This is a guest post by Lori Madeira. Lori is a ME/CFS advocate who posts on social media about a wide range of topics relating to ME/CFS and other chronic illnesses. You can follow her on Twitter '@MECFSWarrior' and Facebook 'Lori Madeira | ME/CFS Advocate'.
Have you been interested in learning more about Jennifer Brea’s recovery but haven’t had the cognitive ability to read more about it? Below is a condensed timeline of important facts taken from her Medium.com blogs, to help you quickly understand about her illness onset, functionality during her illness, surgeries in 2018, and her recovery from ME. This post takes 12 minutes to read.
This information is taken directly from 21 of her blogs from “A New Diagnosis To Add To The List” to “Health Update #4: Recovery Is A %*#*&[email protected]!” This information is condensed and arranged sequentially by Lori Madeira. The closing remarks are by Lori Madeira. All other information is owned and originally written by Jennifer Brea. Please check out her full blogs on Medium.com for more detail if you are able.
In 2008, Jennifer briefly lived in Beijing, China in a house with severe mold infestation, exposing her to massive amounts of black mold over a period of a few weeks. She developed memory and concentration problems. She then collapsed unconscious with a thick, black snot oozing from her nose. She immediately left that house.
After that experience, she started to have hypersensitivity to mold and mildew. She developed food intolerances and a sensitivity to alcohol. These were possibly all symptoms of early Mast Cell Activation Syndrome, which she believes was triggered by her mold exposure. She was otherwise healthy and functional.
Black mold can directly release collagen degrading enzymes. Viruses can trigger collagen degrading enzymes as part of a person’s inflammatory response.
She believes her mold exposure set the stage for a massive inflammatory response in her next major viral infection, by initially causing some connective tissue damage and triggering mild MCAS.
In early 2011, she had a fever for ten days. It peaked at 104.7. She became suddenly ill after this acute viral infection, and developed ME/CFS and PEM.
Tests weren’t done at the time to confirm the virus she was infected with, but Dr. John Chia believes she had Cocsackie B4. She would later test for high titres of autoantibodies.
She believes a hyper-inflammatory response to this acute viral infection damaged the connective tissue and ligaments in her craniocervical junction. This caused brainstem compression and Craniocervical Instability to develop at this point, and was left undiagnosed for seven years.
She became progressively more ill. By 2012, she became bedridden and was incorrectly told she had “conversion disorder.”
She was eventually diagnosed with ME. She met all definitions of ME/CFS, including the ICC and CCC definition of ME/CFS. She prefers the term “ME” even though she thinks ME and CFS are one in the same, see her blogs for more information on her thoughts.
She developed Postural Orthostatic Tachycardia Syndrome. She also developed and was eventually diagnosed with full blown Mast Cell Activation Syndrome.
She had the classic symptoms of ME, POTS and MCAS. As well as those, some of her worst symptoms were sound hypersensitivity, light hypersensitivity, neurological symptoms, and difficulty regulating her body temperature.
From 2011-2018, she lived mostly in her bedroom, only walking to go to the bathroom. At her best, she could leave the house in a power wheelchair, as long as she was careful to pace and not to induce PEM.
She started wearing a fitness watch in 2015. She was averaging 250-300 steps per day between then and 2018. The average for a healthy person is 5,000-7,000.
Life In-between Onset And “Unrest”
During the time she was making Unrest, she spent three years living in tents in her backyard in Princeton from April to October for mold avoidance. She reacted to every building on the East Coast. Before mold avoidance, she was bed bound and able to eat only three or four foods. After, she experienced remission of nearly all food sensitivities, greater cognitive and physical function, and some improvement with ME.
By 2017, the combination of mold avoidance, antivirals- Valcyte and Valtrex, Mestinon, Fludrocortisone, and Ketotifen had improved her overall health. Finding those treatments over the course of a few years took her from very severe, to severe, to moderate, to moderate on the spectrum.
In 2017, she was moderate enough to enable her to travel to promote Unrest with the aid of a power wheelchair and many crashes in hotel rooms.
She included her own MRI taken in 2012 in “Unrest.” In early 2018, a neurosurgeon reached out to her after seeing “Unrest” and told her that her MRI showed an empty sella- an indication of intracranial hypertension. This finding was clear on her MRI but was previously incorrectly considered irrelevant.
