For the first time, the FDA has approved the use of two medications as initial therapy for pulmonary arterial hypertension.  In response to the recently published AMBITION study results, the FDA has approved the use of Letairis (ambrisentan) and Adcirca (tadalafil) to be used as initial therapy in newly diagnosed patients with PAH.  Previously, many physicians would prescribe one therapy then a few months later add a second therapy.

AMBITION Study

I have previously described the results of this study.  As a quick refresher, this study compared Letairis to Adcirca to the combination of the two therapies together in previously untreated PAH patients.  The combination therapy group had a 50% reduction in the rates of clinical worsening.  Exercise capacity was also much improved in the combination therapy arm compared to either individual therapy.

How will this change current therapy for Pulmonary Arterial Hypertension?

A major question in the treatment of PAH has been whether to start multiple medications at the same time or to start them sequentially—start one therapy and see how much improvement is achieved then if needed add a second and then if needed a third medication.  We now have redefined the optimal care of PAH patients.  Newly diagnosed patients in general should be offered dual therapy with Ambrisentan and Adcirca.  A major remaining question is whether other similar combinations are just as effective.  Some experts believe that similar benefit could be obtained with Opsumit and Adcirca.  No definitive answer to this question is available at the present time.

What this means for PAH patients

Many insurance companies and pharmacy benefit companies were reluctant to approve two PAH medications as up-front treatment for newly diagnosed PAH patients.  With the FDA approval and the publication of the AMBITION study results, I am very optimistic that there is no basis to deny authorization for Letairis and Adcirca as initial combination therapy.  This should make it much easier for your doctor to get both medications approved.

One of the most common problems I see on a daily basis is that patients struggle to limit their salt intake to the 2,000mg per day goal for Pulmonary Hypertension patients.  Unlike fluid restrictions where everyone can measure precisely how much fluid they drink, keeping track of salt intake can be very difficult.

Let’s start with some basic terms.  Salt is the same as sodium chloride.  And salt is salt is salt.  On a weekly basis, patients ask me if sea salt is better than other types of salt.  The answer is no, salt is salt.  It does not matter if it is from Hawaii, if it is kosher or if it comes from salt mines in the Himalayas.

One teaspoon of table salt equals 2,300mg of sodium chloride.  A pinch of salt is about an eighth of a teaspoon that equals about 300mg of salt.  It does not matter if you add the salt while cooking or after cooking, it counts the same.

Where do you find out how much salt is in the foods that you eat?

All packaged food items that you purchase in a store are required to have a nutritional facts section on their packaging.  Mid-way down you will find “Sodium” and a number of milligrams.  This is the number of milligrams of sodium chloride (or salt) in each serving.  For example, a small bag of Lay’s Classic Potato chips has 270mg of sodium.  Now let’s consider a can of Progresso Chicken Noodle Soup.  Each serving has 690mg of sodium.  However, each can has 2 servings.  So, if you eat a can of soup you just consumed 1380mg of sodium.

Overall, the biggest problem that I find patients have is that as a culture, we love eating at restaurants.  The food is delicious, easy and quick.  However, the amount of salt in restaurant prepared food is shocking.  Take for example a Big Mac at McDonald’s.  There are over a 1,000mg of sodium in a single Big Mac.  That does not include the French fries, any added ketchup or mustard or a dessert.  If you prefer to dine at the Cheesecake Factory, consider the Thai Lettuce Wraps.  One order that serves 2-4 people has 2,347mg of sodium.  So if you have one quarter of an order as an appetizer, you just started your meal with about 600mg of salt.  Your main course will likely have about twice that.  By the end of your meal, you have probably consumed close to 2,000mg of sodium just for dinner.

Unfortunately, even if you try very hard to choose low salt items on the menu, they usually have more salt than you would think.  Salads are low in salt, but the salad dressing that makes them delicious is loaded with salt.  Grilled fish even has ample salt for “seasoning”.  Chefs seem incapable of avoiding a coating of salt on food.

In short, the only way to avoid eating enormous amounts of salt in a day is to cook from scratch and choose your ingredients carefully.  Just because you don’t add salt to your food does not mean that your food can’t taste good.  You can add herbs and spices to your heart’s content.  Fresh garlic and chili can liven up a meal.  Allow your guests to salt their food individually.

Beware of the high salt foods that we think of as healthful such as cottage cheese, tomato juice, cold cuts and even breads.  Opt for the low salt versions of these.  Avoid pickles, olives, and soy sauce.  Ditch the saltshaker and embrace fresh herbs.

Salt Substitutes

Lastly, salt substitutes usually contain potassium chloride.  For most patients, very small quantities of a salt substitute should not be a problem.  However, if your potassium runs high, then even a small amount may pose a risk.  It is best to check with your treating physician prior to using a salt substitute.

I received a great question from a reader this week.  The reader asked why some patients are quite disabled from Pulmonary Arterial Hypertension when they have only moderately elevated pulmonary artery pressures and other patients can have very high pulmonary artery pressures and yet remain very functional, even working full time.

This question gets at a key concept of PAH.  Even though PAH is defined by elevated pressures within the pulmonary arteries, how a patient feels is really related to how their right ventricle is able to compensate for the elevated pressures and resistance within the pulmonary arteries.

Let’s use an analogy to make this clear.  Imagine a house with an air conditioning system.  When the house was new and the AC system was installed, it ran great and the house in Phoenix was always cool, even during the summer.  Over the years, the windows and the doors in the house became less airtight and heat was able to enter the house.  Initially, the AC system had no problem keeping up with the added work.  It simply had to work harder/longer to keep the temperature inside the house comfortable.  After 15 years, the AC system began to function less well.  The compressor was less efficient and the condenser was not as effective.  Now the residents of the house began to notice that no matter how long the AC system ran, the temperature in the house never reached a comfortable 78 degrees.

In this analogy, the heart is the AC system and the degree of leakiness of the house for keeping out hot air is analogous to the increasing resistance of the pulmonary arteries in PAH.  Thus you feel ok when your heart can keep up with the added demands of diseased pulmonary arteries.  You notice symptoms only when your heart is unable to pump enough blood to allow you to do the activities that you desire.  No one complains that his or her pulmonary artery pressure is too high.  What they notice is shortness of breath and fatigue.  These are symptoms of inadequate blood flow.

The pulmonary artery pressure and blood flow at rest may not be predictive of what happens during exercise.  Some patients have quite mild appearing disease at rest but with minimal exercise they experience dramatic deterioration in blood flow and elevation of the pulmonary artery pressures.  We can sort this out in the catheterization laboratory by measuring your pressures and flows at rest and having you exercise during the procedure.  These patients are often under-treated because they appear to have mild disease when measured at rest.

