Chiari Malformations and the Coronavirus
After being dismissed and disregarded by doctors throughout her life, Neriah England finally found comfort when a Boulder Community Health employee took her pain seriously.
Now, as normal life comes to a halt due to a global pandemic, England’s chronic pain will be extended for even longer.
England, a 20-year-old University of Colorado Boulder sophomore, has dealt with excruciating pain for almost a decade. With symptoms beginning with headaches and migraines, she was always told to stay hydrated and get plenty of rest.
Throughout young adulthood, she experienced a loss of vision and feeling in her arms and vomiting that caused extreme weight fluctuation. Bedridden her freshman year, England felt like she was missing out on life until a year later, she found out the cause of her pain.
A type 1 Chiari malformation was what was hindering England’s daily life and typical college experiences. A Chiari malformation is a congenital condition in which one’s skull base is too small at birth, causing parts of the brain—specifically the cerebellum—to extend downward into the spinal canal once the brain grows to its full size. Patients typically experience a variety of neurological symptoms later in life.
The constriction of the cerebellum, which controls balance, coordination and speech, was the root of all her problems. For Chiari patients, the cerebellar tonsils, the two lower parts of the cerebellum, are pushed into an opening between the skull and spinal canal, which can not only block the release of spinal fluid, but cause extreme pressure build up and severe symptoms. For England, her Chiari malformation extends below the base of her skull.
In cases as severe as England’s surgery is the only solution. England’s surgery was originally planned for late March, but then the coronavirus swept the world.
When Colorado hospitals stopped all elective and planned surgeries for the time being to prepare for the influx of Covid-19 positive patients, figuring out how to patiently wait while in pain was heartbreaking for England.
“I've been suffering in pain like this for 10 years and it's my normal, but obviously someone who gets COVID, that’s not normal and they need help,” she said.
With frustrating past experiences with doctors, chiropractors, massage therapists and emergency room nurses, England felt like giving up.
“Doctors always told me to drink water and that some people just get bad migraines,” England said. “They blamed it on water intake, birth control, they just always had a different answer.”
One doctor even turned down England’s request for an MRI because he thought her pain wasn’t severe enough. She understands she’s young but it still was a slap in the face, she said.
Kimberly Sexton, a Chiari Nurse Practitioner at the Colorado Chiari Institute (CCI) in Aurora says it’s hard for patients to get an MRI to diagnose the condition due to a variable mix of symptoms. She adds that some patients may experience symptoms their entire lives like England, or some may never know they have the condition.
“There’s about 300,000 cases of Chiari malformations, but it’s probably more than that,” Sexton said. “It’s hard to tell.”
In December 2019, England lost feeling in her arms, unable to write notes while in class. Her vision became spotty and began going in and out. It wasn’t until she told her decade-long story to Boulder orthopedic doctors and neurologist’s that she was taken seriously.
“I just felt like I was missing out and I didn’t know why my body hurt and why no one else felt pain like I did,” she said. “I broke down crying [when I found out], I just felt like I wasn’t living.”
She was relieved and said the Boulder doctors made her feel validated for the first time in almost a decade. But now, as her recovery process was on the horizon, her doctors at CCI and delayed England’s surgery date further into the summer.
England’s surgery at CCI is currently scheduled for June 9, but there are no guarantees that The Medical Center of Aurora will be Covid-free and safe for England and the surgeon.
CCI is a world renowned specialty facility that is one of few Chiari institutes in the country. People travel to Aurora to receive specialized treatment for their condition. For Sexton, the most difficult part of working at CCI during this pandemic is prolonging the surgeries of many Chiari patients.
“That’s what the hardest part for me is, is to call and tell patients that we have to postpone their surgery because I know that they’re miserable,” Sexton said.
Across the country, medical staff from all backgrounds are being called to the front lines to care for patients affected by the Coronavirus. Sue Kirelik, MD, a close family friend of England and emergency room pediatric doctor at Rocky Mountain Hospital for Children and medical director at the Center for Concussions, is grateful for the measures taken by Colorado officials and the preparation hospitals around the state established before the surge in cases.
“Right now our focus is completely on how to prepare for COVID, how to take care of these patients, how to shift our resources to take care of these patients,” Kirelik said. “And as you know from Neriah, it kind of puts everything else at a grinding halt.”
Like many health care professionals around the world, Kirelik took major preparations for her emergency room shifts as the virus spread through Colorado. She self-isolated for 15 days with mild symptoms before returning to only one of her jobs. The other, cut her shifts significantly.
Due to her decreased shifts in the concussion clinic, Kirelik realizes that the mental and physical safety and well-being of her co-workers and fellow health care professionals around the world could be much more detrimental during the pandemic.
“I don’t think that everybody understands how incredibly dangerous this is for health care workers,” she said.
Kirelik also noted how she feels fortunate to receive two N95 medical masks per shift at Rocky Mountain Hospital, compared to colleagues in New York who are reusing masks for many consecutive days. The typical medical protocol is to get a new mask, gloves and gown every time after exiting a patient’s room.
“We are now instructed to leave our masks on all day, not to touch them or take them off,” Kirelik said. “This makes it really hard to breathe, eat lunch and drink water.”
Other changes at Kirelik’s hospital include limited entrances, using provided scrubs and limited staffing. The Hospital Corporation of America has provided several opportunities for medical staff laid off during the pandemic, allowing employees to be deployed to a different area if needed or receive paid time off.
On her first weekend shift back in the emergency room, Kirelik observed a considerable decrease in the number of patients she saw. She explained that it is understandable, kids aren’t playing sports and people aren’t driving cars as often but knows that they did keep the patients they saw safe. The staff has been well trained on Personal Protective Equipment (PPE) and how to keep patients safe, she said.
“Actually, I felt better being at the hospital than I did at home for three weeks because when I was at home, I didn’t feel like I had a really good understanding of how things were going,” Kirelik said. “I think the medical centers in Colorado have done so much to prepare for this, which is really helpful.”
For Kirelik, her message is that it is still OK to get medical help if it is necessary. People who need medical care, shouldn’t be afraid to go to clinics and other medical offices, she said.
While Kirelik was on duty helping kids, England was one person she couldn’t help. England, who has been on strict quarantine since March 11, is fighting an uphill battle of her mental and physical health.
“I would say it's been hard because there are so many people going through so much, but it honestly hurts my feelings when people aren’t taking [stay-at-home orders] seriously,” she said. “I think it is ignorant for someone to not follow social distancing rules.”
Both Kirelik and England, have one common goal, to slow the spread of the virus so life can return to normal.
“Even though I’m not allowed to have surgery, I've put it in my head that people with COVID need it more than me and I'll just have to wait.”
