Steven

Name: Steven

Current Age: 30

Occupation: Newspaper photographer and reporter

Your Family: Married, September 2003, 3 y/o son (adoption pending)

When you found out about your condition: I was born with it.

What symptoms you had: "blue baby syndrome" and failure to thrive, was

diagnosed with a congenital heart defect called Transposition of the

Great Vessels

How you prepared for surgery: Too young to remember....

What type of surgery you had: Mustard Proceedure

Medications before surgery: Too young to remember....

Medications after surgery: Too young to remember....

Type of valve you have: St. Jude's Mitral valve, circa 2003

Type of valve you had: none

Hospital where your surgery was done: Buffalo Children's Hospital and

Cleveland Clinic

Your experience there: BCH: Too young to remember.... CCF: Long, boring,

a bit lonely...

Your first few days home: Mmmmmmmmm..... I spent a little over two

months in a hospital, it was SO wonderful to be home again and Spring

was beginnign to bloom...

Your recovery since then: Rough post/op. I spent about 5 weeks on a

ventalator in and out of consciousness. I don't remember any of it. What

i do remember is laying in the step-down unit. I had all kinds of IV

lines and a feeding tube running into me, at times I also sported chest

tubes as a result of internal bleeding incidents, all of which required

surgery to correct, I only remember the last one. I had several blood

transfusions during the initial surgery and afterwards. At one point

early on (after the tube was removed) I had tried to get out of my bed

and I think I had fallen, opening an incision in my left groin. I

remember being helped back into bed and having to lay still, flat, for

about 8 hours. The next few days i had a constant "baby-sitter," a nurse

who stayed by me to make sure i didn't do something I wasn't supposed

to. I didn't get to eat real food until about the last week and a half

of my stay. I got visits from a respiratory tech who administered

breathing treatments every 6 hours, physical therapist, occupational

therapist, daily blood draws and x-rays. As my condition improved I was

able to leave my bed and walk around, tentatively at first, with the aid

of a walker, the OT and the PT plus anurse or two in tow, along wiht my

IV pole. Then I graduated to just using the IV pole then, once I was off

the IV's, I was able to move around on my own, with permission. I took

several walks a day up and down the hallway of the "ward" where I

stayed. At one end of the hall there was a large tropical fish tank and

I spent a lot of time sitting there watchign the fish, that was really

nice. I sat in bed, I slept, watched a lot of TV. I had some reading

material and sketching supplies (I'm an artist) with me though I

couldn't do much because I had lost a lot of fine motor control from

being "asleep" for a month straight. one of my favoirte moments was

during a monring when the team was making their rounds. I had been

sitting in a chair watching TV with my walker nearby, and as they came

to the foot of my bed, I got up out of the chair and moved over to join

them. My surgeon, a specialist in pediatric heart surgery named Dr.

Roger Mee, just beamed. he had fought so hard to save my life and to see

his expression as I showed real signs of improvement was wonderful. The

decision to finally release me came about a bit suddenly. They had been

looking for a place to put me in a nursing facility closer to my home (I

was in Cleveland Ohio, I live in Western New York) when the physical

therapist started pushing for me to just go home. She felt I was at a

point where I was self-sufficient enough to take care of myself. I was

bathing myself and mobile, I even had a shower at one point in the

hospital. About this same time the feeding tube came out. I had been

asperating during swallow tests, a result of vocal cord damage from the

extended intubation. I remember two of these tests, though I gather I

had more. You're placed in a moving x-ray type scanner which takes live

action images of your body as you do what the technicians say, in my

case, ingesting food. The food is laced with barium and is in several

different consistencies, like water, milkshakes, pudding, crackers, etc.

The idea is to watch where the food goes as you swallow it, asperation

means it is travelling into your lungs rather than down your esophagus

towards your stomach. Food in your lungs is extrememly bad and can cause

very serious pneumonia and all other bad things. I was finally given the

OK to eat, I remember the last test well and trying not to get my hopes

up. I had been given swallowing exercizes and was allowed to suck/chew

on chipped ice and a cup of lemon ice twice a day (the tartness helps

trigger your swallow reflex.) They evaluated the results there in the

room while I waited, I watched the recorded exam with them, and

concluded that I was OK to eat, soft foods at first like juice and

chicken broth, then on to firmer stuff within a day or so. I just wanted

a glass of orange juice and it was the best orange juice I had ever had.

Later, I realized it really wasn't all that great... I waited until my

father finished up his semester (college professor) before starting

cardiac rehab. It was 3 days a week in the mornings, about 90 minutes a

session, depending on the wait for machines. They had several stations,

a rower, stationary bike, treadmill, pulleys, and argometer (hand crank)

plus warm up exercises and a daily weigh in. I progressed very nicely

through the summer and stopped about the first week of September as my

wedding closed in (we were married the 14th.) I'm so much stronger now

than I had been over the last year or so. I'm not as good as I used to

be, but I'm worlds better than before surgery. I'm no longer in heart

failure which is real important to me. I'm watching what I eat, trying

to follow a low sodium diet with a lot of my own cooking, guided by a

few decent books on the subject. I stay fairly active. I started taking

kung-fu (something I've always wanted to do) in December, 2003 and I

started back to work at the end of August. my wight's a little higher

than it should be, but I'm working on that one and it's not fluid

retention, just fat. Long-term prognosis looks pretty good. I'm now

seeing my regular cardiologist at 6 month intervals.

How you found out about this site: From an email list serv for adults

wiht congenital heart defects.

