertension can definitely be complicated, and can be due to a combination of causes. And while congenital heart defect is likely the biggest contributor, there have been reports of pulmonary hypertension in approximately 19 percent of cases of unilateral agenesis of the lung (though it is more common in agenesis with someone who also has a left-to-right shunt) (Capuani et al. 1982). And yes, restrictive lung disease can contribute to PH as well. Question: I am 72 years old, and I had Asthma as early as I can remember. In 2008, after frequent admission to the hospital without success of medications, I was then diagnosed with pulmonary hypertension. Do you think I had PH after all these years, or is it possible the Asthma turned into PH? Answer: It is unlikely that asthma would “turn into” PH. What may be the case is that you have two lung conditions, or as you suggest, that your symptoms were in fact due to PH. Without the details of your case I cannot comment specifically on whether either of these is true (or another explanation altogether). However I will say that it is not uncommon that PH goes undiagnosed for years before the correct diagnosis is made. That is one reason we are working so hard to raise awareness about the disease – to physicians and non-physicians alike. Question: The doctors told me I have PH about a month ago, and they said it was mild. I’m very scared and on no medication right now. I have another echo In June, and I currently feel okay and am still going about life. What is in my future with this? Answer: Thank you for sharing your question and what you are feeling right now about having a new diagnosis – and undoubtedly a lot of questions that come with it – is completely understandable and overwhelming. The good news is you “feel ok” and that they said it was mild, but the next steps I would recommend are discussing with your physician more about pulmonary hypertension. Ask them what the next steps are and whether you need more testing or to see a PH specialist (even if only for a second opinion). Also, there is a lot of good information (and not so good) info on the internet about PH (as well as other lung diseases). I highly recommend going to the Pulmonary Hypertension Association website. This is a fantastic organization that can provide you resources both online as well as connect you with people who live with PH and can support you in this. You are not alone. Question: I was diagnosed with IPAH last year and right heart failure which caused three heart attacks. I was really sick all winter but the summer before as I kept my normal walking and physical activities I didn’t have to be admitted. I did nothing in the winter but stay home doing nothing so I was hospitalized five times. I started my walking and am currently doing three miles a day without excretion keeping my Oximeter on and monitoring my HR. Will physical activities cause my PAH to get worse? Thank you for your question and sharing your story. I’m sorry to hear you had a rough winter. It certainly was a rough (and long) one for all of us, especially those with respiratory conditions. In general, I am a big advocate of exercise when PAH is well-treated and the patient is seeing a PH specialist regularly. Many PAH physicians agree that not only is exercise not harmful but that it may even be beneficial. That said, it is always important to talk to your PH specialist before starting an exercise program of any kind, and it is important to not exercise beyond the point where you have symptoms (light-headedness, chest pain, severe shortness of breath). In patients who are starting out, pulmonary rehabilitation programs can be helpful in teaching safe limits of exercise as well as well-rounded programs. Plus, when you can do it, exercise is a part of actively taking an element of quality of life back. Good luck! Question: I have PH and was diagnosed month ago. I also have sleep apnea. My doctor said I have a mild case. What is this disease outlook? Answer: Although I do not have the details of your case to be able to talk to you about overall outlook, I will make a general statement to say that if you have mild PH, talking to your doctor about possible treatments as well as current risk factors that can make it worse would be helpful. One such risk factor is sleep apnea. If you have untreated sleep apnea, which has been associated with pulmonary hypertension, treating it may help your condition. I recommend talking to your primary doctor and sleep doctor about this. Good luck. Question: For those that are newly diagnosed with PH, how does a physician know which drug will be the best fit fit their patient when they determine they are in fact dealing with PAH. Answer: After making that first step of establishing the diagnosis, of course the next challenge is deciding which treatment options are best to start. When a PH doctor makes a diagnosis of PAH – pulmonary arterial hypertension – which means high blood pressure in the lungs due to a disease of the arteries in the lungs, the doctor also assesses other aspects of the patient’s case. How sick is the patient at this moment and how have they felt recently? Is there involvement of the right side of the heart in their disease? Do they have any underlying heart or lung disease or blood clots? Have they been on any therapies before? These are some of the questions that a PH doctor will try to answer before initiating therapies, as different treatments help depending on the patient’s whole condition. Question: My doctor’s philosophy is to wait for transplant until the last possible minute. Other doctors I’ve seen think it’s time now. What markers should be used to decide when it’s time? Answer: In general, when patients are getting worse despite medical therapy, and there seem to not be other options available, it is a reasonable time to at least consider lung transplant. As a transplant physician, I would rather evaluate someone too soon (and if the patient is too healthy for transplant, hold off on listing and just follow them over time in the event that they worsen) rather than too late in the disease (when it becomes more difficult to try to address any medical or other problems that increase a patient’s risk for transplant). Earlier evaluation allows the patient and family to learn more about transplant from the center, and address any concerns that may arise in the evaluation process. Question: Could scarring from pneumonia be a reason for interstitial lung disease? Answer: There are many forms of interstitial lung disease (ILD) (diseases that affect the lung’s structural tissue as opposed to diseases of the airways like COPD or asthma). And there are many known causes of ILD. While I would not necessarily attribute ILD to scarring from a focal bacterial pneumonia, it is believed that lingering active infections or chronic ongoing infections may contribute to developing ILD. Question: What are the factors that prevent you from being listed for lung transplant? Answer: As for factors that prevent one from getting a lung transplant, this is also a complicated question, and varies based on transplant centers. But active smoking or active substance abuse, not going to the doctor or taking medications as prescribed are major “red flags.” There are other factors that – depending on severity – may prevent one from getting a lung transplant such as other serious medical conditions (severe irreparable heart disease, kidney or liver failure, sepsis), but it is best to talk to a transplant center about the individual patient to explore whether transplant is an option. At our center we definitely evaluate patients on an individualized basis as everyone is unique. So while I cannot tell you current life expectancy after lung transplant, I can give you the median survival data from the latest data from the International Society for Heart and Lung Transplantation. In patients transplanted from 2004-2011 is a median survival of 6.1years. This means that at 6.1 years after transplant, 50% of recipients are still alive. This is not life expectancy per se, as we don’t really have good mathematical models to predict life expectancy after transplant. To learn more about pulmonary hypertension or CTEPH or to schedule an appointment with a pulmonary hypertension specialist, call 412-648-6161, or toll-free at 1-877-PH4-UPMC, or email PHprogram@upmc.edu.