ST. ANNE — A union iron worker, Adam Beedle has become quite comfortable playing house and dress-up with his 4-year-old daughter, Annabelle.
While some fathers may recoil at such playtime, Adam could not be happier.
“I appreciate the moments. And I appreciate the moments even more than before,” said Adam, 44, the union iron worker from Local 1 in Chicago. “My focus is one day at a time, one moment at a time.”
On this Sunday afternoon, Adam and his wife, Amanda, 33, a nurse at the Illinois Veterans Home in Manteno, are busy as parents would be raising two daughters, Annabelle, 4, and Abigail, 10 months.
But the household events of play, snacks, story time and naps are far from the only things consuming the thoughts and energies of Adam and Amanda.
The Beedle house has been one filled with great emotional pain and discomfort these past weeks and months. The Beedles’ daughters were both recently diagnosed with an illness more commonly known as the extremely rare Batten’s disease, which is an inherited disorder of the central nervous system known as neuronal ceroid lipfusinoses or NCLs.
Basically, Batten’s is a terminal disease of the nervous system, a brain degenerative disease. No cure exists. Annabelle’s cells lack the needed enzyme which breaks down lipids and proteins.
The failure to break down these structures leaves them to pile up and slowly cause the destruction of her brain cells.
It is estimated by the National Institute of Neurological Disorders that 2 to 4 out of every 100,000 U.S. children are affected by Batten’s disease.
The St. Anne parents learned on Dec. 7 through testing at Lurie Children’s Hospital in Chicago that Annabelle — who had been suffering from seizures dating back to November 2019 — has the rare illness. Because it is a genetic disorder, the parents had Abigail tested as well. On Dec. 28, they learned she also has the disorder.
There are 14 variations of the NCL. Of these variations, Annabelle has CLS2. Of the CLS family of disorders, CLS2 is the only one with any treatment options, this being an enzyme replacement therapy known as Brineura, which can slow the progression of the disease. The treatment program is administered every two weeks and is basically a day-long process at Lurie Children’s.
Through a port, these enzymes are injected into the child’s cerebral spinal fluid of her brain. This process will take place very two weeks for the rest of their children’s lives. One week Annabelle will receive the therapy and the following week, Abagail will receive the therapy. In other words, the family literally and figuratively has many miles to travel.
Annabelle received her first injection Dec. 16. Abigail will receive her first on Wednesday.
Adam and Amanda say that Annabelle is the first with this illness ever diagnosed at Lurie Children’s and Abigail will the youngest patient in the United States to receive the replacement enzymes treatment program, only approved by the U.S. Food & Drug Administration in April 2017.
“It’s been a whirlwind,” Amanda acknowledged. “It’s hard. We’ve had our moments. We take it a day at a time. We’ve had good and bad days, but we still are a family. It just might not last as long as we had thought, but our job is the same. We are here to raise them and give them the best life we can.”
And, she said, despite it all, the girls continue to bring smiles to the house.
“This can really beat you down. That’s not a question,” she said. “But we all have a choice as to how we wake up to face each day. We still have joy for the day.”
And, they have family and friends. To date a GoFundMe account, called “The Beedle Family Fund,” established by a friend has generated $26,316 as of Monday night. The goal was $25,000.
The money will obviously be used for some medical expenses, but also for travel, food and likely lodging for the many impending treatments.
EPILEPSY WAS 1ST THOUGHT
Amanda at first thought Annabelle had childhood epilepsy, which can be managed by medication. The medication seemingly worked, for at least several months.
They attempted to manage the illness in that regard, but by September 2020, the seizures became more and more difficult to manage and other symptoms — such as delayed and loss of skills previously acquired, including speech — began presenting themselves. They traveled to Chicago seeking answers.
On Dec. 7, they unfortunately found it.
Amanda described the phone call from doctors at Lurie as the call which changed their lives.
“I knew we were standing on the edge of something very deep. I don’t know if it’s the nurse in me or what, but I knew. She had responded to the seizure meds so well. Things went back to normal, until it wasn’t,” she said.
Most forms of Batten’s begin at age 2 or 3.
As the illness progresses, children may develop one or more symptoms including personality and behavior changes, clumsiness, learning difficulties, poor concentration and several others. Eventually, blindness and the ability to walk are lost.
Life spans are also greatly impacted for those diagnosed as children. The parents know it is possible neither daughter will live to be 20.
Those thoughts, however, are for another day, another time. Right now the focus is on the two little girls with big smiles and sticky fingers.