Since November is Pulmonary Hypertension Awareness month, we like to reflect on the progress made on this site and in the PAH community in general.  2014 has been an important and busy year for pulmonary hypertension.  First, at Inspire Pulmonary Media, we would like to thank our readers for making this website a tremendous success.  Each month, thousands of people visit this site to learn about pulmonary hypertension and our readership is constantly growing.  We launched a new section of the site—“The Doctor is In”.  Readers have been suggesting PAH topics and we have been posting blogs addressing as many of those topics as we can.  Your feedback has been wonderful.

Continuing Progress in Pulmonary Hypertension Research

Elsewhere in PAH, the FDA approved the first upfront combination therapy for PAH (Letairis combined with Adcirca) and is considering approval of a second oral prostanoid receptor agonist (Selexipag=Uptravi).  Medtronic and United Therapeutics submitted information about the Synchromed II implanted infusion system for intravenous Remodulin.  We await the FDA’s decision.  We heard from a newcomer to PAH, Reata Pharmaceuticals.  This past October they announced positive results in their interim analysis of their phase II study with bardoxylone methyl.  Gilead Sciences also completed enrollment in their phase II study of a novel compound for PAH.

Overall, this year has seen a shift in focus from medicines that relax blood vessels towards medicines that address other aspects of pulmonary hypertension such as inflammation and oxidative stress (metabolic derangements in how the pulmonary artery and heart muscle cells generate energy).

Despite all the progress, we can do more.  We need to continue to strive to increase awareness and lobby our congressional representatives to increase funding for research.  Tell your stories to a friend.  Knowledge is power.  Empower the community.

What Can We Expect From 2016 in PAH?

There are some very exciting studies that are enrolling patients.  Actelion Pharmaceuticals is set to study a combination of three medications together as upfront therapy for PAH.  Reata will launch a phase 3 study of bardoxylone methyl in connective tissue disease associated PAH.  Belleraphon will start enrolling a phase III study of inhaled nitric oxide as add-on therapy in PAH.  We hope that Gilead will announce a phase III study of their newest molecule in the second half of the year.  Many companies are also expanding their studies to include group 3 pulmonary hypertension (PH due to lung disease).  There are many other studies that will start enrolling in 2016, visit clinicaltrials.gov for a complete listing.

2016 is also likely to see more PAH medications become available as generics.  Stay tuned for more information on this topic.

At Inspire Pulmonary Media, we are very excited about 2016.  We look forward to providing both useful and current information about PAH to our readers.  We value your feedback and invite you to suggest topics on PAH.

What is Sarcoidosis?

Sarcoidosis is an uncommon and poorly understood multisystem disease that commonly affects the lungs and lymph nodes.  However, it may affect any organ of the body.  Despite decades of research, the exact cause remains unknown.  The most common manifestation of the disease is enlarged lymph nodes in the chest and nodules and scarring in the lungs.  The skin, eyes, heart, liver and nervous system may also be involved.  Symptoms depend on the organs involved.  When sarcoidosis involves the chest, patients may have cough, wheeze, chest pain or shortness of breath.

For more than 20 years, pulmonary hypertension specialists have recognized that patients with sarcoidosis develop pulmonary hypertension.  Unlike many other causes of pulmonary hypertension, these patients seem to respond very well to treatment with medications approved for pulmonary arterial hypertension.  Unfortunately, there have been no large randomized studies of the treatment of pulmonary hypertension complicating sarcoidosis.  In fact, most studies in the past 2 decades have excluded patients with sarcoidosis.

Treating Pulmonary Hypertension with Sarcoidosis

Despite the lack of high quality data, PH-specialists have amassed substantial expertise in treating pulmonary hypertension in the setting of sarcoidosis.  Our general treatment paradigm involves using PDE5 inhibitors (Sildenafil/Revatio or Tadalafil/Adcirca) first line and then using continuously infused prostanoid therapy (Treprostinil/Remodulin or Epoprostenol/Flolan) for sicker patients.  We have had good results over the years.

We are very excited that Reata Pharmaceuticals, a new company in the pulmonary hypertension space, has announced plans to include patients with pulmonary hypertension due to sarcoidosis in their ongoing phase 2 study of bardoxylone methyl.  This is a major milestone.  Hopefully this will herald a new chapter in pulmonary hypertension studies where sarcoidosis patients are included.

I would like to acknowledge the role of our PH nurse coordinators and support staff.  The care of pulmonary hypertension patients is a challenge.  A typical new patient that I see has already been to a cardiologist and at least one other pulmonologist in addition to their primary care doctor.  They have had dozens of tests and perhaps been hospitalized a few times.

Intake Process

The process of caring for a PH patient begins with our “intake”.  This person is responsible for collecting all the records from all the physicians and hospitals that a patient has visited.  These documents often number in the hundreds of pages.  They are sorted and scanned into our electronic health record.  In parallel with collecting records, my intake team that is led by one of my two nurse coordinators helps to triage how urgently the patient needs to be seen and my authorizations team is ensuring that we have permission from your insurance to see you.  For patients that are doing poorly we try and see them within a day or two.  More stable patients are usually seen within a week or two.  As my schedule is usually booked solid for a couple months in advance, this requires my nurses to create new space (a magical process known as double and triple booking).

Your First Visit

Before the first visit, my team generally has obtained all your old records including recent CT scans and original primary data (not the interpretation of the data but the actual tests themselves).  Prior to your first visit, I review the documents and see if there are any critical pieces that we have yet to receive.  We make a last minute effort to fill any critical holes by calling imaging centers, hospitals, catheterization laboratories and lab facilities to do our best to have all your data ready for your first visit.

When you walk from the elevators into my office you are greeted by a great team of warm, friendly and efficient front desk staff.  They collect your insurance card and ask to take your picture.  Why do we take your picture?  In a typical week I can see as many as eight to ten new patients with similar problems.  I am much better at remembering faces than names.  Thus as a means of creating a personal experience for our patients we collect your picture.

Next, my medical assistants will take your vital signs and review your medication list that you have hopefully already entered electronically prior to your visit.  From there, you are taken back to an exam room if one is open or shown into the waiting room until an exam room becomes available.  At this point, you have yet to see your PH doctor but my team has already spent as many as several hours working to take care of you behind the scenes.

