PH Plus Awareness: Samantha-Anne's Story

This is a long post. If just one person reads this, though, and it helps them to not have to wait 3 years to be diagnosed with Pulmonary Hypertension, then it has been worth it.

The first time I heard about Pulmonary Hypertension was on August 18th during my first appointment with the pulmonologist. It was included in a list of other things I hadn't heard of that might be causing my illness (interstitial lung disease, cardiac asthma, sarcoidosis and PH; I had heard of pulmonary embolism because that is what killed my brother in 1990 at the age of 47.) I knew that all of these things were serious, so I starting learning about them. I'm not sure why it took so long for my doctor to send me to a "lung doctor" when my main complaint for the past 2 years had been shortness of breath (SOB), but I was glad to finally be there.

PH seemed to fit with what was going on with me. However, a lot of other diagnoses had fit before and I honestly didn't want to be disappointed yet again when the doctor told me that I didn't have x,y or z and that he didn't know why my life had changed so drastically since November of 2011. After doing pulmonary function tests, an exam and having me walk in the office while my oxygen saturation level was monitored, the doctor said I would need to have a CT scan to rule out blood clots in my lungs. That honestly was not scary to me because I've been checked for that so many times due to my family history. He said that if there were no blood clots, then I would have to have a right and left heart catheterization procedure to see if I had PH. I had a left heart cath a year ago so I wasn't nervous about that, and since I had no idea what PH was, I didn't worry about it either.

My pulmonary function tests the year before showed that I have a mild restrictive process in my lungs but no obstructive process, so no COPD (which my mom died of in 2000, so I was glad that was ruled out.) I do have mild Alpha-1 Antitrypsin Deficiency, which can affect the lungs, so I knew that lung problems could happen to me. The PFTs he did on 8/18 showed that my lungs were not moving the oxygen in the air I breathe back into my blood (that's poor diffusion capacity). Mine was about 1/3 of normal. An MRI had already shown that my pulmonary artery is slightly enlarged, so I knew that something was going on and all signs were pointing to PH.

Because I really push to get things scheduled quickly and stay on top of my care, I was able to get the CT scans scheduled for 8/21, just 3 days after seeing the pulmonologist. The CT scans showed some mild lung scarring that I already knew about and thankfully no blood clots.

During these weeks I was dealing with a lot of body wide pain (unrelated to the PH) and swelling in my feet, ankles and legs. They basically were just huge stumps, from my toes to my stomach, with no delineation between the three. They hurt and the stinging in my feet was unbearable at times. I was also having to keep an eye on my kidney status because I have Stage 3 Chronic Kidney Disease. Thankfully, there are 5 stages and my disease is mild.

Let me take a second here to talk about the word "mild". I hate that word now. Everything I had going on with me was "mild". Mild means that the doctors don't do much for you and they basically wait until it's not "mild" anymore before they get serious about talking to you or treating you. At least, that was my experience. So even though "mild" is good, it sucks. My cardiologist told me that my heart issues were "mild" and didn't need to be treated and that I should see if the pulmonologist could help me figure things out. That just didn't seem right to me. I started asking myself a lot of questions and realized that I had a lot of questions for the doctors. My life had changed drastically in the past 2 1/2 years and something was causing that. There is no way I could go from being able to hike 2-4 hours at 7000-8000 feet altitude to being SOB and having a racing heart just walking room to room in my house.

I started believing that PH was the illness that was causing my problems so I made an appointment with the PH Specialist in Denver. Neither my cardiologist nor my pulmonologist told me to do that. I did it because I was tired of not knowing why my life had been turned upside down and I wasn't going to wait on them to tell me what to do to get better (since they hadn't done that anyway.)

At the beginning of September, I went back and looked at the sleep study I had done the previous November. I knew I had sleep apnea and was supposed to wear a CPAP, but it made me feel claustrophobic and like I was suffocating so I didn't wear it. I was diagnosed with obstructive sleep apnea in 2006. I got my first CPAP that same year. I had never used my CPAP consistently in all that time. When asked by my doctors if I used it, I told them how it made me feel and that I was not using it very often. No one followed up, no one did any education, no one did any counseling and no one referred me to a sleep disorders specialist. When I looked at the November sleep study (that I had requested be done,) I noticed that my oxygen level was <90% for >99% of the night with and without the CPAP. That didn't seem right to me. I thought that oxygen levels were supposed to be above 90% all the time. The pulmonologist had mentioned getting me compliant with the CPAP so I thought I should look into that. I called the center where I had the study and no one, to this day, has ever called me back. I just kind of figured that it must not be that big of a deal if no one called me back. I was very wrong about that. 