Any other comments you can share: I was born with a condition called

transposition of the great vessels (also known as transposition of the

great arteries, TGV, TGA, or simply transposition.) In transposition the

large vessels that transmit blood from the heart to the lungs and to the

body were switched, in effect causing two "closed loop" systems One

system moved blood from the body to the heart and back out to the body,

the other from the heart to the lungs and back to the heart. Oxygen

couldn't be infused into the blood and carbon dioxide wasn't removed.

This situation is quite fatal unless accompanied by an atrial or septal

defect, a hole between the chambers of the heart allowing blood from

both loops to mix. My first surgery was performed at Buffalo Children's

Hospital by Dr. Subramanian, one of the leading pediatric heart surgeons

of his time, and there weren't very many in the early 70's. After my

heart was repaired I lived a fairly typical childhood and normal life. I

have a brother who is about two years younger than I and we were about

equal in physical ability and activity. I played actively with other

children and while I didn't quite have the endurance that others had, I

could still participate without trouble.

I began to encounter difficulties early in 2001. I had developed a nagging, persistant cough which went undiagnosed for about six months. Early in 2002, during the process of trying to pin down the source of the cough, a chest x-ray was ordered which revealed an enlarged heart. This lead to an echo with my cardiologist who found a leak in part of the original repair to my heart. I was put on an ACE inhibitor, lisinpril, in hopes that the leak would close up. Later that summer, after having had a hernia repaired which was probably induced by a heavy coughing fit, I started to show signs of heart failure, mainly fluid retention in my lower legs and in my lungs. This stuff was hard to diagnose because my presentation of symptoms was very atypical, probably due to the way my heart operates versus a "normal" heart. More medication was added to my list including a pair of diuretics. In December of 2002 I started vomitting after meals, excessive fluid within my system. A discussion had been touched upon regarding the possibility of a transplant through the Cleveland Clinic and when this latest problem developed, my cardiologist set up appointments for me have the evaluation for transplant. It was scheduled for January, 2003 and, after review of an extensive physical exam including echo tests, stress tests, a cath, MRI scan, vascular scans, a final diagnosis was made. I had a failing tricuspid valve, a leak in the baffle which helped reroute blood within my heart, and Stage III heart failure. I was scheduled for surgery in March to either repair or replace the valve. I nearly didn't make it. The next two months found me becoming progressively worse. It was a hard, cold winter and I had to stop working about a month before surgery. While I wasn't bed-ridden, I was nearly so. I spent a lot of time just sitting in one place or another. I was coughing so much I had lost most of my voice and, while it wasn't openly recognized or adressed, my kidneys were failing. I returned to the Cleveland Clinic the day before my scheduled surgery. They performed a number of pre-surgery tests including another echo and thought they had found an infection on the tricuspid vave which would have to be treated before I could have surgery. As it turned out, that "infection' was the remnants of my tricuspid valve which had all but disintegrated during the previous two months. On March 13, 2003 my kidneys and liver shut down and I began to die. I was rushed into emergency surgery at about 9:00pm that night, after my father and fiance' had elft the hospital to where they planned to stay in Cleveland, my aunt's house on the west side. They got word when they arrived "home" and immediately turned back into the city. I spent all night in surgery and the next month fighting for my life while on a ventilator under sedation. I had periods of "wakefulness" but I was never really coherent enough to remember anything. My intial recovery in the hospital was rough. I had a number of setbacks including internal bleeding, damage to my vocal chords, difficulties in adjusting to the medications I was on and in regulating my heart. I spent five weeks laying in bed on my back and had lost a lot of fine motor control and muscle tone. I also lost about 35 to 40 pounds. Once I got moving around I started to improve a lot. Gradually, they pulled back on the medications I was on, the IV poles and lines and finally removed all IV's together. I could walk about on my own (with permission from the nursing staff) around the floor where I stayed and spend a lot of time enjoying that freedom. The first week of May I was discharged and returned home to my family. I had the summer to rest and recouperate. One of my goals before going in for surgery had been to attend the wedding of one of my closest friends. He was married May 31st and I was there, with most of my voice back, and we were all happy for all kinds of reasons. I started cardiac rehab just before than and was attending throughout the summer. That summer i spent a lot of time focusing on other parts of my life that had been neglected for a long time. I've found a renewed passion for my art, and in my music (I play 'cello and electric bass) and I was actively planning a September wedding with my fiance (we had picked a date over the phone while I was in Cleveland about 6 weeks after surgery, I told her wanted to get married in

August...)

I'm back at work. I've always had a part time position, though now I'm back close to my old 30 hours a week. I'm healthy now, I'm not in heart failure. I'm active and I'm enjoying life. I've made a lot of changes in my diet and I'm taking kung-fu, working on a form called "qi-gong" which means, "energy breathing" and works to expand lung capacity and build flexibility. I feel the best I've felt in close to three years. I'm still tired at times, mostly because I don't get enough sleep at night due to my late nights at work and a 3 year old son who insists on waking up before 8am. I'm married, four months now, and I'm looking to the future.

I've always been "above the bar" in my status with a heart condition. I believe I still am. I've heard from a lot of other people who have about the same condition I have and most aren't as healthy as I. They may not have had the valve trouble, but they've had other corrective surgeries for complications that have arisen due to their condition. It's a rather serious condition to begin with and can go wrong in a great many ways. I think I'm pretty lucky to have had only this one (albeit severe) glitch in my heart condition.