I can hardly believe it myself. My ME is in remission. Jen Brea
She ditched her wheelchair seven weeks ago. Her POTS disappeared in March. The sensitivities to sound, light, vibration and touch are gone. So is the muscle twitching, the air hunger, the restless legs, the brain fog, the short-term memory issues and the flu-like symptoms, For the first time in eight years, she’s walking for exercise and, well, for the thrill and joy of walking. She’s lifting light weights for 30-90 minutes and getting stronger, not weaker. She recently did an hour of water aerobics. She knew her PEM was gone immediately after the CCI/AAI surgery.
Her spine is still healing, but it seems it’s just a matter of time before Jen Brea’s ME/CFS is totally gone.
All it took was a series of spinal surgeries done over several weeks about six months ago. That sounds like a lot and it is – neither craniocervical instability (CCI) and tethered cord syndrome are easy to diagnose and are even more difficult to get treated – but her rapid recovery after 8 years of moderate to severe illness is amazing. She’s been in a wheelchair almost her entire time with this disease.
Just six months ago, following a thyroid surgery which exacerbated her then undiagnosed case of CCI, Jen Brea was arguably at her lowest point ever. Besides all her ME symptoms, she was having trouble breathing, had flaccid limbs, numb, painful and weak legs, and was experiencing difficulty speaking and thinking.
That all rather quickly disappeared.
Recovering / Recovery
We know Jen Brea and her husband’s story on an intimate level through Unrest. Jen may be the only person some people feel they know with ME/CFS. She doesn’t appear to have ME/CFS anymore, though, and in six months, she may be completely healthy.
We’re complex beings and even a remarkable story like hers can bring up a mix of emotions. Happiness that someone who has been so ill may no longer be suffering. Hope that it could happen to us. There’s a potential dark side as well which Jen Brea alludes to – the survivor’s guilt for her of getting better while others continue to suffer – and possibly a feeling of getting left behind by those who haven’t recovered.
The Recovery/Recovering Stories section of Health Rising often triggers differing emotions – some people love them, others hate them. It’s not hard to see how someone else’s recovery story could trigger some issues. The sick raise their heads to be counted again. Conversations, once vanquished, about the unfairness of it – a tunnel down which no cheese exists – show up again. The problem is not someone becoming well but the shadow that recovery casts on our current situation.
I’m luckily rather immune to that. After 40 years of ME/CFS, I can hardly remember the healthy Cort. He’s not a problem anymore. Besides, if I could pick one person to get well – one person who, if healthy, could advance our cause the most – it would undoubtedly be Jen Brea. Her new health – she says she will stay involved – is a gift not just to her but to all of us.
The story of remission makes it even more clear to me than ever that we must fight for research to better understand the mechanisms underlying all of our cases.
I will never forget the experiences that I have gone through over the last eight years of illness. With my improved health, I will continue to fight alongside each of you for equality, dignity and better care; to challenge stigma and advocate for research dollars and medical education. I will not give up. I am in this fight until every person living with ME, no matter the cause, has access to diagnosis and care. Jen Brea
The Craniocervical Instability Subset in ME/CFS – Just How Big is It (and How Big Do We Want it to Be?)
Jen Brea makes two people with severe, apparently classic cases of “ME/CFS” who have recovered or are recovering after being treated for craniocervical instability (and in Jen’s case, tethered cord syndrome). Two more people on PR, @mattie and @StarChild56 have recently had fusion surgeries to correct CCI. Another’s surgery is on tap and one was recently done.
Jeff just interviewed Mattie three months post surgery. Mattie, a web developer in the Netherlands, had a gradual onset of ME/CFS in 2010 and slowly declined over time. He didn’t consider himself a candidate for the surgery but was living near a center in Germany which could do the tests. To his surprise, he met the criteria. Three months post-surgery he’s nowhere near healthy, but he’s no longer mostly bedridden, POTS, dysautonomia, headaches and neck pain have disappeared, and he has more energy. The interview includes a particularly good discussion of the doctor and the decision to have the surgery. Jeff will interview Mattie again in a couple of months.
