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6/28/2017 Latest post:
Bethany's story started a couple of years back when her pediatrician, Dr. Bardan, noticed a pretty severe curve (scoliosis) in her spine during a sports physical. Due to her curve being a left thoracic curve vs. the typical right thoracic curve, we were referred on to a neurologist, as this typically means there is something underlying that is causing the scoliosis. After many MRI's, Bethany was diagnosed with Arnold Chiari Malformation Type I.
Chiari malformations (CM's) are structural defects in the cerebellum. They consist of a downward displacement of the cerebellar tonsils through the foramen magnum (the opening at the base of the skull), sometimes causing non-communicating hydrocephalus as a result of obstruction of cerebrospinal fluid outflow. The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in the vasculature of the brain. CM's can cause headaches, difficulty focusing, concentrating, and thinking, as well as difficulty coping (with what most consider and experience as normal, everyday life), fatigue, muscle weakness, difficulty swallowing (sometimes accompanied by gagging), choking and vomiting, dizziness, nausea, tinnitus, impaired coordination, neck pain, unsteady gait (problems with balance), poor hand coordination (fine motor skills), numbness and tingling of the hands and feet, and speech problems.
Less often, people with Chiari malformation may experience ringing or buzzing in the ears (tinnitus), weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis.
In Bethany's case, the only symptom she was having at the time was the scoliosis, which was being caused by syrinxes on her spinal cord. Not everyone with Chiari malformation requires surgery, but when a patient's individual circumstances warrant it, a neurosurgeon may recommend "decompression" surgery (known as a “decompressive suboccipital craniectomy and cervical laminectomy”). As its name suggests, the goal of this procedure is to reduce or eliminate the pressure on the spinal cord caused by the cerebellar tonsils. Nearly all patients who undergo decompression surgery enjoy partial or total improvement in their symptoms, with very little risk.
In decompression surgery, which is performed under general anesthesia, a neurosurgeon makes an incision in the back of the head and removes a small piece of the skull. After this first part of the surgery, the neurosurgeon will evaluate whether or not enough pressure has been relieved. If further decompression is necessary, surgery continues in a second step that involves opening the dura (duraplasty, durotomy), the tissue that covers and protects the brain and spinal cord. Opening the dura results in a more immediate and thorough reduction of pressure on the spinal cord.
On December 31, 2014, Bethany underwent Chiari decompression surgery AND duroplasty. Her recovery was a long road but she is now back to doing her normal activities without many issues other than headaches and some pretty horrific back pain, now due to the scoloisis. At the time of her diagnosis, we knew there was the possibility of spinal fusion surgery in the future. In 2014, her upper curve was measured to be 51 degrees and her lower curve was at 38 degrees. Anything above 50 degrees typically requires surgery.
On Friday, May 5th, 2017, Bethany had a check-up with her orthopedic surgeon. X-rays were taken as they normally are. After the x-rays were read, her surgeon came in to meet with us and gave us the tough news that surgery needs to be performed sooner than later. Her curves have increased to 86 degrees on the upper and 53 on her lower. When he showed us the x-ray, it was so clear as to what has been causing the horrific back pain and headaches. He told us that once scoliosis curves reach between 70 & 80 degrees, there is a significant chance of losing lung function. In her case, in order to improve the pain and symptoms, there really is no other option other than spinal fusion surgery. This is where he will go in and attach screws and a rod to fuse her vertebrae together to help straighten it and prevent it from becoming worse.
As with any surgery, just as the scary risks of brain damage during her Chiari decompression surgery & duroplasty, there are some very scary risks. Bethany not only has scoliosis, but she also has what is called kyphosis (or a more plain term would be "hunch back"). He will need to correct both of these conditions. With the scoliosis surgery, there is a 1 in 1000 chance of neurological and spinal damage. With the kyphosis surgery, there is a 1 in 500 chance. He said that with performing surgery on both, if he had to place a number on it, he would say there is still a 1 in 500 chance of damage. He also told her that this surgery will be tougher on her than the last one and is even more serious due to operating so close to her spinal cord. It will last anywhere from 6-8 hours.
As much as we are scared of the risks, we are also extremely hopeful that this surgery will have a very positive outcome and will give her so much better quality of life in the long run. On a positive note, he said she will instantly grow at LEAST 2 inches, if not more! :) She was pretty happy about that. On a very negative note for her, she will not be able to play her beloved sport of hockey for about a year and the recovery from this surgery will be extremely painful and difficult. She is extremely devastated by this, but she knows that this is something that needs to happen and she's been a total champ in trying to remain positive.
We are graciously asking for prayers and positive thoughts that Dr. Guillaume's hands will be guided by God during her surgery and for courage and strength for my baby girl (and the rest of us). She's been through a heck of a lot in the past few years.
Her surgery date is set for September 14, 2017 at 8:30 am and will take place at Gillette Children's Hospital in St. Paul. Bethany & I will do our best to keep you all updated and I'm hoping she will use the journal on this site as a tool for coping. Thank you all in advance for your support and prayers!