One of the most fortunate things that happened to me was getting sick around September 7th. I ended up in urgent care for bronchitis and an upper respiratory infection. They gave me oxygen and a breathing treatment and sent me home with a ton of meds. I called the pulmonologists office to let them know about my sudden illness. I spoke with Linda, the doctor's nurse, for 41 minutes on the phone. She was the first person who seemed concerned about the results of the sleep study and that I was so sick for so long without a diagnosis. She asked me to come into the office that afternoon so she could see me. She did a very thorough exam and asked a lot of questions. I left the office that day with supplemental oxygen. I could tell right away that adequate oxygen intake is a good thing. It felt so good to breathe. She took copies of my sleep study and my other paperwork to discuss with the doctor before my appointment the following week. Linda really cared, and her taking the time to evaluate me has made all of the difference in the diagnosis of my illness. Just days later, I was set up with 24/7 oxygen at home, including using it with my CPAP at night so I could get adequate oxygenation. On 9/12, when I had my PFTs done again, my diffusion capacity was 2/3 of normal. That was an improvement. My lung function wasn't as good, but I had bronchitis and the URI so that kind of made sense.

So, at 54 years of age, I was on supplemental oxygen 24/7 and taking diuretics to reduce the fluid overload in my body (hypervolemia). I was feeling older than I am. 

On 9/18, one month after first hearing about PH, I was in the cath lab getting my pulmonary artery pressure looked at. The cardiologist told me that I had PH (the pressure in my pulmonary artery was higher than it should be), that my body was overloaded with fluid and that I probably have hypoventilation syndrome (I breathe too shallow) due to being overweight. It was a bit overwhelming. The pulmonologist confirmed the diagnosis on 10/6. The PH Specialist confirmed the diagnosis on 10/7. They both told me that I needed to treat my sleep apnea, continue using oxygen 24/7 indefinitely, lose a significant amount of weight, consider moving to a lower altitude (I live at 6,700 feet) and definitely not travel to higher altitudes and keep my sodium intake to <2000mg/day and my fluid intake to <1.5L/day to combat the fluid overload. That was VERY overwhelming.

Thankfully, I was seeing a counselor to help me deal with being chronically ill even before I got the diagnosis of PH. She has been wonderful and extremely helpful. I also was dealing with the possibility of having lymphedema (causing the swelling in my legs) and had to work through the process of getting that diagnosis ruled out. I had to have a nuclear scan done where they inject the radioactive dye between your toes! Thankfully, it sounds a lot worse than it is. No lymphedema, so that's one less thing to worry about.

I have had a lot of questions for all of my doctors since being diagnosed with PH. There are 5 different groups of PH. It looks like I am in groups 2 (PH due to heart issues) & 3 (PH due to lung issues, which are sleep apnea and hypoventilation syndrome). Group 1 is the only type of PH that has specific medications to treat it. It is also the most rare and dangerous type of PH. Every single patient's experience with PH is different than every other patient's experience with PH. There are similarities, but everyone has something unique to their illness. That makes it difficult to treat PH. In my case, since my PH is due to underlying conditions, those underlying conditions have to be treated to make the PH get better. I have to treat my sleep apnea, my obesity, my fluid overload and my oxygenation issues. I'm hoping that if I treat those things, that the PH will get better and/or go away completely. I may have to deal with PH the rest of my life. To what degree, I don't know.

I have spent a lot of time on the phone with the PH Specialist's nurse. I wasn't supposed to see him again until April, but they want to follow up with me. So, I am going back up to Denver next week. The difficulty with having PH due to other illnesses is that no one is quite sure who is supposed to treat and follow up with the patient. It is a heart disease and it is a lung disease. It is complicated. Thankfully, the specialist is going to make sure that I get my questions answered and that I understand my PH as best as I can.

David and I are feeling overwhelmed right now. We are considering moving to a lower altitude if it would be the best thing to do. We still have so many questions. I am hoping that my appointment with the specialist next week sorts a lot of this out. I am looking at doing a Pulmonary Rehab Program 3x/week for 3 months to get me active again. I have basically been sedentary for the last 3 years. I'm hoping to get a portable oxygen concentrator (a machine that makes its own oxygen) so I don't have to bring oxygen cylinders with me everywhere and have to constantly be refilling them. I want to be mobile and get out again. I want to understand what my flavor of PH means to me. What can I do to feel better? What can I do, if anything, to halt/reverse/eliminate PH in my life?

I am going to see the ENT/Allergy doctor this afternoon to help with the constant nasal congestion I have. That keeps me from wanting to wear my CPAP mask at night. Wearing oxygen full time affects your nose and sinuses. What can I do to make sure I comply with wearing oxygen 24/7? I am going to see the sleep disorders specialist tomorrow. I know that being compliant with my CPAP, treating the sleep apnea and reducing the amount of time that my oxygen level is below 91% is crucial. I need to know if I do have the hypoventilation syndrome. I'm not sure if he would diagnose that or not but I'll ask him. And I definitely need to sleep better. Being rested is important, too.

