When growing up Angela would often stop what she was doing to ask us if we smelled something strange but my wife and I were unable to sense what she did. After a few years we started asking doctor after doctor what could be causing such sensations but no one could explain why she was smelling things that did not exist or why she was having feelings of déjà vu. Then, in October 2012, my freshly-minted sixteen year old started to repetitively seize. After two days of non-stop seizures and EEG monitoring Angela was diagnosed with temporal lobe epilepsy; it was only then that her neurologist was able to confirm that the sensations Angela had experienced for years were actually simple partial seizures, otherwise known as auras. At this time Angela was also diagnosed with a congenital condition called Chiari Malformation Type 1, but this neurological disease was not yet causing problems and her doctors were more concerned with getting her seizures under control. Angela was put on antiepileptic drugs to try to control the seizures but even when taking three different prescription medications she continued to have six auras a day and two larger seizures a week. Four months after graduating high school and almost exactly three years after diagnosis, in October 2015, Angela underwent a temporal lobectomy to remove the damaged area of her brain. Thankfully, this surgery was a tremendous success –she has been seizure-free since the surgery and has not had an aura in over six months.
Unfortunately, her seizures were replaced by a constant severe headache, sensitivity to light and sound, nausea, dizziness and fatigue. Angela started attending the University of Washington studying neurobiology in January 2016 and was able to push through the pain to successfully complete three quarters. In February 2017, however, her condition became so serious that she had to temporarily stop attending the university.
Angela’s doctors insisted her new symptoms were a result of her temporal lobectomy and worked to treat only the pain as they did not believe there was another underlying problem. From July 2016 to February 2017 her doctors unsuccessfully tried to treat her pain with fifteen different methods, ranging from oral medications to injections to nerve blocks. After fifteen attempts Angela’s doctors informed her that there was nothing else they could do for her and that she would just have to deal with life with chronic pain. My daughter is extremely stubborn and always wants to live life to the fullest so she was not satisfied by this answer, instead reaching out to experts across the country for other treatment options.
In April a neurosurgeon in New York examined Angela’s case and informed her that her symptoms were classic for Chiari Malformation Type 1. The disease she was diagnosed with nearly five years ago is now causing problems. Essentially the hole at the base of Angela’s skull, through which the spinal cord passes to attach to the brain, is too small. Her brain does not have enough space to sit within the skull so her cerebellum (the part that controls balance) is falling out of her skull and into her spinal cord. This is blocking the flow of spinal fluid, with minimal flow in the front of her cerebellum and no flow in the back. This neurosurgeon has forty years of experience treating Chiari Malformation and has never seen a case like Angela’s, but he is certain that her temporal lobectomy triggered the onset of her symptoms and the rapid progression of her disease.
Angela needs a second brain surgery, called a posterior fossa decompression, in order for her symptoms to be alleviated. In this procedure bone will be removed to make the hole at the base of her skull bigger, thus relieving the pressure this condition is putting on her brain and restoring spinal fluid flow. This procedure will take place in Long Island, New York on July 11. She will need to be in New York for a few days prior to surgery for preoperative tests and will stay in the hospital for four to five days after surgery. Receiving this treatment means traveling three thousand miles across the country so this money will be used to pay for travel and medical expenses.
The goal of this surgery is to halt the progression of this condition so no permanent nerve damage occurs, but surgery is not a complete cure. Chiari Malformation is a chronic illness Angela will battle with for the rest of her life. Any remaining funds will be donated to the Chiari and Syringomyelia Patient Education Foundation, an amazing group of researchers who are working to fully understand this rare disease and to find a cure to help Angela and her fellow Chiarians conquer this debilitating illness.
Angela is a compassionate young woman who never hesitates to help those in need. More than anything she wants to be healthy enough to return to school in the fall so she can continue working to become a neurologist. She believes her experiences will help her better relate to her patients and give her extra motivation to find answers for the people she treats as she knows what it is like to not be given any.
I thank you from the bottom of my heart for any help you can give to my daughter. We greatly appreciate all of your love and support!
Watch the video below to see the New York surgeon discuss why diagnosing this condition is so difficult and how he goes about treating patients with Chiari. He starts talking at the 15:00 minute mark of the video.