When you finally make your way back to the exam room, the medical assistant will ask you to sit in a chair that is designated for the “patient”.  We usually have two chairs in each exam room and one is strategically positioned so that I can sit at my computer and look directly at you.  This way I can input any data into the chart and still keep eye contact with you.  In the pre-electronic health record era, doctors spent more time looking at patients but now that the federal government has mandated that we use and electronic health record, we have had to make small sacrifices including spending a bit more time with our heads in our computers.

During our first visit, I will take a detailed history (even though I generally have already read about you in detail and have a pretty good understanding of your situation).  You would be surprised by the inaccuracies in the medical records.  Next, I will examine you and then go over the key results from the data that I have gathered.  We often do a six-minute walk test on the first visit and sometimes do breathing tests and a chest X-ray.  Then I will put together my impressions about your diagnosis and outline a plan for further diagnostic testing and treatments.  I often draw pictures on the white paper that covers the exam table.  I am no artist but I have had a lot of practice at drawing pictures of the heart and lungs.  I will do my best to answer all of your questions.  I write down the plan on a special sheet of paper that has a copy underneath that gets scanned into your chart.  This insures that if you forget the plan and lose the sheet of paper we have an exact copy.

Pulmonary Hypertension Nurse Coordinators

My nurse coordinators are often in the exam room for some of your first visit.  This allows them to get to know you a bit.  They also provide much of the education about salt and fluids and will explain the process of easily communicating with us through our web-based health portal or the old-fashioned telephone.

Once you leave the office, our work has just begun.  My authorizations team obtains permission from your insurance for any testing that I have ordered.  My nurse coordinators work to obtain authorization for your specialty medications, a process that can take half an hour or several days.   At the end of each day in the office, my team meets together to review each patient’s plan and identify key action items.

Just because you left the office does not mean that the work is done.  There is often hours of behind the scenes work.  My nurse coordinators push through prior authorizations and appeals to ensure that your expensive medications are approved.  They call and check up on you and answer those questions that you forgot to ask during your visit.

In between visits, there is also a beehive of activity.  When you call and ask for help with disability paperwork or are not feeling well, my nurses do the heavy lifting.  They work tirelessly to insure that your care is moving forward.

In short, I have the easy job.  My team does the heavy lifting.  Hats off to a great team that allows us to deliver great care to our patients.

The question of whether anticoagulation (thinning of blood with medications) should be used in the treatment of pulmonary hypertension dates back more than 35 years.  Very early observations of small blood clots in the lungs of PAH patients propelled interest in using warfarin (our oldest blood thinner).

In the 1980’s and 1990’s several small studies were completed that suggested that in patients with severe idiopathic pulmonary hypertension (formerly referred to as primary pulmonary hypertension) there was a survival advantage to using blood thinners.  During this time not all the data was consistent and 2 small studies showed no benefit to warfarin.

Two new studies were recently published adding further confusion to the question of whether blood thinners are helpful in PAH.  Both studies are registries (patient data was entered into a database in real time and they were tracked prospectively). Before I describe the study results, it is important to acknowledge that registry type studies can never definitively address whether a treatment is effective.  Because patients are not randomized to specific treatments or interventions, there is a high probability that the doctors caring for each patient made specific choices based on each patient’s clinical situation and these are not evenly distributed between the groups.

For example, imagine that there are two patients with PAH.  They both have severe disease with similar heart catheterization, echo and six minute walk findings.  One patient, however, has a problem with bleeding from her bowels.  As a result, this patient would not receive anticoagulation whereas the other patient would probably receive anticoagulation.  Now imagine that bleeding from the bowel is linked to a worse outcome in PAH.  The net effect is that it appears that blood thinners are an effective therapy but in fact, the blood thinners may just be a marker for identifying patients at lower risk for death.

COMPERA Registry

In 2007, a collaboration of European PAH centers embarked on a prospective registry study.  The COMPERA Pulmonary Hypertension Registry recently reported on their experience with anticoagulation in PAH.  This large database included 1,283 patients who were followed for 3 years.  Survival was better for idiopathic PAH patients treated with anticoagulation.

In contrast to the results seen in idiopathic PAH, patients with non-idiopathic PAH (patients with all other causes of PAH—such as connective tissue disease, liver disease, congenital heart disease), survival was worse in the PAH patients taking thinners.

REVEAL Registry and Anticoagulation in PAH

The REVEAL registry is the largest database of PAH patients with more than 3,500 patients included.  Dr. Preston and colleagues used this database with a nested-design study (a subgroup of patients from the total database were selected using careful criteria) to create 4 groups of patients.  Idiopathic patients treated with blood thinners and a comparison group not treated with blood thinners as well as a scleroderma associated PAH group of patients treated and a comparison group of scleroderma associated PAH not treated with blood thinners.

Their analysis showed that blood thinners made no difference in outcomes for idiopathic PAH patients.  In contrast, amongst the scleroderma associated PAH patients, warfarin use was associated with significantly lower survival.

How Do We Put All this Data Together and Make Good Clinical Decisions?

In my practice I do not treat patients with scleroderma-related PAH with blood thinners unless they have been diagnosed with blood clots in the legs or lungs or have atrial fibrillation (an irregular heart rhythm that increases stroke risk).  I also do not treat liver disease or congenital heart disease-related PAH with blood thinners.  Amongst my idiopathic PAH patients, I tend to treat those with more severe PAH with blood thinners but I now engage in a discussion that highlights the uncertainty in the literature.

As we look forward, we clearly need a high quality prospective randomized placebo controlled clinical trial of blood thinners in idiopathic PAH.  Unfortunately, we are unlikely to see this any time soon.

FDA has Approved Uptravi to Treat Pulmonary Arterial  Hypertension

We can add another drug to our arsenal of treatments for PAH.  On December 21st, the FDA approved Actelion’s newest medication called Uptravi (Selexipag).  This oral medication is a prostacyclin receptor agonist.  The efficacy of the medicine was established in the largest pulmonary arterial hypertension study ever conducted.  1156 patients were enrolled.

Uptravi reduced the rate of clinical worsening by 40% compared to placebo.  The medication is dosed starting at 200mcg twice daily and gradually increased to the highest tolerated dose or 1600mcg twice daily.  As with other PAH medications, side effects are common.  These include headache, diarrhea, nausea, jaw pain, leg and muscle pains, flushing and joint pains, all consistent with prostacyclin type activity.

Where Does Uptravi Fit into our Treatment Algorithm?

The most direct competitor for Uptravi is Orenitram (oral Treprostinil).  In comparison to Orenitram, Uptravi is dosed twice daily not three times daily (best results with Orenitram are with three times daily dosing, though it may be given twice daily).  Furthermore, the period of gradually increasing the medication dosage (titration schedule) is substantially shorter with Uptravi.  We do not yet know how much Uptravi will cost but we suspect it will be priced less than Orenitram.

