BSBN Brigid Costello vs. Neurosarcoidosis

Purpose: Due to Brigid’s condition, she currently has no income. This fund will assist with Brigid’s expenses - medical and otherwise.

Brigid’s Story:

My name is Brigid Costello. And I'd like to share a story with you. A story of my recent personal experiences. This is not a sob story. This is not a fairytale. This is a real story based on hard facts. I do not want you to "feel sorry" for me. That is not the point. The point of my story is to illustrate how quickly the trajectory of a person's life can change without notice. How fragile human life is. And how I intend to navigate forward through the challenges in front of me. I'm a science nerd, so I'll include some science-y stuff. I hope you find my story educational, but more importantly, inspirational. This story begins on a Friday…

Friday, March 25th, 2022 was a good day. I went with my boyfriend, Matt, to John Pessoni's 50th birthday party. John is the drummer in my boyfriend's band, plus a lot of other bands - probably most notably, The Urge - so the party was packed with popular local musicians. It was fun. As we left the party, we walked to our cars with another couple. I expressed my excitement about upcoming events in my life. I'd turn 39-years old on April 23rd - a Saturday(!) I was scheduled to compete in a martial arts tournament - the Kuk Sool Won Midwest Tournament - on April 30th. I was scheduled to test for my 4th degree black belt on May 7th. Up until Friday, March 25th, I'd led a normal life. A life with some adversity, of course, like most, but fairly normal nonetheless. And I had worked hard to achieve some life goals. I had earned a Bachelor's Degree in Astrophysics from UMSL. I had earned a Bachelor's Degree in Education and a Master's Degree in Early Childhood Education from Maryville University. After 15+ years teaching children at a Daycare, I'd earned a nice promotion to a management position. After 15+ years of passionate work and dedication, I'd earned my 3rd degree black belt in the martial art of Kuk Sool Won. I loved helping kids. I'd work with kids at my Daycare job during the day, then drive from St. Louis to St. Peters a couple nights a week to teach both kids and adults in martial arts classes. So, yes. On Friday, March 25th, life was good. Then came Saturday, March 26th. I got a headache.

I can tell you now what no one knew on March 26th… and what no one - including doctors - knew even several weeks after: On Saturday, March 26th, my headache was the first symptom of an extremely rare autoimmune disease called "neurosarcoidosis". Neurosarcoidosis attacked my central nervous system (CNS) - my brain, spinal cord, and "meninges" - a 3-layer membrane that covers and protects my brain and spinal cord. Neurosarcoidosis is a subcategory of "sarcoidosis". Sarcoidosis is rare. The U.S. population is around 330 million. Sarcoidosis affects less than 6% of 1% of the entire U.S. population. Sarcoidosis most often affects the lungs, a condition called "pulmonary sarcoidosis". Neurosarcoidosis is even more rare. Only 5-10% of sarcoidosis patients have neurosarcoidosis. In short, neurosarcoidosis affects less than 1% of 1% of the entire U.S. population. My primary care doctor, who is based out of a popular St. Lous hospital, and who has seen me since I was 5-years old, said he's never seen a single case of the disease. Now I know why.

In my particular case of neurosarcoidosis, onset was rapid and severe. From the time I got that headache on March 26th through April 7th - in the span of less than two weeks - my headache persisted and increased in severity. I experienced numbness and weakness from my waist down, collapsed and lost my ability to walk. I experienced complete vision loss in my left eye. And then as a result of “cranial neuropathy”, a condition called “bilateral facial palsy” set in, essentially breaking the connection between my brain and my facial muscles. I could no longer move my lips or close my mouth. My face froze still, unable to convey expression. I could not blink. Even when I slept my eyelids would be open. Eventually, as my condition spiraled downward, I became unresponsive and was held in a deep sleep most of the time. Then, on Thursday, April 7th, I had a grand mal seizure. After my seizure, I was transferred to St. Louis University Hospital, where I was finally stabilized. Unfortunately, by that time, several areas on my central nervous system had been severely damaged.

