What is Periodic Paralysis??

Hello All,

"Periodic Paralysis is a disease unlike any other. It is in a category all its own and needs to be treated in non-conventional ways." (Quote from one of the doctors who diagnosed me.)

As I have been busy building this blog, I have been trying to decide the best way to describe just what Periodic Paralysis is to our audience. Rather than re-inventing the wheel, I decided to post an article I wrote about it on PublishUs. It is a good overview of my story and Periodic Paralysis. Then I hope to break down the components and write about a different aspect of it each day. Please feel free to ask questions and that may help to guide me to a topic for research and for a future post.

What is Periodic Paralysis?

I am 69 years old and was diagnosed with an extremely rare, hereditary, debilitating, genetic disorder called Periodic Paralysis 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? The following is my story, information about this cruel disease and how and why I control the symptoms.

I have had episodes of partial and total full-body paralysis for much of my life. Due to a series of misdiagnoses and mistreatment with improper medications, I have become totally and permanently disabled with weak muscles throughout my body including those involved with my vision, digestion, breathing and my heart. I must be on oxygen constantly and cannot exert myself in any way. I have had a heart loop monitor inserted in my chest to monitor the tachycardia and arrhythmia, which include life-threatening long QT interval heartbeats. I sit in a recliner for most of my day and can walk (with a walker) only from one room to another or stay on my feet only short periods of time to do simple tasks like brushing my teeth. I must use a motorized wheelchair if I leave home or need to go any distance. If I did not have the help of my husband, I would have to live in an assisted living program.

Through the past years of my physical decline, I have had to give up my career as a special education teacher, my hobbies to include hiking, walking, swimming, exercising, fishing, camping, traveling, shopping, cooking and baking. I had to sell, and move away from, a beautiful home in the mountains of Utah.  I can no longer drive. I have lost many friends, because I could not keep up with them or entertain any longer. I have lost contact with family members who did not understand or did not want to watch my decline or who thought I was a hypochondriac. I have lost the connection I once had with my grandchildren because I can no longer keep up with them or continue a meaningful relationship with them. The relationship with my husband has changed from husband and wife to caregiver and patient. Most of the over 30 doctors I had seen in the 6 years before my diagnosis treated me poorly and as if I were mentally ill.

 I have spent the past several years working diligently to get a diagnosis and treatment for the ailment that cruelly stole the quality of my life. The most difficult part of this, for me, is knowing that I may not have became this seriously ill if just one of the over 30 doctors I have seen in the last 6 years in Oregon and the many years before, would have taken me seriously.

 One Sunday morning after recovering from yet another full body paralytic episode, I searched once again on my computer for “periods of paralysis”. I was shocked and dismayed to read about a disease, which actually had all of the components many of my family members and I had been experiencing for so many years. Once I realized what I actually had, the struggle became even more difficult trying to convince my doctors. By this point, everything else had been ruled out, but no one wanted to diagnose me. I heard I was “too old” to have it. I was ignored. I was dismissed and told to go have a “good time” as long as I was in Portland, after driving 250 miles for the results of a muscle biopsy (The test did show myopathy (muscle disease), change in shape and size of muscle fiber but I was told it was normal). I was given lidocaine after telling my primary care physician (PCP) I could not have it during a mole biopsy. It caused an episode of paralysis but I was treated as if I were a naughty child behaving badly. I was left alone in the room in paralysis. I was in metabolic acidosis, twice in front of my PCP and sent home rather than to the hospital. My heart was in tachycardia and I could not breathe. After discovering that I was having long QT interval heartbeats on a Holter monitor (a marker for ATS), this was dismissed by my PCP, even after being told it meant I could go into cardiac arrest at any given moment. After two months, I had to request a referral to an electrocardiologist. The referral took two more weeks to get from my PCP and the insurance company.

During this time, I continued to decline as I had more and more severe total paralytic episodes. I had tachycardia and palpitations of my heart and I was having difficulty breathing. Sometimes my breathing would actually stop for a few seconds at a time. It felt like an elephant sitting on my chest. It was very frightening. Soon the difficulty of taking breaths in and out began to happen when I was not in paralysis. I found it more and more difficult to breathe. Every time I stood up, ate a meal or exerted myself in anyway, the breathing got worse and my heart would speed up until it was beating 130 to 140 beats per minute, even while I was eating.