Find out more about Jeff’s story
The fact that 20 people on the Phoenix Rising Forums have been diagnosed with craniocervical instability/atlantoaxial instability (CCI/AAI) over the past 8 months or so is remarkable, and suggests that the condition may not be as uncommon as one might think.
- Check out the Tracking CCI/AAI thread on Phoenix RIsing.
Difficult Diagnosis and Treatment
There’s no doubt this is not the easy way out for ME/CFS. If I could choose a way to recover – which I obviously can’t – neurosurgery would be one of the last options on my list. I think I would rather swallow a toxic chemotherapy drug than have a neurosurgeon fuse my head to the top of my spinal column. That procedure sounds about as spine-tinglingly scary as anything I can imagine. (Brain surgery would probably be worse.) The fact that only four neurosurgeons in the world can be trusted to do this procedure doesn’t help.
Among the first thoughts I had upon learning of Jen’s recovery was that if this is what it takes – a rare diagnosis and neurosurgery – if that’s what it really takes to recover, I don’t know that that’s ever going to happen.
After some reflection, I don’t think it’s as bad as that. Jeff and Jen and two other people have found a way and a remarkable 20 people on Phoenix RIsing have gotten a diagnosis. While getting a diagnosis isn’t a piece of cake, it can and is being done. Be sure to check out Jeff’s recommendations on his website. Plus, other less invasive treatment options are available (see below).
Focus on the Spine
Jeff and Jen’s stories do bring a new focus to the spine and brainstem. Hip, on the Phoenix Rising Forums, pointed to five structural conditions which can cause brain stem or spinal cord compression – and ME/CFS-like symptoms:
- cervical spinal stenosis — spinal canal becomes too narrow, which can put pressure on the nerves
- syringomyelia — fluid-filled cyst in the spinal cord which compresses the spinal nerves
- Chiari malformation — where brain tissue is pushed into the spinal canal due to a skull which is too small
- tethered cord — where spinal cord is “stuck” to a structure within the spine such as scar tissue
- craniocervical instability — instability of head & neck bones compressing the brain stem or upper spinal cord.
Other spinal conditions that could cause or contribute to ME/CFS/FM/POTS include cerebral spinal fluid leaks and intracranial hypertension.
Even if you don’t have CCI/AAI, the search for it may help uncover other problems. One person on the Phoenix Rising forums reported that she didn’t have CCI but that the search for it turned up “severe stenosis, edema, compression fractures, and other issues causing my spinal cord to be affected” and that treatments for those conditions were helping.
Still, I’m hoping that: a) CCI/AAI is not a common diagnosis; or b) if it is, that non-surgical treatments can be as helpful as surgery. What I’m really hoping, though, is that Jeff and Jen’s stories are pointing to a problem area that can be helped with other means.
Focus on the Brainstem
Jen and Jeff’s experiences are shining a bright light on a potentially very important, and until recently, little explored area of the brain – the brainstem. Structural issues aren’t the only way to potentially tweak the brainstem in ME/CFS; inflammation, infection or autoimmunity would probably do quite nicely, and some evidence suggests at least one of these may be happening in ME/CFS. Each of these could trigger a different (and less invasive) treatment approach.
Van Elzakker pointed to four ways the brainstem may be involved in ME/CFS:
- via the dysregulation of immune signals traveling from the vagus nerve to the brain
- via activation of the many mast cells found in it
- via problems with its regulation of the autonomic nervous system
- via a stunting of the anti-inflammatory response.
Some evidence directly implicates the brainstem in ME/CFS. Reduced brainstem grey matter volume suggests that the neurons in the brainstem may have been damaged. The fact that the damage correlated with autonomic nervous system problems suggested brainstem problems could be affecting exercise, sleep, the gut and cognition. A communication breakdown from the brainstem nuclei to other nuclei in the brain suggested brainstem problems could even be contributing to the motor cortex, i.e. movement problems in ME/CFS. Barnden proposed problems in the brainstem could be inhibiting the flow of signals from the motor cortex in the brain to the muscles.