In my practice, I anticipate using Uptravi as a third oral agent when patients are progressing despite treatment with an ERA and a PDE5 inhibitor.  Uptravi has not been studied in very sick patients and should not replace continuously infused Flolan or Remodulin in our sickest patients.  It may well be a more convenient alternative to Tyvaso (inhaled Treprostinil).

Important Questions About Uptravi

As Uptravi comes to market in January 2016, a few important issues remain to be addressed.  First, we do not yet have a clear understanding how to transition patients from Uptravi to pump-based therapy if they are unable to take pills.  Actelion is working on some guidance for the PAH community.  Second, perhaps rather than starting two medications initially in newly diagnosed PAH patients, three treatments might be better.  This question will be studied in an upcoming clinical trial called TRITON.  Lastly, as the complexity of PAH treatment continues to increase, so does the cost.  As a PAH community, we need to continue to advocate for cost-effective treatments.

As we look back to 2015 it has been a good year for pulmonary hypertension patients.  We have made steps forward.  We have an expanding number of medications to treat PAH.  Patients are living longer and feeling better.  We have taken further steps toward understanding the genes involved in the disease.

Despite our progress, we remain ineffective in answering some very basic questions about PAH, and we lack a comprehensive strategy for research and development.  All of our approved medications work to either improve exercise capacity or delay clinical worsening.  However, no one medication is effective for all patients.  Some patients respond very well to one therapy and not well to others.  We have made no progress in understanding how to match our available therapies with individual patient characteristics.

Personalized Medicine

Much has been made about personalized medicine.  This is the concept of making patient level decisions that optimally match treatments to each patient.  In order to do this we need much better quality research.  The community of PH doctors has been clamoring with increasing fervor for drug companies to collect blood samples and analyze the information in a way that allows us to choose the optimal medication regimen.

Unlike many other diseases where research studies involve many thousands of patients, most PH studies have a few hundred patients up to a thousand patients.  Furthermore, as more community doctors are comfortable prescribing oral therapies for PAH patients, fewer patients are being referred to research centers.  This has complicated the process of efficiently enrolling research studies.

Here is my Pulmonary Hypertension wish list for 2016

1.     Clinical research studies will standardize the collection of blood and information to allow scientists to begin exploring how to choose the best treatments for each patient

2.     The Pulmonary Hypertension Association should continue to expand their leadership in advocating for federal funding for PAH research

3.     The expansion of treatment options that target new pathways

4.      Improved diagnostic tests that allow us to monitor our patients’ progress

Last but not least, I would like nothing else than to be out of a job when we find a cure for PAH.  Until then, we have much to do and many patients to help.

I recently lost a friend and patient of almost a decade.  Her battle with familial pulmonary hypertension started when she was a young girl and her mother died from the disease.  I met her in her early twenties when she already had severe PAH.  Her brother had been treated for severe PAH for several years prior.

When I first met her, she had a young daughter, the light of her life.  With the help of a supportive family and an aggressive treatment regimen, she improved greatly and experienced overall good health for almost a decade.  She raised her daughter to be a lovely young woman.  In the past year, though, she started declining.  We adjusted her therapies but she continued to decline.   We were able to list her for urgent lung transplantation but her heart failed before we had donor lungs.

As a doctor, I am learning all the time.  My patients teach me how great the human spirit is.  They teach me how the power of positive thinking can propel even very ill patients forward and allow them to make deep and lasting friendships and find joy and satisfaction.  Simply getting out of bed and putting one foot in front of the other may be a challenge.  It may cause shortness of breath, fatigue, lightheadedness.  This particular patient navigated a very difficult road.  Nonetheless, she never complained about her lot in life.  She never sought pity from those around her.  She chose to focus her positive energy on raising her daughter and connecting with those around her.  Despite severe PAH she traveled and lived life to its fullest.  It was an honor and a privilege to care for her for almost a decade.

As a physician that has cared for PAH patients for much of the past two decades, I am reminded that despite our advances PAH remains a deadly disease.  We must redouble our efforts to raise money and awareness.  We must continue to improve our care systems.  Most importantly, we must push forward with basic science, translational and clinical research until we don’t lose any more patients like her to PAH.

We are lucky to have some patient contributors to our website.  I have known Donna and Patricia for many years.  They have been very active in the local pulmonary hypertension community.  They offer the following insight into the role of support groups.

By Patricia Harrington and Donna Green

The support of family and friends is a key element of coping with any complex medical problem.  Patient education within support groups often helps the patient and family to understand their condition better.  Information and connections within the support group often encourages new patients to further research their condition in order to make better choices about doctors, family support and treatment.  The friendship and comradery found in the support group provides important support during good and bad times.  The ability to help fellow patients with PAH is also an uplifting experience.

The Phoenix Pulmonary Hypertension Support Group has been a vital part of this rare disease community.  Beginning Saturday, February 6, 2016, this group will meet on alternating months for the purpose of sharing experiences and furthering our knowledge and understanding of PAH.  Lunch is provided and free to patients and caregivers.  Come join the community!  The first meeting for this year will be held at University of Arizona Medical Center (formerly Banner Good Samaritan Hospital) on Saturday, February 6, 2015, at 12noon (Hospital Provides Parking Shuttle).

For more information please visit us on Facebook at Inspire Pulmonary Media.

Your Questions Answered.

Our readers have submitted some great questions.  We value your questions and encourage you to continue to tell us about your interests and questions.

What is happening with the implanted Remodulin Pump?

The FDA recently announced they are not ready to approve the implanted pump system for the delivery of continuously infused Remodulin.  They are asking that more work be done prior to approving the system.  Many patients across the United States have been eagerly awaiting approval of the implanted system.  Medtronic and United Therapeutics continue to work to bring this important treatment to patients.  No timeline has been released.

Is a right heart catheterization necessary to diagnose pulmonary arterial hypertension?

The emphatic answer is absolutely.  If your physician wants to diagnose you as having pulmonary arterial hypertension without performing a right heart catheterization then you would be best served by seeking a more expert opinion.

While an echocardiogram may point to the diagnosis, it is never enough to make a firm diagnosis or initiate treatment.

The Pulmonary Hypertension Association has developed a center of excellence label “Comprehensive Care Center” that has been awarded to more than 30 programs across the country.  These programs have a demonstrated track record of outstanding care in all aspects of PAH.

