Jenny's Story: PH and Lupus, Fibromyalgia and Sjogren's syndrome

In August 2010, Jenny Klick of Winter Springs, Florida found herself out of breath – she couldn’t even walk to the bathroom. She attributed her symptoms to smoking and her limited airway disease. However, she wasn’t quite sure and went to the doctor anyways. Her doctor ruled out upper GI problems and sent her to a pulmonologist who concluded she just wasn’t taking her Advair correctly. Still out of breath, Jenny goes to a cardiologist. He performed an echocardiogram where he determined Jenny may have pulmonary hypertension. Like too many patients, she was already in respiratory failure and immediately went to the emergency room. 

In order to confirm pulmonary hypertension, the cardiologist performed a right heart catheterization. However, Jenny’s doctor could not complete the procedure because her pressure was too high. Nonetheless, Jenny finally had a diagnosis – Class IV PAH due to the lupus that never really caused her too many problems. 

Though the diagnosing cardiologist noted she would eventually pass away from the disease, he didn’t faze her. Having several other chronic illness including lupus, fibromyalgia and Sjogren’s syndrome, Jenny understood she could not be cured but knew she could continue life with the positive outlook she’s always had. Once Jenny was placed on Revatio and Tracleer, she was released from the hospital and began to feel a lot better and continued life as usual.  

A few weeks later, Jenny made an appointment with Dr. Tarver, a well-known PH specialist in Orlando. Like many patients, Tracleer was beginning to affect her liver and began taking Tyvaso instead. Jenny began feeling better and stopped thinking about her new diagnosis. As Jenny describes, living with PH became her “new normal.”

It wasn’t until Jenny came across an issue of Pathlight did she realize how devastating the disease could be. Soon after, she attended her first support group where she met other patients and caregivers. Months later, she became a support group co-leader and is now in charge of scheduling guest speakers for the group. Jenny loves her role citing “everyone in the PH world is eager to lend a hand and tell their story.” Jenny’s involvement in the PH community extends to her participation in PHA’s 10^th International PH Conference and Scientific Session last summer. There, she met more supportive individuals who directed her to PH Family, a community-operated Facebook page. She remains active with the community on Facebook, PH Central, and goes to the PHA website frequently. Jenny is also a Tyvaso Peer Mentor for United Therapeutics’ Peer Network.

Though Jenny has been fortunate throughout her journey with PH, in January she was hospitalized for a week with a serious respiratory infection. Since then, she reports feeling like “a typical PH patient” as simple task like going up stairs is now very difficult for her. Her doctor concluded her limited airway disease decreased the effectiveness of Tyvaso so her dosage was increased from 12 puffs to 15 puffs though the average dose is 9 puffs. However, her heart rate is still out of control and hopes to find a solution soon. 

It is important to mention Jenny has a strong faith in God and has kept a positive outlook throughout the last couple of years. Though she lives with other serious chronic illnesses, she admits PH has been another beast and is thankful for the support she has received from her family and friends. Her husband especially has been a great caregiver to Jenny and has worked to learn as much as he can about PH. He even called family and friends to talk to them about her new diagnosis so she wouldn’t have to. Jenny knows she is “very blessed to have support and understanding” from those who care about her. She is also thankful for PHA and wishes for a day when she can receive the same great support for her other illnesses. 

By Imani Marks, Patient & Caregiver Services Intern

Based on an interview with Jenny Klick 

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 ...