“We are striving to provide these two precious girls with a wonderful childhood ... Every day and every moment are so precious,” Amanda said.
But still, it is hard not to look back.
“Everything had progressed so perfectly,” Amanda said. “Looking back it’s so hard to believe. It hard to believe such chaos and disfunction are going on.”
BRADLEY — At a cost not to exceed $75,000, the Bradley Village Board approved the hiring of Kankakee-based architect Bright Architecture.
The board unanimously approved the contract and the firm has already developed preliminary design concepts for the project expected to be in the $800,000 range.
In addition to the architect, the village administration will also be hiring a project manager within the next 30 days or so, Mayor Pro Tem Mike Watson said at Monday’s village board meeting.
The project manager will oversee the project from beginning to end.
Doug Bright, president of Bright Architecture, said at the meeting that it is a wise move to have a project engineer. He said that engineer can bring specifics to such a project.
“[Architects] can give an idea of what it could cost,” he said, noting that is not the architect’s role.
Bright did note as a resident of Bradley in the Hunter Run subdivision along Armour Road east of Illinois 50, the distance for quick response times are simply too far.
“I’ve lived in Bradley for 29 years. This has been needed for a long time,” he said.
That sentiment was also expressed by former Bradley Fire Chief Steve Wilder. Wilder, who served as chief from 2000-08, said there have been many starts and stops regarding the development of a second fire station.
“It seemed like it always got put on the back burner. Don’t let it go the back burner anymore,” he said during the public comment section of the meeting. “Please keep this alive. If not, you are punishing only those on that side of town.”
Preliminary plans call for a 3,500-square-foot garage to be added to the former Bradley-Bourbonnais Chamber of Commerce site at 1690 Newtowne Drive, a property the village has owned for several years.
The village is also considering constructing a community room onto the former chamber building for use by outside organizations, but that concept has not been finalized.
On-again, off-again plans to construct a second fire station in Bradley are very much on and are about to move forward as construction could begin by late spring.
At issue is response time for fire and ambulance calls. While response times are to be in the 4- to 5-minute range, according to industry standards, the village response times for north and northeast Bradley — the farthest points from the station — are in the 8- to 10-minute range.
BRADLEY — Students returned to Bradley-Bourbonnais Community High School on Monday for the first day of second semester with two major changes — an hour more of class time and a return to the every-other-day attendance schedule.
With the possibility of teachers and staff getting vaccinated for COVID-19 in the coming weeks, the every-other-day schedule could change to five days per week as soon as March, school officials say.
“It was a fantastic day having students in the building,” Principal Brian Wright told the BBCHS School Board Monday evening.
In-person students are divided into “red” and “white” attendance groups, each consisting of about 530 students.
Also for second semester, 560 students have opted for a fully remote schedule and 250 students with individualized education plans attend school daily.
So, roughly 800 students walk the halls of BBCHS on a given school day. Classes are capped at 17 students.
“We’re excited to have students back,” Wright said. “We haven’t had that in a long, long time.”
The high school started the school year on the every-other-day schedule and with 30-minute class periods.
As the year went on, COVID-19 numbers fluctuated in the school and in Kankakee County, and the school board and administration discussed and adjusted plans several times.
Eventually, the attendance groups were reduced from one-half to one-third of in-person students daily.
For the second semester, the every-other-day schedule is back in place and class periods have been lengthened to 40 minutes.
The district has also outlined criteria by which a switch to full remote learning would be reimplemented. The criteria are re-evaluated every three weeks.
Superintendent Scott Wakeley said it was amazing to see students in the “red” attendance group fall back into their routines when returning to school Monday. The “white” group returns Tuesday.
“It feels like they never left,” he said.
It was once a concern whether or not high school students would comply with wearing face masks. Wakeley said this has not been a problem at BBCHS.
“It’s just a way of life right now,” he said.
Wakeley added that teachers were excited to return as well.
“The [teachers] that I talked to, it felt like the first day of school because they were going to have more than two kids sitting across from them throughout the day,” he said. “So, it was a good day.”
The Kankakee County Health Department is still working through vaccinating the 1A group, but Wakeley said he is optimistic that teachers and school staff, who are included in the 1B group, will be able to get their vaccines soon.
“My goal is by the beginning of March, hopefully, we’ll be able to have all kids who aren’t full-remote back in school every day,” Wakeley said. “It’s kind of out of our hands right now, but it’s coming.”