Malignant Hyperthermia and Its Connection to Periodic Paralysis

 Malignant Hyperthermia and Its Connection to Periodic Paralysis

By Susan Q. Knittle-Hunter, Periodic Paralysis Network, Inc.

Malignant Hyperthermia (MH) is a rare but life-threatening reaction to certain anesthetic agents. Many people with Periodic Paralysis (PP) have never been warned about this danger, yet it is essential information for patients, families, and medical professionals — especially because many in the PP community cannot tolerate anesthesia or medications at all.

Below is a clear explanation of what MH is and how it relates to the channelopathy behind Periodic Paralysis.

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What Is Malignant Hyperthermia?

Malignant Hyperthermia is a genetic disorder of skeletal muscle calcium regulation. It is triggered by:

• Certain general anesthetics (such as volatile gases)
• Succinylcholine, a commonly used muscle relaxant

When exposed to a trigger, the person’s muscles release uncontrolled amounts of calcium, causing the muscle cells to go into overdrive. This leads to:

• Rapid rise in body temperature
• Severe muscle rigidity
• Dangerous heart rhythm abnormalities
• Breakdown of muscle tissue (rhabdomyolysis)
• Life-threatening metabolic crisis

MH is considered a medical emergency requiring immediate treatment with dantrolene and intensive care support.

The main genes associated with MH are RYR1 and CACNA1S. Both are ion-channel genes responsible for calcium handling in muscle cells.

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How Is Malignant Hyperthermia Connected to Periodic Paralysis?

Although MH and PP are not the same condition, they share several important connections:

1. Both Are Ion Channel Disorders (Channelopathies)

Periodic Paralysis is caused by defects in ion channels — often sodium, potassium, or calcium channels — including the SCN4A gene that many PP patients carry.
MH involves RYR1 and CACNA1S, which also regulate calcium flow in muscle cells.

Because these systems work together, dysfunction in one can affect the others.

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2. Some PP Mutations Create Overlapping Risk

Certain calcium-channel mutations have been linked to both:

• Hypokalemic PP
• Malignant Hyperthermia susceptibility

This means that people with PP — especially those with calcium-channel variants — may be at higher risk for MH during anesthesia.

Even those with sodium-channel disorders like Andersen-Tawil Syndrome or Hyperkalemic PP have increased anesthetic sensitivity, arrhythmia risk, and metabolic instability, which can resemble or trigger MH-like crises.

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3. Many PP Patients Cannot Tolerate Standard Anesthesia

In her books, Susan describes that PP is not simply “muscle weakness”; it is a mineral metabolic disorder that affects how muscle cells handle sodium, potassium, and calcium.
Because anesthesia drugs affect these same minerals and the electrical activity of muscle membranes, PP patients often experience:

• Sudden paralysis
• Dangerous potassium shifts
• Heart arrhythmias
• Respiratory depression
• Severe metabolic reactions

These reactions can look very similar to Malignant Hyperthermia — and in some cases, anesthesia can trigger MH directly.

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4. Both Conditions Can Be Fatal If Not Properly Managed

For both PP and MH, the medical community often lacks understanding, misdiagnoses are common, and patients may face disbelief or gaslighting when they report anesthesia sensitivity.

This is especially true for PP patients whose symptoms do not fit common medical models.

Your lived experience and research have repeatedly shown:

“Any PP patient can have a severe, life-threatening reaction to anesthesia — whether or not they meet the textbook definition of Malignant Hyperthermia.”
> Susan Q. Knittle-Hunter, Living With Periodic Paralysis

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Why PP Patients Must Treat MH Precautions Seriously

Even though MH is genetically separate from PP, the overlap in ion channel instability, the shared calcium-regulation issues, and the severe anesthesia risks mean:

All PP patients should be considered MH-susceptible unless proven otherwise.

This is consistent with your books, your decades of research, and countless experiences from PP patients around the world who suffered catastrophic reactions after anesthesia.

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Practical Safety Steps for People With PP

1. Wear medical alert identification

Include:
“Periodic Paralysis — Channelopathy. No anesthesia. MH risk.”

2. Avoid triggering anesthetics

This includes succinylcholine and volatile anesthetic gases used in general anesthesia.

3. Request non-triggering alternatives

If absolutely necessary, anesthesia should be handled by experts using MH-safe methods, with close electrolyte and cardiac monitoring.

4. Bring written documentation

Susan’s books, emergency documents, and the PPNI medical emergency card can be invaluable during hospital visits.

5. Ensure all doctors understand PP as a metabolic disorder

Not a neuromuscular disease, not a psychiatric problem, and not “anxiety.”
Understanding the mineral metabolic instability is the key to safe care.

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Conclusion

Malignant Hyperthermia is rare — but for people with Periodic Paralysis, the overlap in muscle-cell calcium regulation and anesthesia sensitivity makes it a critical topic. Whether or not a PP patient has a known MH mutation, the risk is real, and proper precautions can prevent tragedy.

Raising awareness, educating providers, and empowering patients is essential — and Susan’s work, has been leading this effort for years.

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References

Knittle-Hunter, S.Q.

