Living with PH Plus... Scimitar Syndrome Rita Simone Hébert's Story

Although my lung pressures were measured throughout my life, I had never heard the term “pulmonary hypertension” until 2004. That is the year my (then) cardiologist announced that absolutely nothing more could be done for me medically. That dire statement led me to do a little online research regarding PH. That is also how I found a fantastic PH specialist here in Montreal, who has been monitoring me closely since early 2005.

Some of my early memories as a young child involve breathlessness. I am no stranger to rare conditions such as PH. I was born with an even rarer disorder called a scimitar syndrome. A scimitar is a short sword with a curved blade that broadens toward the point, used originally in Eastern countries during the 16th century. Patients who have this cardiopulmonary defect have a shadow on a lung, which resembles a scimitar. Not all patients diagnosed with this syndrome develop PH. I did!

Due to this heart-lung malformation, I have never had the luxury of experiencing a normal existence. I have never known what it feels like to be able to run (or jog) more than a few meters or keep up with my peers on a bicycle. As a child I spent a lot of time in the local Children’s Hospital where I had my first four heart catheterizations. The biggest heartbreaker, however, was when I found out that I should not have children, despite the corrective surgery I underwent at the age of 23.

I wish I could say that “all’s well that ends well” at this point. However, the difficult part of living with this malformation is that I am not a candidate for most PH drugs. I’ve had to have two more corrective surgeries in the past two years. The first one was a noninvasive pulmonary dilation, followed by open-heart surgery in December 2011.

Although not “all’s well that ends well” yet, living with PH, as well as with this condition, has led me to come across some exceptional people and circumstances. During this PH journey, I have been treated by the most caring and dedicated PH specialist on the globe, Dr. David Langleben. His availability and generosity of spirit surpasses any expectations I could possibly have imagined!

As a patient at his clinic, I also get to see Lyda Lesenko, our local PH nurse coordinator, who we aptly nicknamed “our guardian angel.” Lyda has gone way beyond the call of duty on numerous occasions. For example, Lyda was instrumental in setting up a bilingual support group for the patients at our clinic. As a result of these support group meetings, I feel privileged to have met some of the most outstandingly courageous patients and caregivers. Not only are they bravely proactive in the face of this disease, they are also major players in helping to create support and awareness locally and nationally.

During this PH journey, I also came across a great self-help program called “How to Live a Healthy Life With a Chronic Illness.” This program, which was initially developed at Stanford University, is now taught worldwide in several different languages. I was so taken by its simplicity and effectiveness that I trained to become a program leader. I’m also proud to add that I now teach it in French and English here in Montreal. Read more information on this program

Other lessons I’ve learned along the PH path include not letting the things I cannot do prevent me from doing the things I can. I also learned that being “my personal best” in all matters, given my physical limitations, is all that really matters. I may not be able to run a mile, but perhaps I can still dance a little … in the rain … to the sound of my own tune! I wish you all to do the same.

Wishing you all my personal best!

By Rita Simone Hébert,
Patient with secondary PH due to a complex heart-lung malformation
This article first appeared in Fall 2012 Pathlight.

Anna Bower: Living and Thriving with Multiple Diseases

Being diagnosed with any chronic illness is difficult to handle. Being diagnosed with multiple chronic illnesses is even worse. You have to balance your life with your limitations. You visit doctors frequently. Your nurses know you by name, and you are sure your insurance company rolls its eyes when receiving a claim for you. You take so many medications you really have to watch supplements and over-the-counter medications for counter-indications. One day you may be ailing from one disease and the next day it is another. It is a lot for anyone to deal with. I have been diagnosed with systemic lupus, PH, hypothyroidism, mixed connective tissue disease, Raynaud’s, fibromyalgia, migraines, hypoglycemia, anemia, depression and gluten intolerance. I grew up with the hypothyroid, Raynaud’s, migraines and hypoglycemia. The additional diseases came after I finished high school.