We received a question about PAH and pregnancy

Pregnancy is very dangerous for women with PAH.  Depending on the severity of a woman’s PAH, mortality may be as high as 50%.  As a woman moves through the first trimester of pregnancy the blood volume increases by about 50%, peaking in the third trimester.  In patients with PAH, the weakened right ventricle is unable to accommodate the increased blood volume and may further decline.  This results in worsening right heart failure.  A second period of great risk involves delivery.

Lastly, in the first week after delivery there is a process where edema fluid moves back into the blood vessels and results in rapid increases in blood volume.  This can precipitate abrupt right heart failure.  Adding to the risk of pregnancy is that all endothelin receptor antagonists are 100% contraindicated in pregnancy due to risk of birth defects.  Any woman with PAH who is pregnant should immediately be seen by a high-risk obstetrician and a PAH expert at a center of excellence.

We had another successful Symposium this past weekend. This year, in addition to discussing Pulmonary Arterial Hypertension topics, Dr. Feldman also presented on Idiopathic Pulmonary Fibrosis. We had great attendance by numerous medical professionals involved in the daily care of patients with PAH & IPF.

We’d like to thank all of those who helped make the Symposium possible and those who are involved in the daily care of patients with these diseases. We are living in exciting times with many new treatments available and other progress in the pipeline. But it’s clear that promoting awareness and research will continue to be essential to helping those suffering from these diseases.

One of the great challenges in medicine in general and Pulmonary Arterial Hypertension in particular is finding a doctor and treatment team that will provide you with the best care.  This is particularly important as treatment choices for PAH medications have become plentiful and more doctors are comfortable with some of the oral medications.  Defining quality medical care is also complicated.  Everyone wants to believe that their doctor is great.  And all doctors want to believe that they are great at what they do.  However, with 15 treatment options and numerous clinical trials ongoing, staying abreast of the latest development is more than the average physician is capable of doing.

Comprehensive Care Centers for Pulmonary Hypertension

In response to growing concern that many patients are receiving care from physicians that are not truly expert in PAH, the Pulmonary Hypertension Association set out to develop a certification system to recognized different levels of expertise in treating physicians.  This is designed to help patients receive the best care.

Two levels of certification have been developed.  The highest level of care is Comprehensive Care Center (CCC).  When we first wrote about CCCs in February of 2015 there were 11 accredited centers. There are currently 32 CCCs across the country with more to come.  These programs have a physician leader that is expert in all available treatments, a nurse coordinator who is knowledgeable in helping patients navigate their way through PAH hurdles.  All CCCs are required to participate in research and in educational activities.  These 32 programs have well-developed collaboration with surgeons, anesthesiologists, rheumatologists and other specialists to provide care for PAH patients under any circumstance.  They can provide expert care to patients in the office and hospital.  They are also affiliated or have relationships with lung transplantation programs.

Regional Care Centers for Pulmonary Hypertension

Recognizing that not every patient will have access to a Comprehensive Care Center, a second designation was developed called a Regional Care Center (RCC).  These programs have less expertise than Comprehensive Care Centers but still are able to begin the diagnostic process and initiate treatment for PAH patients.  They are required to collaborate with a CCC.  In general RCCs will be prescribing oral therapies and will send sicker patients to CCC for further evaluation and treatment.

We have included an updated list of Comprehensive Care Centers below.  The Pulmonary Hypertension Association will start recognizing Regional Care Centers soon.  Stay tuned for an update about Regional Care Centers.