So what is neurosarcoidosis? Here's where I'll tell you some science-y stuff. At the onset of sarcoidosis, the body's immune system - mistakenly - begins to react as if there is an infection to attack. The immune system prepares for battle. Ferocious cell clusters called "granulomas" develop, increase, divide, and spread throughout organ tissue. At the heart of granulomas are "macrophages". In Greek, the word "macrophage" literally means "giant" (macro) "eater" (phage). Without a virus or any other foreign abnormality to attack, the macrophages - these "giant eaters" - turn on the human body and begin to attack human tissue. In neurosarcoidosis, this means the central nervous system is attacked. As you can imagine, unchecked, this condition can lead to severe physical consequences. Like a scene straight out of a horror movie, some “thing” is literally feasting on the human brain, spinal cord, and their valiant protector, the meninges. Inflammation begins. Lesions form and grow. The connection between the brain, nerves, and muscles begins to break down and deteriorate.

Is there a cause? Here's the thing: There is nothing I could have done to prevent my disease. I've always led a clean, healthy, and active life. Although sarcoidosis has been recognized as a disease since the late 1800s, no cause has been found. Because it predominantly affects the lungs, it's believed the disease is triggered by an antigen that is inhaled. However, despite decades of research, no connection to any particular chemical or substance has been found. There is no proven genetic connection. There are no solid answers. Neurosarcoidosis can affect anyone. Any time. Any place.

Is there a cure? No. Currently, there is no cure for neurosarcoidosis. The disease can only be treated by medicine to hopefully contain its adverse effects. For the remainder of my life, I will receive medication and my condition will be monitored. Because of the severity of my case, this means I'll receive chemotherapy (cyclophosphamide) for at least six months then, hopefully, transition to milder chemotherapy (methotrexate and infliximab). This depends on how my body reacts to the treatment. At the same time, I will continue intensive physical rehabilitation.Trying to reconnect my brain to my body. Trying to teach myself to talk again. Trying to teach myself to walk again.

I'll finish my story with a timeline. The timeline illustrates how quickly my life changed. The timeline is from the onset of my first symptoms on March 26th, 2022 forward, and is compiled from a wide variety of sources - texts with friends, audio recordings from friends, doctor's notes, etc. It's the only way an accurate timeline could be compiled, because honestly, I really don't remember the first half of April.

IT’S MORE THAN A HEADACHE: March 26th through March 31st

Saturday, March 26th, 2022:

Upon awakening, Brigid experiences a headache. As the day progresses, Brigid’s headache persists. She attends martial arts class in St. Peter’s, and then visits her boyfriend, Matt, in De Soto. They binge-watch Netflix and have dinner. Brigid's headache grows more severe. She takes ibuprofen, which is ineffective. Brigid comments that her headache feels unusual.

Sunday March 27th, 2022:

Brigid's headache pain increases. She attempts to rest at home. No activity.

Monday, March 28th, 2022:

Brigid goes to work at her Daycare job. Feeling worse, she leaves work early to see her Primary Care Physician at St. Mary’s in St. Louis. She reports her whole body hurts as her headache persists. The doctor diagnoses her with hypertension and prescribes blood pressure medication.

Tuesday, March 29th, 2022:

Brigid goes to work at her Daycare job, but stays home from her Tuesday evening martial arts classes, as her headache persists. She experiences the onset of a NEW SYMPTOM: DIZZINESS.

Wednesday, March 30th, 2022:

Instead of going to work, Brigid goes to St. Mary’s ER in the morning. She reports her persistent headache and dizziness to the doctor. She also reports difficulty sleeping due to her headache. The hospital performs a chest x-ray and a CT scan of the head without IV contrast. “Without IV contrast” is significant, as contrast is extremely important in revealing abnormalities in the brain associated with neurosarcoidosis. Brigid’s chest x-ray is declared clear. No abnormalities are revealed by the CT scan. Brigid is given a migraine cocktail and released in the afternoon.

Thursday, March 31, 2022:

Feeling worse, but also feeling a sense of duty, Brigid goes to work at her Daycare job. Symptoms persist. NEW SYMPTOM: DOUBLE VISION. Brigid arranges for a ride to work the following morning, because she doesn’t feel it’s safe to “drive with blurred vision.”

