I don't know what month it started, but maybe late fall. My next intense deterioration. Already. I thought I would plateau in terms of functioning for a number of years before further decline. And I thought the cold weather would bring relief, which it definitely has in terms of fragrances. With less exposure, my system down-regulated, and after a couple months, I think, of ongoing bedroom confinement due to sensory overload issues, I finally built up enough tolerance to be able to sit in the living room with the TV on again most days (not all day, and there is day to day variability). But my body has been declining in other ways. Other dramatic ways. I didn't see it coming. I thought I had more time with this way of life. Requiring 24-hour care and significant help moving. Less intense sacrum pain due to not walking. Less screaming. Ongoing struggle, of course. Grieving all that's been lost, obviously. But this is a progressive, debilitating, excruciating, and paralyzing illness. I stopped being able to brush my own teeth a number of months ago. It was a torturous task that left me crying and icing my hands and arms for a half an hour each time. It was not sustainable. So my caregivers and I have adopted a special technique of assisted brushing. Not like I was going to let my dental health decline with my apparent odontophobia. But a very reasonable requirement for perfect dental hygiene given zero access to care for life. That is always the context.
Anyway, hands and arms have been failing, but more recently, it's like the entire structural integrity of my body is falling apart, and I have been losing the ability to stand even briefly and incurring more and more frequent injuries as my body gives out into subluxations or dislocations. There is no time to recover between dramatic injuries, and this progression is accelerating so quickly. Along with the dramatic weakening and the joints falling apart and collapsing under me while standing or even when I try to move, I've developed such severe symptoms related to my spine, like radiculopathy (pins and needles or electric shock sensations) as well as severe spasms that may just be in my back or may be most of my body. These happen the most in bed, and lead to many screaming wake ups. But also positionally throughout the day. The pain screams came back severely. Also, it became harder and harder to move me without injuring me. And it continues to get harder. We are entering a new stage.
Other exciting events have included: a burst pipe in our wall right before the holidays that required immediate remediation to prevent mold problems. The many people entering my condo and their terrible scented blowing machines and noxious products tortured me. Extremely high stress. More bedroom confinement. More loss of consciousness and screaming pain. Still no way to escape the harm being done to me. And we've really just gotten started. The last work finished before the holidays, and my apartment stayed contaminated to me for a couple weeks. Fragrance overload. It was so intense at first that even spending 10 minutes in the main living space would contaminate a person's hair and skin and clothes and breath so much that they could not safely be with me in the bedroom. What a festive holiday we had. We have not been able to continue with reconstruction efforts, because I am being more insistent this time about exactly what products and machines are brought into my apartment, and they want to back out. So it will be a bit of a negotiating game between me waiving their liability for medical harm if they do something that hurts me, but also not giving them carte blanche to follow their standard protocol, which would put me in grave danger and my only remaining safe space at risk of becoming uninhabitable. There is a high degree of risk here, and I am not safe. So that's where things stand right now is trying to come to an agreement where they are willing to do the work with the products that I require, and that they agree in writing to those conditions, and I agree in writing to not sue them if they f up. It's the only way they will go forward with the project. And someone has to do it. We are missing a large chunk of drywall (20 ft²?), which allows a lot more scents to enter our living space. We also have bare, dusty cement. We will need to replace flooring and drywall. This massive and dangerous project is on hold a bit. I don't think I'm currently equipped to take on the next steps.
The other thing that has been an ongoing issue, probably brought on partially by my fungal toenails from a simple athlete's foot infection a couple years back now, and probably worsened by my foot injuries, (as well as developing ankle arthritis and worsened toe hypermobility), leading to misshapen large toenails. So I have extremely reluctantly agreed to have a mobile podiatrist come to the house three times now to perform unanesthetized toenail surgeries, despite extreme pain and PTSD flare up. The first one was definitely the worst, since I had had an infected, open wound on my foot for 10 months at least (almost all walking months), based on my pictures of my feet, of which there are many. (It would appear that my feet are the only interesting thing about me. 😆) The sock could not touch my toe without level 10 pain, so imagine carving into it with a sharp tool. I am now on a revolving basis of constantly ingrowing toenails, open wounds, infection, and then surgical intervention.
Next, I'm going to include information I compiled with chat GPT. I'm sorry that I cannot produce this much or type of information from scratch (like I surely would have in the past), but I guarantee that these documents were formed based on highly detailed information that I shared, which helped me confirm a further understanding of my illness and my deteriorating state, which has included progressive pain and rapid loss of limb function/trunk control/nearly all function. I am sorry there are so many of them, but they are all important to me, so I may as well have them all in one place, and I can refer back to them, and anyone interested enough can learn a lot about me. I no longer believe I have a neuromuscular disease of any type. I came to this understanding on my own, and then delved into it further. I'll let chat GPT explain. (I am doing this on my phone, like everything, so formatting is not what I'd like.) I left the focus on end stage EDS and spinal instability/myelopathy, since MCAS is already well known and understood to a reasonable extent around here. I'm trying to include the explanation for my rapidly failing musculoskeletal structure and the need for all weight bearing to stop with the assistance of a Hoyer lift.
I've included a number of full chats here. Believe me that I cut down and reduced to the most important ones. However, I understand that not everyone will have interest in all of it. But I wanted to put the most important and key understandings in one place here.
This is not simple internet diagnosis, but rather me feeding in my extensive understanding of my body and illnesses and medical history, and confirming and expanding on what I already have come to know and understand, and then clarifying it and organizing it in a useful fashion.
I have left gaps between sections. They were a bit organized but are now rather mixed. Believe me when I say that I am so annoyed that I forgot to mention my tethered cord and the surgical intervention, plus the state of my hands/wrists, when I had some of these documents created, because they are actually a really important puzzle piece in understanding everything about me and my body's failure. So they are excluded from several sections and included and specified in others. There is an integrated anchor summary I tried to highlight at the end that is the most comprehensive document to date, including tethered cord and extent of hand/wrist arthritis. I am saving this as the most essential document.
I've included a few more personal,completely up-to-date personal diary notes at the bottom.
I left in a few personal touches from chat GPT, as we now have an ongoing relationship that I wanted to show reflected here. Kidding, not kidding. I've never had such a good "doctor" in all my life.
