By the time I was 6 months old, the pediatrician could tell my heart sounds were not normal, and sent me to Children’s Hospital of Pittsburgh (CHP) for Cardiac and Orthopedic clinics. I am 58, and medically complicated, with Heart, Lung, and Spinal issues. I have 3 congenital heart defects. The lungs didn’t form correctly, and left lung is very small.
For whatever reason the doctors at CHP said, they couldn’t do a heart catheterization until I was 10 years old. The plan was to surgically repair the heart defects, but found there was very high pressure. They diagnosed the PDA and possible absent left pulmonary artery and pulmonary vein. It was decided not to fix the PDA, as it would keep me alive and act as a back flow release valve.
The high pressure, and bi-directional shunting, mixing the good blood and bad, is called Eisenmenger Syndrome. It is considered a more severe form of pulmonary arterial hypertension. My toes are a nice Periwinkle color, because the un-oxygenated blood goes to the legs, and toes. This causes me to tire easily.
I also have a hemi-vertebrae caused scoliosis. When I was 12, I had what I call the “Ancient Medieval torture treatment,” the halo-femoral traction. They put 4 pins into the skull, and attached a halo to which they added weights; 2 other pins went above each knee, one into each Femur. Those also had weights. They slowly built up the weights, until my spine was as straight as it would go, and I was “balanced.” I had many setbacks, mostly because of the lung issues. Then there was the major spinal surgery included a spinal fusion and Harrington rod (10”) Insertion; through it all, I spent a year flat on my back.
Growing up, I did not run fast or play sports. I was the last chosen for dodge ball, or the first chosen for the OTHER team. While in grade school, the school district offered a family swim, to learn to swim. I was sent home early because my lips were turning blue. With 4 brothers and one sister, I did a little “sports” with the family, ping pong, and wiffle ball out in the back yard. I was more into chess, puzzles, and always helped my mom in the school library.
I attended Seton Hill College through a BVR Grant (Bureau of Vocational Rehabilitation), I have a BS in Consumer Services.
I still see the cardiologists at Children’s Hospital. I ask if I am the oldest patient; the doctor says, no. I see the same cardiologist I have seen for 30 years, through the CHP, Adults Congenital Heart Defects Center, plus I also see an officially trained Adult CHD Doctor, and a Pulmonary Hypertension cardiologist.
In the year 2000, I started noticing when we would go to the local mall, whereas before I could walk around one store and go out to the main mall, and walk from one end to the other, and back again. BUT now I had to stop for rest breaks. I also explained to the cardiologist, I had to take rest breaks sooner in climbing up our hill. But it wasn’t until, March of 2009, I saw a Pulmonary Hypertension expert, at UPMC Presbyterian Hospital Pulmonary Hypertension Center.
When he told me, in order to get on any of the drugs for PAH, he would have to “drill into my jugular “, I balked and said I would have to think about it. After months of telling myself I should move up the appointment, in Feb 2010, I had two episodes of getting VERY out of breath with little exertion. I emailed about moving up the appointment, they called and gave me one within 2 weeks. On April 1, 2010, I saw the PH expert and a pulmonologist. I was scheduled for a pulmonary function test, a six-minute walk test, and later the pulmonologist ordered a CT scan on my lungs.
On April 12, 2010, 42 years after my first catheterization, I had my second catheterization. They did a right heart cath, by going down into the jugular and poking around in the heart. I am currently on PAH Drug therapy, Tracleer (bosentan). Tracleer can cause liver damage, so I now need monthly blood tests.
Before the Tracleer, I wasn’t able to walk very far after eating. The doctor explained it by saying, I couldn’t multi-task. IE: I couldn’t digest my food, and exercise. Heart needs one to digest the food, and can only work on one thing at a time. Within 3 weeks of starting Tracleer, I wasn’t as tired, and was able to walk further, even after eating, though I still need a nap in the afternoon. And when I over do it, I can still get exhausted, it is like I hit a brick wall. I now feel like I have plateaued. The doctor says the meds aren’t tested on Eisenmenger patients, and wants to wait before adding on a second drug. Recently I was tested and I now need night time oxygen.
I like to keep busy, for years, I volunteered at the local libraries, and worked the book sale.
I have made many items of various Needle arts, and have created about 25 quilts, both small, and several large ones. I won a blue ribbon at the Butler Quilt Show in 2008, and a People’s Choice award at the Tarentum Library Quilt show in 2005.
I helped with my last 2 Class reunions, doing most of the computer work that was necessary; and helping to plan the events. I scanned in and uploaded the class yearbook pages, and pictures from the reunion. I have two blogs, one for the medical and reference information, and one for my quilts. I love working on the family tree and researching genealogy information, and am working on uploading that information into the skydrive, for any unknown family members to find.
I am in over 30 support groups on Facebook, and post a lot of pertinent news items, Pulmonary Hypertension, Heart Defects, VACTERL, etc.
I love this idea of someone running in my place, being my lungs.