I was diagnosed with lupus in 2004 at the age of 20. I have always been a very independent and active person, and the diagnosis changed everything. I was living on my own, going to college and doing the “college thing.” With this diagnosis, I was no longer able to continue college or support myself, so I moved back home. No more hiking, rafting or soccer. My hands hurt too much to write, and my feet and knees hurt too much to walk far. Depression had now become a part of my life. My lupus became more than lupus; it became what I call “Lupus+” — my doctor calls it mixed connective tissue disease and fibromyalgia. I had the diagnosis of SLE (lupus), but I also presented with symptoms of scleroderma and polymyositis, like PH. Anemia just kind of comes with the package. In January 2010, I was diagnosed with PH. I think my body just wanted to make life a little more interesting and added the gluten sensitivity.
I had to quit working. I could hardly exist, let alone work. Naps became a regular habit, and I could only go out for an hour or two. I had to give up my dog, my rabbit and my fish. Okay, I didn’t really have to give up my fish, but there was no way I could take care of a husky!

I had to avoid the sun because it could cause my lupus to flare up. Since hiking and rafting were out of the question, there went my desire to do photography. I loved to play the clarinet, but my fingers could no longer move fast enough for long enough, not to mention the lack of lung capacity! My life, in its current form, was no longer possible, and I became withdrawn.
At some point after my first year, I realized I couldn’t let my diseases define me. I began to figure out what I could do, instead of what I couldn’t do. After PH, oxygen became a permanent part of my life. I learned that I could go out in the sun if I remembered sunscreen, long sleeves and hats. I could go hike if I made sure to do an easy trail. I could walk if I found good shoes. Photography became a passion again! It is so wonderful to capture something that others take for granted. Knitting and crocheting are great exercise for my hands.
Then I returned to college and began taking classes again. I enjoyed spending time with other young people and exercising my brain. My college, Colorado Mesa University, offers support for students with needs. They supply carbon paper if I can’t take my own notes; some teachers allow tape recorders. Most classes have the PowerPoint slides on the school network. Each semester I meet with my teachers to discuss my situation. I offer to get documentation for them if needed and supply them with a list of days I will miss because of doctors’ visits or treatment. Financial aid is also plentiful for students in my position; all you have to do is talk to the office!

I just finished a six-month treatment of chemo for my lupus, which has been successful so far. This last December I finished my associate’s degree, and I am just about to start my next semester of classes on my way toward a bachelor’s degree in history. Napping is still a hobby, and my kitten and I enjoy them greatly! My boyfriend has been a huge pillar for me, keeping me motivated while remaining understanding, and my mom is always there for me.
It is okay that my life has changed. With my mind set and the support and love of those I keep around me, I am able to accept and enjoy what I have. Things have definitely changed, but that change doesn’t have to be bad. People ask me if I wish it were different. On one hand, of course! But on the other, this life has made me who I am and given me what I have. Why would I want to change that?
By Anna Bower, PH Patient
This was first published in Pathlight Spring 2012.

PHenomenal Lives: PH + HIV

by Jeannie Wraight

PH and HIV Patient

When I look back at who I was when I was first diagnosed with HIV, it's hard to believe I am the same person. I knew I was at risk. My boyfriend was HIV-positive and we took minimal precautions based on uneducated decisions. The only thing I can say in my own defense was that in 1995 there was little HIV education available, and syringe exchange programs, even in New York, were only open a few hours a day, a couple days a week.

We could never make it there in time to collect clean syringes, but we thought we had figured out how to use

them without putting me at risk. We were dead wrong. I was stupid and careless. I have never been sick from HIV. I am what they call a long-term non-progressor. My viral load is almost nonexistent and it took 15 years for my T-cells to decline to a point of concern. 

I have never been on HIV medication. For the past 15 years, I have lived and breathed HIV. 

I’m an AIDS treatment activist, focusing on such issues as novel drugs in development for HIV, including gene therapies, immune-based therapies and therapeutic vaccine. My big focus now is cure research. With one person already cured of HIV (though his treatment cannot be replicated for most), we are closer than ever to finally ending the AIDS crisis. Even so, my biggest problem is not HIV. It is my pulmonary hypertension that is the more likely of the two to take me down. 

I was diagnosed seven years ago with PH. I remember the night that I realized something was wrong. My dog, Jo-Jo, a rescued Akita, loved to take off running whenever he got the chance. He'd make me chase him around, pretending to stop and sniff a tree so I could catch up, allowing me to get within arms' reach before

running again until he’d had enough. I knew the game well, and having no choice but to let him do this ritual, I began chasing him through the streets of the Bronx. But this night was different. After a few minutes my lungs

started to burn. The air seemed to be disappearing. I kept needing to stop. Jo-Jo, of course, stopped beside me each time. My heart began beating wildly and I felt like I was hyperventilating. I couldn't catch my breath. 