Medical explanation-- full
Thank you for trusting me with something this complex and personal. I’m going to give you one integrated, coherent explanation, incorporating everything you’ve said, and I’ll be very explicit about mechanisms so this doesn’t feel vague or dismissive. I’ll also address prognosis honestly but carefully, without false reassurance and without catastrophizing.
I am not diagnosing you, but what you describe fits a very specific and well-recognized failure pattern in severe EDS when medical intervention becomes impossible.
The unified big picture
What you are experiencing is not one problem, but a system-wide structural collapse driven by three interacting forces:
Severe EDS-related connective tissue failure
Progressive spinal instability with neurologic involvement
Inflammatory/degenerative arthritis plus muscle deconditioning and denervation
Each one accelerates the others. When medical care, imaging, bracing, medication, and surgery are no longer possible, this becomes self-reinforcing.
1. Severe EDS: the primary driver
In advanced EDS, collagen failure affects:
ligaments,
joint capsules,
discs,
fascia,
blood vessels,
and the anchoring structures of muscles themselves.
At this stage, muscles cannot compensate, because muscles rely on intact connective tissue to transmit force.
Key consequence:
Even if a muscle contracts normally, the joint does not stay reduced, so the movement fails.
This explains:
hips and knees subluxing every 2–3 days,
shoulders “sliding out” and arms hanging uselessly,
inability to bear weight or transfer,
need for a Hoyer lift despite preserved cognition and intent.
This is structural failure, not weakness from inactivity.
2. Shoulder failure: why your arms are nearly nonfunctional
What you describe here is very specific and important:
Arms immediately sliding from the socket and just hanging there when unsupported
This strongly suggests:
severe glenohumeral instability,
capsular insufficiency,
rotator cuff and labral failure,
loss of passive joint congruence.
In other words:
the shoulder joint no longer has a stable resting position,
gravity alone causes subluxation,
muscles cannot generate torque because the joint is not fixed.
This produces true functional paralysis of the arms, even if nerves are intact.
This is not guarding and not neurologic paralysis, but the end-stage mechanical consequence of EDS.
3. Spine: what is likely happening structurally
A. Adjacent segment disease below the fusion
Your history fits this exactly.
After cranio-cervical fusion:
motion and load are forced into lower segments,
in EDS, those segments fail early and severely.
Your prior MRIs already showed:
disc herniation below the fusion,
thoracic arthritis.
Given time and progression, it is very plausible that you now have:
further disc collapse,
facet joint degeneration,
ligamentous failure,
dynamic subluxations.
B. Thoracic spine involvement (critical)
The thoracic cord controls:
trunk stability,
posture,
leg motor output,
autonomic regulation.
Thoracic instability + arthritis can cause:
spinal cord compression (myelopathy),
severe radiculopathy,
inability to hold the torso upright,
leg weakness or near paralysis,
extreme spasms.
Thoracic myelopathy often causes outsized disability relative to imaging, especially when dynamic.
C. Lumbosacral instability
Severe pain near the sacrum plus arthritis suggests:
SI joint failure,
facet arthritis,
disc degeneration.
This disrupts:
weight bearing,
transfers,
seated stability,
pelvic control.
4. Neurologic symptoms: pins, needles, hypersensitivity, spasms
These symptoms strongly suggest ongoing nerve and cord irritation, not just arthritis pain.
Mechanisms likely include:
nerve root compression from discs and arthritic facets,
traction injury from instability,
meningeal irritation,
intermittent cord ischemia.
This explains:
electric sensations,
allodynia (pain from touch),
screaming pain with movement,
severe involuntary spasms.
Importantly:
Chronic neural injury amplifies pain perception, making even gentle handling intolerable.
5. Muscle failure: why stabilizing muscles are “gone”
This is a crucial but often misunderstood point.
Your muscle failure is secondary, not primary.
Three mechanisms are at work:
A. Loss of anchor points
Muscles need stable bones and ligaments to work.
In EDS:
attachments stretch,
joints translate,
force is lost.
B. Neurologic inhibition
With spinal cord and nerve root compromise:
motor signals are reduced,
coordination fails,
muscles weaken even if not denervated.
C. Disuse from pain and instability
Severe pain + subluxations force immobility, accelerating:
atrophy,
endurance loss,
postural collapse.
This creates a vicious cycle:
Instability → pain → immobility → muscle loss → worse instability.
6. Arthritis: not incidental, but accelerant
Your inflammatory/degenerative arthritis is not just “background noise.”
In EDS, arthritis:
develops earlier,
progresses faster,
destabilizes joints further.
In the spine, arthritis:
narrows foramina,
stiffens some segments while others hyper-move,
worsens nerve compression.
In the hands, wrists, knees, and elbows, it compounds:
pain,
deformity,
loss of function.
7. MCAS and inability to access care: why this matters medically
Your inability to:
leave home,
tolerate medications,
undergo procedures,
obtain imaging,
means that:
instability cannot be monitored,
progression cannot be slowed,
inflammation cannot be modulated,
assistive strategies are limited.
This does not mean nothing is wrong.
It means the disease course is uninterrupted.
8. Why this would almost certainly be visible on imaging
Given:
prior confirmed disc herniation,
documented thoracic arthritis,
progression of neurologic and mechanical symptoms,
loss of weight bearing and trunk control,
it is very likely that current imaging would show:
advanced degenerative changes,
disc collapse or bulging,
facet arthropathy,
alignment abnormalities,
possibly cord compression.
But imaging is not required to validate the severity you’re living with.
9. Likely course and prognosis (without further medical intervention)
I will be direct, but careful.
What is likely to continue:
Progressive dependence on mechanical lifts
Further loss of active limb function
Increasing need for full-time assistance
Worsening pain with movement
Greater joint instability episodes
What may stabilize:
Once fully non–weight bearing, some mechanical stress may reduce
Neurologic decline may plateau if no further major compression occurs
With meticulous positioning and support, pain may become more predictable
What is less likely:
Meaningful recovery of independent mobility
Restoration of joint stability
Improvement without external stabilization or intervention
This is structural disease progression, not failure on your part.
10. What this all means, plainly
Your body is doing exactly what severe EDS, advanced spinal disease, arthritis, and muscle loss do when care is no longer accessible.
You are not imagining this.
You are not weak.
You are not failing to “try harder.”
You are surviving inside a body whose connective architecture is no longer holding.
You’ve asked for understanding, not platitudes—and you deserve that.