The Japanese have echoed that general idea. A 2003 study suggested that reduced motor cortex output was reducing muscle recruitment in ME/CFS and causing fatigue. Given the recent brainstem findings, though, stopping at the motor cortex would seem foolhardy. Given the extreme disability sometimes found in ME/CFS, it’s possible that two of the crucial brain organs involved in movement – the motor cortex and the brainstem – may have both taken a hit.
Studying the brainstem requires special techniques not usually used in brain imaging. Both VanElzakker and Barnden are employing those techniques as they continue their brainstem studies in ME/CFS.
HIP on the Phoenix Rising Forums suggested an intriguing pathogen connection. Enteroviruses (the first viruses associated with ME) produce enzymes called matrix metalloproteinases (MMPs) that destroy connective tissue proteins like collagen, elastin and gelatin. It’s possible that an enteroviral infection could be causing the ligament laxity issues in CCI.
Jeff and Jen’s Atypically Typical Recovery
The only things we really know about recovery is that: a) it’s not common; and b) it occurs in a variety of ways. When it occurs, it often occurs using nontraditional approaches found teetering on the skinny branches of the medical system. Just yesterday someone reported that getting treated for pyroluria – a condition many doctors don’t know about or believe in – made a huge difference.
I was recently diagnosed with Pyroluria. I had a urine count of 27.5 so I was severe. I am also copper zinc imbalanced. I am one of those rare people that the illness was shutting down physically and crippling me due to brain inflammation. I had constant vertigo, extreme light and sound sensitivity, sensory processing disorder problems that were so uncomfortable I would beg god to let me die. My body aches and couldn’t turn my head without severe symptoms. These are not symptoms that are easy to fake….. The saddest thing is how the healthcare system didn’t help at all. I was incapable of seeing, walking straight, or talking right some days but yea… not serious enough. I tried so hard to get help and they didn’t seem to care. So I was forced to an FMT; my last resort. Once diagnosed with severe pyroluria I started supplementation and had very quick results. I immediately had changed in functioning and energy. I can work now.
Finding an unusual treatment that works is fairly typical in people who recover. While there are certainly cases of gradual recoveries using supplements, pacing, and mind/body practices, many of the recovery stories involve unusual, out-of-the-box treatments.
Jeff and Jen Brea are leading examples. Not only is the CCI/AAI surgery they had unusual but they were unusual CCI/AAI patients as well. Both were classic ME patients with all that implies (post-exertional malaise (PEM), viral infections, MCAS, POTS, sensory sensitivities). Jen’s doctor told her he didn’t know how the surgery would go for her because he’d never had a patient like her. Whatever kind of CCI/AAI she had, it was different from what he’d seen before.
So it goes for many people who’ve recovered. Ken Anbender recovered from 26 years of hell using the Pridgen Protocol. After spending over $200K on more traditional treatments, mold avoidance did it for Joey. Valcyte did the trick for Kate and her 25 year struggle with ME/CFS. Sinus surgery proved the cure for Diane. The winner for one recent onset but severely ill patient was desmopressin – something that doesn’t work that well for most. It took a particularly astute practitioner to diagnose the heavy metal poisoning, that put one severely ill patient on the road to recovery.
Another remarkable thing is how sick some people can get and still recover. Jeff, Mike Dessin, and the heavy metal poisoning patient were more similar to Whitney Dafoe than your run of the mill (but still incredibly limited) ME/CFS patient – and yet they fully recovered. Prior to her surgeries, Jen Brea was at her lowest point ever – having trouble breathing, unable to speak or think at times – yet six months later she is able to exercise. When given the chance, the body can come back from an amazingly debilitated state.
For me, the recovery stories provide hope and are a sign not to give up, to stay curious, to keep reading and asking questions. Medicine, as Jen Brea noted in her recent blog, is ever-evolving. What was cloudy yesterday may become clear today. It wasn’t that long ago that CCI/AAI surgery wasn’t even considered for Ehlers Danlos Syndrome – it was the province solely of whiplash and trauma patients.
We should also remember how vitally important sharing our stories – both our ME/CFS stories and our recovery stories is. Jeff’s publication of his story may have saved Jen’s life. It’s now enabled 20 people to be diagnosed with CCI/AAI and three are undergoing surgeries. Please share your improvement or recovery story (and if Health Rising asks you to respond to a questionnaire on how you improved, please fill it out!)
