By Jenny Neyman
Photo by Jenny Neyman, Redoubt Reporter. Marcia Jacobs looks at a book of Disney fairies with her daughter, Emily, in their Soldotna home Friday. Emily suffers from Chiari malformation of the brain and had to undergo brain surgery earlier this month to relieve pressure on her brain stem. Jacobs said Emily has been doing better since they got home Oct. 13.
Redoubt Reporter
At 2 years old, Emily Jacobs can count to 14. She can name all the Disney fairies. She can tell you about the sun being up and going down. She can point out a shark — “aaah!” — and a kitty — “so cute.” She can look at a syringe and tell you it’s for medicine — “icky.”
What she can’t yet do isn’t measured in terms of cognitive or educational milestones, like counting to 20 or writing the alphabet. It’s more basic than that.
She can’t swallow thin liquids without choking. She can’t toddle down the hallway without resembling a pinball careening into the walls. She can’t quite sleep through the night without waking up in pain. She can’t look forward to a childhood without the very real possibility of needing major brain surgery.
In June, Emily was diagnosed with Chiari malformation of the brain, where space in the lower rear portion of the skull is smaller than normal. Some people live with the condition symptom-free for their entire lives. But in Emily’s case, the base of her brain is compressed and her cerebellum is putting pressure directly on the top of her brain stem. The pressure has restricted cerebrospinal fluid flow around her brain, and the excess pressure has forced a hole in her spine where the fluid is leaking out into her spinal column.
Emily has been sick almost from birth, showing signs of increased intracranial pressure that her mother, Marcia Jacobs, of Soldotna, recognized all too well. Jacobs’ first child, Anjuli, died of brain cancer in 2001 at 4 years old.
In May, Jacobs took Emily to Children’s Hospital in Seattle, where Anjuli was treated. Doctors told her Emily would need brain surgery to remove part of the base of her skull to relieve the pressure. It’s a massive, risky surgery, involving taking patches of bone from elsewhere in the skull and cauterizing the cerebellar tonsils, a portion of the cerebellum shaped like the tonsils in the neck, at the base of the brain. The risk of infection, paralysis and even death are greater the younger the patient is, so Emily’s doctors wanted to wait until she was 3 to try it. She turned 2 on June 10.
Doctors sent Jacobs back home to watch Emily’s condition and wait, hopefully at least until December, when they’d do more tests and re-evaluate when it would be safer to do surgery. Emily did well through July, but in August, her condition deteriorated. Her eyes started to cross and she started having double vision, reaching for a crayon but grasping for it off to the side. Her balance lessened, her appetite waned and her choking got worse. Sleep was more frequently interrupted by pain and bouts of dry heaves. She was losing sensation in her hands and feet.
“And everything else bugged her. Heaven forbid you try to comb her hair or wash her face,” Jacobs said.
She knew Emily was in pain, even without the half-hour tantrums Emily started to throw.
“They told me that the development of all those symptoms indicated she was starting to have impacts on her nerves through her whole body,” Jacobs said. “And the good thing was it was still intermittent. If any of those things had become constant, there was the high likelihood they would remain constant, because if they were constant, that indicates permanent nerve damage.”
Doctors in Seattle wanted to see Emily in a matter of weeks, no longer months. Jacobs took her down Sept. 23. They did a brain scan and fluid flow test, and determined they couldn’t wait any longer for surgery. But Emily was nowhere near 3, and the surgery’s risks were still substantial. Doctors instead opted to do a sort of phase one of the surgery — remove a piece of skull, part of her C-1 vertebrae and cut some of the ligaments restricting the brain — rather than opening up the dura — the protective outer covering of the brain.
“In doing that, potentially that would be all she needs, or at the very least it would buy her time for the big surgery,” Jacobs said. “It was so scary because we’ve been told so many times before that she needed to be closer to 3 and all the risks of the surgery.”
Emily underwent the procedure Oct. 5. After a lot of pacing and finding a waiting-room bookshelf to manically reorganize, Jacobs was told the surgery went well.
The recovery, however, was not as smooth.
“She is 2, and she’s done a real good job of reminding everybody of that, that she’s 2,” Jacobs said.
The afternoon and night of her surgery went fine. But the next day, in ICU, Emily reached her limit of tolerance for all of it — being in pain, being in a scary new place, being too little to understand or control what’s being done around and to her. While in her ICU bed, she started to throw a massive tantrum. Jacobs tried to lift her out of the bed to cuddle and hopefully calm her, but in the process, Emily yanked out the IV line in her arm and, much worse, the arterial line catheter inserted into her artery.
A nurse in the room clamped her hand around Emily’s wrist to stop the blood flow, but neither she nor Jacobs could reach the call button at first to summon help. In the ensuing tussle, Emily also yanked out her catheter and spilled her catheter bag, and started banging the base of her skull — transected by her surgery wound — against Jacobs’ collarbone.
“She’s screaming, she’s bleeding bloody murder and the nurse, I can see her hand holding Emily’s, and I can see the blood just squirting through her fingers, and it’s dripping down her arm and running into a pizza-sized puddle on the floor,” Jacobs said. “There was just blood everywhere. I can just so vividly see the blood squirting through the nurse’s fingers and going, ‘That’s my baby’s blood. That’s by baby’s blood. Put it back!’ And Emily’s this totally raging octopus. It seemed like she had eight arms and eight legs.”