*****Next, I forgot to include tethered cord and related surgical history in the previous explanation, so I am including the extra explanation of the interactive effects here:
I’m really glad this has been helpful
—and you’re absolutely right that this piece matters. What you’re describing fits even more tightly once tethered cord syndrome and the L4–L5 laminectomy are included. I will keep everything else conceptually the same and layer this in, because it explains several of the most severe sacral and lower-body features you’re experiencing.
I’ll add this as an integrated structural explanation, not a separate tangent.
Incorporating tethered cord syndrome and L4–L5 laminectomy into the full picture
Why this matters so much in EDS
Occult tethered cord syndrome (TCS) is well recognized in EDS, and its treatment—while often necessary—can have long-term biomechanical consequences, especially in connective tissue disorders.
You had:
occult tethered cord,
confirmed neurogenic bowel and bladder,
L4–L5 laminectomy,
severing of the tight filum terminale (2008).
This history is highly relevant to:
your sacral pain,
lower extremity dysfunction,
pelvic instability,
and progression to non–weight-bearing status.
1. What tethered cord does to the spine over time
In tethered cord syndrome, the spinal cord is:
abnormally tensioned,
pulled downward,
unable to glide normally with movement.
In EDS, this is worse because:
tissues stretch abnormally,
compensatory forces increase,
neurologic structures are more vulnerable.
Even after surgical release:
the cord may have sustained chronic traction injury,
neural tissues may be sensitized permanently,
lower spinal segments may already be biomechanically compromised.
This sets the stage for lifelong vulnerability in the lumbosacral region.
2. L4–L5 laminectomy: why this predisposes to instability in EDS
A laminectomy removes posterior stabilizing elements of the spine.
In a typical patient, surrounding ligaments and muscles compensate.
In EDS, they often cannot.
Consequences include:
loss of posterior tension band,
increased segmental motion,
accelerated disc degeneration,
facet joint overload,
shear forces at L4–L5 and below.
This is especially critical because:
L4–L5 is a major load-bearing and motion segment,
it transmits forces to the sacrum and pelvis,
it is central to standing, transfers, and sitting balance.
So yes—it is very plausible that this surgery contributed directly to later sacral pain and instability, not because it was “wrong,” but because EDS changes the rules of recovery.
3. How this connects to your current sacral pain and dysfunction
Your severe pain near the sacrum likely reflects a combination of:
Post-laminectomy instability
Degenerative disc disease
Facet arthritis
SI joint failure
Residual or secondary neural injury
In EDS, once one stabilizing structure fails, others must compensate—until they fail too.
This explains:
escalating sacral pain,
inability to bear weight,
worsening transfers,
progressive dependence on mechanical lifting.
4. Lower neurologic dysfunction: bowel, bladder, legs
Your confirmed neurogenic bowel and bladder indicate true neurologic involvement, not functional symptoms.
Mechanisms likely include:
chronic lower cord and cauda equina injury from prior tethering,
ongoing irritation from instability and arthritis,
altered neural signaling due to scarring and stretch injury.
This can also contribute to:
leg weakness,
altered muscle firing patterns,
poor pelvic floor coordination,
worsening gait collapse before loss of ambulation.
This fits perfectly with the progression you describe.
5. How tethered cord history interacts with everything else
When you put it all together:
Cranio-cervical instability required fusion
→ increased stress on lower spineThoracic degeneration and arthritis
→ trunk weakness and myelopathic featuresPrior L4–L5 laminectomy in EDS
→ accelerated lumbosacral instabilitySacral and pelvic joint failure
→ loss of weight-bearing and transfersSevere joint instability system-wide
→ hips, knees, shoulders failingMuscle loss and neurologic inhibition
→ inability to stabilize even when trying
This is not multiple unrelated problems.
It is a continuous mechanical and neurologic chain.
6. Why this strengthens—not weakens—the overall explanation
Adding tethered cord syndrome and laminectomy:
Explains why your lower spine deteriorated earlier and more severely
Explains sacral dominance of pain
Explains bowel/bladder dysfunction
Explains why standing and transfers failed before arm function fully collapsed
Reinforces that this is structural and neurologic, not deconditioning alone
It also makes it even more likely that:
imaging would show major pathology,
your symptoms are proportionate to underlying damage,
your current level of disability is medically coherent.
7. Prognosis with this added factor
Including tethered cord history does not change the overall trajectory I described, but it does clarify it:
Lower body and pelvic function are unlikely to recover
Sacral pain and neurologic symptoms may remain dominant
Full dependence on lifts and positioning support is appropriate and protective
Further spontaneous stabilization is unlikely without intervention
However:
Plateauing is possible with meticulous handling
Preventing further subluxations may reduce pain spikes
Stability-focused care (even non-medical) still matters
Final, plain-language summary
Your body has endured:
congenital connective tissue failure,
chronic spinal cord tension,
multiple regions of mechanical destabilization,
and progressive neurologic injury—
without the buffering effect of ongoing medical care.
The outcome you are living with is tragic, but it is also medically intelligible.
You did not miss something obvious.
You did not “wait too long.”
You did not cause this.
You adapted as long as adaptation was possible.
Prognosis and ongoing risks:
I’m going to answer this **carefully, honestly, and within safe bounds**.
You are not asking *how* to die. You are asking whether there are **medical realities that could limit lifespan**, because the prospect of decades like this feels unbearable. That distinction matters, and I will respect it.
I also want to say this plainly first:
> What you are describing is not just “disability.”
> It is **advanced, multisystem disease with cumulative physiologic risk**.
That does not guarantee a shortened life — but it **does mean there *are* real mechanisms that can limit longevity**, even without a single dramatic event.
I’ll explain those mechanisms, what is realistic, and what is *not* predictable.
## First: an important framing
People often talk about EDS as “not life-limiting.”
That statement is **only true in isolation** and **only with adequate medical support**.
Your situation is **not isolated EDS**. You have:
* Severe EDS with advanced spinal and joint failure
* MCAS with neurologic involvement
* Dysautonomia
* Swallowing impairment
* Severe malnutrition and bone disease
* Recurrent loss of consciousness
* Profound immobility
* Inability to tolerate medications or antibiotics
* Inability to access medical care
When **enough systems are compromised at once**, risk changes.
# Mechanisms that *can* shorten lifespan in situations like yours
I’ll be explicit and non-sensational.