THE DOWNWARD SPIRAL: April 1st through April 7th

Friday, April 1, 2022:

As she gets ready for work, Brigid collapses in the shower. NEW SYMPTOM: Due to NUMBNESS AND WEAKNESS FROM THE WAIST DOWN, Brigid is unable to stand on her own. At approximately 9am, Brigid is taken to St. Care’s ER in Fenton, MO. She is examined in the ER and then by a neurologist. Symptoms include headache, dizziness, and diplopia (double vision). ER notes she is so lethargic she falls asleep before answering questions. Brigid is lucid when she is seen by the neurologist. The neurologist states he isn’t sure what is wrong, but insists he will figure it out. Brigid undergoes several tests: lumbar puncture, CT scan with IV contrast, and bloodwork. Lumbar puncture (spinal tap via L3-L4 interspace) is performed. 1st lumbar puncture attempt by DO is unsuccessful. 2nd attempt by MD is successful. A small amount of cerebrospinal fluid (CSF) is obtained and sent to pathology for study. CT scan of both neck and head with IV contrast is performed. A subtle beaded appearance of the extra cranial right internal carotid artery (ICA) is noted. Brigid is placed on a steady regimen of three separate antibiotics via IV.

Saturday, April 2, 2022:

NEW SYMPTOM: Brigid experiences COMPLETE LOSS OF VISION IN LEFT EYE. Examination confirms 20/800 vision (blindness) in left eye. More CSF is needed per neurologist. Therefore, Brigid undergoes a repeated lumbar puncture. Approximately 8 cc of CSF obtained. CSF sent to pathology for study. Brigid is seen by Speech Therapist and a Physical Therapist, who confirmed Brigid’s lower body deficits: Bilateral lower extremity (BLE) reliant on BUE (bilateral upper extremity) for support. PT notes: Patient will need intensive PT upon discharge. Neurologist reviews preliminary bloodwork and brain MRI imaging, with the following impression: 1) Meningoencephalitis. Etiology unclear. 2) Multiple cranial neuropathies due to #1. 3) Bilateral lower extremity weakness due to #1. Imaging reveals lesions in bilateral cerebellar hemispheres. Ultrasound of abdomen performed. No abnormalities found in abdomen. MRI of spinal cord planned.

Sunday, April 3, 2022:

Previously mentioned symptoms persist. Brigid receives several visitors - family and friends. Although unwell and fatigued, Brigid’s communication is normal. She continues to be treated with three separate antibiotics via IV. Additional bloodwork.

Monday, April 4, 2022:

Brigid continues to be treated with three separate antibiotics via IV. Additional bloodwork. A hematologist/oncologist (blood/cancer doctor) performs a brief physical examination of Brigid. MRI of Brigid’s spinal cord is performed. MRI of spinal cord reveals lesions at multiple levels around the cervical and thoracic spinal cord. Further review of brain MRI imaging reveals multiple, additional lesions, most prominent in the left occipital region, left temporal, right temporal, and bilateral frontal regions. Per doctor’s notes: “Findings are concerning for infectious inflammatory etiology. Neurosyphilis or neurosarcoidosis are in the differential. Metastatic disease cannot be excluded.”

Tuesday, April 5, 2022:

Per early AM phone conversation with St. Clare’s nurse: All visitors restricted per doctor’s orders. Brigid has been diagnosed with meningitis. The nurse is unsure which type - viral, bacterial, etc. A CT scan with IV contrast of Brigid’s chest, abdomen, and pelvis is performed for malignancy evaluation. No malignancy is found. At 10:00am Matt has a five minute phone call with Brigid. He has difficulty understanding her words. He attributes the lack of clear speech perhaps to a bad phone connection. Per 5:49pm text message thread from Brigid: “Lunch was okay. I didn’t eat much.” “The ultrasound said I probably don’t have cancer.” “They might do a biopsy.”

Wednesday, April 6, 2022

At 12:29pm Matt has a seven minute phone call with Brigid. Her speech difficulty was clearly apparent. He asks her to text what she is trying to communicate. Brigid responded via text: “I said Im having a lot of trouble contacting my lips. It’s hard to enunciate.” Brigid’s sister, Amy, is with her. At approximately 4:30, Brigid is cleared for a transfer to SLU Hospital in St. Louis “as soon as they have a bed.” Matt visits Brigid for a few hours in the evening. NEW SYMPTOM: FACIAL PALSY. Immobility of Brigid’s outer facial muscles is clearly noticeable. Inability to move lips. Matt asks her if she has difficulty swallowing, to which Brigid responds “A little bit.” Brigid’s physical condition is clearly deteriorating rapidly as her symptoms progress.

