Patient story

Karen Janjanin

I have pulmonary hypertension secondary to scleroderma. I was first diagnosed with PH in 2002 after having congestive heart failure. Prior to that time, I worked as a legal assistant and was experiencing no symptoms of scleroderma, Raynaud’s and some morphea. I went to bed one night and woke up the next morning in so much pain I could not breathe. After spending three days in the hospital, my rheumatologist told me I had congestive heart failure. When I was released from the hospital and followed up with my rheumatologist, he told me to see my pulmonary doctor. I saw Ronald Kotler (Pennsylvania Hospital) the same day and Dr. Kotler told me that I had PH. He told me that I had to see a specialist at Presbyterian Hospital, Harold Palevsky and that he (Dr. Kotler) could not help me.

It was then I got got really afraid for the first time. I met with Dr. Palevsky and Christine Archer-Chicko in December 2002 and I was told that I needed a heart catheterization in January. Dr. Palevsky told me about PH and, at the time, my condition did not look good at all. After meeting with Dr. Palevsky and Chris, I was scared out of my mind. I had a heart cath (femoral and Swan-Ganz) in January 2003 and I was put on Tracleer, Digoxin, Norvasc, and Lasix. I was told to apply for disability, which I got after about eight months. If not for my husband and parents I would have been homeless and helpless!

The Tracleer really helped me but it in a short time it affected my liver function and I had to come off of it. I remained on the regular meds until Ventavis was approved. Once Ventavis was approved, I was admitted to the hospital and my husband and I were trained in how to use and maintain the equipment. Ventavis and I did not get along together at all. Unless I had a new screen, each treatment took over twelve minutes and the headaches were terrible. By the time I got done the treatment, cleaned the machine, and got over the headache it was time for another treatment.

In the midst of all of this, we learned that my husband has Myasthenia Gravis (a rare neuro-muscular disorder that requires infusions of IVIG) and our world really began to get worse. Neither one of us could work and we had a hard time just getting to the store, cooking, cleaning, running routine errands. Life was very hard. In 2006, my parents helped us get a home in Rehoboth Beach, Delaware and we moved in July. By then, I was really very sick. I was gaining weight, not able to exercise, on oxygen 24/7 (even in the shower) and I got more and more depressed. By November of 2006, I was going into CHF again. By the time I was admitted to Presbyterian in January 2007, no one really thought I would make it. My weight was 206 pounds and I couldn't breathe. Once I was admitted to the hospital, the doctor began to diurese me and I started to feel better.

On January 4, 2007, while I was in the hospital in Philadelphia, my husband was driving home to DE and the hood of our car flew up while he was on the highway. When I got the phone call that he had almost been killed in an accident I thought I would rather give up and die. Nothing was going right and everything was going wrong! I went into the bathroom of my room and gave my life to Jesus Christ. I asked Him to take my life and use it for His purposes. I surrendered everything to Him - the diseases, the worry, the ups and downs. I got better from that point on!

It was not easy but I was determined! When I got out of the hospital I began to really take care of myself. I went on as low a sodium diet as I could live on and I began to really educate myself about my diseases and how to help myself. By April 2007, I was about 35 pounds lighter and feeling better. I was waiting to hear about the approval of either bosentan or Ambrisetan and I just kept living my life. My husband would take me to the beach and I slowly began to put my life back on track. I began to attend a Church and on June 10, 2007, my husband and I were baptized into Christ. I kept my positive attitude, kept to my diet, and kept determined to live life and serve Christ. By November 7, 2007, the date I started Letairis, I was about 70 pounds lighter than the 206 I had started the year with and I felt great.

In the summer of 2008, I began to notice that my abdomen was distended and I was having pain in my spleen. My PH doctor sent me to my liver doctor and then I went to another doctor who does just liver and not all of gastro issues. It was decided that I had to have another right heart catheterization, a liver cath and another liver biopsy. (I had had a liver biopsy done via my jugular vein in 2005 and learned that I had Primary Biliary Cirrhosis from the Scleroderma). My PH doctor did not think I needed a heart cath, but my liver docs insisted that the pressures in my belly were coming from "above."

So, on Monday, October 13th, 2008, I had another Swan cath. We learned that my pulmnonary pressures, which were once over 100, were in the 30s!! Praise the Lord! On Wednesday, October 15th, I had a liver catheterizaton and some biopsies taken. I learned that I have mild portal hypertension and we had the original diagnosis of PBC confirmed. We are waiting to hear more about the biopsy results.

The main message I have for all PH patients is that there is HOPE! I remember when was first diagnosed and given a very grim outlook and prognosis and I have survived! Since 2002 I had CHF twice, had to have my gallbladder removed, had a cancerous tumor removed, and I have only improved. God has worked a miracle in me and I live each day to serve His purpose. I eat healthy, I watch my sodium intake (like a hawk!), I restrict my fluid intake, and, most of all, I try to remain positive. I take Norvasc, Digoxin, Lasix, Aldactone, Letairis, and Ursodiol and my PH is wonderfully under control!

I truly believe that a large part of the living with PH has to come from within us. We have to educate ourselves about our disease and advocate for ourselves. We have to take charge of our own health and be willing to do what we know we have to do to feel better (like passing up those salty foods and eating healthy). I am not proposing that this is all it takes, we are all in different phases of PH and in different situations. All I know is that there is HOPE. In this life and the next.

By the way, I also call and write to our senators and congress members to advocate for PH! Each and every one of us should be doing the same thing because we need to bring awareness of PH to the world!

Thank you


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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.