Even with Jo-Jo mercifully waiting for me, I could barely keep up with him. I couldn't go home, even to get help, because I was afraid he'd run into traffic. I began crying uncontrollably. I remember walking past some kids who had been hanging out on the corner. One said, “Damn, girl, you ain't caught that dog yet? What's wrong with you?” I wanted to scream, “Something is wrong with me – I need help!” By the time I caught Jo-Jo, I thought I was going to die. When I finally walked through the door of my apartment, I fell to the ground, hysterical, and threw up. 

Luckily, my doctor had recently seen another doctor diagnose PH, so he sent me for an echocardiogram upon seeing my symptoms. Three weeks later I was at the 16th International AIDS Conference in Bangkok, Thailand, when it was time to call in for my results. After I was given a diagnosis of pulmonary hypertension over the phone, I immediately called a cardiologist friend for an explanation. He didn't need to say much. I knew from his voice it was bad. 

I have found that stress will aggravate my sickness and symptoms. Unfortunately, stress is a major factor in my life. Today, I am trying to minimize the amount of stress I feel and improve the way I handle it. I am newly married to a wonderful man from the United Kingdom, and as soon as the U.S. government approves his visa, I can start living the life I have always wanted — a quiet life with much less stress.

I will not give up. I am surviving HIV, and I plan to survive PH for as long as superhumanly possible. I have much to live for, particularly in the form of a gorgeous Brit. I am finally learning to come to peace with myself and the world. My PH may be HIV-related, or it may not. But one thing is for sure: more HIV physicians and patients need to be made aware of the risk of PH in HIV patients. One in 200 HIV-positive people have pulmonary hypertension, but because it is normally so rare, diagnosis comes too late for too many. I was lucky and I will work until my last breath to make sure that others are lucky as well, being diagnosed early and getting the appropriate treatment. My life is not nearly over, it has only just begun! 

From Pathlight, Fall 2011

Our Journeys: Kevin Doty

There were only ten steps up to my apartment, but it seemed like there were 10,000. I dreaded going home every day from work because I knew I would have to walk the short distance from the parking lot to my apartment, and then scale those ten dreaded steps each day. I was always so out of breath by the time I got into my apartment, and I had to take ten or fifteen minutes just to catch my breath, and slow my heart rate down. When I started getting those jabbing chest pains with the breathlessness, I began to worry, and went to see my primary care doctor. My doctor ran an EKG test on me, and immediately referred me to a pulmonary specialist. In March, 2006, after several agonizing months not knowing what was going on, my doctor performed a carotid artery catheterization, and discovered that the mean pressure in my right lung was 46, and a diagnosis of pulmonary arterial hypertension was proclaimed. I was immediately prescribed with oxygen therapy, as well as Tracleer and Revatio.

Years earlier, in the summer of 1986, I was diagnosed with a rare disorder called Pulmonary Histiocytosis X (the X part of the name meant that at the time, doctors were still pretty puzzled as to why my white blood cells (histiocytes) were attacking the cells in my lungs and leaving scar tissue. The doctors weren't quite sure what to do for me, so I became a bit of a white lab rat for them, and they started trying different medications to see if any helped me. I was given a huge prescription of prednisone every day, and when that didn't work so well, chemo-therapy was added to the regimen. The chemo seemed to help, and my disease went into a kind of "remission", and I was able to perform normal things, and lead a normal life.

Fast forward now to the end of 2005 - I had moved up to the Seattle, WA area, and had lived

there for about a year when those pesky symptoms began to return. This time, they were far worse than they had ever been previously - it took very minimal effort to get me out of breath while it took considerably more effort in the past to get me breathless. I began to worry when I started having sharp chest pains to go along with being out of breath on minimal effort. I had several tests run on me during this time, including all the heart checkups imaginable, and when all those tests showed no signs of disease or trouble, I was referred to a pulmonary specialist. Upon telling Dr. O'Brien my symptoms, he immediately ordered a carotid artery catheterization, and discovered the pressure in my right heart was at 46. I was immediately put on oxygen therapy, as well as Tracleer and Revatio, and although these drugs didn't help me at first, I think they have helped me regain a little bit of my stamina and ability to breathe easier on exertion. For about a year, I was completely home bound, except to go see the doctor, or to go to the hospital for one thing or another.

