joe's story



 

"I was deteriorating pretty rapidly," Jennifer says.   "My husband, Tim, and I were concerned because my right leg was starting to drag, and I had developed so much pain in my neck and the back of my skull.

 

 

chiari syrinx

Jennifer's chiari malformation was further complicated by collections of fluid called syringomyelia (arrows) in her spinal cord.

 

 

Jennifer had been suffering from recurring headaches, accompanied by numbness and heaviness in her right arm and leg, for many years when the second wave of symptoms began.

"In the past the numbness and headaches had generally happened together," Jennifer recalls. "Sometimes I'd get the headaches without the numbness, but any time I had the numbness I always got a headache with it."

Around the end of the summer of 2006, Jennifer started to experience numbness in her arm and leg without headaches. "This was different, plus I started getting back and neck pain, and I had never had that before."

Jennifer, a fourth-grade teacher from Huntingburg, Indiana, also began to have trouble swallowing, and occasionally her coordination was impaired. At the time, she had no idea that all of her symptoms were the result of a Chiari malformation in her brain and resulting cysts in her spinal cord. A Chiari (pronounced kee-AR-ee) malformation occurs when the indented bony space at the back of the skull is smaller than normal, causing the cerebellum – the part of the brain responsible for balance -- to migrate into the spinal canal.

When this occurs, the flow of cerebrospinal fluid, which bathes and cushions the brain and spinal cord, can be blocked, and enlarging cysts can develop inside the spinal cord. In addition, with the protrusion into the spinal column of the descending cerebellar tonsils -- two small portions of tissue in the cerebellum -- pressure is exerted on the lower stem of the brain.

"I was deteriorating pretty rapidly," Jennifer says. "My husband, Tim, and I were concerned because my right leg was starting to drag, and I had developed so much pain in my neck and the back of my skull. I continued working despite my symptoms, taking a day off here or there, but I was struggling."

Jennifer consulted her primary care physician, who ordered an MRI of the lower back. The MRI revealed some slightly bulging discs, but nothing severe enough to cause her symptoms, so the doctor then ordered an MRI of her brain. That MRI revealed the Chiari malformation, and Jennifer's doctor then referred her to a neurologist.

The neurologist did not think the Chiari malformation was connected to Jennifer's problems, but, concerned about the numbness and weakness in her arm and leg, he ordered an MRI of her cervical spine. The third MRI demonstrated a serious disorder: Jennifer's spinal cord contained a cyst, or syringomyelia (pronounced sear-IN-go-my-EEL-ya) cavity. Blockage caused by the Chiari malformation was forcing cerebrospinal fluid into the center of the spinal cord.

Jennifer's next step was to investigate whether her problem could be treated. "I visited a neurosurgeon in Louisville who was recommended by a friend," Jennifer says. "This doctor agreed that I needed to have the surgery, but he had performed only about two or three of this kind of surgery. My husband and I were confident in him as a doctor, but we didn't want someone operating on me who had done this only a few times. So we did a lot of research on the Internet. We found that there are a handful of major Chiari and syringomyelia centers in the United States."

Jennifer learned that Dr. John M. Tew, a neurosurgeon with the Mayfield Clinic and The University of Cincinnati Neuroscience Institute at University Hospital, had extensive experience in treating Chiari malformations. Jennifer then called the Mayfield Clinic, which asked that she send her scans and other test results.

"Dr. Tew saw me almost immediately, and everything happened pretty quickly after that," Jennifer says. "My husband is the best judge of character I know; he can read a person. He told me Dr. Tew is the person we need to deal with. And that was good enough for me."

Jennifer recalls feeling reasonably calm before her surgery. "I think my friends and family were more worried than I was. I think I knew what could happen with this condition – it can eventually cause paralysis – and I knew how I was feeling. So I was less afraid of the surgery than I was of what could happen. Of course, I was nervous about it, but I wanted to get it done."

Says her husband, Tim: "The best thing she did was to investigate. She took the time to research the disease. I think that made her feel better."

While Jennifer was in surgery, Dr. Tew and his team removed the bottom part of her occipital bone (the lower skull). He removed the upper cervical vertebra and the tough outer membrane of the brain (the dura), and replaced it with a larger patch, derived from the inner scalp, to make more room. He also shaved the cerebellar tonsils, which were protruding into her spinal column.

By freeing up the flow of cerebrospinal fluid and relieving pressure on the brainstem, Dr. Tew was hopeful that the cysts would eventually shrink.

Jennifer underwent surgery at Cincinnati's University Hospital on a Tuesday morning and went home on Friday. A month later, most of her symptoms had subsided. Her neck and back pain were gone, and her headaches were mild compared to those that preceded her operation. Six weeks following her operation, despite a residual feeling of numbness in her right arm and leg, she was ready to return to work.

"Jennifer's story provides a classic account of a severe, rapidly progressing Chiari disorder associated with brain compression and syringomyelia formation," Dr. Tew says. "She is fortunate to be experiencing a good recovery following early recognition and treatment."

Jennifer doesn't remember much about her stay at University Hospital, but she says she remembers "every name and every face of every nurse" who cared for her.

"They were just awesome," she says. " Katie, Holly, Rhonda, Florence, Brian and Nancy. When I came back to Dr. Tew's medical office to get my staples removed, I brought basket of treats and took it over to the hospital. Katie, one of the nurses I had, was there. She said, ‘It's so good to see you looking so well.' They love it when patients come back and they get to see them up and about."

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Hope Story Disclaimer - "Jennifer's Story" is about one patient's health-care experience. Please bear in mind that because every patient is unique, individual patients may respond to treatment in different ways. Results are influenced by many factors and may vary from patient to patient.