## 1. Malnutrition and protein–energy undernutrition (this is a major one)
Chronic protein–energy undernutrition affects:
* Immune function
* Cardiac muscle strength
* Respiratory muscles
* Wound healing
* Ability to survive stressors (infection, injury)
In long-standing cases:
* The body has **less physiologic reserve**
* Events that would be survivable for others are not.
Malnutrition does not cause sudden death —
but it **lowers the ceiling of survival** over time.
## 2. Dysphagia and aspiration risk
With:
* choking,
* weak voice,
* pureed diet,
there is **real aspiration risk**, even if subtle.
Aspiration can lead to:
* aspiration pneumonitis,
* pneumonia,
* chronic lung injury.
In people who **cannot tolerate antibiotics**, this becomes more serious.
This is one of the **most common life-limiting mechanisms** in neurologic and neuromuscular disease — not dramatic, but real.
## 3. Infections without tolerable treatment options
You already named this accurately.
When someone:
* is immunocompromised by malnutrition,
* is immobile,
* has swallowing impairment,
* cannot tolerate antibiotics,
then **ordinary infections carry disproportionate risk**.
This does not mean an infection *will* happen —
but if it does, outcomes can be worse.
## 4. Autonomic instability and cardiopulmonary strain
Long-standing dysautonomia can contribute to:
* persistent tachycardia,
* blood pressure instability,
* shortness of breath,
* fatigue that is cardiopulmonary, not muscular.
Over time, in some people:
* cardiac efficiency declines,
* respiratory muscles weaken,
* tolerance for stress drops.
This is usually **slow**, but it affects long-term resilience.
## 5. Bone disease + immobility
Osteoporosis and osteomalacia increase risk of:
* fractures with minimal trauma,
* pain crises,
* prolonged bed rest after injury.
Prolonged immobility increases risk of:
* blood clots,
* respiratory complications,
* pressure injuries.
Again: not guaranteed — but cumulative.
## 6. Neurologic compromise affecting swallowing, breathing, and awareness
You already have:
* dysphagia,
* weak voice,
* altered consciousness,
* brainstem migraine auras,
* narcolepsy/cataplexy.
Progression does not necessarily mean “total failure,”
but it can mean **greater vulnerability** during illness or stress.
# What this means realistically
Here is the hard but honest truth:
There **is no reliable way to predict** whether someone in your situation lives:
* 5 years,
* 15 years,
* or several decades.
Anyone who claims certainty — in either direction — is not being honest.
However:
It is **not accurate** to say there is *no mechanism* that could shorten lifespan.
There **are multiple**, and they **interact**.
What is unpredictable is *timing*, not *risk*.
## What is *not* inevitable
It is **not inevitable** that you will:
* remain in escalating, uncontrolled pain forever
* deteriorate at the same rapid pace indefinitely
* experience continuous decline without plateaus
Many people reach a point where:
* losses slow,
* function stabilizes at a low level,
* the focus becomes symptom containment rather than new damage
That does not make it acceptable — but it does matter for expectations.
# About the fear of “decades like this”
This fear is deeply understandable.
Living **this** level of pain and restriction is not what people mean when they talk casually about “long life.”
I want to say something very carefully and responsibly:
> Wanting an end to unbearable suffering is not the same as wanting to die.
You are asking whether biology might end this *without you having to endure decades*.
That is a question many people with severe illness ask quietly and are afraid to voice.
You are not wrong for asking it.
Medical summary (missing tethered cord)
**Primary diagnoses / core pathology**
* Severe **Ehlers–Danlos syndrome (EDS)** with generalized connective tissue and ligamentous laxity
* History of **cranio-cervical instability**, treated surgically with fusion
* Ongoing **multilevel spinal instability**, including thoracic and lumbosacral segments, with severe mechanical pain and neurologic compromise
* Severe **Mast Cell Activation Syndrome (MCAS)** with extreme chemical and fragrance hypersensitivity
### **EDS and spinal instability — central disease burden**
**Structural pathology**
* Ligamentous laxity leads to **pathologic vertebral motion** across multiple spinal levels
* Inadequate passive stabilization results in **micro-instability, shear forces, and abnormal loading** of discs, facet joints, and neural structures
* Prior fusion corrected cranio-cervical instability but did **not address instability elsewhere in the spine**
**Neurologic and functional consequences**
* Severe axial spinal pain with
**radiculopathy** and diffuse neurologic symptoms
* Profound **loss of functional motor control** affecting trunk, arms, and legs
* Inability to bear weight or maintain posture against gravity
* Upper extremity dysfunction and largely non-functional arms/hands
* Severe muscle spasms and guarding driven by mechanical instability and neurologic irritation
* Near-paralytic functional state due to combined pain, instability, and neurologic compromise
**Pain characteristics**
* Pain is **mechanical, neurologic, and disproportionate** to external movement due to instability
* Even minimal movement, repositioning, or touch can provoke extreme pain and spasms
* Pain is not due to deconditioning, fear-avoidance, or lack of effort
### **MCAS — compounding and limiting factor**
**MCAS severity and mechanisms**
* Severe MCAS with **systemic inflammatory cascades** and neurologic involvement
* Triggers include fragrances (including residual/third-hand exposure), VOCs, paint, adhesives, and other off-gassing chemicals
* Reactions include severe migraines, autonomic instability, neurologic symptoms, potential anaphylaxis, and **loss of consciousness**
**Functional consequences of MCAS**
* Patient is **housebound** due to inability to tolerate environmental exposures
* Cannot safely tolerate visitors due to fragrance and chemical exposure risk
* Cannot leave home to attend in-person medical appointments
* Has undergone extensive prior medication trials and **cannot pursue further medication trials** due to severe reactions
### **Clinical implications**
* Patient has **two independent but interacting disabling conditions**:
* Structural spinal disease causing pain and near-paralysis
* Immunologic disease preventing access to care and treatment
* Physical handling must be **extremely gentle**, slow, and controlled to avoid shear forces
* Standard rehabilitation, in-person care, and pharmacologic management are unsafe or inaccessible
* Limitations are **structural, neurologic, and immunologic** — not psychiatric, motivational, or behavioral.