Arizona

Arizona Pulmonary Specialists, LTD

3330 North 2nd Street, #300

Phoenix, AZ 85012

Director: Jeremy Feldman, MD

Appointments: (602) 443-0184

California

Cottage Health System

Cottage Pulmonary Hypertension Center

2403 Castillo Street, Suite 206

Santa Barbara, CA 93105

Director: Jeffrey S. Sager, MD

Appointments: (805) 898-8840

Stanford University

Stanford Adult Pulmonary Hypertension Clinic

Chest Clinic 300 Pasteur Drive, A13, Ambulatory Care Clinic 2

M/C 5351

Stanford, CA 94305-5351

Director: Roham T. Zamanian, MD

Appointments: (650) 723-1474

University of California, at San Francisco Medical Center

University of California, San Francisco (UCSF) Pulmonary Hypertension Program

400 Parnassus Avenue

Level B-1, APL 094

San Francisco, CA 94143-0124

Director: Teresa De Marco, MD

Appointments: (415) 353-9088

Colorado

University of Colorado Denver | Anschutz Medical Campus

Pulmonary Hypertension Program

12605 E. 16th Ave, Room 3.2203

Aurora, CO 80045

Director: David Badesch, MD & Todd Bull, MD

Appointments: (720) 848-6518

Connecticut

Yale University School of Medicine

Yale Pulmonary Vascular Disease Program

333 Cedar Street

New Haven, CT 06520

Director: Terence K. Trow, MD

Appointments: (203) 785-4196

Florida

Mayo Clinic Florida

Pulmonary Hypertension Clinical Program

4500 San Pablo Road S

Jacksonville, FL 32224

Director: Charles D. Burger, MD

Appointments (Patient Line): (904) 953-2272

Appointments (Physician Line): (904) 953-0321

Orlando Health Heart Institute

Pulmonary Vascular Disease Program

1222 South Orange Avenue

Orlando, FL 32806

Director: James H. Tarver, MD

Appointments: (407) 650-1347

Iowa

University of Iowa

Heart and Vascular Center – Pulmonary Hypertension Program


200 Hawkins Drive

Iowa City, IA 52242

Director: Linda Cadaret, MD

Appointments: (319) 356-7102

Kansas

The University of Kansas Hospital

University of Kansas Pulmonary Hypertension Program

3901 Rainbow Blvd

MS 3007

Kansas City, KS 66160

Director: Timothy Williamson, MD

Appointments: (913) 588-4098

Kentucky

Kentuckiana Pulmonary Associates

100 West Market Street

Suite 2

Louisville, KY 40202

Director: John Wesley McConnell, MD

Appointments: (502) 587-8000

Louisiana

University Medical Center New Orleans

Comprehensive Pulmonary Hypertension Center

2025 Gravier Street, Suite 615

New Orleans, LA 70112

Directors: Matthew Lammi, MD & Shigeki Saito, MD

Appointments: (504) 903-2387

Maryland

Johns Hopkins University

Johns Hopkins Pulmonary Hypertension Program

601 North Caroline Street

7th Floor, Suite C

Baltimore, MD 21287

Director: Paul Hassoun, MD

Appointments: (410) 614-6311

Massachusetts

Brigham and Women’s Hospital

Pulmonary Vascular Disease Program | Center for Pulmonary Heart Disease

Watkins Clinic – Carl J. and Ruth Shapiro Cardiovascular Center

70 Francis Street

Boston, MA 02115

Director: Aaron Waxman, MD, PhD

Appointments: (857) 307-4000

Michigan

University of Michigan

1500 East Medical Center Drive

Ann Arbor, MI 48109-5853

Director: Vallerie V. McLaughlin, MD

Appointments: (888) 287-1082

Minnesota

Mayo Clinic

200 1st Street SW

Rochester, MN 55905

Director: Robert P. Frantz, MD

Appointments: (502) 587-8000

Missouri

Washington University at Barnes-Jewish Hospital

4921 Parkview Place

Suite B

St. Louis, MO 63110

Director: Murali M. Chakinala, MD

Appointments: (314) 454-8917

New York

Columbia University-New York Presbyterian Hospital

3959 Broadway

CH-2N, Division of Pediatric Cardiology

New York, NY 10032

Director: Erika Berman Rosenzweig, MD

Appointments: (212) 305-4436

Weill Cornell Medical Center-New York Presbyterian Hospital

520 East 70th Street

New York, NY 10021

Director: Evelyn Horn, MD

Appointments: (212) 746-2381

North Carolina

University of North Carolina at Chapel Hill

University of North Carolina Pulmonary Hypertension Program

102 Mason Farm Road, CB 7020

Chapel Hill, NC 27599

Director: H. James Ford, MD

Appointments: (984) 974-5775

Ohio

University of Cincinnati

University of Cincinnati Medical Center Pulmonary Hypertension Program

UC Health Physicians Office (Clifton)

222 Piedmont Avenue

Suite 4300

Cincinnati, OH 45219

Director: Jean Elwing, MD

Appointments: (513) 475-8523

Pennsylvania

Allegheny Health Network

Allegheny General Hospital

320 East North Avenue

Pittsburgh, Pa. 15212

Director: Raymond L. Benza, MD

Appointments: (412) 359-6739

University of Pennsylvania

Perelman Center for Advanced Medicine at the Hospital of the University of Pennsylvania

3400 Civic Center Boulevard

Philadelphia, PA 19104

Penn Presbyterian Medical Center
51 North 39th Street

Philadelphia, PA 19104

Director: Steven M. Kawut, MD, MS

Appointments: 800-789-PENN (7366)

Rhode Island

Rhode Island Hospital – Brown University

Rhode Island Hospital Pulmonary Hypertension Center

593 Eddy Street

Providence, RI 02903

Director: James Klinger, MD

Appointments: (401) 444-3570

Tennessee

Vanderbilt Medical Center

Vanderbilt Pulmonary Vascular Center

1301 Medical Center Drive

Nashville, TN 37212

Director: Ivan Robbins, MD

Appointments: (615) 322-5879

Texas

University of Texas Southwestern Medical Center

UT Southwestern Pulmonary Hypertension Program

5939 Harry Hines Blvd, Ste 600

Dallas, TX 75390

Director: Kelly M. Chin, MD, MSCS

Appointments (New Patients): (214) 645-8300

Appointments (Established Patients): (214) 645-5505

Virginia

Inova Fairfax Hospital

Inova Advanced Lung Disease Management Program

3300 Gallows Road

Falls Church, VA 20042

Directors: Steven D. Nathan, MD & Oksana A. Shlobin, MD

Appointments (New Patients): (703) 776-6168

Appointments (Established Patients): (703) 776-2986

Virginia Commonwealth University

411 East Marshall Street

Richmond, VA 23298

Director: Dan Grinnan, MD

Appointments: (804) 828-2161

Wisconsin

Froedtert Hospital and Medical College of Wisconsin

Froedtert Hospital and Medical College of Wisconsin Pulmonary Hypertension Service

9200 West Wisconsin

Suite E5200

Milwaukee, WI 53226

Director: Kenneth Presberg, MD

Appointments: (414) 805-6633

Having a surgical procedure is always stressful.  Having Pulmonary Arterial Hypertension and needing a surgery is even more stressful.  Over the years, centers of excellence in PAH care have figured out ways to minimize the risk.  Below I outline some of the important areas to consider when planning for a surgery or invasive procedure.

1.      Is the procedure necessary?

The best way to avoid harm is to avoid unnecessary procedures.  For example, if your cardiologist wants you to have a trans-esophageal echocardiogram (ultrasound exam of your heart from the inside of your esophagus) there are alternatives that do not require sedation and are not invasive.  Don’t be shy.  Always feel comfortable asking about alternatives to the procedure.  Don’t be afraid to ask what are the risks of choosing not to undergo an invasive procedure.

2.      Where is the procedure going to take place?

In the world of medicine, procedures can take place in a doctor’s office, in outpatient surgical centers (surgi-centers) or within a hospital.  In general, procedures that do not require any sedation can be performed in any setting.  Procedures that require any sedation should not be undertaken in the doctor’s office or in outpatient procedural/surgical centers.  The safest place to perform a procedure that requires sedation is in a hospital that has a full complement of PAH capabilities including a PAH expert.

3.      Does the procedure require sedation or anesthesia?

There are several different levels of sedation for invasive procedures.  The lightest sedation carries less risk and requires less monitoring.  However, any PAH patient that is to receive any sedation needs a continuous oxygen saturation monitor, blood pressure monitoring and a designated staff member to monitor breathing.  Moderate sedation involves more risk and requires an anesthesiologist to be present for PAH patients.  This is the type of sedation that is used for colonoscopy.  Moderate sedation should only be performed inside a hospital with expert PAH capabilities.  There are different medicines that may be used to achieve moderate sedation. I generally avoid propofol due to the risk of lowering your blood pressure and depressing your breathing.  Versed and fentanyl are much safer.

The deepest level of conscious sedation is called deep sedation and should only be undertaken by an anesthesiologist with skill and training in managing pulmonary hypertension patients.  This type of sedation requires more monitoring such as an arterial line to measure your blood pressure continuously.  Deep sedation should only be done in a hospital with expert PAH capabilities.  Lastly, general anesthesia is the deepest level of sedation.  When you are under general anesthesia, you are completely unconscious and not breathing on your own.  You will have a breathing tube and be on a breathing machine.  This type of anesthesia carries the highest risk for PAH patients and requires an anesthesiologist with special skill and training in the care of PAH patients (typically a cardiac anesthesiologist).  I always have an arterial line in place and sometimes a central venous catheter (big IV catheter in the neck).

