She’d wake up screaming, shrieking from the pain until her small voice slipped away. Afterwards, three-year-old, Racheal Opaczewski would lay limp, whimpering. “It was almost too much to take,” recalls her mother Crystalina.
The year was April 2004 and the Elkhart, Indiana family was in the throes of a medical mystery. They knew their toddler was having severe facial pain at night but they didn’t know why.
Little Racheal’s screams would last for hours. “And when she started, nothing would help,” recalls Crystalina. Numerous middle-of-the-night ER visits were nonproductive with doctors diagnosing the child with everything from inner ear infections to dental issues. Racheal was always plied with potent antibiotics and sent home—just in time to have the pain return.
“After two months of ER visits and five rounds of different antibiotics, I took Racheal to our family doctor--and spent some time on Google,” recalls Crystalina. “As soon as we walked in, I asked, ‘is this trigeminal neuralgia (atypical facial pain)?’”
While he couldn’t confirm it (since the condition can be difficult to diagnose), the doctor had strong suspicions. So, he prescribed Racheal an anti-seizure medicine commonly used to treat the condition. The first dose was effective: “Her pain vanished,” says Crystalina.
But after the second dose, things went downhill. “Racheal fell into a coma,” she says.
Crushed, the couple dialed 911. Ultimately, a blood test revealed that Racheal had overdosed on the drug. “Initially, the hospital accused me,” says Crystalina. “Thankfully, our family doctor admitted that it was his mistake. Being a family doctor and not a neurologist, he had underestimated the potency of the drug.”
What followed, says Crystalina, can best be described as discouraging.
“Racheal received an MRI, which revealed nothing. We then bounced around from one doctor to another. Each had their own theory. I was told everything from ‘your daughter is faking her pain and needs to see a psychiatrist to we need to extract her molars.’ Racheal was four years old at the time. It was infuriating.”
Crystalina continued to believe her daughter was suffering from trigeminal neuralgia. Racheal’s symptoms seemed to align with the condition, she says. Unfortunately, the family was told there was no test that could be run to pin down this diagnosis—and Racheal was so young that it was difficult for her to fully describe her pain.
Ages 4 to 9 were challenging for Racheal and the whole family, recalls Crystalina. Racheal’s pain increased from only nights to all hours of the day.
The family finally found a neurologist who would prescribe Racheal an anti-seizure medicine to quell her pain. “The drug worked until it didn’t,” recalls Crystalina. Then, Racheal turned 15 years old. “And last October, the drug no longer helped. The pain became unbearable. Racheal got to the point where she regularly asked God to take her life. It was devastating.”
In a quest for answers, Racheal’s neurologist referred the family to Riley Hospital for Children. “Dr. Kristyn Tekulve immediately prescribed Racheal a new medication and ordered an MRI with contrast,” says Crystalina. The addition of adding contrast to the test, she says, was a first. “So many times I had asked for that but was told no,” she says.
The result: The MRI revealed compression between some of Racheal’s blood vessels and a nerve, says Crystalina, a characteristic of trigeminal neuralgia.
Why was this case so difficult to diagnose? “Racheal’s condition, trigeminal neuralgia, is an irritation of the trigeminal nerve, which supplies sensation to most of the face. It usually occurs in adults and rarely in children,” explains Andrew Jea, MD, director of pediatric neurosurgery at Riley Hospital for Children. “The overall incidence of this disorder is around 10 new cases per 100,000 people each year. Because this condition is so rare in children, it likely wasn’t top of mind for some providers.”
Symptoms for trigeminal neuralgia include sharp shooting pain in the distribution of the trigeminal nerve which can be as innocuous as lightly touching the skin of the face, brushing the teeth, or a cold breeze hitting the cheek, explains Dr. Jea. “Untreated trigeminal neuralgia may be more difficult to treat as time goes on because the pain circuits get re-wired and spread.”
“In Racheal’s case, medications, namely anti-seizure medications, had worked to calm the irritation of the trigeminal nerve for a while,” explains Dr. Jea. “But they gradually lost their effectiveness. It was at that point that I encountered Racheal.”
Dr. Jea was incredibly compassionate, maintains Crystalina. “During our first meeting, he came over to Racheal, looked her in the eyes, held her hand and said, ‘I believe you, Racheal. I believe you have been and are really in pain and we are going to stop it.’ With everything we had been through, to hear a doctor say that was so significant.”
So, what caused the three year old to wake up in those early days? “Her condition had been triggered by the course polyester fabric of her pillowcase,” says Crystalina.
Dr. Jea told the family that a surgery called microvascular decompression could be a solution. The stakes were high, however, with blindness or deafness existing as potential risks. “But Racheal said she couldn’t live with the pain anymore,” says Crystalina. “So, we moved forward with the surgery.” Her operation occurred on July 25th, 2017. “The surgery involved creating a small window of bone through the skull just behind the ear to find the trigeminal nerve as it courses back to the brainstem, and looking for anything that could be compressing or irritating it, such as a pulsating artery,” explains Dr. Jea. “Once we found the artery pushing on the trigeminal nerve, we placed a pledget of Teflon to cushion the nerve from the artery. The fact that Racheal’s pain resolved so quickly after surgery is an excellent prognostic sign. It is always possible that there could be a recurrence of facial pain. But I think the odds are in Racheal’s favor that she will have long-lasting (life-long) pain relief.”
The team spirit and collaboration at Riley also helped Racheal. “At Riley, neurosurgery works together with our operating room nurses, anesthesiology, occupational and physical therapy, and neurology colleagues. It was our close relationship with our partners in neurology that brought Racheal to my attention,” says Dr. Jea.
Today, Racheal has a new life. “I can brush my teeth now for the first time in six years without pain and I ate sour candy for the first time recently and opened the car window and let the air hit my face. It’s awesome,” says Racheal.
Crystalina says the family is indebted to Dr. Jea. “Over the years, I had gotten so used to fighting doctors, there were always battles. But, Dr. Jea was so calm and considerate, even after we discussed the diagnosis he asked us to go home and think about it. To find a neurosurgeon who was sure of what was wrong but was humble and respectful to us as parents--it was an incredible thing.”
-- By Sarah Burns