After a lifetime of headaches, Karrie learned at age 45 she would need brain surgery, but she never expected it would be the first of dozens
Any patient, especially one with a rare disease, wants to know she's in good hands when it comes to her medical care.
So it was more than a little distressing for Karrie Johnson when, at a primary care appointment, she was asked to spell the name of her condition, which the provider had never heard of before.
Karrie uttered the letters slowly, patiently: "C-H-I-A-R-I."
Karrie was born with a Chiari malformation, an anatomical abnormality involving the posterior portion of the brain called the cerebellum. In people with Chiari malformation, the cerebellum extends down through an opening in the skull, into the space normally occupied by the spinal cord. This can obstruct the flow of cerebrospinal fluid, causing it to build up. The skull is a closed space, so any increase in the volume of its contents results in high intracranial pressure and compression of brain tissue, a condition known as hydrocephalus.
Karrie also has an additional complication called syringomyelia, which is a collection of fluid within the spinal cord, and can lead to debilitating symptoms like arm and leg weakness, balance problems and pain. Karrie suffers from all of these.
Many people with Chiari are asymptomatic and, if they go their lives never having any imaging of their brain, like a CAT scan or MRI, may never find out they have it. Karrie got diagnosed at age 45, after more than two decades of symptoms.
For as long as she could remember, she had had headaches. They were mild during childhood and adolescence, but in her early 20s she developed severe migraines. These happened a few times a month, and they knocked her flat.
"It felt like something was being pressed into the side of my skull, where my temple is," she said. Then came the tunnel vision, nausea and vomiting. Normally a very active person, she would sleep the day away, hoping the pain would be gone when she awoke.
"That was the only thing I could do. I was not like on those commercials, where people are pushing swings, having a migraine."
She started noticing a pressure-like sensation at the back of her head, which worsened with movement. Laughing, coughing and heavy lifting in particular would provoke intense pain.
Besides migraines, though, she was in good health. "I really hardly ever got the flu," she said. She got a shot of Imitrex, a migraine medication, twice a month. She would occasionally have to take a day off work because of headaches. One job, as a preschool teacher where she spent her days surrounded by toddlers, was particularly triggering.
Over time, the nature of her headaches changed. She would still get the warning signs that a migraine was coming, but the headache itself would be manageable and short-lived. At the same time, she started noticing a pressure-like sensation at the back of her head, which worsened with movement. Laughing, coughing and heavy lifting in particular would provoke intense pain.
"My girlfriends and I would get together one Friday night a month, and we would laugh so much," she remembers. "I was the only one that kept grabbing the back of my head and telling everybody, 'Guys, stop making me laugh, you're killing my head.'"
Still, Karrie was able to cope and go about her normal life, more or less. When she wasn't in the throes of a headache, she didn't think about them much. In fact, she just assumed that everyone had headaches similar to hers.
"I just thought that was normal, I thought everybody dealt with that stuff," she said. She suspected she might have tension headache, brought on by the stress of being a mother of young children.
The hurt besieged her brain daily. It wasn't until her late 30s that it dawned on her that this wasn't normal. "I started to meet people that said, 'I've never had a headache before,' and I just looked at them like, 'What are you even talking about? How could you not?'"
She carried ibuprofen with her wherever she went; she found that if she took it at the onset of symptoms she could avert some of the worst ones. But the ibuprofen lost its effectiveness over time. The headaches started waking her up at night.
Then one day something new and unfamiliar happened: Her left arm went numb. This was unnerving. "I thought maybe I was having early signs of a heart attack," Karrie said. She rushed to make a same-day appointment with her primary care doctor. He assured her that her heart was fine, but he sent her to get a CAT scan of her head.
In her more than 20 years of headaches, it was the first brain imaging she'd ever had. It showed her cerebellum pushing down into her spinal canal, the telltale sign of Chiari. A follow-up MRI demonstrated severe syringomyelia of her cervical spinal cord.
