PPNI Support

Susan Q. Knittle-Hunter

My name is Susan, the managing director of PPN. I was diagnosed with an extremely rare, hereditary, debilitating, genetic disorder called Periodic Paralysis (PP) on February 7, 2011 at the age of 62. The form I have is a variant of Andersen-Tawil Syndrome (ATS). I was misdiagnosed for over 50 years. How could such a thing happen in today’s world with all of the modern medicine and technology? Follow this link to read my story, information about this cruel disease, and how I manage my symptoms.

PPNI Mission Statement

The Periodic Paralysis Network Forum provides assistance to individuals with Periodic Paralysis (PP) through Awareness, Support, Education and Advocacy (A.S.E.A.) and by providing a hands-on approach to understanding the condition, getting a proper diagnosis, managing the symptoms in all natural ways and assisting their caregivers and family members.

What is Periodic Paralysis? (PP)
PP is a rare, mineral metabolic disorder, called an ion channelopathy. It is generally inherited and characterized by episodes of muscular weakness, tightness or flaccid paralysis without the loss of sensation or consciousness (though both can happen). Dysfunction of the ion channels for potassium, sodium, or calcium is involved. Regular activities such as sleep, exercise, eating, and taking medications can trigger muscle paralysis. Episodes of muscle paralysis can be full body lasting minutes, hours or days. Permanent muscle weakness may occur over time. During periods of muscle paralysis a person may experience difficulty breathing, heart rate fluctuation and arrhythmia, blood pressure fluctuation, and choking. It can be life threatening. PP is often misdiagnosed and mistreated by medical professionals.

Common Forms of Periodic Paralysis (PP)

Hypokalemic PP (HypoPP or HypoKPP)
Also known as Westphall Disease, in HypoKPP, paralysis results from potassium moving from the blood into muscle cells in an abnormal way due to the calcium channel not signaling for appropriate release of calcium. It is associated with low levels of potassium in the blood (hypokalemia) during paralytic episodes.Normokalemic

PP (NormoPP or NormoKPP)
Paralysis results when potassium shifts within in normal ranges. This can happen in any form of PP; Hypokalemic PP, Hyperkalemic PP, Normokalemic PP and Andersen-Tawil Syndrome. The paralysis may result from the shifting itself, rather than low or high potassium or it may occur due to the shifting of the potassium, which can happen very quickly and is undetectable in lab testing.

Hyperkalemic PP (HyperPP or HyperKPP)
In HyperKPP, also known as Gamstorp Disease, Paralysis results from sodium flowing into the cells because the channel remains open inappropriately. It is associated with high levels of potassium in the blood (hyperkalemia) during paralytic episodes.

Andersen-Tawil Syndrome (ATS)
With ATS, also known as Long QT Syndrome 7, paralysis results when the channel does not open properly; potassium cannot leave the cell. This disrupts the flow of potassium ions in skeletal and cardiac muscle. During paralytic episodes, ATS can be associated with low potassium, high potassium or shifts within the normal (normokalemia) ranges of potassium. An arrhythmia, long Qt interval heartbeat, is associated with ATS as well as certain characteristics, such as webbed or partially webbed toes, crooked little fingers and dental anomalies.

Paramyotonia Congenita (PMC)
With PMC, also known as Von Eulenberg’s Disease, the skeletal muscles can become stiff, tight, tense or contracted and weak when the sodium channels close much too slowly and the sodium, potassium, chloride and water continues to flow into the muscles. It is actually considered to be a form of Hyperkalemic PP, however, the symptoms can appear from shifting of potassium into low or high ranges or even if potassium shifts within normal levels.

Products | Services | Features

If You Have Periodic Paralysis
If you have PP, are seeking a diagnosis, are a family member or caregiver you are invited to join the private support group.
The Periodic Paralysis Network Support, Education and Advocacy Group

Information About Periodic Paralysis: The Blog
The Blog: Living With Periodic Paralysis Blog

Periodic Paralysis Network Facebook Page
Facebook Page: Periodic Paralysis Network

PPNI Publications
Living with Periodic Paralysis: The Mystery Unraveled
The Periodic Paralysis Guide And Workbook
A Bill Of Rights For Periodic Paralysis Patients
What Is Periodic Paralysis?: A Disease Like No Other

Awareness | Support | Education | Advocacy

Awareness
PPNI is here to provide awareness of PP around the world for individuals with the condition and their family members. This is accomplished through ASEA services and features.
Go To PPNI ASEA Information Page

Support
PPNI provides support to individuals with PP and their family members and caregivers. We do this in many ways for more than 1,000 members worldwide.
Go To PPNI ASEA Information Page
Go To Facebook Periodic Paralysis Network Support Group

Education
PPNI provides educational materials to individuals with PP, caregivers, family members, medical professionals and anyone else interested to learn about PP.
Go To PPNI ASEA Information Page

Advocacy
PPNI encourages community healthcare professionals to provide informed and compassionate care to individuals with PP and their family members in an unbiased manner.
Go To PPNI ASEA Information Pagee your text here...

Blogs Written About The Effects of Drugs/Medications/Pharmaceuticals/Anesthesia in PP

Read Susan's Story