Carson's Journey with PH+

    Carson Smith was diagnosed with Pulmonary Hypertension (PH) and Pulmonary Veno-Occlusive Disease (PVOD ) her freshman year of high school and has not let the disease slow her down. She is currently enrolled in a Masters of Public Health degree program.  Upon graduating this will be her second Master’s degree.

   

    Since we last heard from Carson in her “PHA Journey’s” article, she has faced a number of great achievements, difficulties, and eye-opening experiences, all allowing her to grow as an individual.

   

    In 2009, Carson graduated from Washington University with a degree in Anthropology. She worked for a year and used this time to adjust to her limits. During this time she was faced with the stress of having her files transferred from a children’s, to adult hospital, and meeting with new doctors who were not aware of her medical history.  Being a natural born athlete however, physical activity helped her cope. Soon, Carson was alerted that she was no longer considered sick enough to remain on the lung transplant list. The news left her with bittersweet feelings. While there was a breath of relief to know she was healthy enough to be kept off of the list, it also left her with a lack of security. Carson had already experienced the process of being added to the transplant list. With this background she knew that if one day she did need a transplant, she would have to go through the strenuous process of getting back on the list, and waiting for a donor.

  

  In August of 2010, Carson enrolled in Morehead State University in Kentucky where she pursued a Master’s degree in Public Administration.  By December of 2010, she found herself being rushed to an emergency room and later airlifted to a hospital in St. Louis. Doctors had discovered an accumulation of fluid in Carson’s lungs, leaving her sedated for 6 days and in the hospital for a total of ten. Carson had always considered herself lucky because she did not experience a lot of the things other PH patients did. She was never reliant on oxygen, and was always able to take oral medication. Upon being discharged from the hospital, Carson realized once again that while she was not on oxygen, and was able to continue in certain physical activity, she still suffered from a severely dangerous disease. Even with this setback, she graduated in 2012 and enrolled in a Master’s in Public Health program in August of 2012.

   While faced with the complications of living with a chronic disease, Carson has sustained a positive attitude, and has learned to appreciate the little things in life. She has maintained an attitude of resilience, and believes it is a trait that all PH patients should adopt. She states “it is so much better to try and have to quit, than to not try at all. You have to continue to fight, and use your support network.” For doctors, Carson believes you should “allow patients to test things.”

Josiah's Journey with PH+

by Jan Windz

When we met Josiah he was living in a group home for medically fragile children. At that time he was 4 years old, very tiny, non-verbal and had just started to walk. He was wholly g-tube fed and on oxygen 24/7. He would become cyanotic when you took off his oxygen to pull his shirt over his head. He was considered to be terminally ill and non-adoptable. His prognosis was that he would be dead within a year without a heart lung transplant. However, he was not considered a candidate for a transplant because he was in the foster care system. He carried diagnoses of primary pulmonary hypertension, complex congenital heart disease, severe infantile scoliosis, and Noonan's syndrome. He was also, at that time, thought to be autistic.

We had been approached about adopting Josiah because we already had some experience with transplantation. On August 31, 2001 I was blessed to be our adopted son, Felix's kidney donor!

As soon as we brought Josiah home I got him in to the pediatric cardiologist at UC Davis Medical Center in order to have them prepare the packet for transplant evaluation at Stanford. About a month after we sent the package to Stanford I received a call from Dr. Jeff Feinstein, at the Wall Center for PH, Lucille Packard Children's Hospital. He said "before we jump to transplant, why don't we actually try treating the PH!"

Since Josiah has begun being treated by Dr. Feinstein he has become a new child. He has now been on Tracleer (Bosentan) for almost three years. For us this has been an absolute miracle drug. He has never had any side effects. The child who became cyanotic in moments of being off of his oxygen now needs to be reminded to wear it. He can go outside without it and ride his bike for 30 minutes at a time! He still gets most of his nutrition from his g-tube feedings, but he is taking food orally as well. He hasn't stopped talking in the past two years (except when he is asleep)! He is learning, loves school and has only had one, very brief hospitalization since he has been with us. A year ago Sildenifil was added to his regimen. While the affect hasn't been as dramatic as with the Tracleer, it does seem to be helping as well. We are currently anticipating surgery in December for his scoliosis. Dr. Feinstein will work with the surgeon, also at Stanford, to be sure that Josiah will tolerate the surgery.

We don't know what the future will bring. Josiah still has severe pulmonary hypertension. However, he is currently doing incredibly well. And every day brings new advances in medicine.

We are truly blessed.

My Life with PH + Lupus

by Stacey Gausling

I have two children named Lupus and PAH. Lupus is 14 years old and PAH is 7. Lupus tends to be on the quiet side but when she gets upset, she confuses everyone. PAH came into my world with forceful energy. She demanded so much attention that I was wiped out like never before. Her tantrums were so bad in the beginning. She requires a lot of daily care and has her special “buddy” that has to be with her at all times, ever since she turned 2. I have to carry her “buddy” in my purse or a fanny pack 24/7. She also requires a special diet and I can’t run too far without her tugging at me wanting attention.  On top of that, I am trying to be a super mom and working full time. 

Last year, Lupus got tired of all the attention that PAH was getting and decided it was her turn. She threw a major tantrum and accidentally kicked me in the kidneys. Then she got involved with a bad influence named Anemia which got so bad that it did a number on my blood pressure.  I had to take a one week vacation at La Resort Hospital to get over that one! Well, PAH was having none of that. She demanded to have a makeover for her “buddy” which required another one week stay at La Resort.

As a parent of a teen and an adolescent, I am challenged daily with these 2. The level of care is more that I would have ever imagined, but it gets easier with each year. I am looking forward to seeing them in their 20’s and hopefully they will find their own lives and leave the nest. 

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.