Thursday, April 7, 2022

Brigid’s best friend, Jeri, flies in from Mexico and arrives around 8am. Brigid’s sister, Amy, visits in the afternoon and communicates with several people trying to speed up Brigid’s transfer to SLU. Brigid is in a deep sleep with minimal response. She is non-verbal from at least 8am forward throughout the day/evening. Her eyes are closed most of the time. When her eyes are open, the motion is very slow and there is very little movement before she slips back into sleep. Brigid is cleared for transfer from St. Clare’s to SLU Hospital around 4pm. However, a transfer ambulance is not available until 8pm. NEW SYMPTOM: SEIZURE. At approximately 7:20pm, Brigid experiences a grand mal seizure. The seizure lasts approximately one to two minutes. Shortly thereafter, a Basic Life Support (BLS) ambulance arrives to transport Brigid to SLU Hospital. Given Brigid’s dire condition, the BLS ambulance crew seeks the advice of their supervisor. The supervisor decides an Advanced Life Support (ALS) ambulance is needed. An ALS ambulance arrives a few hours later. Brigid is transported to SLU Hospital around 11pm.

FINDING ANSWERS: April 8th through April 28th

Friday, April 8 - Thursday, April 28, 2022

Brigid is admitted to SLU Hospital. Upon arriving at SLU, the urgency of attention to Brigid’s care increases. Brigid is given a very high 5-day course of methylprednisolone via IV followed by prednisone (steroids), which proves effective in halting her downward spiral. Steroid treatment is continued and, although her symptoms remain, Brigid becomes less lethargic and is more responsive. Brigid undergoes a barrage of tests (bloodwork often, MRIs, CT scans, repeated lumbar puncture, PET scan, nerve conduction tests). She is examined/visited by a wide variety of doctors - neurologists, ophthalmologists, infectious disease doctors, rheumatologists, and neurosurgeons. She is also placed on an EEG monitor for her first few days. A thorough clinical exam by the ophthalmologist is performed on Friday, April 8th. The exam reveals Brigid has no light perception in her left eye. A neurologist visits on April 9th. The neurologist states Brigid has multiple lesions in her CNS and informs Brigid there will be a repeat lumbar puncture and possibly a biopsy of Brigid’s spine or brain. On Monday, April 11th, the ophthalmology team visits and informs Brigid the blindness in her left eye is permanent. “That’s not going to change. That’s forever.” Multiple MRI scans of the orbits (eye sockets) reveal the optic nerve to her left eye is dead. The goal is to now do a “full court press” to prevent damage to Brigid’s right eye. After tests rule out a multitude of infectious diseases, the doctors consult and determine Brigid’s condition is more likely an autoimmune disease. She is taken off antibiotics and the emphasis turns to steroid treatment. The lumbar puncture reveals elevated ACE levels in Brigid’s CSF. After more test results are received and further discussion, a preliminary diagnosis is made. A neurologist visits Brigid on Tuesday, April 12th and informs her she is being treated for a condition known as “sarcoidosis”. A reading of the PET scan shows no signs of malignancy and no other areas of Brigid’s body affected outside of her CNS. The results of the PET scan further narrow the diagnosis to likely “neurosarcoidosis”. On Wednesday, April 20th, Brigid undergoes surgery. A brain biopsy is performed to confirm neurosarcoidosis. A neurosurgeon drills through Brigid’s skull and extracts a small piece of tissue from the dura (the outer layer of the meninges).

LEARNING TO WALK AND TALK AGAIN: April 28th forward

Thursday, April 28 - Tuesday, May 24, 2022

Brigid is transferred to St. Mary’s Acute Rehabilitation Hospital in Richmond Heights. She receives daily speech, physical, and occupational therapies during inpatient stay. During this time her physical condition improves to the point where she is able to walk with a walker and assistance. Her ability to move facial muscles also improves.

Tuesday, May 24, 2022

Brigid is released from St. Mary’s.

A WORK IN PROGRESS: June 2, 2022 forward

Thursday, June 2, 2022

Brigid begins Physical Therapy (PT), Occupational Therapy (OT), and Speech and Language Therapy rehab at the Day Institute in Kirkwood five days a week.

Saturday, June 11, 2022

Brigid’s first day back at Kuk Sool Won St. Louis since March! Hopefully lots more to follow.

Brigid will have frequent follow up appointments with Neurology, Rheumatology, Neuro Optholomolgy, and Otolaryngology.

The team of doctors outline Brigid’s treatment plan: She will receive a six-month course of cyclophosphamide via IV infusions (every four weeks) along with several other oral medications. If all goes well, after the full course of cyclophosphamide, Brigid will transition to a combination of methotrexate (via weekly shots) and infliximab via IV infusions indefinitely.