4.      Who will be delivering the sedation?

Anesthesiologists are doctors that specialize in delivering the sedation and putting patients to sleep for procedures.  Within the broader specialty of anesthesia there are subspecialties—such as cardiac anesthesiology.  Cardiac anesthesiologists specialize in the sedation of patients with advanced cardiac disease including pulmonary hypertension.

5.      What should take place before the procedure?

Before I give the green light for my patients to proceed with an invasive procedure, I see the patient in the office and review how they are doing.  I pay particular attention to any signs of heart failure such as fluid retention.  If the planned procedure involves deep sedation or general anesthesia then I often plan a right heart catheterization prior to granting permission for the procedure to proceed.  This allows me to make sure that my patient is doing well enough to tolerate the planned procedure.  A thoughtful preoperative visit will include a plan for blood thinners, all PAH medicines and a discussion of how to minimize the risk of blood clots if the patient is to be hospitalized after the procedure.  I generally talk with the physician that will be performing the procedure if the procedure will involve general anesthesia or deep sedation.

6.      What should take place before larger surgeries?

For patients with more advanced PAH who require major surgical procedures I often admit the patient the night before the surgery and place an arterial line and a central venous catheter prior to the surgery.  I also prepare the medications that may be needed during the procedure and personally talk with the anesthesiologist.  I often will accompany the patient into the operating room and be present for induction of anesthesia (putting the patient to sleep).  Having an experienced set of hands to help can minimize risk dramatically.  We work hard to avoid excessive fluid administration during surgery and minimize blood loss.  Pulmonary artery catheters are not helpful despite what many older anesthesiologists will tell you.

7.      Cancelling surgery?

Sometimes the right thing to do is to cancel a patient’s surgery.  This often creates frustrations for the patient and the surgeon.  However, it is always better to have a live patient and a frustrated surgeon than a bad outcome.  Good surgeons will understand and appreciate your pulmonary hypertension doctor’s attention to details.

8.      Are there medicines to avoid?

Depending on how severe the pulmonary hypertension is, we often avoid certain types of anesthesia.  It is best to talk in advance with your pulmonary hypertension specialist and your anesthesiologist to make a plan before the procedure.

9.      Should I skip my medicines the morning of surgery if I am told to take nothing by mouth?

In general we have our patients skip their diuretics but we strongly encourage them to take their other pulmonary hypertension medications in the early morning with a small amount of water.  If you are taking Orenitram then you will need to have a very clear plan as missing even a single dose of Orenitram is not advised.  If you are on a pump-based therapy (Flolan/Remodulin/Veletri) then you should bring all your supplies and your back up pump.  Make sure that you have enough medicine in your pump so that it does not run out.  If you are on an inhaled PAH medication, then do your treatments as scheduled.  If you are unable to do a treatment on time due to the surgery, then just do the treatment when you are next able.

We are excited to feature a delicious, heart healthy, low salt and low carb recipe from Meghan Hunt.  Send us your favorite healthy recipes and we will share them with our readers. Or feel free to post them on our Facebook page.

Zucchini Lasagna

Ingredients

• 2-3 zucchinis cut length wise into 1/4 inch slices
• 1 1/2 cups ricotta cheese
• 2 eggs
• 2 tsp flour
• 1 cup mozzarella cheese
• Spaghetti sauce (recipe below)

Directions

Partially cook zucchini slices and dry with paper towels. Mix eggs, ricotta cheese, flour and half of the mozzarella cheese together in a bowl. Place zucchini slices on the bottom of a 9 x 11 inch cake pan. Spread ricotta cheese mixture evenly over the top of the zucchini, then spread some sauce on top of the ricotta cheese mixture. Place another layer of zucchini slices, top with another layer of sauce and the rest of the mozzarella cheese. Bake uncovered at 375 for 30-40 minutes or until cheese is brown. Let set for 10-15 minutes and serve. Approximately 3 carbs and 200 mg per serving (based on 8 servings).

Sauce recipe

• 1 pound ground beef or ground turkey
• (4) 6oz cans tomato paste
• 1 tablespoon garlic powder
• 1 tablespoon anise seed
• 1 tablespoon Italian seasoning
• 1/2 tsp pepper
• (7) of the tomato paste cans filled with water
• (1) package fresh sliced mushrooms (optional)

Directions

Brown and drain meat.  Return to pan and add all ingredients.  Bring to boil.  Cover and reduce heat and simmer for 3 hours (crack lid just a bit if needed) stirring occasionally.  Put individual servings into ziplock baggies and freeze to pull out as needed.

Your Questions Answered

Is there an oral treatment that can replace continuously infused therapy (Flolan/Remodulin)?

Flolan and Remodulin are also called Prostanoids.  This family of medicine has been the backbone of PAH therapy for our sickest PAH patients more than for 15 years.  In the past 2 years, the FDA approved 2 oral medicines that are in the same class (prostanoid and prostanoid-like).  These two medicines, Orenitram (oral treprostinil) and Uptravi (selexipag), are more convenient to take but are not as effective as pump-based therapy.  Many patients are eager to switch from their pump-based medicines to pills to avoid site pain and the inconvenience of having a catheter and pump.  However, this is not advisable for most patients.  Although in the short term carefully chosen patients may be able to switch, our experience over 18-24 months is that many patients will worsen and there is a risk of not being able to regain the lost ground with resuming pump-based treatment.

Other considerations include the fact that it is easy to miss doses of pills but hard to miss “doses” of pump-based treatment.  Furthermore, many patients experience more side effects with pill versions of the prostanoid class medications than with continuously infused therapy.  I am encouraging my patients to hang in there with pump-based treatments with the hope of an implanted pump becoming available in the next year.  Of course, there are certain patients who have had hard to manage side effects from pump-based therapies such as refractory site pain and catheter infections who may be candidates for the oral options.

One of the most common questions that we receive from our readers is where to get high quality pulmonary hypertension care.

On a prior post we included a list of the Comprehensive Care Centers.  These programs have been evaluated by the Pulmonary Hypertension Association and have deep expertise and well developed infrastructure to diagnose, treat and conduct clinical research.

Each month we receive a couple of questions about whether or not a patient should have a hole in their heart closed.

The two most common types of holes in the heart in PAH patients are called PFO (patent foramen ovale) and ASD (atrial septal defect).  These are both considered atrial (top heart chamber) level communications.  Cardiologists often suggest closing these holes when found.  However, our practice is never to rush into closing such a hole.  In general, we almost never close the smaller PFO holes.  In patients with severe pulmonary hypertension we never close PFO or ASD communications.  In patients with very mild pulmonary hypertension we consider closing the larger ASD communications.  In patients without pulmonary hypertension, the decision to close an atrial level hole is best undertaken by a cardiologist with special training and experience in adult congenital heart disease.