Karrie says her primary care doctor didn't quite know what to do with this information—he had never had a patient with a Chiari malformation before. She took it upon herself to do some research online, and in doing so learned that many cases of Chiari require surgery. She located a neurosurgeon in Chicago and made an appointment in order to get an opinion on her case.
She'd had a relatively clean bill of health previously, and now suddenly she was being told she was actually quite sick.
After reviewing her imaging, the doctor confirmed that she would need a decompression surgery, which involved removing part of the back of her skull to create room for her cerebellum. Karrie recalls her initial gut reaction to this recommendation: No way.
"Nobody's going to cut open by head—nobody," she thought.
Too much was happening all at once. Karrie wasn't ready for such a weighty diagnosis, followed in quick succession by a recommendation to operate. She'd had a relatively clean bill of health previously, and now suddenly she was being told she was actually quite sick.
"It was kind of a panicky time of my life," she said. "Here I have this cyst on my spinal cord, and something's wrong with my brain, and I'm supposed to have brain surgery."
"It was hard for me to even comprehend that I had this going on in me."
Once the panic subsided, she took stock of her situation. Her symptoms were terrible, and limiting her ability to do just about anything. She was popping ibuprofen like candy, yet it was doing nothing for her. She felt she owed it to herself and her family to try to correct the problem before it was too late.
She scheduled her surgery. Little did she know at the time it would be the first of 24.
Waking up from the decompression, Karrie found herself in the grip of the worst pain she had ever felt in her life. "I didn't even want to breathe because it hurt so bad," she said. "I've had five children. I would give birth a hundred times over before having to go through that again." The surgeon told her the operation had been successful, and that her recovery would be swift.
If the goal of the surgery had been to relieve her headaches, it had not achieved that. The pain continued to pound her skull daily, crunching like a vice. She told her doctor this at her post-op appointment. "He would tell me, 'You're just having migraines.' And I said, 'These are not migraines. I know what migraines feel like. They're all in the back of my head, so severe.' And he kept telling me I was fine, I'm fine, I'm fine."
Karrie's outlook began to shift after this first operation, as she adapted to a new normal of not feeling well. She went back to work after six weeks with the same symptoms she had suffered before the decompression. Despite what her surgeon told her, she knew this couldn't be the expected post-operative outcome. Why had she gone through the surgery, she thought, just to be back at square one?
This became Karrie's routine: If she wasn't recovering from a surgery, she was planning for the next one.
A co-worker of hers researched Chiari specialists and found one who agreed to see Karrie for a second opinion. This doctor told her too much of her skull had been removed and would need to be replaced with supportive mesh. After that, one surgery cascaded into another. The mesh placement was complicated by a cerebrospinal fluid leak into the back of her head that "looked like a baseball." To correct it, she needed a shunt placed to divert excess fluid from her ventricles. Despite this, she continued to leak, so a surgeon placed a bovine patch to plug the leak; her immune system rejected the patch and it had to be removed. The ventricular shunt in her brain eventually stopped functioning and she had to have a second one implanted in her lumbar spine. The dual shunts ended up decompressing her ventricles too much, causing her to feel constantly dizzy, weak and nauseous. The lumbar shunt was subsequently removed.
This became Karrie's routine. If she wasn't recovering from a surgery, she was planning for the next one. At one point in her seemingly endless series of operations, she had a heart-wrenching flashback to her former state of being. When her body was rejecting the bovine patch, she was prescribed a high-dose steroid in an attempt to suppress her immune response. Steroids often increase energy levels and can even produce a mild euphoria. Taking the steroid, she almost felt back to her normal self. It made her cry. "I thought, 'Oh my gosh, I'm supposed to feel like this,'" she said.
The feeling didn't last, but Karrie does occasionally have days when she feels well enough to do the things that used to bring her joy: gardening, walking the shores of Lake Michigan, refinishing furniture. A few years ago she took up winter hiking; she found peace in the crunch of snow beneath her boots and the stillness of the empty wilderness around her.
