OUR JOURNEYS

Patient story

Ami LongAmy Long

My name is Amy Long and I am 23 years old. I was diagnosed with PPH in January of 2005. My journey has been a long and complicated one that has no doubt changed my perspective on life and strengthened me. At the same time, however, the long journey to my diagnosis has challenged my faith in doctors, even very good ones, and it has been difficult for me to trust that my doctors know what is best for me. You will see how this distrust developed as you read my story. It is not a sad story though, but one of hope and grace, as I truly feel I have been blessed to be diagnosed so early and that I will have time here to figure out what PH means to me, and what greater purpose it has in my life. I am currently getting my Master's degree at UT to be a speech pathologist.

My first experience with PH came when I was a sophomore in college. While skiing with my family in New Mexico, I passed out while trudging up an incline to wait for a ski lift. My family was very concerned and I was put on oxygen at the ski base for 30 minutes. However, this isolated incident was easily explained away by altitude sickness. I thought nothing of it and continued life as usual. When I went back to school, I noticed that sometimes if I got up to go to the bathroom at night, I would nearly pass out. Sometimes I would make it back to my bed and would actually end up passing out for a few minutes in bed. I thought it was strange, but it was also very inconsistent and only happened a few times. Later, I began to notice that it was difficult for me to climb stairs. At the top of the stairs, my heart would pound and I felt like I needed to take a break for a few minutes. One time I actually passed out at the top of the stairs. Luckily my boyfriend was there to catch me. Again, I attributed this to just being out of shape, even though I am quite small, 5'3" and 112 lbs. When I told my mom about the passing out episodes and that climbing stairs was difficult for me, she was very concerned and wanted me to see a doctor. I live in Austin and go to UT, and my family lives 4 hours away in a small town in northeast Texas called Sulphur Springs. I saw my general practitioner who was baffled by my inconsistent symptoms: stairs are difficult sometimes, don't really feel out of breath, racing heart, random passing out. He had me see a cardiologist who did an ekg, an echo and a stress test and all were NORMAL! Even the echo. So, I continued life as normal, thinking no news was good news....funny how wrong you can be about that, and I just lived with the symptoms.

All my friends and family knew, Ami has this weird heart thing and the doctors don't really know what it is. Sometimes she needs to rest after stairs and inclines, and she sometimes passes out. End of story. My boyfriend's mom is a nurse in Austin, and she recommended I see an electrophysiologist (a heart doctor that specializes in arryhthmias and electrical heart problems) that she worked with at the hospital. Apparently, it was fairly common for electrophysiologists to treat young people like me who complained of racing hearts and fainting. It sounded exactly like what I was experiencing, so I went to see him. I wore holter monitor after holter monitor, some for 24 hours, some for a month, trying to see if there was some type of electrical problem with my heart. They never found anything. I was told that it couldn't be anything life threatening, so that was enough to keep me calm. They decided to go in and see if I did have an extra electrical pathway, even if the monitor didn't find one. I underwent 2 procedures called cardiac ablations, where they go in through a catheter and zap any abnormal or extra electrical pathways that they think may be causing the symptoms. For a short while after each procedure, I felt a little better, but it didn't last. Now I think my improvement was truly psychological. After these procedures I was even told that it may just be all in my head. They couldn't figure out what was going on.

Maybe it was because I was getting around just fine and didn't feel terribly short of breath. Maybe I just got so used to the way I felt that I didn't know what short of breath felt like and I couldn't remember what normal was. My doctors were stumped. I was told that getting exercise to strengthen my heart to get it used to beating fast was the best thing I could do. As it turned out, that was probably not very good advice. I eventually had a CT scan to rule out any congenital defects and the dye they used in the procedure, the one that makes you feel hot all over, made me pass out on the table! All my tests were inconclusive so AGAIN I returned to life as I knew it, just trying to live with the symptoms, hoping that I would eventually out grow it. Then, on the night of the presidential election in November, I woke up in the middle of the night with pain in the left side of my chest that wouldn't go away. I called my boyfriend's mom and she said she would meet me in the ER at the hospital where she worked in Austin. It turned out that I had idiopathic pericarditis (inflammation of the pericardium, the sac surrounding the heart), and I was given some medicine for the pain and to reduce the inflammation. One of the cardiologists who had seen me before while I was undergoing the electrophysiology tests came by to see me, and decided to do another echo, just to make sure that there was no fluid around my heart. This echo, two years after my normal one, showed that the right side of my heart was slightly enlarged. My cardiologist said I had pulmonary hypertension and that I needed to see a specialist immediately.

My family came to Austin to see me, and of course I got on the internet and scared myself to death trying to find out what this vague title meant. That was a very dark time for me, probably the roughest week of my life. I went to see Dr. Fernando Torres in Dallas, the most wonderful and caring doctor I have ever known, and had the right heart cath to confirm my diagnosis of PPH. My pressures were phenomenally high, around 120. Because of my age, my heart was compensating quite well, and I did just fine on the 6 minute walk tests. I was classified as functional class 2 and joined a research study to approve Tracleer for earlier stage PH patients. I lucked out and got the drug, and have been taking it (and coumadin)for about 7-8 months now and I definitely feel better. I haven't passed out since! I also feel much better when I stand up after sitting for a long time and my heart doesn't pound when I get up in the middle of the night. I can climb 2 flights of stairs without feeling terrible. I just feel better overall.

I just had another cath as part of the study in July, and my pressures have come down quite a bit. My mean PAP is now 55, down from 75, and the highest reading they got was 64, down from 120. My heart is also smaller. I am doing my best to enjoy each day, and not worry about things that haven't happened yet. I do get a lot of chest pain, and I suffer from occasional panic attacks and high anxiety. I think my 3 year journey to be diagnosed finally took its toll on me and came out in the form of panic attacks. I see a wonderful therapist and I have a tremendous support group that keeps me going. I am so thankful that I get to continue living my life the way I want to, continue going to school, and being relatively active (I walk for exercise). I am eternally hopeful that a cure is on the horizon.

 

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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.