I have a lot of huge lifestyle changes to make. I'm working on making those changes every day. I am looking at every option from surgical weight loss to moving from my beloved Colorado back to sea level (or as close as I can get). Being diagnosed with PH has changed my life. It has changed David's life, too. I am sad knowing that I cannot go back to Waldo Canyon to hike. My goal since I got sick 3 years ago was to get back to Waldo Canyon to hike again. It's at 7000 feet, though, so that is not an option right now. I can't go up to the summit of Pikes Peak, 14,114 feet, and get a "high altitude" donut (they just taste different up there!). Life is different now. I'm relieved to finally have a diagnosis so that I can move forward with my life, but it's slow going right now. I know that, quite honestly, one of my options is to do nothing and just get worse or at least, no better. I don't think I will do that, though. My pulmonary rehab program evaluation is next Thursday. I may be able to start the program on Friday, Monday at the latest. It will feel good to be active again.

The PHA website has been an invaluable resource since hearing about PH. Not only do they provide lots of information but they also provide a lot of support through online chats, telephone support groups, one on one email and telephone support and educational resources. I can't thank the people and other patients on the PHA website enough for being there for me and helping me to navigate through all this since being diagnosed with PH.

Thank you for reading this very lengthy post. I hope it helps you to understand the journey to a PH diagnosis and the affect it has had on my life. I hope it helps someone who needs to be diagnosed or has been recently diagnosed with PH. 

Written by Samantha-Anne Wagoner

Rare Disease Day 2014

Across North America and Europe more than 60 million patients have been diagnosed with one or more of the 6000+ "rare" diseases. If you were to broaden the inquiry to encompass the world you would need to add millions more. Almost every patient has family or friends that help them. I think it would be safe to say that worldwide, more than 1 billion people are affected by rare diseases!! That is staggering to think about! About half of those affected are children.
 
So what exactly is a rare disease? In the European Union, a rare disease is one affecting fewer than 1 in 2000 patients. In the United States, it is defined as affecting fewer than 200,000 Americans at any given time.

Symptoms vary, not just between diseases, but also largely from patient to patient with the same diagnosis. 80% of rare diseases have identified genetic origins. The remaining 20% result from infections (both viral and bacterial), allergies and environment, and most are degenerative and proliferate.
Misdiagnosis is a major problem for rare diseases, often because a common symptom will hide the underlying disease.  In Pulmonary Hypertension patients, we are often diagnosed early on as having asthma, or COPD. Lupus patients are frequently thought to have the flu in the early stages of diagnosis, or simply an allergy.

What is Rare Disease Day? Rare Disease Day began in Europe in 2008, coordinated by EURODIS on the international level, and National Alliances and Patient Organizations (“umbrella organizations that regroup several rare disease organizations in a given country or region”) on a regional level. The goal for the campaign is to raise awareness, mainly in the general public, about the impact of rare diseases. Last year, in 2013, more than 70 countries participated, with more expected this year. This year’s objective is “for WHO (World Health Organization) to recognize the last day of February as the official Rare Disease Day and raise increasing awareness for Rare Diseases worldwide.” 

Join Together for Better Care! Caring for those living with a rare disease is multi-faceted, requiring medication, physical assistance or equipment, medical consult, physical therapy, social services, respite for the family, and more. Worldwide there is a broad lack of scientific knowledge and quality information on rare diseases, resulting in delayed diagnosis, along with increased financial and social burdens.
Because of the increased awareness created by campaigns like Rare Disease Day things have gotten, and can continue to get, better! Awareness of rare diseases has allowed the implementation of more comprehensive approach to care. Better public health policies have been developed across the world. Cooperation in clinical and scientific research and an international sharing of scientific knowledge on all rare diseases has led to the development of new diagnostic and therapeutic procedures!

Now, how can you get involved? There are several ways YOU can get involved, and increase awareness… without even leaving your desk!

            Become a Friend- www.rarediseaseday.org/become-a-friend/

Patient organizations, health care professionals, drug developers, public authorities, and any association with an interest in rare diseases are encouraged to become Friends of Rare Disease Day! Just fill out the form, and join the network!

            Raise and Join Hands- www.rarediseaseday.org/join-your-hands/

Raise your hands in a symbolic gesture before The Day to show your solidarity with rare disease patients, take a picture, and upload it to the site!

            Tell Your Story- www.rarediseaseday.org/tell-your-story/

Tell us how your life has been affected by a rare disease, share words of encouragement, or an uplifting story.