Your Questions Answered

Is it safe to travel to high altitude with Pulmonary Hypertension?

This is an important question for patients with any chronic heart or lung problem, and especially important for patients with PAH.  As we ascend to higher elevations a variety of changes take place in our heart and lungs.  Heart rate increases immediately.  Pulmonary artery pressures increase at rest and even more so with exercise.  Oxygen levels decline the higher the elevation.  Most patients don’t experience any problems with elevations of less than 3,000 feet (approximately 1,000 meters).  Patients with mild PAH (mean pulmonary artery pressure less than 35 and normal cardiac output) generally do well with elevations less than 6,000 feet though some patients are particularly sensitive.  Patients with more severe PAH are less tolerant of elevation.

What can I do to travel more safely to higher elevations?

First, you should always check with your PAH physician.  There is a test called a High Altitude Simulation Test that allows your doctor to see if you drop your oxygen saturation at oxygen conditions that mimic commercial airline travel (8,000 feet elevation equivalence). This test accurately identifies patients that require supplemental oxygen but does not tell us how your right ventricle handles the added stress of high elevation.

Second, if you have severe PAH, avoid exposure to elevations greater than 3,000 feet when possible.

Third, travel with oxygen if you meet the appropriate criteria.

Fourth, return to lower elevation if you are feeling poorly.

If I have a cold, what medications can I take for nasal congestion and cough?

Unfortunately having PAH does not protect you from all the common illnesses such as seasonal colds and respiratory infections.  There is no magic medicine to treat the cough, nasal congestion and achiness.  There are some medicines that should definitely be avoided and these include Decongestants such as Afrin, Oxymetazoline, Phenylephrine, Sudafed, and Pseudoephedrine.  These medicines are vasoconstrictors—this means that they act by squeezing the blood vessels.  Unfortunately in addition to squeezing the blood vessels in your nose to decrease your runny nose, they also squeeze your pulmonary arteries and can lead to worsening pulmonary hypertension.

Another strategy to dry your nose and congestion is first generation antihistamines such as Benadryl or Chlorpheniramine.  These are safe for PAH patients though if you are an older man and have trouble urinating you should be careful.  Cough and mucous symptoms can be very frustrating.  Over the counter Guaifenesin offers mild benefit and is very safe.  Many patients find that it loosens secretions.  Cough suppressants include Dextromethorpan, Ricola and other cough drops that are over the counter may provide relief and are quite safe.  There are a variety of prescription cough suppressants that may be tried if your symptoms persist.  Time and patience are the best medicine.

What can I take for seasonal allergies?

All of the antihistamines are safe for PAH patients.  The first generation antihistamines like Benadryl and Chlorpheniramine are inexpensive and effective but are sedating. The newer agents (second generation antihistamines) are also very effective and don’t cause sedation. These agents include Zyrtec (cetirizine), Allegera (fexofenadine), Claritin (loratadine) and others.  Avoid the “D” which is the added decongestant that can cause increased pulmonary artery pressure. 

Nasal steroid sprays are very effective for treating allergic nasal symptoms and are now available over the counter.  In general they are very well tolerated and extremely safe.  They don’t work instantly, so many patients find that they benefit most when they use this type of medicine for a period of time when they are experiencing more allergy symptoms.

As we look back to 2015 it has been a good year for pulmonary hypertension patients.  We have made steps forward.  We have an expanding number of medications to treat PAH.  Patients are living longer and feeling better.  We have taken further steps toward understanding the genes involved in the disease.

Despite our progress, we remain ineffective in answering some very basic questions about PAH, and we lack a comprehensive strategy for research and development.  All of our approved medications work to either improve exercise capacity or delay clinical worsening.  However, no one medication is effective for all patients.  Some patients respond very well to one therapy and not well to others.  We have made no progress in understanding how to match our available therapies with individual patient characteristics.

Personalized Medicine

Much has been made about personalized medicine.  This is the concept of making patient level decisions that optimally match treatments to each patient.  In order to do this we need much better quality research.  The community of PH doctors has been clamoring with increasing fervor for drug companies to collect blood samples and analyze the information in a way that allows us to choose the optimal medication regimen.

Unlike many other diseases where research studies involve many thousands of patients, most PH studies have a few hundred patients up to a thousand patients.  Furthermore, as more community doctors are comfortable prescribing oral therapies for PAH patients, fewer patients are being referred to research centers.  This has complicated the process of efficiently enrolling research studies.

Here is my Pulmonary Hypertension wish list for 2016

1.     Clinical research studies will standardize the collection of blood and information to allow scientists to begin exploring how to choose the best treatments for each patient

2.     The Pulmonary Hypertension Association should continue to expand their leadership in advocating for federal funding for PAH research

3.     The expansion of treatment options that target new pathways

4.      Improved diagnostic tests that allow us to monitor our patients’ progress

Last but not least, I would like nothing else than to be out of a job when we find a cure for PAH.  Until then, we have much to do and many patients to help.

I recently lost a friend and patient of almost a decade.  Her battle with familial pulmonary hypertension started when she was a young girl and her mother died from the disease.  I met her in her early twenties when she already had severe PAH.  Her brother had been treated for severe PAH for several years prior.

When I first met her, she had a young daughter, the light of her life.  With the help of a supportive family and an aggressive treatment regimen, she improved greatly and experienced overall good health for almost a decade.  She raised her daughter to be a lovely young woman.  In the past year, though, she started declining.  We adjusted her therapies but she continued to decline.   We were able to list her for urgent lung transplantation but her heart failed before we had donor lungs.

As a doctor, I am learning all the time.  My patients teach me how great the human spirit is.  They teach me how the power of positive thinking can propel even very ill patients forward and allow them to make deep and lasting friendships and find joy and satisfaction.  Simply getting out of bed and putting one foot in front of the other may be a challenge.  It may cause shortness of breath, fatigue, lightheadedness.  This particular patient navigated a very difficult road.  Nonetheless, she never complained about her lot in life.  She never sought pity from those around her.  She chose to focus her positive energy on raising her daughter and connecting with those around her.  Despite severe PAH she traveled and lived life to its fullest.  It was an honor and a privilege to care for her for almost a decade.

As a physician that has cared for PAH patients for much of the past two decades, I am reminded that despite our advances PAH remains a deadly disease.  We must redouble our efforts to raise money and awareness.  We must continue to improve our care systems.  Most importantly, we must push forward with basic science, translational and clinical research until we don’t lose any more patients like her to PAH.