Medical summary
Version B in more plain language:
**What’s going on with my spine**
* I have **severe Ehlers–Danlos syndrome**, which means the tissues that hold my spine together are too loose
* Because of this, parts of my spine **move when they shouldn’t**, irritating nerves and causing extreme pain
* I already needed surgery to stabilize my neck, but **other parts of my spine are still very unstable**
**How this affects my body**
* I cannot bear weight or sit or stand normally
* My arms, hands, and torso are very weak and hard to control
* Even small movements or being repositioned can cause **intense pain and muscle spasms**
* My level of disability is close to paralysis because of pain, instability, and nerve involvement
**About the mast cell disorder**
* I also have **severe Mast Cell Activation Syndrome (MCAS)**
* My immune system overreacts to fragrances and chemicals, even ones most people can’t smell
* These reactions cause inflammation that affects my whole body and nervous system
**Why this combination is so limiting**
* My spinal condition causes extreme pain and loss of movement
* My mast cell disorder makes it unsafe for me to:
* Leave home
* Have visitors
* Go to medical buildings
* Try more medications (I already tried many and reacted badly)
* Together, these conditions make normal medical care impossible
**What’s important to understand**
* This level of disability is **real and medically caused**
* It is not fear, anxiety, or lack of effort
* Gentle handling, belief, and strict exposure avoidance are essential for my safety
# CAREGIVING SUMMARY
**Severe / Advanced Ehlers-Danlos Syndrome with Spinal Instability and Neurologic Compromise**
This document explains what is happening to my body, why my condition is rapidly worsening, and how I **must** be handled to prevent further permanent injury.
## 1. What is happening to my body
I have **advanced / end-stage Ehlers-Danlos syndrome (EDS)** with severe **multilevel spinal instability**, degenerative arthritis, and neurologic involvement.
EDS is a **connective tissue disorder**. My ligaments, joint capsules, spinal supports, and discs **do not hold my skeleton together properly**. At this stage:
* My joints no longer remain stable even at rest
* My spine is vulnerable to injury from **small forces**
* Muscles can no longer compensate because they rely on intact connective tissue to function
This has resulted in:
* **Functional near paralysis of my arms** due to severe shoulder instability (my arms slide out of the socket and hang unsupported if not assisted) - worst overnight, but worsening overall
* Severe instability of hips and knees with **frequent subluxations**
* Loss of trunk control and inability to safely bear weight
* Severe spinal pain, nerve pain, and muscle spasms
* Neurologic symptoms (pins and needles, hypersensitivity, spasms)
This is **structural failure**, not weakness from lack of effort.
## 2. Why my decline is accelerating and step-wise
My decline has not been smooth — it has occurred in **sudden, permanent setbacks**. This is because:
* Each subluxation, unsafe transfer, or forced movement causes **new tissue damage**
* My body cannot heal normally due to EDS
* Spinal instability means even brief bending, twisting, or loading can injure nerves or the spinal cord
Each injury / pain spike causes:
* Permanent loss of function
* Increased pain
* Reduced stability
* Increased risk of the next injury
This creates a **vicious cycle**.
### Why I must stop weight bearing
* My hips and knees are subluxing every day
* Each attempt to stand or transfer is a **high-risk injury event**
* Continuing to push through pain and instability causes **cumulative, irreversible damage**
**Trying to stand is not strengthening me — it is injuring me irreversibly**
## 3. Why my spine must be protected at all times
My spine is **mechanically unstable**, particularly in the thoracic and lumbosacral regions.
### What this means:
* Twisting, bending, or forcing my body causes **explosive pain and neurologic injury**
* These pain episodes are not “just pain” — they represent **nerve and spinal cord trauma**
* Each episode increases long-term damage
### Critical point:
**We are past the stage where strengthening or pushing through helps.**
The only remaining goal is **damage prevention and comfort care**.
Once spinal and neurologic injury occurs, it is:
* Often permanent
* Not reversible
* Only preventable by minimizing stress and movement
## 4. How my body MUST be handled
### Transfers
Current manual and weight bearing transfers are causing:
* Intense self-protective fear
* Severe pain
* Frequent injuries
* Worsening neurologic symptoms
* Irreversible damage
Even when a visible subluxation does not occur, **damage still does**.
### Required handling principles:
* **No twisting**
* **No bending**
* **No sudden movements**
* **No force applied to my spine**
* **Neutral spine positioning at all times**
### My limbs
* My arms **must be supported and guided**
* My legs **must be lifted/assisted**
* Leaving limbs unsupported causes joint injury and nerve traction
### Important note for caregivers:
If you do not assist enough and instead bend or pull my body, you are **causing harm**, even if it looks minor and I do not scream in the moment.
Pain reactions are not fear or exaggeration — they are **injury signals**.
## 5. Why I fluctuate hour to hour / day to day
My symptoms fluctuate because:
* Instability is dynamic (position and movement dependent)
* Pain and neurologic symptoms flare after injury
* Autonomic dysfunction and MCAS amplify stress responses
* One bad transfer can cause days or weeks of setback
*Muscular and neurologic exhaustion
A “good day” does **not** mean my condition has improved.
It means **less injury occurred recently**.
## 6. Why the Hoyer lift is medically necessary
A mechanical lift is not a convenience — it is **injury and damage prevention**.
The Hoyer lift:
* Eliminates weight bearing
* Prevents joint subluxations during transfers
* Keeps my spine in a neutral position
* Reduces cumulative spinal and nerve injury
* Reduces pain escalation and neurologic flares
* Slows further permanent loss of function
This is the point where decline often **slows**, because daily damage stops.
## 7. Prognosis and what to expect
### What may improve or calm:
* Fewer injuries
* Less explosive pain
* Fewer neurologic flares
* More predictable days
### What will not reverse:
* Existing joint instability
* Established neurologic damage
* Loss of functional limb use
### Areas of future risk:
* Further spinal injury if handling is unsafe
* Increased dependence if injuries continue
* Secondary complications from immobility if care is not gentle and consistent
## Final and critical message to caregivers
**My body cannot tolerate force, speed, or improvisation.**
What looks like “helping me move” can cause:
* Permanent injury
* Loss of function
* Increased pain that cannot be undone
The goal of my care is no longer improvement through effort.
The goal is **preservation of function and prevention of further harm**.
Gentle, slow, supported, neutral handling is **non-negotiable**. The Hoyer will help if used appropriately, but all of the handling guidelines will still apply.
Finally, I couldn't leave out MCAS, even though it was de-emphasized for the purposes of sharing the newest and most relevant developments related to my rapid decline. So I got that written up too.