She had the symptoms of intracranial hypertension for years: headaches and the feeling of pressure behind her eyes. In hindsight, she now attributes the crash after the parade in “Unrest” to intracranial hypertension.
In March of 2018, she sought diagnosis of intracranial hypertension by getting a type of MRI with contrast to image her veins. She had a high volume lumbar puncture to see if temporarily reducing the spinal fluid volume in her brain would improve her symptoms. It did temporarily, most notably, her ability to read and dizziness improved.
These tests lead to a suspected diagnosis of intracranial hypertension, something she had had since the early stages of her onset, but had gone undiagnosed until then. She needed further testing to confirm this.
These tests and findings also lead to the first doctor to suspect she had Craniocervical Instability, aka CCI, that needed further investigation.
: Jennifer Brea, from her Medium.com blogs
Thyroid Surgery And Aftermath
In June of 2018, she had a total thyroidectomy due to Stage 1 thyroid cancer. She had put off having this surgery until after the promotion from “Unrest” was over. During the surgery, her neck was hyperextended to intubate her. She theorizes that this exacerbated a mild to moderate undiagnosed case of CCI that she had had since her ME onset, and turned it into a severe case.
She awoke from the thyroid surgery with excruciating pain in her jaw. She was weaker, now experiencing numbness and difficulty speaking. She couldn’t sit up without falling over. She was possibly experiencing spinal fluid leaks. All of her ME and POTS symptoms got much worse.
She developed the new and disturbing symptoms of stopping breathing (central sleep apnea) while she was completely awake, every time she laid on the back of her head. She had to lie on her side to remain breathing. She became non-responsive and looked like she had passed out every time she turned her head to the left.
All this resulted in a huge crash in July 2018. Coupled with the new symptoms, this was the most symptomatic and debilitated she had ever been.
Between Thyroid Surgery And Her Three Neurosurgeries
On the advice of her friends with Ehlers Danlos Syndrome, she began wearing a hard neck brace: a Philadelphia collar. She found that it relieved some of these new symptoms. It did not relieve her ME or POTS.
Her physician, Dr. David Kaufman, ordered an upright MRI. He was the second doctor to believe she had CCI.
She underwent two invasive tests: cervical traction test and a 24 hour intracranial pressure bolt test. Two bolts attached were surgically implanted to be attached to metal handles. A small hole was drilled into her skull and a probe was inserted to monitor intracranial pressure. She then underwent 24 hours of testing, measuring and monitoring her symptoms.
The invasive cervical traction test was performed, using the two bolts and handles to attach to a weight and pulley system. They used this to lift her head off of her spine. At thirty pounds of traction, she felt all her symptoms lift. She temporarily had complete remission of the agony of ME she had experienced for seven years. She knew then that brainstem compression was the underlying mechanism of her ME.
The intracranial pressure testing monitoring definitively confirmed her intracranial hypertension diagnosis.
She was definitively diagnosed with Craniocervical Instability and Atlantoaxial Instability, aka CCI/AAI, and Tethered Cord Syndrome. Her abnormal breathing was caused by cranial settling.
Starting in November 2018, she had three neurosurgeries over three months. One on her upper cervical spine, a craniocervical fusion, and two more surgeries in her lumbar spine.
On November 28th, 2018 she underwent a craniocervical fusion. The skull is pulled upward and placed into a corrected position, and then the occipital bone on the skull is fused to the upper cervical vertebrae to hold in the corrected position. Essentially, this lifts the head upwards permanently via surgery. This is very serious surgery, typically taking 4-8 hours and recovery from it takes a full year.
She woke up from her craniocervical fusion surgery with her ME symptoms in remission. It corrected her breathing problems/central sleep apnea, numbness, and weakness.
After the craniocervical fusion, she woke up with temporary remission from POTS symptoms. Her POTS symptoms went away and came back repeatedly over the course of a few months, she believes due to post surgical swelling.
In December of 2018, one week after her craniocervical fusion, she had surgery for tethered cord release. She believes she was born with a tethered cord. After that surgery, her POTS temporarily worsened, likely due to swelling.
