Phyllis' Story

My health problems all started sometime in 1995. The first thing I noticed was that my index finger on my right hand kept turning white from the last joint to the tip. I went to my Primary Care Physician, and he did some blood work. He said I had Lupus and wanted to put me on Prednisone. I said I wanted a second opinion. I went to a Rheumatologist, and he said I didn’t have Lupus but a Positive ANA and Raynaudes. He said that I probably had an unidentifiable Connective Tissue disease, but that right now he didn’t have enough pieces of the puzzle to diagnose it. He said to go home and forget about it. I had a Doppler of my arm because they couldn’t get a pulse on my right ulnar artery. The doctor said there was no blood flow from mid-arm to the middle of my hand. So, I had an arteriogram of my arm. It revealed that the right ulnar artery was missing mid-arm to middle of hand, but it was OK up the other side. They didn’t know what happened to it, but they didn’t think it was a birth defect. They said it had atrophied, been attacked by my own body or was in a deep spasm and wouldn’t let the blood pass through. The next thing that happened was I developed Irritable Bowel Syndrome, followed by loss of speech in both ears. I had to have a Hysterectomy, and then later my Gallbladder removed. I also started having swallowing problems which was diagnosed as GERD or acid reflux. In 2001, I had pneumonia, and after it was supposedly gone, I continued to have chills, low grade fever and strange food cravings. I went to a Rheumatologist, and he said that my immune system was turned on and couldn’t turn off. He gave me a dose pack of Prednisone, and the symptoms went away. He said at that time that I had Undifferentiated Mixed Connective Tissue Disease and Fibromyalgia. I also had dry eyes. In October of 2006, I started having shortness of breath. I had been troubled with shortness of breath on exertion for a few years before, but my recovery was quick and I thought I was just out of shape. This time, my recovery wasn’t so quick, and I became worse and worse. My chest felt heavy like it was weighted down. My back hurt between my shoulder blades. I was very tired. I went to my Primary Care Physician. He did a lot of tests, and put me on Thyroid medication for low thyroid, but I didn’t get better. My legs were swollen and hurt a lot. I was getting weaker and weaker. I went to a Cardiologist, and she said she couldn’t find anything wrong. I had several tests there, including a Nuclear Stress test. I could hear my heart beating in my ears. I thought something was very wrong. I was sent to a Pulmonologist. I told him my medical history and asked if he thought I could have PAH. I had been doing research on the Internet, and that seemed to be a fit as far as everything I knew to be true of my symptoms. He said it was a likely possibility. He suggested I see a PAH specialist at Vanderbilt University Medical Center in Nashville, Tennessee by the name of Dr. Ivan Robbins. I went to see a Rheumatologist instead. He did a lung scan, and he said I am calling your Pulmonary doctor. You need to get to Vanderbilt ASAP. I think you are in danger and have PH. I was put on a list to see Dr. Robbins on March 13 of 2007. On February 9th, I woke up about 1:17 a.m. (one week to the day prior to this, I had passed out) and told my husband I needed to go to the ER as I thought I was having a heart attack. I told him if I waited until March 13th, I would be dead. He called an ambulance. I went to the local hospital ER. They admitted me, and started me on Lasix. They called my Cardiologist, who in November couldn’t find anything wrong with me except for a slight heart murmur. She was shocked at my current condition. My Pulmonary doctor came as well. He called Vanderbilt, and Vanderbilt said I could come straight to their hospital as soon as they had a bed. I was taken by ambulance the next evening. Once I arrived, they assigned me to a team of doctors. Dr. Robbins also came to see me. They did a lot of tests including a V-Q Lung Scan, Echocardiogram and Right heart catheterization which showed PAH. I was diagnosed as a high stage III. I went home after five days on Revatio, Lasix, Potassium, Warfarin, and Ventavis was ordered for me. Although, Dr. Robbins thought I needed Flolan. While I was in the hospital, they pulled 26 pounds of fluid off my body. No wonder, I hurt everywhere. After a month on Ventavis, I agreed that I was backsliding and needed Flolan. So on April 1, 2007, I went on Flolan. I have progressed so much since then that now I am a candidate for either Inhaled Remodulin when it comes out of trials or Letaris and Revatio combination therapy. Time will tell. Thanks to Vanderbilt, I am alive and showing signs of progress. As far as my Connective Tissue Disease goes, they think I may have a form of Scleroderma or a form of CREST, but they still aren’t certain.