Tyler was born February 18th 1997 at 9:35pm by emergency cesarean section. He was delivered c-section due to fetal distress. He weighed in at 7 lbs 12 oz. and 20" long. Due to aspiration of fluid during birth Tyler was put on IV antibiotics a few hours later
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Tyler, Summer of 1998
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From the start, Tyler was a very "spitty" baby. He spit up during and after most feeds. After five days in the hospital we came home. Tyler seemed to progress normally for awhile. His weight gain was slow because he continued to vomit after every feeding. Despite his lack of nutrition Tyler was a very happy smily baby.
At about 3 months we noticed that Tyler could not hold his head up. He was very floppy. He picked his head up long enough to look the other way but could not hold it up for any length of time. With his poor weight gain, lack of strength and "floppiness", our family doctor sent us to a pediatrician with our concerns. From there we made an appointment to see a pediatric neurologist.
August 18th 1997 at exactly 6 months old we went to Hershey Medical Center for our neurology appointment. At this age Tyler is still not able to hold his head up. He is rolling from back to belly once or twice but mainly from side to side to get toys. He scoots in circles on his back to look at things. At his first visit we were told Tyler had developmental delays and failure to thrive and low muscle tone. We were referred to the Early Intervention Program in our area. He also had a series of tests run, including an MRI. This showed that Tyler's cerebellum did not develop properly. This resulted n a "Dandy-Walker Variant" finding in the brain, giving him a diagnosis of Dandy Walker Syndrome. We were referred to pediatric GI.
Tyler had his early intervention evaluation at about 9 months. He was finally rolling consistently from belly to back and from back to belly. he started PT, OT, Speech and home visits by a preschool teacher. We focused on strengthing, sitting and pushing up on his arms while on his belly. Tyler was still not gaining good weight. He had several formula changes to increase calories and try to reduce vomitting but it didn't help. Neither did the reflux meds Reglan and Zantc.
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Megan, 6 - 8 weeks old
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We finally decided on g-tube placement. September 4th, 1998 Tyler was admitted to HMC for g-tube placement and nissen fundoplication surgery at almost 19 months. After starting supplemental feedings through the g-tube Tyler's strength and energy increased, as did his weight. He gained 17 lbs in 6 months. The amount of tube feeds was decreased. The surgery was a success. Tyler was sitting independently, creeping, pulling to stand at the couch and taking steps in a walker.
At 22 months, Tyler got a baby sister, Megan. Megan was born November 24th 1998 at 01:05 pm by c-section. She weighed 6 lbs 11oz. Megan too, had problems from the start. I was not able to see her for nine hours after she was born.. She had problems with feeding. Her heartrate and breathing increased during feeds, she had a poor suck and vomitted from the very first feed. She could only tolerate 1/2 ounce of food. Whe she left the hospital she weighed less than 6 lbs.
Knowing the problems Tyler had we noticed Megan was following in his footsteps to a worse degree. We got started right away with diagnostic testing. At about 6 weeks a home nurse inserted a tube in Megan's nose for feedings. I was shown how to reinsert the tube because her vomitting made the tube come out. This tube did not work. We went to HMC to have an NJ tube inserted through her nose directly to her intestines. This reduced some vomitting but not enough to increase her weight.
January 27th 1999 Megan had an MRI. She was shown to have an underdeveloped cerebellum. At this time she was given the Dandy-Walker Syndrome diagnoisi just as Tyler was. At this point I got into the Dandy-Walker website and checked things out. The only similarity Tyler and Megan had to the other children was the cerebellum development, no other physical or developmental similarities existed.
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Tyler and Megan, Spring / Summer 1999
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At 3 1/2 months Megan was admitted to HMC for failure to thrive. After 8 days of observation she had surgery for g-tube placement and a nissen fudoplication on March 16, 1999. Even after these surgeries she continued to profusely vomit. Even through all her troubles Megan managed to have a smile that lit up a room. Throughtout the next few months Megan continued to vomit every feeding. We resorted to the j-tube placement along side her g-tube to try to reduce vomitting by going directly into her intestines. A few months later we thought we were safe to remove the j-tube because she was gaining weight. We thought wrong. She started vomitting again. Over the last few months I had been in contact with other parents on the Dandy Walker listserv asking questions about Megan's continued problems. We were beginning tht think that Tyler and Megan had something other than Dandy Walker Syndrome. A parent said I should check out the CDG website so I did. I printed the information for further reference. In the meantime Tyler had surgery for an undescended testicle in early February of 2000.
March of 2000 after another few months of continued vomitting Megan was once again admitted fo the hospital for a battery of tests. I took the information I had printed from the CDG website because every characteristic on how CDG presents Tyler and Megan showed from; failure to thrive, hypotonia, inverted nipples, long fingers and toes, peculiar fat pads, and the list goes on... I asked the doctors to read the information and then asked if they would run the test even though it may be a long shot. They did. March 24, 2000 we got the news Megan tested positive for CDG ! So at 16 months exactly, we had a correct diagnosis for Megan.
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Tyler and Megan, October 2004
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A few weeks later at one of Tylers visits we asked to have Tyler tested knowing what the results were going to be. On May 18th, 2000 we got the news confirmed. Tyler too had CDG. After being in contact with the CDG listserv I was put in contact with Dr. Donna Krasnewich from the N.I.H. She helped us get the typing done. A few weeks and a few vials of blood later we knew that Tyler and Megan were both CDG Type IA!
Although getting a definite diagnosis was heartbreaking it was also a relief to know exactly what we were dealing with. Even though we know that there is is no cure for CDG this diagnosis shed a whole new light on our situation. Making decisions came alot easier now. At first everything revolved around nutrition.
In November 2001 we spent 6 weeks at HMC for an extensive day treatment stay at feeding clinic. During this stay Tyler learned to consistently feed himself and increased hi tilerance of textures. When we started Tyler could only eat smooth textures. By the time we left he was eating a large variety of foods that were fork smashed or chopped. Megan, on the other hand, we tried to increase the amount of food she could tolerate. This was a tough thing for her but we learned the signs to look for when she got full. She also learned to tolerate more food from the spoon. Over all it was a good experience.
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Tyler and Megan, February 2005
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January of 2002, Megan started preschool with Tyler she too enjoyed the experience. The other children really taught her alot. The kids are thriving! Eating, drinking, slowly gaining weight, walking in walkers and fighting with each other like typical kids. Here we are in 2005 Tyler is in mainstream "total inclusion" first grade. He is in 1st grade with his peers yet he has a special ed teacher to teach him also. He's also got a personal aide. He is learning to tell time and spell. Megan is in a full time life skills class and also has a personal aide and is learning very well.
We hope our stories about Tyler and Megan are an inspiration to other parents! No matter how tough things get we can NEVER give up on our children. We as parents have to keep pushing them to make their lives as productive as possible no matter what the obstacles are.
eff: 04/08/2005