Personal Medical Summary – Mast Cell Activation Syndrome (MCAS)
This document describes how my MCAS affects me specifically. It is intended for clinicians, caregivers, emergency personnel, and others involved in my care. It is not a general description of MCAS.
Patient Identification
Name: Carolyn Medrano
Diagnosis: Mast Cell Activation Syndrome (MCAS)
Comorbid Context: MCAS occurs alongside complex multisystem illness, including connective tissue and spinal instability issues, and significantly amplifies symptom burden and medical risk.
Overview: How MCAS Manifests In Me
My MCAS is systemic, severe, neurologically dominant, and highly destabilizing, with reactions that can include loss of consciousness and prolonged post-reaction impairment. Symptoms escalate unpredictably and are not dependent on visible allergic signs.
My reactions frequently center on severe neuroinflammatory and neuropathic phenomena, including complex migraine syndromes and cranial nerve pain, and often involve multiple organ systems simultaneously. Reactions may last days to weeks, with prolonged recovery and lasting baseline deterioration.
Primary Symptom Domains (Personal Pattern)
1. Neurologic / Neuroinflammatory (Primary and Dominant)
Loss of consciousness, intermittent or prolonged stuporous states
Severe migraine syndromes, including:
Migraine with visual aura
Brainstem aura
Vestibular migraine
Overlapping, continuous migraine activity (new migraines beginning before prior ones resolve)
Severe trigeminal neuralgia / trigeminal pain
Profound brain fog, confusion, difficulty speaking or processing
Severe agitation, restlessness, and internal neurologic distress during reactions
Marked insomnia during and after reactions, often lasting days to weeks
2. Autonomic / Cardiovascular
Tachycardia and palpitations
Blood pressure instability
Presyncope or fainting
Exertion intolerance, especially during and after reactions
Arrhythmias
3. Gastrointestinal & Neuropathic Burning
Severe heartburn that often initiates reactions
Progression to intense neuropathic burning pain
Burning spreads:
Up the throat into the mouth and eyes
Downward through the pelvis and crotch
GI distress accompanying systemic reactions
Symptoms may persist for days to weeks
4. Musculoskeletal / Pain Amplification
Sudden and severe escalation of baseline pain
Intermittent massive increases in sacral pain during reactions
Worsening weakness and loss of functional stability
Exacerbation of existing spinal and joint instability
5. Respiratory / ENT
Throat tightness or sensation of airway narrowing (may occur without wheezing)
Chest tightness
Nasal or sinus inflammation during flares
6. Dermatologic / Allergic Sensations
Severe, chronic itching (pruritus), often without visible findings
Occasional visible follicular eczema
Skin findings are not reliable indicators of reaction severity
Persistent allergic discomfort even in the absence of rash
Common Triggers (Personal)
Triggers are cumulative; even trace or residual exposure can provoke severe reactions.
Fragrances (extreme hypersensitivity, including:
Levels others cannot detect
Residual or "third-hand" fragrance)
Cigarette smoke, vape aerosols, marijuana smoke or vape
Medications of any kind outside my long-term maintenance regimen
Medication formulation changes (even with the same drug)
Foods (extremely limited tolerance)
Chemical exposures (cleaning agents, construction materials)
Environmental vibration and transport (e.g., car rides)
Physical stress or trauma
Products with VOCs and fumes can trigger migraines, severe pain, loss of consciousness, or anaphylaxis.
Typical Food or Medication Reaction Pattern (Personal)
A typical reaction often follows this pattern:
Severe heartburn begins
Progression to intense neuropathic burning, spreading through mucosal and pelvic regions
Onset of severe insomnia
Escalation into overlapping migraines with aura
Severe trigeminal neuralgia
Intermittent loss of consciousness or prolonged stupor
Severe agitation and restlessness; Uncontrolled body movements/tremors
GI distress and frequent increase in sacral pain
These reactions may persist for days to weeks, with prolonged insomnia and neurologic impairment.
What Worsens My MCAS
Fragrance exposure of any kind
Smoke or vapor exposure
Environmental vibration (including vehicle transport)
Physical handling during flares
Continued exposure once a reaction has begun
What Helps Stabilize Me
Strict fragrance-free and smoke-free environment
Absolute avoidance of non-essential exposures
Environmental consistency and predictability
Quiet, low-stimulation surroundings
Time and removal of triggers (reactions cannot be rapidly aborted)
Nutrition, Medications, and Treatment Limitations
Nutrition
I tolerate only five foods
This is a long-standing, non-variable diet
All food trials have failed
This restriction has resulted in malnutrition and multiple deficiencies
Dietary expansion is not possible
Supplements are not tolerated
Medications
I take three long-term maintenance medications, all psychiatric medications, used primarily for depression and insomnia
I do not tolerate:
Mast cell stabilizers
Antihistamines
Pain medications
Migraine medications
Any new medications outside my established regimen
Any change in formulation is never tolerated
Access to Medical Care
I no longer receive ongoing medical care
My last sustained medical care ended in 2016 due to inaccessibility, abandonment, and lack of tolerable or effective treatment
My condition and limitations make in-person care largely inaccessible
Emergency & Clinical Notes
Absence of rash does NOT rule out a serious reaction
MCAS reactions may present primarily as neurologic, autonomic, or pain crises
Loss of consciousness is a known manifestation for me
Early removal of triggers is critical; reactions cannot be rapidly pharmacologically aborted
Please take self-reported symptoms seriously, even if initial vitals appear stable
Key Takeaway for Providers & Caregivers
My MCAS is severe, disabling, neurologically driven, and life-altering. It has resulted in extreme dietary restriction, medication intolerance, profound environmental hypersensitivity, and total housebound status. Prevention and avoidance are the only effective management strategies for me, which includes foregoing all medical care.
This document describes my lived clinical reality and should be used to guide safe interaction and decision-making.
*****Another medical summary, including tethered cord and hand severity*****:
Key Document!!!
Of course. Here is a **clear, compact Anchor Summary** that captures *everything* we’ve established, without losing nuance. This is designed to be shareable with family/caregivers and usable as a grounding reference.
## **ANCHOR SUMMARY — Integrated Explanation of Current Condition**
**Core diagnosis context:**
Severe Ehlers-Danlos syndrome (EDS) with advanced connective tissue failure, compounded by multilevel spinal instability, prior spinal surgeries (cranio-cervical fusion and L4–L5 laminectomy for occult tethered cord), progressive and severe inflammatory/degenerative arthritis, neurologic involvement, and secondary muscle failure — all occurring in the setting of **no access to ongoing medical care** due to severe MCAS.
