Bilateral perisylvian polymicrogyria.
Most have never heard of it. Neither had Carol and Stan Drainer of Jerseyville until their daughter, Tess, was diagnosed with the disorder.
Tess, 11, was born 16 weeks early. She weighed 1 pound, 6 ounces and was 10¼-inches long. She wasn’t given a chance to survive past the first day.
“I never left her side,” Carol Drainer said. “After two surgeries and 98 days in the neonatal intensive care unit, she got to go home.”
Tess had some physical and developmental difficulties, which her parents — and the doctors, at first — thought were due to her being a “micro-preemie.”
As it turns out, they now believe the bilateral perisylvian polymicrogyria caused her to be born ahead of schedule, but they didn’t know that until much later.
Before she turned 3, they were told she had cerebral palsy.
“It was quite the blow, and we were upset, but we figured out how to deal with it and help Tess with therapies,” Drainer said.
At 5, she began to have seizures. She was put on medication, but it didn’t work.
“She was having as many as 50 visible seizures a day, but a monitor showed she was actually having much more,” Drainer said.
A neurologist tested her for bilateral perisylvian polymicrogyria, and they were given the diagnosis 4½ years ago.
Bilateral perisylvian polymicrogyria is a rare neurological disorder that affects the cerebral cortex — the outer surface of the brain. Symptoms include partial paralysis of muscles on the face, tongue, jaws and throat, difficulty in speaking, chewing and swallowing, or seizures. While the cause is not known, it is believed the result of improper brain development before birth and can occur in people with no family history of the disorder.
State Rep. C.D. Davidsmeyer, R-Jacksonville, took an interest in Tess, and drafted a resolution to be presented at the state Capitol to help bring recognition of bilateral perisylvian polymicrogyria and other rare diseases. The hope is to get a full medical code for the condition, which would help support the medical necessity for related services.
“We have been trying to raise awareness because it is so rare,” Drainer said. “The year she was born, there were only 40 known cases. We need to spread the word about this horrible life-threatening disorder.”
For a few years, Tess could get around to some extent by herself. She used a motorized wheelchair, she could eat, and went to school half days, with the other half reserved for therapies.
But because her immune system also is low, she can easily catch any “bugs” or viruses that are around, and with weakened lungs, it often develops into pneumonia. In 2012, she became very ill and was in the hospital for weeks on a respirator. During the following year and a half, Tess spent 64 weeks in the hospital.
“Two different times, she was close to not pulling through,” Drainer said. “It was so scary for us.”
By March 2014, doctors felt it was important for her to have a tracheotomy and use a ventilator full time to help keep her lungs inflated.
“Again, we learned to live with it,” Drainer said. “We have learned to be nurse, doctor and therapist, along with being parents. It’s not easy, but we will always be there for Tess.”
But even while in the hospital, Tess has a smile, her mother said.
“She is always a happy girl. One day in the hospital while on the vent Stan had her laughing and her alarms were going off like crazy,” she said. “Doctors and nurses came running to see what was happening. When we said she was laughing, they said kids don’t usually laugh while on the vent.”
Because she is so weak and susceptible to infections, the Drainers don’t venture out much. She has had pneumonia three times just this year.
About a month ago, they lost their home care nurse, and Carol Drainer has been caring for Tess on her own. They do have frequent doctor’s appointments, and it is especially hard to go anywhere now because Drainer needs a third person in the car, in case there is a problem with the ventilator.
“I’ve learned so much medically,” Drainer said. “But I still second-guess myself about some of her health issues. I love taking care of her. She is such a joy, but I want her to be able to do more, and we would like to find a home nurse again.”
Because of her increasing physical difficulties and weakness, Tess can no longer use her motorized chair. She can’t eat with the trach and ventilator, and gets her nutrition from formula. She can’t attend school; a teacher now comes to the house three hours a week.
“It makes me sad for her because she doesn’t get to know what it’s like to be a little girl and play outside,” she said. “People think I don’t leave the house because I’m afraid of leaving her, but truthfully, I want to be with her as much as I can.”
Just when they feel they have it all figured out, new difficulties arise. Recently, Tess’ left eye began to turn, and may require surgery. She has developed scoliosis that makes it hard to sit up straight. They now have a chair contoured to her back to help keep it from getting worse.
“Since the first day she came into our lives we have been on a roller coaster — always with love,” Drainer said. “She is special in so many ways, and speaks volumes without saying a word. She actually communicates very well with her eyes, eyebrows and her hands. She understands everything, and she’s had many wonderful experiences, too.”
The Drainers have an annual benefit, “A Night for Tess” at the American Legion in Jerseyville. Tess’ cousin is a professional wrestler, a sport she loves to watch, and he gathers fellow wrestlers who appear at the benefit. This year’s event helped them to make a down payment on a van with a ramp, because as Tess has grown — she is now 5 feet tall and 90 pounds – it is impossible for Drainer to lift her in and out of an unaltered vehicle. Nursing and equipment rental cost thousands of dollars each year, and her 11 medications are about $4,000 a month, not to mention the supplies needed for daily care.
“I thank God every day that I have her,” Drainer said. “I’m so glad I was chosen to be her mom.”