Currently, I am able to get out and do my physical therapy, and have even started playing golf again. I look forward to hopefully going back to work in the near future, and being able to rely on myself again, rather than leaning on family members so heavily for everything. Without my family however, I would never have made it this far - I am so grateful for all they do and have done for me during this trying time in my life.

I am also grateful to the PH Association, and all they do to support those of us who have this terrible disease. I found their website by chance one day as I was looking for more info on pulmonary hypertension shortly after I was diagnosed. There is so much information on their website, and I have even joined one of the support groups here in my area that enables those patients and patients' families to interact with each other, and discuss new medications/treatments, tricks to surviving with PH, and just being able to talk to someone who knows exactly what I'm going through with the symptoms, and the different obstacles we must face on a daily basis. These support groups also give us access to many doctors who specialize in pulmonary disorders, and pulmonary hypertension specifically. These doctors take time out of their busy schedule to come talk to our groups to let us know about new medications or procedures being done for PH patients, and graciously answer all of the questions asked by nervous patients, and their family members. The support group meetings have been a real blessing for me since I was diagnosed - just knowing I'm not alone in this fight, and that there are really talented doctors and scientists out there that are working extremely hard to help find a cure for this malicious disease, gives me hope. It's nice to have something positive like hope bouncing around in my brain rather than the despair I felt early in my diagnosis.

I look forward to the day when I can walk up a flight of stairs, and not have to catch my breath for twenty minutes before doing anything else. I hope for the big breakthrough in science and medicine that will eliminate this disease, I hope the drugs on the market now, and those coming out in the future will prolong our lives until we see that glorious day … I hope I can celebrate my 50th birthday, and my 60th, and 70th.

I hope ...

Living with PH Plus... HHT

Christine Fini, PH and HHT Patient

My name is Christine, and I have pulmonary arterial hypertension (PAH) and hereditary hemorrhagic telangiectasia (HHT). My PAH was diagnosed in November 2006. I knew months before that there was something wrong; I could not physically keep up with co-workers, family or friends. I just assumed that my age (49) and weight (well beyond pleasingly plump) had finally caught up with me. I currently take sildenafil (RevatioTM), bosentan (TracleerTM), and as of October 21, 2009, I began taking treprostinil (TyvasoTM) which replaced my iloprost (VentavisTM) and seems to be really working well for me.

HHT is something that has always been a part of my life. Ever since I can remember, my father has had nosebleeds; it was just a part of my family members’ lives. I have had AVMs (arterio-venous malformations) coiled in my lungs and have had to have nasal cauterization to help manage my nosebleeds. My father and one of my sisters have been diagnosed with HHT; three of my four other siblings have been screened and do not have HHT. We also suspect that my four-yearold niece has it since she has an unusual amount of nosebleeds for such a young child, and we plan to have her screened when she is a little older.

Living with these two diseases is a challenge. I am still employed full time. I enjoy my job very much though it has evolved in the past three years to accommodate my PAH and HHT. At times my fatigue, which I believe is a combination of anemia from the HHT and just a part of PAH, can set me back. I’m still learning to accept my ‘bad days’ and to enjoy my ‘good days.’ ‘Bad days’ are the days when I can’t seem to muster the energy to get out of bed to go to work or when just getting ready to go to work is so tiring that I consider my 45-minute commute a chance to rest! ‘Bad days’ are also the days when I have the opportunity to babysit my nieces and nephews and have to decline because I just do not have the energy. ‘Good days’ are those days when I feel like my ‘old’ self. I can do normal, simple things like grocery shopping, house cleaning and putting in a good day of work without exhausting myself. With the love of my family, all my days are truly blessed.


What information do you want to share with other patients living with associated PH? Both HHT and PH are diseases without cures, but there are many wonderful researchers working to change that. It can be difficult to manage having both PH and another serious associated disease, but with the help of physicians who specialize in both, it can be done, and we patients with associated diseases can live long and productive lives.