On October 26, 2002 Azaria Sage Lucille Baker was born. (Azaria is pronounced ah-z-AIR-ee-ah, sounds like the word “area”.) She weighed 8lbs 12oz, and was 20 inches long. Azaria came into the world to meet her parents TJ and Debi, and big brother Christopher (14). Debi had a normal pregnancy, until she was about 7 months pregnant when she noticed the baby would “shake” in her belly every so often. We were told not to worry, the baby probably had an immature nervous system. Looking back, we know the baby was having seizures. Two months later, Azaria was born and it was the best day of our lives! The next day, our world would turn upside down.
On October 26, 2002 Azaria Sage Lucille Baker was born. (Azaria is pronounced ah-z-AIR-ee-ah, sounds like the word “area”.) She weighed 8lbs 12oz, and was 20 inches long. Azaria came into the world to meet her parents TJ and Debi, and big brother Christopher (14). Debi had a normal pregnancy, until she was about 7 months pregnant when she noticed the baby would “shake” in her belly every so often. We were told not to worry, the baby probably had an immature nervous system. Looking back, we know the baby was having seizures. Two months later, Azaria was born and it was the best day of our lives! The next day, our world would turn upside down.
When Azaria was born, we noticed that she would quiver, but we were told that she was probably just cold, not to worry. The next day we were ready to take our newborn home. The doctor came in to discharge us and while examining Azaria, she had a seizure right in front of the doctor! Azaria was transported to Valley Children’s Hospital NICU. She was given Phenobarbital and had a lot of tests done. A CT Scan showed Agenesis of the Corpus Collosum. We were told this meant she is missing the part of the brain that connects the left and right. We couldn’t believe this was happening to us.
A neurologist suggested an eye exam by a Pediatric Ophthalmologist. We were told Aicardi Syndrome was a possibility, but is very rare and they wanted to rule it out. Before Azaria’s eye appointment we were able to go home and look up Aicardi Syndrome on the internet. After doing some research we couldn’t believe that our baby would be diagnosed with it. The agenesis of the corpus collosum we could handle, but the Aicardi Syndrome, no way! However, on November 5, 2002, at ten days old Azaria was diagnosed with Aicardi Syndrome. It felt like being kicked in the stomach. We were sent home with our baby and a referral to a genetic doctor. On November 12 we saw the genetic doctor and everything was confirmed. We were told that the prognosis was grim. The doctor said Azaria would probably never walk, talk, or live past a few years. We were told to take our baby home and enjoy the time we have with her. We cried and cried.
After being home with our baby, all we could do was hope for the best and take it one day at a time. We continued with the Phenobarb and Azaria was doing fine. At 2 months old we noticed she started having these continuous repetitive jerks. Fortunately we had been in contact with other Aicardi parents through an email group and were able to ask them what they thought this was. The response was infantile spasms. We took Azaria to see her pediatric neurologist and sure enough, he diagnosed her with infantile spasms. We were told infantile spasms are a form of seizure and almost impossible to control. At this time Azaria was having 25-50 infantile spasms a day, lasting anywhere from 10-45 minutes each.
Azaria’s neurologist suggested ACTH injections to control the spasms. We had done research, talked with other parents, and then decided we were not comfortable going that route. It was a hard decision to make but we felt the side effects of ACTH outweighed the benefits, especially since ultimately the seizures would probably return. So instead he put her on Topamax, and we increased her Phenobarb. After a month of that and an increase in seizures we were back to see the neuro. By this time she was having 60-80 seizures a day. We asked if we could try Vigabatrin to help control the infantile spasms since the Topamax was not working. Vigabatrin is not federally approved in the US so we had to purchase it from Canada. Azaria started Vigabatrin in January and it worked wonderfully for the first 2-3 weeks. The infantile spasms came back but not with the intensity that they had been. Azaria was having 20-30 a day, lasting 5-30 minutes each.
When Azaria was 4 months old she started having a new kind of seizure that made the infantile spasms look like a piece of cake. She was admitted to the hospital twice for severe seizures, convulsions, or grand mals. We added another drug Zonegran in hopes to control ALL the seizures she was having. We saw a Pediatric Neurologist at UCLA and she suggested also adding Vitamin B6. We started her on B6, and about the same time Debi removed all dairy products from her diet since she wad breastfeeding Azaria and it was felt the dairy products may be causing some of the seizures. After all of these changes Azaria’s seizures went down and seemed to be in control, only having one or two a day!
Azaria continued to do well. She was weaned off Phenobarbital and Vigabatrin, and stayed on Vitamin B6 and Zonegran. The seizures came back, on and off. She would do great for a few weeks, and then the next week she would need emergency seizure control for status seizures. It was a roller coaster! We tried to figure out why she would do well one week, and then have severe seizures the next. In July we took Azaria to see Dr. David Simon, a Professor of Neurology at Deepak Chopra’s Center for Integrated Medicine in Carlsbad, CA. Dr. Simon gave us a more holistic approach with some wonderful suggestions on how to try and control the seizures. He suggested daily massage, herbal supplements and other lifestyle changes. At the end of our visit, his assistant came in and whispered a mantra in Azaria’s ear. I don’t know what was said but after we left there, Azaria was the best she had ever been!!
Azaria lost her smile when was 2 months old, when the infantile spasms started. When she was 8 months old she started smiling again! It was so depressing not being able to see our baby smile. We are so excited now and appreciate every smile she gives us. Now Azaria is 11 months old and is still only on Vitamin B6 and Zonegran. She still has days were her seizures are out of control, but most days she only has one or two mild seizures, lasting no longer than 5 minutes. Azaria is developmentally delayed for an 11 month old. She can not sit up on her own and she has no interest in toys. She can not grasp any objects, anything put in her hand she will drop. Azaria does not put weight on her legs, roll over or crawl. In August Azaria really progressed with her head control and now has about 90% control! She also learned to do raspberries, and blow kisses. Her progress has been so amazing.
Azaria is legally blind, however she can see us coming from across a room, so we know she is able to see. On September 16th Azaria saw her pediatric ophthalmologist. We were told that the vision in her right eye is very bad. The lesions have taken over most of her vision in that eye. The vision in her left eye is great though. We were given a prescription for glasses so Azaria will soon have cute little baby glasses! Lately, teething has been our big issue. (I have to admit it has been nice dealing with an issue that all parents have to deal with.) Azaria has gotten her two bottom teeth and is now getting her top teeth. She has been very fussy and cranky, she has also had a few more seizures brought on by the teething.
Azaria has regular physical therapy and occupational therapy at UCP, and also at Cypress Rehab. She regularly sees a pediatrician, a pediatric neurologist at Children’s Hospital and UCLA, an endocrinologist at Stanford, a pediatric ophthalmologist, and a pediatric orthopedic doctor. She has a wonderful Vision Specialist who comes to our home for vision therapy. As well as a great physical therapist and a nurse who both come to our home regularly. She also gets Cranial Sacral Therapy, Aqua Therapy, Music Therapy (Kindermusik), and sees a Traditional Chinese Medical Doctor and a Nutritionist. We work with Azaria everyday and we hope one day she will be seizure free, and learn to walk and talk. We would settle for her just smiling everyday and being happy though. Azaria has so many people who love and pray for her. We are all truly blessed having this little angel in our lives! Please feel free to email us using the link below, we love sharing our story. And we are so thankful to have such a wonderful support group with the other Aicardi parents.