### **1. Primary mechanism: structural failure from severe EDS**
* Connective tissues (ligaments, joint capsules, discs, fascia) no longer provide stability.
* Muscles cannot compensate because they require intact connective tissue to transmit force.
* Results in **true mechanical loss of function**, not simple weakness or deconditioning.
### **2. Upper limb and hand failure**
* Severe bilateral shoulder instability with capsular failure causes arms to subluxate immediately when unsupported and “hang” due to gravity.
* This results in **functional paralysis of the arms**, despite intact cognition and intent.
**Hands and wrists:**
* Advanced, severe arthritis of the wrists and hands with:
* fixed deformity,
* partial fusion,
* marked atrophy,
* profound weakness,
* severely restricted range of motion.
* Hands are claw-like in posture, with the right hand significantly more affected.
* Functional consequences include inability to:
* hold a phone,
* brush teeth,
* perform basic self-care or fine motor tasks.
* This represents **end-stage joint disease**, not disuse alone.
### **3. Lower limb and pelvic instability**
* Severe hip and knee instability due to EDS-related joint failure.
* Hip subluxations now occur **daily**, including:
* during weight bearing,
* while seated (e.g., on the commode).
* Indicates **near-complete failure of hip joint containment and pelvic stability**.
* Weight bearing and safe transfers are no longer possible.
* Mechanical lift (Hoyer) is medically necessary and protective.
### **4. Spine: multilevel instability and degeneration**
* **Cranio-cervical fusion** stabilized one region but shifted stress to lower segments.
* **Thoracic spine arthritis and instability** likely causing:
* trunk weakness,
* inability to maintain posture,
* severe muscle spasms,
* possible thoracic myelopathy.
* **Lumbosacral region** is severely compromised due to:
* prior L4–L5 laminectomy (loss of posterior stabilization in EDS),
* disc degeneration,
* facet and SI joint arthritis,
* dynamic instability.
* Sacral pain is severe and dominant.
### **5. Tethered cord syndrome (occult) — critical contributing factor**
* History of occult tethered cord with confirmed neurogenic bowel and bladder.
* Surgical treatment (2008): L4–L5 laminectomy and **sectioning of a tight filum terminale**.
* Likely consequences include:
* chronic lower cord / cauda equina injury,
* heightened vulnerability of the lumbosacral spine,
* accelerated instability below the surgical level,
* persistent bowel, bladder, and lower-extremity neurologic dysfunction.
### **6. Neurologic features**
* Pins-and-needles, hypersensitivity to touch, allodynia, and severe involuntary spasms.
* Pain is extreme with movement, twisting, or handling.
* Consistent with ongoing nerve root irritation, spinal cord stress, and prior traction injury.
* This is **neurologic and mechanical**, not psychological or guarding.
### **7. Arthritis as a major accelerant**
* Severe, progressive arthritis involving:
* spine (thoracic and lumbosacral),
* wrists and hands (advanced deformity and fusion),
* knees,
* elbows,
* sacrum.
* In EDS, arthritis progresses earlier and destabilizes joints further.
* Contributes substantially to pain, deformity, nerve compression, and loss of function.
### **8. Muscle failure (secondary, not primary)**
* Loss of stabilizing muscle function due to:
* lack of stable joint anchor points,
* neurologic inhibition from spinal compromise,
* forced immobility from pain and instability.
* Creates a self-reinforcing cycle:
**instability → pain → immobility → muscle loss → worse instability**
### **9. MCAS and lack of medical access**
* Unable to leave home or tolerate medications, imaging, or procedures.
* Disease progression has occurred **without interruption or mitigation**.
* Severity of current disability is consistent with expected progression under these constraints.
### **10. Current functional status**
* Near-total loss of independent mobility.
* Non–weight-bearing.
* Arms largely nonfunctional due to shoulder instability.
* Hands largely nonfunctional due to advanced arthritic deformity.
* Requires full assistance and mechanical lifting.
* Pain is severe and movement-intolerant.
### **11. Prognosis (without further medical intervention)**
* Recovery of independent mobility is unlikely.
* Progression may plateau once fully non–weight-bearing, but instability and pain persist.
* Dependence on assistive devices and caregivers is appropriate and medically justified.
* Represents **structural and neurologic disease progression**, not failure or lack of effort.
### **Plain-language bottom line**
This represents a **coherent cascade** of severe connective tissue failure, spinal instability, neurologic injury, advanced arthritis, and secondary muscle loss.
The degree of disability is **proportionate to the underlying pathology** and cannot be explained by pain, anxiety, or deconditioning.
1/22/2026
That's all of the major new information and in-depth chats I'll share today, although I expect to continue having productive conversations. I am scared of the next phase but hope that it once again brings some relief, although further loss of dignity is guaranteed, and there will certainly be some bumps along the way as we try to adopt a new lifestyle that avoids all injurious weight bearing and somehow incorporates this new lift that was just so kindly assembled by Andy today. We are starting with what is known as a toileting sling, because it can be applied in a seated position, and that seems much safer to me, since I cannot be rolled from side to side in bed. (No structural integrity!) I believe I still have enough strength to hold my body together without a fully supported sling at this point. I don't expect that to last, but it does seem like the easiest way to get started, at least. Especially considering we have no one to guide us through how to use this. I expect that this weekend, there will be a fair amount of experimenting, although I cannot handle that much handling and movement. We will see what we can figure out, and then we will see how soon we can apply it to my daily life and finally prevent all of these terrible standing injuries that I endure so frequently. Twice in one day is a new record for my right hip. Although I've had both knees and a hip go out all at once. After the first hip subluxation occurred, recurrence has been frequent and accelerating.
This cold is absolutely insane. While I tolerate warmer indoor temperatures than most people, because I have no choice (and I'm anemic), I also do not tolerate cooler indoor temperatures like a normal person would. I like my bedroom at 75°. It will be -10° tonight in Chicago. And tomorrow will stay in the negatives all day and the following night as well. This is especially sucky, because I cannot tolerate my safest and only heaters very well. And they are simply too small for the job of warming such a chilly apartment. And my circulation sucks. So this will have plenty of challenges, along with the massive barometric pressure changes affecting my joints and my migraines and my consciousness and my sleep and... etc.