Download and Share Communications Material- www.rarediseaseday.org/get-involved-download-our-communication-materials/

Social Media-

            Facebook- www.facebook.com/rarediseaseday

            Twitter- www.twitter.com/rarediseaseday

            YouTube- www.youtube.com/rarediseaseday

            Flicker- www.flicker.com/rarediseaseday

            Thunderclap- www.thunderclap.it/projects/8543-rare-disease-day2014-feb-28

                                    Wait… what’s that?!

Share a single message (In this case “Today is #RareDisease Day, the day to put rare diseases in the spotlit! Show your solidarity today http://thndr.it/1fkAYmz”) simultaneously in one THUNDERCLAP of awareness!

Want to learn more? Go to www.rarediseaseday.org for more information on Rare Disease Day, for a list of sponsors, and to become involved.

Improper Diagnosis: The Challenge of PVOD

Improper Diagnosis: The Challenge of PVOD

Pulmonary Veno-Occlusive Disease (PVOD) is a disease of pulmonary venules and small veins, characterized by fibrous thickening and abnormal intimal cellularity, leading to vascular obstruction and pulmonary hypertension (2013, Istanbul, Pulmonary Vascular Research Institute Workshops and Debates). It is an incredibly rare form of pulmonary hypertension. With doctors knowing so little about the disease, it is often improperly diagnosed, or completely undiagnosed. Patients and caregivers share their stories and reflect on their journey.

Mysterious Symptoms—

“I started being seen by numerous doctors who all had different diagnoses. I spent hours sitting through medical tests and waiting for results. I felt like a lab rat always being poked and prodded. My freshman year (of high-school) the doctors’ suspected severe asthma. Today, they, and I know they were mistaken. I actually have Pulmonary Veno-Occlusive Disease (PVOD.” –Carson; St. Louis, Missouri    

 “..At every move, I was still gasping for breath. In late August we all knew I had reached a plateau as far as getting better. I was terribly worried since I could not breathe if I did anything strenuous – just walking and talking were exhausting. Finally in November, after many medical tests, and visits to various specialists, a biopsy and heart catherization needed to be done. Armed with those results they sent me to Boston to see another specialist. By the end of November, we had a diagnosis – Pulmonary Hypertension Veno-Occlusive Disease.” –Marie; Maine

“In November of 2010, my mom and I were flown out by Flight for Life and the University of California hospital for surgery of what doctors thought to be a pulmonary embolism. After 10 minutes of surgery, the doctor said it was something more serious.” –Kullie (as told by daughter, Lakesha; Colorado)

“We had spent the last year of her life (2009-2010) trying to sort out why she did not feel well, why she was so thin, why she didn’t want to eat, and why she complained of not being able to get her breath. We watched her slow down physically, finding new hobbies like reading lots of books instead of riding her bike or playing soccer. She had many appointments with several doctors... wrongly she was given a clean bill of health.” –Aine (as told by family)

Challenges and frustrations…

“Adjusting from having my parents around to take care of me, to taking care of myself during my first year of college was difficult. I struggled to do things for myself, and realized that I needed to take better care of myself. I also struggle with knowing when to tell people of my disease, or who I should tell and who I should not.” –Carson

 “For years, life had been too busy – I had lived on a diet of promises to myself that my time to relax would come when I retired; so I was ready and eager to start my new life. Instead on May 2 I found myself in the hospital emergency room.” – Marie

 “We know now that had the doctors looked further into just that one symptom—shortness of breath with exertion—or had the cardiologist seen her for a follow up; or had they not ignored her low blood states; or had they not labeled her with anxiety; or had the pediatrician followed up where the specialist left off, they would have found pulmonary hypertension...a lung transplant could have saved her life had they not ignored her.” – Aine’s family

Coping…

“Being physically active helped me adjust with stress.” –Carson

“My mom turned to her family, specifically my brother and I; we were her pillars, her strength and support.” –Lakesha

Why do you believe it is important to keep looking for answers?

“I think we put a lot of trust into doctors, but we need to make sure doctors are giving us the best [care]. We need to press doctors to keep working and finding information. It was a nightmare to find out there was a PVOD specialist right here in Colorado after being flown out to California for treatment.” –Lakesha

 Advice for Others…  

“Always keep a positive attitude and appreciate the little things. Cherish being able to do things and be optimistic. Keep trying to do things, it is so much better to try and have to quit, than to not try at all. You have to have an attitude of resilience.” –Carson

“My attitude was good and maybe that’s what helped me cope. I discovered there was life after diagnosis. This acceptance does not mean I liked it, I can dislike something and still accept it.” –Marie

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 ...