GROWING UP WITH CONGENITAL CMV DISEASE

I guess I had a pretty typical childhood with some exceptions.  Here is what my mother has to say:

“Kristen was born 10:58am on April 5th, 1982 weighing 6lbs 1oz after a full term, non-eventful second pregnancy.  I was 33 at the time.  When she was first brought to me I noticed her skin was covered with little red dots.  The nurse told me it was from the pressure of the delivery and the fact that her skin was so fair.  Later I learned that this was petechiae and can be a sign of a congenital CMV infection.

She cried all the time and nothing consoled her.  At 8 weeks she had an inguinal hernia repair..  A week later her pediatrician, on a routine exam, found a grossly enlarged liver and spleen.  She was admitted to Albert Einstein Hospital in the Bronx and after a liver biopsy we were given the diagnosis of CMV. 

She responded to spoken words by smiling but had little physical movement.  As time progressed I felt something was wrong because she was not holding up her head or attempting to roll over.  At 8 months of age she was diagnosed with a dislocated hip and was referred to Helen Hayes Hospital in West Haverstraw, NY, a local rehabilitation hospital, where she was evaluated as having the physical ability of a 3 month old.   She was placed in a Pavlik Harness to keep the hip in place and started physical and occupational therapy and responded quickly.  At age 16 months I was told that the Pavlik Harness did not do what had been hoped and she would need surgery to release the muscles and tendons.  Through all of this we still did not have a definitive diagnosis.

It was at Helen Hayes Hospital that we first met Dr. David Roye.  Over the course of the next 6 years of rehab she had 2 additional hip surgeries (one at age 4 for femoral osteomoties and one at age 6 to remove hardware) and continual physical and occupational therapy.  There is a day I will never forget.  At the age of 2 ½ I had her at clinic waiting to see Dr. Roye.  She was at the point where she would “cruise” furniture and loved to push things.  She wanted her stroller so I stood her down on the floor and she took her first steps!  I cried because I had my doubts as to whether she would ever be able to walk.  She fell down a lot but she always got up again.”

 My brother, Peter, is 4 ½ years older than me, and I went through a phase of wanting to do whatever he did. That’s why I played several years of Little League baseball (mostly T-Ball), until it became very clear that sports were not my thing. I was in Brownies for a year, because Peter was in Cub Scouts. That also didn’t last.  It kind of took a while for me to realize that trying to fit in with “normal” kids wasn’t going to work. I just had to accept the hand I was dealt, and try to deal with it as best I could.

School was always a challenge for me. Not so much academically, although I struggled in math quite a bit. The worst for me was definitely gym. It was always frustrating to see other kids do things like make a jump-shot, or hit a home run, and think, “Why can’t I do that?” But luckily, I found my strengths in the classroom, and that helped even things out. I was always very good in language arts and reading. So whenever I would feel tempted to get discouraged over some of the things I couldn’t do, I would remind myself that I had talents, just in other areas.

In first grade, I was put in a Special Ed. class for most of the day, and I would only join my peers for things like art, music and gym. I don’t think I really should have been kept separate from the others because it made me feel like an outsider. I never had a chance to get to know my classmates all that much. Plus the fact that my Special Ed. class was doing reading that was far below my abilities. I could easily have kept up with a regular first grade class, in that area at least.

After that year, I was placed in a regular classroom, but was given special help, such as being able to sit close to the board, extra time for tests, etc. Most of my teachers were very willing to accommodate my needs. Things became even better in 4th grade, when I finally met with a teacher of the hearing impaired (due to the congenital CMV infection I am deaf in my left ear).  This teacher helped make sure all my teachers were aware of what my needs were, as well as helping me to stay organized, which was never an easy task! She basically stayed with me from then through high school. That was a great comfort to me when I moved from one grade to the next, knowing that I was already familiar with at least one of my teachers.

For many teenagers, a driver’s license is a major achievement, offering independence and freedom from parental control. Unlike a lot of my peers, I never really felt any great need to learn to drive. I rode the bus to school and to my part-time job. Plus I had a number of nearby relatives who could offer rides in a pinch. So I never even got serious about getting behind the wheel until after high school. In retrospect, I wish I had acted sooner. From past experience with things like riding a bike, I already knew I would take longer to learn than most people. But because of my late start, I don’t have as much confidence in my abilities as I should, in spite of having my license (after six attempts at the road test!).

When it was time to go to college I didn’t want to go far from home.  My parents and I were totally shocked when on senior awards day I was presented with a full scholarship to a local college that was literally “down the hill” from my high school!  I was fortunate in that the college that had a program for students with special needs, and I did receive a certain amount of accommodations there. Mostly I was given extra time for tests, or being able to type assignments rather than handwrite them. Initially, I planned to become a teacher, and possibly use my experiences to help other students in a similar situation to mine. When that didn’t work out, I changed my major to English, and managed to get a B.A., with a minor in psychology, thanks to all the education courses I took.