My husband became so concerned with the lack of caring being displayed by my PCP and our insurance company, that he walked into a medical supply company and told them what was happening and asked if they could help me to get oxygen because I could not breath. After speaking with him for a few minutes, the manager told my husband that she would give all of the information he had carried in with him, to one of the technicians and that they would see what they could do for us. She told my husband that they find it is best to get all the information together and then, “Hit them (doctors) between the eyes with the facts”.

They hooked me up with a recording oximeter. It was discovered that my oxygen saturation levels were dropping dangerously low during my episodes of paralysis and it was apparent that they were low every time I exerted myself in any way. The technician took the information to my PCP and she had no choice but to sign a referral for me to get oxygen. At that point, we began to look for another PCP and decided to change insurance companies to avoid the need for referrals.

 A month or two before this point, I was in despair over trying to find a doctor who knew about Periodic Paralysis. Then on the evening news, I saw their weekly feature of offering direct calls to doctors with any medical question. I quickly picked up the phone. After a wait of only a few minutes, I was speaking with one of the physicians. I asked her if she had heard of PP or knew of any doctors who might know about it. As luck would have it, she herself had a patient with it. She gave me the name of the neurologist the patient sees.

I went to my PCP with this information and talked her into giving me yet another referral. The neurologist eventually diagnosed me with “probable” Periodic Paralysis. He wrote a letter telling my PCP that I needed to see an electrocardiologist right away. It was several months before I got the referral. He described my heart condition, by that point as serious with no treatment, but insisted I needed to have a heart monitor implanted. He also set up a renal specialist to help diagnose what he believed was Andersen-Tawil Syndrome based on all the information being presented to him by my PCP, the neurologist and me. I did get the diagnosis while in the hospital for the implant after going into paralysis and being observed by the doctors. The paralytic episode was caused by a mistake. They gave me a saline drip and lidocaine during the procedure. My diagnosis was actually based on an accident.

What is Periodic Paralysis?

 One of the neurologists who diagnosed me recently said, “Periodic Paralysis is a disease unlike any other. It is not a neuromuscular, mitochondrial or autoimmune disease nor is it a muscle myopathy. It is in a category all its own and needs to be treated in non-conventional ways.” He further stated, “Doctors need to keep an open mind and think ‘outside of the box’ when it comes to diagnosing and treating Periodic Paralysis.”

The following is an overview of the condition gleaned from years of research and my own experience.

Periodic Paralysis (PP) is an extremely rare, hereditary disease characterized by episodes of muscular weakness or paralysis, a total lack of muscle tone without the loss of sensation while remaining consciousness. It is passed from either the mother or the father to any of the children, male or female. It is a mineral metabolic disorder, also known as an ion channelopathy, a disease involving dysfunction of an ion channel for potassium, sodium, chloride or calcium. Ion channels regulate ions as they flow in and out of the cells.

 

The Most Common Forms

Hypokalemic Periodic Paralysis (HypoPP)

Paralysis results from potassium moving from the blood into muscle cells in an abnormal way. It is associated with low levels of potassium in the blood (hypokalemia) during paralytic episodes.

Hyperkalemic Periodic Paralysis (HyperPP)

Paralysis results from problems with the way the body controls sodium and potassium levels in cells. It is associated with high levels of potassium in the blood (hyperkalemia) during paralytic episodes.

Andersen-Tawil Syndrome (ATS)

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. 

Normokalemic Periodic Paralysis (NormoPP)

Paralysis results when potassium shifts within in normal ranges. This can happen in any form of Periodic Paralysis; Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis, Normokalemic Periodic Paralysis 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.

Paramyotonia Congenita (PMC)

The skeletal muscles can become stiff, tight, tense or contracted and weak due when the sodium channels close much too slowly and the sodium, potassium, chloride and water continue to flow into the muscles. It is actually considered to be a form of Hyperkalemic Periodic Paralysis, however, the symptoms can appear from shifting of potassium into low or high ranges or even if potassium shifts within normal levels.

Thyrotoxic Periodic Paralysis (TPP)

Intermittent paralysis results from low potassium due to an overactive thyroid or hyperthyroidism.  It can occur spontaneously or can result from a genetic mutation. Unlike the other forms of Periodic Paralysis, TPP can be treated and cured by removing or treating the thyroid.

