Lydia

4 Yrs Old

More pictures of Lydia

Welcome Ava Reese, Born - June 7, 2007

Ava has tested negative to having IVA

Isovaleric Acidemia? How could our precious little girl have a rare, genetic disorder? That was our immediate reaction when we heard our 5-day-old daughter had IVA. Unfortunately, she was already in a coma at the time and fighting for her life. Here’s our story:  

My husband Curt and I were married two years when we decided it was time to start a family. After only one month of trying, I became pregnant. Nine months later, we delivered the most precious baby girl we had ever laid eyes on. When Lydia Nicole entered our lives at 4:36AM on May 18, 2003, she was bright-eyed and beautiful. She scored a 9 on the Apgar test. All those months of worrying were for naught. She was perfect.

Two days after we brought her home from the hospital, she stopped eating. I was breast-feeding and I couldn’t wake her up long enough to feed her. She felt very cold to the touch and seemed extremely limp. I called her pediatrician, who advised me to try giving her a bottle. Maybe she was going to be a “klunky” breast feeder. She drank about an ounce and fell right back to sleep. She slept through the night again (everyone thought we were so lucky) and didn’t want to eat in the morning. I decided to attend a breastfeeding class in the area – maybe I was doing something wrong! It was there that the nurse weighed her. Lydia had dropped from 6 lbs. 12 oz. to 5lbs, 3 oz. in three days. We rushed her to the hospital.

 It was while we were at the hospital getting processed that the Newborn Screen results came in. It showed Isovaleric Acidemia. We were immediately transported to Rainbow Babies and Children’s Hospital in Cleveland, Ohio (we live in a nearby suburb). Lydia was rushed to the NICU and surrounded by doctors and nurses. This turned out to be the worst night of our lives. Lydia’s ammonia level was over 400 and she was in a coma. The only way to get it down was by hemodialysis (a cleansing of the blood technique where a catheter was inserted into her neck through a surgical procedure and her blood was circulated through a machine). I’ll never forget the sight of her tiny body laying there, hooked up to all those machines, fighting for her life. Her doctors couldn’t guarantee she would make it through the night.

Thank God she did but it was a long road to recovery. Even though the doctors were starting to get her metabolically stable, they were still concerned about her long-term health and possible brain damage. After 16 days in the hospital, we were finally able to bring her home. It was like bringing her home for the first time all over again. We couldn’t have been happier.

Today, Lydia is a thriving, happy 4 year old. There have been no signs of brain damage from her initial crisis. She is developing like a normal toddler including forming words into sentences, learning her colors and numbers and showing the first signs of being ready to be potty trained.

Her diet plan consists of 16 ounces of formula a day (a mixture of X-Leu Analog, 80056 and Similac with Iron). She gets approximately 1020 milligrams through food. She eats a lot of potatoes, veggies, fruits, macaroni and snacks (fruit snacks, gelatin gels, popsicles, suckers, etc.). She also takes Glycine and Carnitine three times a day.

We’ve had three stays in the hospital since her initial crisis due to minor illnesses, but overall, Lydia is doing wonderfully. We go to clinic with her doctor, Dr. Arthur Zinn and dietician, Judy McConnell every four months. They look at her labs and determine any changes to her diet or medication dosages. Due to her initial crisis, she’s also followed by a pediatric neurologist, Dr. Nancy Bass. However, because of how well she’s doing, Dr. Bass graduated Lydia from her care after her last visit in August.

I know we’ve had some new IVA parents join OAA recently. Please feel free to contact me with any questions or concerns. Living with a child with IVA is definitely a challenge but the rewards are endless. We feel so thankful to have Lydia in our lives and cherish every day with her.

Sincerely, Tina & Curt Baranski Parents to Lydia, 4 yrs  - IVA tinab1940@wowway.com