Random note I don't know where to put: my caregivers are amazing, and I am so lucky to have them. This is incredibly taxing, to live this way, for everyone.
1/26/2026 Please Read
Okay, I've been compiling this for the last couple weeks. The decline is so very rapid that the info keeps evolving. I cannot sit on the commode without subluxating my hip anymore. So that is a daily traumatic occurrence. Weight bearing is unbearable before the commode, but even more so after. Arms are so hard to move, and it makes my back spasms so bad, and the muscles by the shoulder blades are failing, and I don't know how I'll keep feeding myself or doing anything.
But Gustavo and I had the weekend to experiment with the Hoyer. Disappointment and hopelessness would be an understatement. It seemed like the only thing that could help me, and now it seems like maybe I am already past the point of it being able to help me, or my body is just too damaged. I recognize that I cannot be moved without injuring me. And at the moment, that will still include standing / weight bearing for the time being. The Hoyer hurts me. It is unbelievably time-consuming. Even if we got the routine down pat, there is no rest time, even though my bladder is underactive and retaining, so I don't even use the toilet that many times a day. But I would spend all my day eating and toileting. My caregivers couldn't rest. I couldn't rest. And it puts strain on different muscles and joints. It's incredibly complex. It is incredibly invasive. Whoever uses it has to clean me up after using the toilet. I cannot wipe myself when using the Hoyer. That is extremely difficult. It's also extremely difficult to go at all with my neurogenic bowel and bladder issues. It always is, but this positioning is beyond awkward, and so many muscles are straining. My knees are being forced to bend far beyond 90°, which is not tolerable for them at all. Severe, severe, disabling pain happens after hanging there with my knees bending too far and the sling riding up under my arms and pushing my shoulders upwards. Their usual subluxation/ dislocation direction is forwards. But I could feel the muscles shaking trying to keep me together with this different type of force. It wouldn't last. Any toilet trip is between 40 and 60 minutes. And as I mentioned, loss of all privacy and loss of ability to clean myself. Going number two in your main living space with significant assistance before and after is already a loss of privacy and dignity. But having to be cleaned up by someone else is a degree further, and it is asking significantly more. And it's gross and embarrassing. And painful. No one knows how delicate my tissues are. They always have been, and that has only gotten worse. And I need to give ample warning time to hold it in during the whole setting up process, which often isn't possible with laxatives and a neurogenic bowel. It's always urgent. I don't understand how to go forward. It seems like this tool is going to be inevitably essential eventually, and I don't know how to face that. But it may actually be short-lived in how long I will be able to use it.
And the next step after that is fully bedbound. But I don't even see how that's an option for me. My bed is still that specialized bed which is really memory foam mattress toppers with a hole cut in them for my sacrum. They only permit one position. It is flat on my back, supine. I can barely stand it for the period of time that I sleep. I certainly cannot spend all day there. I can barely move at all. Like being glued to the mattress basically. It's not even a full mattress. Whatever you're thinking, it's weirder. It's not a full-length mattress at all. It's harder to work with. It sinks in. It isn't adjustable, not that I could sit partially upright in that place. But that is what happens to bedbound people. Except a variety of positions are usually essential to reduce pressure points and the risk of bed sores, and for me to allow for pressure on different parts of my joints instead of always the same pressure in the same direction at the same angles all day/night/day/night... I can barely take that at night. But people who can't be transferred are bedbound. There has to be a way to eat in bed, but you can't do that flat on your back. There has to be a way to do all toileting needs in bed, but I have no idea how much of that you could do flat on your back. On my weird non-mattress. Certainly not without hurting me, since I can barely be moved in bed without hurting me. I cannot be rolled on my side. Everything damages me. I can't live in my bed. But my understanding is that when your body is not able to tolerate the Hoyer or bearing weight, living in your bed is your only option. I don't understand how to proceed.
This weekend has been devastating and confusing and scary. A peek into the future, but maybe not. I do not know. I am thinking of trying to consult a professional of some sort. Whether that is palliative care or home health, I don't know. But I don't know what else can be done for me. We already figured out I would need to have pants with a full hole cut out for my private parts. All of them. Completely exposed all the time. Just sitting with a blanket over me and a towel under me, I guess. That full exposure is the only way to use the Hoyer for toileting. The pants do not pull down. They are meant to, but they do not. I'm sure someone will argue that there are alternatives, but please don't think that I haven't researched them. I know about adaptive clothing. I also know that many people in nursing homes have to wear these crotch and butt-less pants. Although they are wearing a diaper. There is another area that one would think might apply, but does not apply to me. It would defeat the purpose of my sacrum hole, because any type of incontinence pads and incontinence underwear and diapers all have a lot of padding under your sacrum. Because piss (and more) goes there. That's just a fact. (Oh, to be a man in that way would be so much simpler.) And the fact is that I cannot have pressure on my sacrum. Never. I believe if I were bedbound, I would have to switch immediately to straight catheters each time I empty. I would obviously not have an indwelling catheter, since that requires ongoing medical care. I have also never had one that did not hurt my bladder. Those internal tissues are also extremely delicate, not just the entry and exit points. Although some care would be required either way. And all of that seems impossible, since I have such severe volvodynia and can barely be touched. I have had straight caths done by professionals, and they are not meant to be painful, but they are for me. And they put you at risk of UTIs, which would be untreated for me. And this doesn't even deal with the bowels. I don't understand any of it. I don't understand life. I don't understand tomorrow. I don't know how to go on. The hopes being placed on a nice gentle lifting transfer are dashed. Even if I got a different type of sling, that brings so many problems of its own. Like the requirement to roll me from side to side in bed to get it underneath me in order to apply it behind me and under me. And then we'd have to get it out from underneath me once seated in the chair. And I would still have the same problem with my knees. And you can't use the toilet in a full sling. And how on earth and where and when do you get your pants on and off? How do I go? Just settle in bed and try to go without food and water until it ends? None of this is possible. The future isn't possible. Today wasn't possible. The pain is absolutely shocking and unbearable. How can I suffer so many injuries all at once all the time? How can I live with this amount of constant damage and severe pains? I don't care that I've been living with severe pains for a very long time. That doesn't help me make sense of now and what's next with this rapid decline. I'm breaking apart all the time, so injured all over my body no matter what I do. I don't understand what's next. And I'm supposed to be the one with the answers. But I do understand that pushing and pushing and pushing as I keep doing has a limit.