Help finally came and they were able to get Emily sedated and stable, through a remaining, auxiliary IV in her foot.
“I just started to cry, and, literally, I can’t control my child, I can’t comfort her, I can’t sit. I was trying to hug her, kiss her, sing to her,” Jacobs said.
That afternoon, Emily was transferred out of ICU, and the move elicited another tantrum. This time she pulled her remaining IV out of her foot and started vomiting. Just two days later, doctors told Jacobs she could take Emily home. After the tantrums, with Emily fighting taking her medication and still on an IV, Jacobs was suddenly almost as nervous to take Emily out of the hospital as she was to check Emily into it.
“It was really scary leaving Children’s, because I’d been through so much, she’d been through so much. I was like, ‘OK, if I have to go, can you guys all come with?’” Jacobs said.
She and Emily returned home Oct. 13, and Emily is doing better and better by the day. She still has trouble eating and doesn’t have much of an appetite, her balance is a little wobbly and she’s still fussy. But she sleeps better now, is off pain medication, her energy comes in longer and longer spurts, and she seems to be feeling better. She’s got some rosiness to her cheeks, some sparkle in her eyes and a quick smile that isn’t as dulled by sickness and pain. And there are signs the effects to her nervous system are abating.
“She has rediscovered her feet,” Jacobs said. “She was all about her feet the few days after surgery. She even stood on one foot a few times since we’ve been home. She’s never done that before. She complains of cold feet. It used to be the sight of a sock would totally send her into a hissy. She hated socks and shoes — hated them, hated them, hated them. Now there’s times she even asks for socks because her feet are cold.”
If her recovery goes well, Jacobs doesn’t have to take Emily back to Seattle until December, and they’ll do another brain scan six months later to determine if she still needs the bigger surgery.
“Until then, the way that we find out how she’s doing is, how is she doing? She will let us know how she’s doing, and she’s doing better, so I’m thinking that must mean that the surgery worked and everything is getting better. I’m just hoping that we’re done,” Jacobs said.
There’s a chance Emily won’t need any more surgeries. Doctors said she’ll need to be watched closely for the next two years, have a brain scan every year until she’s a teenager, and be monitored depending on symptoms after that.
“This is lifelong. There’s the potential for more surgery the rest of her life. This could be the first of many, this could be the last of all. You just don’t know,” Jacobs said.
For the time being, Jacobs is taking each day as it comes.
“It’s been exhausting. I’m much better this week. Last week you could have mopped the floor with me. All the sleep deprivation, the worry, the fear, the emotions, the physical strain. It’s really hard, but it’s better this time because she’s getting better. There’s hope,” she said.
Doctors told Jacobs she’d need to keep Emily at home for four to six weeks, so she could recuperate and build up her immune system. Friends have been helping with grocery shopping and running other errands, and Brian Heath, who worked with Jacobs to organize the St. Baldrick’s cancer fundraiser, is planning a fundraiser for Jacobs. It’s a holiday bazaar from 10 a.m. to 7 p.m. Nov. 6 in the Denali Room at Central Peninsula Hospital. Local vendors of Pampered Chef, Avon, Creative Memories and others will donate a percentage of their sales during the bazaar to help cover Jacobs’ travel and medical expenses.
“She just got back and they still have to go down in December again,” Heath said. “She hasn’t really been able to work. Even though she has insurance, it doesn’t cover all the costs. Twenty percent when you’re talking about neurosurgery and CT scans and all the time in the hospital is a lot. She’s already racked up thousands of dollars in bills. She’s a person who gives a lot of time to the community.”
Jacobs hasn’t been able to work since early October. She’s a single mother, and already had her finances devastated by Anjuli’s medical bills, not to mention all the medical care Emily has needed. That just complicates the situation for Jacobs, worrying about her daughter’s health, as well as the family’s finances.
“I’m trying not to panic about the mortgage payment, because I’ve been in worse situations than I’m in now. And back then, Anjuli was dead, so this time Emily’s here, she’s well, she’s doing good. So I just look at this as a tough spot we’ve got to get through and get to the other side of it,” Jacobs said.
Still, it weighs on her. She had a dream recently that she and Emily were homeless, trudging through mounds of snow.
“I was carrying our possessions and so I couldn’t carry her, and she was having to trudge through the snow post brain surgery. And she was so wobbly and falling face down, and then I’d have to put everything down and go cover her up,” Jacobs said.
“And obviously it stayed with me, because it was a week ago and I’m still like, ‘Oh, that dream, that dream, that scary dream.’”
There is no cure for Chiari, and there is no immediate answer for whether Emily will need further surgery. There’s also no Disney fairy that can speed her recovery with the flick of a magic wand.
As nice as that would be, Jacobs would settle for life just returning to some sort of normal.
“It’ll be good to get things back to normal,” she said. “I look forward to normal. I want a T-shirt that says, ‘Normal is paradise.’”
Anyone interested in being a vendor at the holiday bazaar for the Jacobs family can call Heath at 398-9690.