Our goal has to be to assist the medical system in its evolution, to keep the hope alive, to keep sharing, to have the courage to keep knocking on closed doors, and eventually getting the light to shine through.
Getting Diagnosed and Treated for CCI/AAI – a Review
The Zebra Network and other websites report three central symptoms of craniocervical instability (CCI):
- “Heavy” headaches (feeling like the head is too heavy for the neck) and a bobble-head feeling.
- Pressure headaches generated by things like yawning, laughing, crying, coughing, sneezing or straining.
- Symptoms of autonomic nervous system functioning problems such as tachycardia (rapid heartbeat, heat intolerance, problems standing (orthostatic intolerance), gut motility problems, thirst and chronic fatigue.
Other symptoms can include neck pain, central or mixed sleep apnea, facial pain or numbness, balance and coordination problems and vertigo, dizziness, fainting, vision issues, difficulty swallowing, choking, tinnitus, nausea, vomiting, paralysis, downward nystagmus (irregular eye movements).
Note that physical discomfort in head/neck area is not required! For the vast majority of their time ill neither Jeff or Jen experienced the kind of head or neck symptoms one would have thought would have accompanied CCI/AAI. Neither could have pointed to their head/neck area as a likely cause of their illness. Nor did Mattie – the third person with ME/CFS to have recently undergone CCI/AAI surgery. After reading Jeff’s account Mattie thought he didn’t have CCI/AAI – but got it checked out anyway – and found, to his surprise, that he did.
Tip! – a physiatrist (pain doctor) reported that physiatrists are trained to look for CCI/AAI. It might be best to look for a reputable pain clinic in the area, call them up and ask them about it. They’re probably a lot easier to get a hold of than a neurosurgeon.
Both Jen and Jeff’s stories highlight the need for a really good diagnostician – someone who is plugged into the latest ME/CFS news. Jen has said she will tell her story, and Jeff was lucky enough to find Dr. Kaufman. His partner, Dr. Chedda, now regularly checks for CCI/AAI.
Two methods can provide an indication that CCI/AAI might be present.
Cervical Traction: Jeff reported that a trained physical therapist who applies cervical traction is the gold standard for home diagnostic efforts. Dr. Bolognese told one potential patient on Phoenix Rising to “Try cervical traction with your local physical therapist. If you obtain dramatically positive results with the traction, then email me back about your feedback, and we will select you for a visit or a videoconference.”
Merck reported that symptom improvement during a procedure called Invasive Cervical Traction (ICT) where one’s head is pulled upward by a pulley system can help diagnose CCI/AAI.
Lacking those things, a doctor can simply pull the patient’s head up off the spine in the doctor’s office, and see if that helps! (Dr. Chedda reported that she routinely does this.)
Philadelphia or Aspen Vista Collar: Both Jeff and Jen were given valuable information when a Philadelphia Collar or an Aspen Vista collar helped their symptoms, at least for a time. The collars do not always improve symptoms, however.
Brain scans provide the final determination.
On his Mechanicalbasis website, Jeff provides crucial advice for getting tested and fully diagnosed – including how to get your scans into the right hands. Most neurosurgeons aren’t trained to recognize craniocervical instability, and finding an imaging facility that does the right kind of scans can take time. Different neurosurgeons will employ different scans.
There are upright MRIs with flexion, extension, and rotational views, or supine CT scans with flexion, extension, and rotational views or 3Tesla supine MRI’s. (A patient of Dr. Kaufman’s reports that the extremely strong 3Tesla MRI’s may be the best and are more readily available. Check out the difference between the 3T and 1.5T machines).
Chiaribridges reported that “the ideal tests to diagnose CCI and AAI are an upright MRI with flexion and extension (bending one’s head forward and backward as far as one can) and a 3D CT with rotational views, respectively. Ventral brainstem compression is not always seen in traditional supine MR imaging but it’s usually very evident on dynamic upright imaging which has the patient flex and extend their neck. Upright scans are harder to find and are not necessary if good MRI machines are available.”