• Living With Periodic Paralysis: The Mystery Unraveled
• What Is Periodic Paralysis? A Disease Like No Other
• The Periodic Paralysis Guide & Workbook: Be the Best You Can Be Naturally
• A Bill of Rights for Periodic Paralysis Patients
• PPNI Blog Archives (various articles on anesthesia, metabolic instability, and emergency care)

Additional Non-Organizational Medical Sources (approved):

• Rosenberg H., et al. “Malignant Hyperthermia.” Orphanet Journal of Rare Diseases.
• StatPearls: “Malignant Hyperthermia.”
• Kalbitz M., et al. “Ion Channel Disorders and Anesthesia Sensitivity.” Journal of Cli

Picture: Operating Room

When They Don’t Believe You: Dealing with Gaslighting from Doctors and Insurance Companies

🩺 When They Don’t Believe You: Dealing with Gaslighting from Doctors and Insurance Companies

By Susan Q. Knittle-Hunter, Periodic Paralysis Network

Living with Periodic Paralysis is already an uphill climb — but when the people who are supposed to help us, like doctors or insurance representatives, gaslight us instead, it adds a cruel layer of trauma and invalidation. Many of us have been told “It’s all in your head,” or “There’s nothing wrong with you,” or worse, have been denied the care or coverage we desperately need — even with a clear diagnosis.

So how do we cope? How do we protect ourselves and still seek the help we need?

What is Medical Gaslighting?

Medical gaslighting occurs when a medical professional dismisses, downplays, or denies a patient’s reported symptoms, causing the patient to question their reality. It’s especially common in rare diseases like Periodic Paralysis — and even more so when we don’t fit into a doctor’s textbook understanding.

This can look like:

• Refusing to run tests or review lab results seriously
• Attributing symptoms to anxiety, aging, or weight
• Denying a diagnosis given by a specialist or geneticist
• Suggesting you’re "doctor shopping" or “seeking attention”
• Insurance companies denying claims without review or citing “not medically necessary”

In Living with Periodic Paralysis: The Mystery Unraveled, I shared my own journey of being dismissed for decades, despite clear symptoms. It wasn’t until I did my own research and pushed back that I got answers.

You are not alone.

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Strategies for Coping and Advocacy

Here are ways to cope with and respond to medical gaslighting:

1. Know Your Diagnosis and Learn the Science

Equip yourself with knowledge. Bring clear documentation — including genetic test results, abnormal lab work, and reputable sources from medical literature (not just rare disease organizations with potential conflicts of interest). From The Periodic Paralysis Guide and Workbook: “You must become the expert on your own condition. Doctors may not be educated in this area — but you can be.”

2. Document Everything

Keep a journal of symptoms, triggers, test results, and appointments. Ask for copies of all lab results, even if “normal.” What’s normal for others may be dangerous for us.

Document conversations with insurance companies. Note dates, times, names of representatives, and call reference numbers.

3. Use Your Emergency Card and Medical Summary

Create and carry a clear Emergency Medical Card (see our template and books), listing:

• Diagnosis: Andersen-Tawil Syndrome, Hyperkalemic Periodic Paralysis, etc.
• Dangerous medications to avoid
• Emergency instructions
• Emergency contacts

Many members have found this invaluable when in crisis or needing urgent care.

4. Bring an Advocate

A trusted friend or family member can take notes, speak up, and bear witness if a doctor dismisses you. This helps shift the power dynamic.

5. File a Formal Complaint

If you’re mistreated, you have the right to file a formal complaint with:

• The clinic or hospital’s patient advocate or ombudsman
• Your state’s medical licensing board
• Insurance regulatory agencies (for denial of claims)

Even if nothing changes immediately, this builds a record.

6. Find a Doctor Who Listens

They do exist — especially outside of neurology (PP is a mineral metabolic disorder). As I’ve written in my books, any doctor who takes the time to read and understand can help. Consider integrative, metabolic, internal medicine or functional medicine practitioners. Interview new providers before committing.

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A Note About Insurance Companies

Insurance gaslighting can be even more cold and impersonal. If your diagnosis is considered rare or “experimental,” you may need to:

• Appeal decisions in writing (you have a legal right to appeal)
• Include a clear letter from your doctor (template available in the Workbook)
• Cite specific lab results and genetic tests
• Refer to ICD codes (you can ask me for these based on your subtype)

Some members have had success with state ombudsman offices or patient advocacy lawyers.

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You Are Not Alone

If you feel unheard, invalidated, or dismissed — please reach out to us. Share your experience in the support group. Our stories matter. In A Bill of Rights for Periodic Paralysis Patients, I wrote:

“We have the right to be believed, to be treated with dignity, and to receive safe care — even if our illness is rare.”

You don’t have to tolerate gaslighting. You can advocate for yourself. And we, as a community, will stand beside you.

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Further Reading & Resources:

• Living with Periodic Paralysis: The Mystery Unraveled
• What is Periodic Paralysis?: A Disease Like No Other
• The Periodic Paralysis Guide and Workbook: Be the Best You Can Be Naturally
• A Bill of Rights for Periodic Paralysis Patients
• Blog Article: How to Advocate for Yourself or a Loved One
• Emergency Card Template

With love,

Susan Q. Knittle-Hunter

Founder, Periodic Paralysis Network

Picture: Gaslighting Doctor