Only about 50% of the above known types of Periodic Paralysis have identified genetic markers. This means they can be diagnosed by DNA testing. The remainders of the cases are diagnosed by the symptoms and characteristics of the patient. This is called being diagnosed clinically. Those who are diagnosed clinically have symptoms and characteristics identical to others who have known genetic codes. One who is diagnosed with ATS clinically is said to have ATS Type 2 to differentiate.

Although Hypo PP, Hyper PP and ATS are forms of periodic paralysis the mechanism, which creates the muscle weakness and paralysis, is different as described above and the symptoms before and accompanying the paralysis vary. Symptoms can range from simple weakness to total body paralysis with life-threatening heart arrhythmia and tachycardia, breathing problems and choking. Death can occur in some rare cases. The episodes may last from a few minutes to several hours or many days. The speed with which the potassium shifts may cause symptoms to occur suddenly and without warning or there may be a gradual progression into the weakness or paralysis.

Abortive attacks may also affect some individuals. Occasionally, the common symptoms may begin but the full attack or paralysis may not occur. The person is left with extreme weakness and other symptoms such as extreme fatigue. This may last for hours, days or weeks.

The following symptoms may accompany the paralytic attacks. Most are based on my own experiences and the experiences of others I have talked with. Some may cross over.

One who suffers with Hypokalemic Periodic Paralysis may experience a variety of symptoms in relationship to their hypokalemic paralytic attacks including but not limited to the following:

 

Paralysis, partial

Paralysis, total

Muscle weakness

Muscle stiffness

Muscle aches

Muscle cramps

Pins and needles sensation

Pulse issues-fast heart beat
High blood pressure

Breathing problems-barely breathing

Hypoventilation

Irritability

Severe thirst

Nausea

Vomiting

Constipation

Excessive urination

Irregular heartbeat

Sweating

Tiredness

One who suffers with Hyperkalemic Periodic Paralysis may experience a variety of symptoms in relationship to their hyperkalemic paralytic attacks including but not limited to the following:

 

Paralysis, partial

Paralysis, total

Muscle weakness

Muscle cramps

Fasciculation

Tightness in legs

Strange feeling in legs

Tingling sensations

Pulse issues - absent, slow, or weak
Low blood pressure

Heart palpitations

Irregular heartbeat

Breathing problems-fast breathing

Mild hyperventilation

Nausea

Feeling hot

Slurring words

Sleepiness

One who suffers with Andersen-Tawil Syndrome may experience a variety of symptoms in relationship to their ATS paralytic episodes including but not limited to any of the symptoms from above depending on whether the attack is hypokalemic, hyperkalemic or normokalemic. They also have long QT interval heartbeats, a life threatening arrhythmia, which is a marker for ATS. Ventricular arrhythmia is common as is fainting.

The periodic muscle weakness or paralysis is triggered by a wide variety of activities such as exercise or sleep; foods such as carbohydrates or meat; conditions such as heat or cold; medications such as antibiotics or muscle relaxers; compounds such as caffeine or salt or simply resting after exercise. Many of the triggers are the same for most people but some of the triggers can be unique to each person or the type of Periodic Paralysis.

It is important to discover one’s triggers because of the need to stop the episodes, if possible, in order to regain some quality of my live and to prevent further damage to the organs as the potassium shifts and depletes in my body. This damage can lead to permanent weakness and disability as well as tachycardia and serious arrhythmia, including long QT intervals, which can lead to cardiac arrest. Avoiding paralysis is absolutely necessary for me, due to these life-threatening conditions.

Every moment of my present life I must control my symptoms. The following plan was created after trial and error in my own quest for treatment and management. I had no doctor assisting me and gleaned as much as possible on the Internet and in discussion with other people who live with Periodic Paralysis and Andersen-Tawil Syndrome. Anyone with Periodic Paralysis may follow this plan to see promising results.

Discover triggers:

Simple carbohydrates,

Complex carbohydrates,

Most meat,

Wheat,

Gluten,

Salt,

Sugar,

Caffeine,

Medications including over-the-counter medications,

Exercise,

Exertion,

Rest after exercise,

Sleep, all aspects: falling asleep, during sleep, waking up and napping.

Stress (good or bad),

Dehydration

Msg

Food in general

Large meals

Fatigue

Fasting

And ?  I still have not discovered all of my triggers. No matter how careful I am, I can still go into paralysis without knowing why.