"If I'm feeling good, I seem to go non-stop," she said, "because it feels so good to be able to do that, and I know it's not gonna last."
She worries, though, that she might eventually forget what "good" feels like, and if that were to happen, then how can she gauge if she's improving? If she loses sight of the target, how will she know where to aim?
"Gosh, do I even know the difference now?" she sometimes asks herself. "You doubt yourself, like, 'OK, is everything that I'm feeling really real?' It's not until I feel better—there's such a huge difference—that I say to myself, 'Why would you doubt yourself?'"
On her rare good days, when she is able to gain some perspective, she reminds herself: "I'm not supposed to feel like that. And I wouldn't trade the way I feel right now for anything. So I know it's not in my head."
Instead of focusing on minimizing or eliminating her pain, Karrie has developed strategies to manage it. She attended a 12-week rehabilitation program, where she learned about mindfulness and meditation techniques. She dislikes pain medicines. She has found a good deal of relief from marijuana, however. It doesn't get rid of her pain, she says, but "it makes me not care about my pain." These days, the thing that brings her the most physical comfort is smoking and then soaking in the hot tub.
"Sometimes you've gotta treat the mind, because that can help control your pain," she said. "If you can't control that, you've gotta control your mind."
The past few months have been some of her worst, and not just because of pain. She gets weak and shaky if she stands for too long. She feels that her mental acuity and memory are slipping, too. In scheduling our interview, we settled on a tentative date but she requested that I check in that morning to see if she was up to it (she was, thankfully). She said if it had been one of her bad days, which occur fairly regularly now, she would have had to cancel. I asked her near the end of our talk if she had been experiencing pain during it. "Oh, yeah," she answered. "It never, ever, ever goes away."
Karrie is currently on her fifth neurosurgeon, due to a combination of relocations and frayed relationships. Her surgeries these days are mostly revisions to her shunt, to tweak it when it's draining either too much or not enough. She still trusts her doctors, but she has adopted a firmer stance with them. She is not one to complain, and she makes sure they know that, so that if she does report something out of the ordinary, it's not dismissed. Back when she had two shunts, it took her a year and a half of repeated visits to convince one surgeon that something was wrong with them before she was taken seriously, and the lumbar shunt was taken out. Karrie regrets this lost segment of her life—irreplaceable time during which she was barely able to function. She is determined not to let it happen again.
"This is my life, my days that I can still see my kids and my grandkids," she said. "I can't keep wasting all this time in my life because a doctor won't listen."
Still, she is wary of disclosing new symptoms or concerns, because of the high likelihood it will mean another day in an operating room, another scalp incision, another recovery, and another period of waiting with anticipation to see if this time she gets better. Nowadays, she's not holding her breath.
"I've pretty much accepted that this is gonna be my life."
I asked her if, in recent memory, she has gone into surgery thinking it will be her last—the one that stabilizes everything and allows her to return to the way things were before. She laughed.
"Friends or relatives will say, 'Maybe this will be the one,' and inside my head I'm just kind of laughing and I think, 'Gosh, I thought that about the last 20,'" she said.
No doctor has ever told her outright that she's unlikely to get much better. But Karrie is smart enough to read between the lines. She knows that at this point, each successive surgery is a patch, not a cure.
"I've pretty much accepted that this is gonna be my life," she said. "It seems like every time I have surgery I'm faced with my own mortality, and it kind of freaks me out. I cherish every single day that I'm alive now. I try really hard not to ever take anything for granted."
She's determined to make hers a life worth living, and to continue to defy the limitations her illness imposes. When we spoke, winter was approaching, and I suggested, presumptuously, that she might have to forego hiking for the season. I was wrong.
"Oh, I plan on it. I'm gonna keep doing it," she said. "I'm the type that I don't give up, I'm kind of stubborn, and I refuse to give in. If I can keep going, I do."
"If it brings me some kind of joy, I do it, because what other option do I have?"