The interview subject of the following narrative has granted permission for this content to be shared publicly on the course website and is aware they can alter this permission at any time. The individual has been assigned a pseudonym to protect their identity.
After a long workout, John will prepare a coffee and cook himself an omelet before he must head out for classes. He is a busy graduate student; studying industrial/organizational psychology; however, he will always make time for exercising and cooking in the morning. Although the cup of coffee may be scalding, he cannot feel the heat when he holds it in his right hand. As he cuts an onion to prepare it for his omelet, he must be extra careful and avoid holding it to close to his hands. He wouldn’t feel or notice even a simple cut. When turning off the stove he must be extra careful not to touch it while it is still hot; he would never know if he were to get a burn. Most people take for granted the ability to be feel sensations in their hands, but for John not having this ability is a normal part of his everyday life. Although it may be challenging at time his symptoms have never altered his charismatic and caring personality. John has learned to live with the side effects of the medical conditions Chiari malformation and syringomyelia. Chiari is a medical condition in which the brain tissues extend into the base of the skull (referred to as the cerebellum) causing blockage (Mayo Clinic para. 1). The most common symptoms are headaches, dizziness, and neck pain (Fischbein, Rebecca, et al. p 1617). Syringomyelia is a cyst that forms in the spinal cord because of the blockage caused by Chiari (Mayo Clinic para. 27). For John, his main symptom from both conditions were migraines and nerve damage. In this interview I wanted to learn from John’s perspective what has been done to research and improve the lives of those living with Chiari malformation and syringomyelia.
John started experiencing symptoms of Chiari malformation as a toddler. His initial symptoms appeared when he would collapse to the floor and declare that he could not get up. Since little is known about this medical condition, John went through several different tests as a toddler. His parents took him to the ER where they took a CT scan, which had no results. He was then referred to a neurologist that took an MRI scan of his brain and determined he had Chiari. The neurologist then referred John to one of the few neurosurgeons in the area that would treat his medical condition. After another MRI, this time examining the full body, it was officially determined he had Chiari and syringomyelia (commonly referred to as syrinx). At three-years-old John had his first surgery. This surgery, often referred to as duraplasty, extends the brain structure allowing fluid to flow (Hidalgo, Eveline Teresa, et al. P 544). The road to recovery was complicated, John had a spinal tap (lumbar puncture), where he was diagnosed with meningitis. Regardless, the MRIs soon determined that the surgery was successful; the cerebellum was expanded, and the syrinx could no longer be seen in MRI images. The MRI scans were stopped, but this was not the end of John’s medical journey.
From eighth grade to his sophomore year of high school John began to experience frequent migraines, vertigo, and loss of sensory sensation in the right side of his body. Taken to the hospital, it was confirmed that syringomyelia had reoccurred. Only one MRI scan was needed to confirm his condition, the spinal blockage was severe enough to negate other testing. The symptoms of syringomyelia were a result from the first surgery he had as a toddler. The scar tissue and bone regrowth caused compression in the spinal cord causing a cyst to form in the spine. The diagnosis was difficult to grasp. “I felt horrible. I went back to high school and had to pretend like everything was going to be okay when I know it most likely wouldn’t be.” As complicated as it was, he finished out the end of his sophomore semester and prepared for his surgery that took place over the summer.
Little is known about this medical condition, which makes the anticipation of surgery frightening. “I didn’t know what to expect, in high school it was very nerve wracking. I was also told a corrective surgery has an unknown mortality rate, that was stressful to hear.” By the time he had surgery, the syrinx was the full diameter and length of the spinal cord. Even though the surgery was a success, John still experiences symptoms of his medical condition such as nerve damage on the right side of his body, a loss of touch sensation on his right face all the way to his torso. His neck and base of skull has nerve damage from the incision during surgery. Doctors couldn’t determine one solution on how to reduce the symptoms. They gave him Gabapentin, a medication that is supposed to reduce nerve pain. Although initially effective it was not as successful long-term as was hoped by the doctors. John then turned to physical therapy and exercise which has proven to be a better alternative in maintaining the symptoms he experiences daily. “Exercise has helped my neuropathic symptoms significantly. I used to have random nerves firing causing pain and itching sensations that wouldn’t go away. With exercise and balanced dieting, I have reduced those symptoms.”
The lack of research goes beyond the treatment options provided and impacts family members of those with Chiari and syringomyelia. It is unknown how the medical condition is inherited, but one theory is that it may be genetic. For John spinal conditions run in his family. His mom was diagnosed with Chiari shortly after he was as a toddler, and his sister has scoliosis. Spinal ailments are not unknown to the family, but no genetic factor has been confirmed. Genetics may play an important role in this condition, yet not enough research has been done to support this claim.
John now understands what to look for when he begins to experience symptoms from his medical condition, but many people, both in the medical field and outside do not understand Chiari malformation and syringomyelia. Medical professions have indicated that studies are not representative of the general population, but rather are only investigated when individuals begin to experience neurological symptoms requiring the patient to get neuroimaging’s, such as MRIs (Kahn, Elyne N., et al p 1). Not enough research has been conducted on this medical condition. “Research, research, research. Prevalence rates are high for Chiari, but few people have adverse reaction requiring intervention. But research is of utmost importance for this disease.” 92.9% of children who are diagnosed with Chiari malformation are likely to live with the condition without needing surgery. However, those who are diagnosed with Chiari and syringomyelia are likely to be considered as surgery candidates because of the high rate of improvement shown in patients once surgery has been performed (Pomeraniec, Jonathan I, et al. p 343).
Chiari malformation and syringomyelia have played an important part in John’s life. “[Chiari and syringomyelia has] made me more empathetic to others and gave me an appreciation for my life.” John has learned to live with his condition. Because of physical therapy he has discovered that he loves to exercise and enjoys maintaining a balanced diet. This has piqued his interest in cooking, discovering new recipes, and creating food for himself and others to enjoy. After graduate school he hopes to work in organizational management for airline companies. Life may be different for John, but his illness does not define him. It has made him into the person he is today.
“Chiari Malformation.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 24 Sept. 2021, https://www.mayoclinic.org/diseases-conditions/chiari-malformation/symptoms-causes/syc-20354010.
Fischbein, Rebecca, et al. “Patient-Reported Chiari Malformation Type I Symptoms and Diagnostic Experiences: A Report from the National Conquer Chiari Patient Registry Database.” Neurological Sciences, vol. 36, no. 9, 2015, pp. 1617–1624., doi:10.1007/s10072-015-2219-9.
Hidalgo, Eveline Teresa, et al. “Time to Resolution of Symptoms after Suboccipital Decompression with Duraplasty in Children with Chiari Malformation Type I.” World Neurosurgery, vol. 117, 2018, pp. 544–551., doi:10.1016/j.wneu.2018.06.073.
Kahn, Elyne N., et al. “Prevalence of Chiari I Malformation and Syringomyelia.” Neurosurgery Clinics of North America, vol. 26, no. 4, 2015, pp. 501-507. Google Books, doi:10.1016/j.nec.2015.06.006.
Pomeraniec, Jonathan I, et al. “Natural and Surgical History of Chiari Malformation Type I in the Pediatric Population.” Journal of Neurosurgery: Pediatrics, vol. 17, no. 3, 20 Nov. 2015, pp. 343–352. doi:10.3171/2015.7.peds1594.
Smith, John [pseudonym]. Personal communication with author. 10 Nov. 2021.
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