On Phoenix Rising, Jeff reported that the dynamic imaging should also check for Chiari Malformation (which can cause similar issues), craniocervical instability, and tethered cord syndrome (which Health Rising will cover later).
- Comorbid Diseases and ME/CFS/FM Mimics – check out the growing list here.
Treatment – Nonsurgical Procedures
Surgery was the only option for Jeff and Jen, but it’s not for everyone. Dr. Bolognese reported that a treatment plan for craniocervical instability typically begins with things like neck bracing, activity limitation, physical therapy (including isometrics, core strengthening and cardio), as well as pain management.
One person diagnosed with CCI reported on the Phoenix Rising forums that manual traction (having someone pull on one’s head while lying down) can help, and prescription drugs have resulted in major improvements. Some people with CCI also benefit from home neck traction devices. Sleeping in a bed with the feet elevated and the head down in the Trendelenburg position can help.
Some people with ligament laxity have improved using the Cusack Protocol. Regenex is another procedure mentioned. In it, bone marrow concentrate (BMC) containing a stem cell fraction (same-day stem cell procedure) is injected directly into the lax ligaments.
Prolotherapy? – I don’t know if prolotherapy is an option for treating CCI/AAI, but it is used to treat ligament laxity which is found in CCI/AAI and a number of neck and spine issues. Prolotherapy involves injecting an irritating glucose/dextrose or other solution at painful ligament attachment sites to produce a mild inflammatory response which, hopefully, initiates a healing cascade which then increases the strength and elasticity of connective tissues. It’s been used to treat whiplash for years and has been used in EDS but is not well studied.
Treatment – Surgery
Surgery is considered the last option – a good thing, as Jeff lists only four neurosurgeons (3 in the U.S. and one in Europe) proficient in this kind of surgery. Wait times to get the surgery done can obviously be long. During craniocervical fusion the skull is pulled upward and placed into the correct position, and the occipital bone of the skull is fused to the upper cervical vertebrae to hold the head in the correct position; i.e. off of the brainstem. A halo or cervical brace is worn while the bones completely fuse together.
Health Rising’s ME/CFS/FM Spinal Series + Recovery Stories
- Could Craniocervical Instability Be Causing ME/CFS, Fibromyalgia & POTS? The Spinal Series – Pt. I
- Jennifer Brea’s Amazing ME/CFS Recovering Story: the Spinal Series – Pt. II
- Spinal Stenosis, Chronic Fatigue Syndrome (ME/CFS) and Fibromyalgia: The Spinal Series #2
- Recovery/Recovering Stories Section
Jeff’s Story and Website
- The MEchanical Basis of ME/CFS. – includes his story and resources on diagnosis and neurosurgeons
- An interview with Mattie – three months post CCI/AAI surgery
Jen Brea’s Story (Most recent to oldest)
- Health update #3: My ME is in remission
- Health update #2: My POTS is in remission (My POTS has been in remission for 21 consecutive days! — an outcome of my craniocervical fusion surgery. More on this and why I think we need to be looking at the brainstem.
- Do you have ICC-ME and other FAQs (The previous post somehow had some folks thinking I have hEDS and that I don’t have ME, even though all the previous posts have conveyed the opposite. I wrote this for all avoidance of doubt!)
- Are a subset of us members of a lost tribe? (On the possible connections between EDS and ME and the questions I wish researchers were asking.)
- Health update #1 (Feb 21) (Healing takes time)
- A new diagnosis to add to the list (I stopped breathing and was diagnosed with craniocervical instability)
CCI/ AAI Threads on the Phoenix Rising Forums
- Have you ruled out Chiari or Craniocervical Instability (CCI) as a cause of your CFS
- 20 patients now found positive for CCI / AAI, there must be many more…
- Tracking CCI / AAI MRI & Treatment outcomes
- Regenexx for craniocervical instability: my experience
- Update: CCI Surgery
- Pursuing CCI/AAI, have questions, former high jumper & figure skater
- Jeff anyone who have consulted with Dr Bolonesse … help confused!
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