Although I am working full time now, I am not sure what I ultimately want to do with my life. But my parents raised me to have a “can-do” attitude, and to focus on my abilities, rather than my disabilities. So whatever I end up doing, I’m sure it won’t be defined by my CMV.

For nearly a year, my mom had been worried about me. Last summer, during a trip to Washington, D.C., she noticed a lump on my rib as I sat in my wheelchair.

My name is Kevin.  I am a 16-year-old, who was born with cerebral palsy that left me a quadriplegic and legally blind.  When I was examined by Dr. Roye, it turned out that I was suffering from such severe scoliosis that my rib cage had rotated 70 degrees, affecting my lungs, stomach and other internal organs.

My mom was devastated. Her first reaction to the scoliosis diagnosis was, “Why didn’t someone find this before? How did it get this bad”? But her anger soon turned to anxiety when she learned that extensive, challenging surgery was the only cure, and that if my spine rotation reached 100 degrees, nothing could be done.

It took her a long time to agree to the surgery. She was so depressed and lacked confidence in the outcome. She increased my physical therapy, bought a body brace and prayed I would be okay until the end of my freshman year at Nutley High School, where I am mainstreamed with a one-on-one nurse as my aide.

Thankfully, the brace and therapy helped some and my curvature of the spine remained at 70 degrees until my school closed for summer. But when I finally went into Morgan Stanley Children’s Hospital in New York City in June 2007, I experienced such extreme “white coat” hypertension, that the surgery almost had to be called off. Fortunately, anesthesia lowered my blood pressure.

For 11 hours while I was under the knife, my mom walked around the hospital and prayed and asked God for a miracle.

When Dr. Roye finally came to talk to her, he warned her about my appearance. He told her she may be scared when she saw me. My whole body was full of tubes, I was on a respirator, and because I had been laying on my stomach for such a long time, my lips and face were swollen. I stayed in the hospital for 6 days and everyday I would get a visit from a caring and hardworking staff member who wanted to do everything in their power to make sure that I was not in pain.  I couldn’t believe how many doctors and nurses came to see me on July 4th even though it was a Holiday.  

It turned out, I did not need weeks of post-surgical morphine; in fact I was sitting and laughing a few days later. Although I have still a way to go, my ribs are completely straight now, and I look better, breathe better and feel better.

I am just so grateful to Dr. Roye and his staff for talking my parents into having this procedure. My mom has told me that the one main thing that convinced her was when she heard Dr. Roye visiting the elderly homes and seeing people who are suffering from scoliosis he wishes that he had been able to help them out when they were younger.  The last thing my mom wanted for me was to be stuck in a home when I am older where my body is stiff and crooked and I can’t fit in any chair or move or breathe because of scoliosis.   

I am extremely grateful to my therapists, doctors and nurses for caring for me.  I am so happy to be surrounded by my family and friends who send us cards and their encouraging words.  I still go to therapy and my therapist keeps telling my mom that I laugh a lot more now than I used to.  My mom and I go down the shore every weekend even though the weather is now cold.  I love walking on the boardwalk.  Since my surgery, I can tolerate sitting in my power chair for hours.  So I make my mom walk for over 4 miles while I enjoy the freedom of driving along the beach front and having the ocean breeze and the warm sun shining in my face.

Jeff

Sonya

Samantha

Like many young female athletes, Samantha is a force.  Samantha can traverse a soccer field in record time, outmaneuvering her competition.  But also like other female athletes, she is susceptible to ACL injuries.  At the Center for Pediatric and Adolescent Sports Medicine, Christopher Ahmad, MD is conducting research to determine why the gender difference exists.  He instructs his patients that one of the reasons is women tend to increase their quadriceps strength more than hamstrings – creating an imbalance which leads to undue stress on the ACL.  Another reason is women tend to perform their sports in more upright positions.  Samantha will change her training regimen to include strengthening of her hamstrings and acquire a more flexed position.  And she will continue to run circles around the competition.

Amanda and John

Amanda and John, two years apart and brother and sister, were both born with Spinal Muscular Atrophy (SMA).  This is a group of inherited diseases that lead to progressive muscle degeneration and weakness.  Both children underwent spinal deformity correction and fusion to correct severe scoliosis and to improve their upper body alignment.  While the surgery was not exactly kid stuff, thanks to the surgical expertise of Dr. David P. Roye, Jr., both children are now able to hold their own bodies in an upright position, and get rid of their body braces.  And they are enjoying just normal kid stuff -- like going to Yankees games, fishing, playing music and hanging out with friends.

Chris