Control symptoms:

Avoid triggers

Following a proper ph balanced diet, eating from the farm; not the factory

Take no medications including over-the-counter medications

Avoid stress

No exercise

No exertion

Get plenty of rest

Stay well hydrated

Constantly monitor vitals

Take potassium when needed (if low potassium) (If under 3.0 go the the hospital)

Take sugar or glucose tablets as needed (if high potassium) (If over 6.5 go to hospital)

24/7 oxygen

Monitor vitals:

I use several pieces of medical equipment for measuring my vitals. These items are necessary for my caregiver to monitor me while in paralysis or for me to know which direction my potassium shifts for proper treatment.

Cardy meter (potassium reader),

Finger pulse oximeter,

Blood sugar monitor,

Stethoscope,

Wrist blood pressure monitor,

Thermometer and a digital

pH balance reader

Litmus paper

Gather a team of medical professionals:

I have an MD as my primary care provider and a cardiologist. I no longer have a renal specialist or neurologist.

Continue to gather information: I must educate myself, my family, my friends, my neighbors, my community, my doctors, my hospitals, my dentist, my optometrist and my local first responders about every aspect of my condition. Knowing and understanding this disease and syndrome eases my fears and the fears of those around me and assists me with proper management and treatment. Knowing others will be able to aid me during paralysis episodes is essential.

Join Periodic Paralysis social groups: Being part of a Periodic Paralysis community is vital. I have discovered I am not alone. I receive encouragement, support, sympathy and empathy. I gain information and knowledge from others who live with the same enemy daily. I ask questions and share ideas.

The above plan is followed constantly and diligently.  It is a constant “tightrope” I must balance, day-by-day, minute-by-minute; second-by-second. I cannot let up for even a minute or I can go into paralysis and the cycle begins anew.

Prognosis: Most individuals with Hypokalemic Periodic Paralysis are able to control the symptoms and paralytic attacks with one or two forms of potassium and avoiding the things that trigger them. Individuals with Hyperkalemic Periodic Paralysis symptoms and paralytic attacks can control their symptoms and paralytic attacks with a diet high in carbohydrates and sugar and by avoiding the triggers.

Controlling the symptoms and paralytic attacks in people with Andersen-Tawil Syndrome is much more difficult. This is due to the fact that these individuals suffer from paralysis due to potassium levels that can be low, high or in normal ranges. Taking potassium may make the symptoms worse. Also individuals with ATS are usually unable to take any forms of medication. Managing the heart issues by surgery is also a problem because anesthesia can trigger paralysis and deadly arrhythmia. For these individuals, natural methods are the best way to manage the symptoms.

For most people with Periodic Paralysis the weakness and paralysis are intermittent. There is a beginning and end and between the episodes the individual is normal. Some individuals, however, may experience only gradual muscle weakness. Others experience periods of paralysis and gradual, progressive, chronic weakness. For some individuals the weakness can linger or become permanent. Some people will become disabled and require the use of a power wheelchair. Some individuals may become terminal if the disease weakens the breathing muscles.

The Periodic Paralysis Network:

My husband, Calvin, and I have created and now manage the Periodic Paralysis Network. We are an organization with an online community of people who are affected by Periodic Paralysis. The Periodic Paralysis Network provides a hands-on approach to understanding the disease, getting a proper diagnosis, managing the symptoms, and assisting caregivers and family members. Our focus is on educational resources and self-reliance. Our approach to treatment focuses on the self-monitoring of vitals and the management of symptoms through natural methods. We continue to do research and provide the latest information to our members. Everyone is welcome. Members will receive encouragement, support, sympathy, empathy and validation. Members will also gain information and knowledge about all aspects of Periodic Paralysis. Members   ask questions and share ideas. We are usually on in real time, answering questions and providing support as needed for our members.

We have also written and published the following books,
"Living With Periodic Paralysis: The Mystery Unraveled"
"The Periodic Paralysis Guide And Workbook: Be The Best You Can Be Naturally"
"A Bill Of Rights For Periodic Paralysis Patients"
"What Is Periodic Paralysis?: A Disease Like No Other"

They can be purchased at:
https://www.amazon.com/Susan-Q-Knittle-Hunter/e/B00HVEBSSQ/ref=dp_byline_cont_pop_book_1

If you wish to know more about Periodic Paralysis, you may visit the Periodic Paralysis Network.

Until Later...