In January 2019, she had another lumbar spinal surgery to release a hematoma that was compressing her spinal cord, a complication from a prior blood patch.
These three surgeries were brutal and the greatest traumas of her life. While her ME symptoms remitted during her craniocervical fusion, her full remission and permanent remission wasn’t clear at that point because of the impact of these surgeries was so severe. She needed significant recovery and physical therapy. And after seven years mostly in bed and wheelchairs, she was deconditioned.
During the recovery process from her surgeries, she slowly regained her ability to walk. She was able to walk 4,000-7,000 compared to her average of 250-300 pre-craniocervical fusion. By March, physical therapy wasn’t causing PEM, but instead gave her an endorphin rush.
In March of 2019, she went into full permanent remission of POTS and announced this publicly. She could easily stand without her heart rate going over 94. She theorizes that her POTS was caused by brainstem compression.
What was clear to her since receiving thirty pounds of cervical traction and how she felt when she first awoke from craniocervical fusion surgery had held true: ME had gone into remission. In May of 2019, she announced that she no longer had ME.
Since then, she is now well enough to hike, exercise, lift weights, dance, hold a plank for two minutes, drink alcohol, read and write long form, and multi-task.
She has experienced significant complications since undergoing surgery. She has had swelling that recompressed her spinal cord, trouble with recurrent spinal fluid leaks, and jugular vein entrapment by her omohyoid muscles. She has had to deal with the significant emotional trauma from all this.
Now, the only illness she still has is MCAS, which she still takes Ketotifen for. She attributes her mold sensitivity to MCAS and is still reactive to mold, but that seems to be improving. She takes no other of her previous ME and POTS medications. She takes thyroid replacement hormone due to her thyroidectomy.
A growing number of clinicians believe that intracranial hypertension may be an important part of the pathophysiology of ME/CFS and Fibromyalgia.
She theorizes that an acute viral infection made worse by prior mold exposure caused the damage connective tissues and collagen. Infection can damage the cervical spine. The region where her skull connected to the upper cervical spine had “lax” ligaments, causing cranial settling into the brainstem. This caused brain compression. She believes that brainstem compression caused her ME symptoms, POTS and autonomic dysfunction, considering they were relieved upon releasing the pressure on her brainstem when she had the craniocervical fusion lift her skull up off her compressed brainstem.
Craniocervical fusions are not a cure for ME. They are a treatment for people with ME who have CCI.
None of this means that she didn’t have “true ME” or “true CFS.” Jennifer Brea absolutely, factually had ME/CFS and was scientifically confirmed as having it. Her other diagnoses uncovered a previously little-known possible cause, complication, or underlying mechanism of ME/CFS. Her remission proved the factual basis of all this.
A lot of these symptoms, diagnoses, and surgeries are common in Ehlers Danlos Syndrome. She has some hypermobility but doesn’t meet any diagnostic criteria for EDS. She suspects she has a connective tissue disorder, due to changes brought about after her viral onset. She believes that connective tissue damage and collagen degradation was a part of her ME, and research indicates that this may be true for a large number of PwME as well.
A Swedish study of 234 patients who met the CCC definition of ME/CFS found 49% had hypermobility, and 20% met the criteria for EDS. 67% met Fibromyalgia criteria. Three different signs of intracranial hypertension were found in patients at rates of 83%, 55%, and 23%. Herniation of the cerebellar tonsil was found in 56% of patients. Chiari malformation was found in 13% of patients. This study confirmed that structural neurological abnormalities are present in a significant subset of ME patients.
Jennifer Brea’s recovery has ushered in a new era of discovery and hope for the ME/CFS community. We have a long way to go to understand the mechanisms that caused her particular problems and led to her recovery. We also have a tremendous amount to learn about how these mechanisms may affect the ME/CFS population as a whole. What’s clear now is that connective tissue damage due to viral infection or MCAS may be causing brainstem compression in ME/CFS.
We must keep an open, inquisitive mind as we move forward. We must learn as much as we can, piecing these puzzle pieces together. We must support one another in our journeys to seek answers for our own personal health. And we must not attack one another over disagreements about this topic.
Hopefully more and more answers will become available in the near future. Let’s hope that a clearer path of what PwME should be doing in response to this possible mechanism behind ME/CFS becomes apparent soon.
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