What is an Ion Channelopathy?

Hello All,

Periodic Paralysis is a channelopathy and channelopathies are considered a class four metabolic disorder. Periodic Paralysis is a fourth class mineral metabolic disorder. Today's blog is about ion channelopathies.


What is an Ion Channelopathy?

Periodic Paralysis is a rare condition like no other. It is called an ion channelopathy, which is a dysfunction of an ion channel. Ion channelopathies were first recognized in 1971 and Periodic Paralysis was one of the first to be discovered. ^{1, 2}

 Ion channels are like a microscopic tunnel in the cells of muscles. The tunnels are called muscle fibers. Ions, which are molecules or atoms, flow in and out of the muscle cells through membranes or gates. Each of the gates is shaped exactly for the correct ion or molecule to enter. The ions are made up of what we call minerals, electrolytes or proteins. Some of the common ions are potassium, sodium, magnesium, chloride and calcium. They are electrically charged and each has its own size or shape, so to speak. If the gates or membranes are faulty in size or shape, an inefficient or improper flow through the membranes can and does cause muscle weakness and paralysis because they regulate contraction and relaxation of the muscle.

Through research, I have discovered that channelopathies are classified as a class four metabolic disorder. Disorders of metabolism are usually inherited and are involved in  chemical and physical processing, which use and make energy in the body. These processes include: breathing, circulation of blood, food and nutrient digestion, elimination of waste through bowel and bladder and temperature regulation.

Unfortunately, ion channelopathies are not usually categorized nor listed in medical writing or studies as metabolic disorders. This poses a problem for recognition, diagnosis and treatment by physicians and other medical professionals. I have found and heard Periodic Paralysis, which is a channelopathy, referred to as, a neuromuscular disease (affecting muscles and/or nerves also known as myoneural), a muscular dystrophy (wasting of muscle and eventual early death), and a disease of the nervous system (nothing to do with the nervous system), therefore, none of these is correct. Periodic Paralysis is a metabolic disorder, a condition which is based in the faulty cellular level of how energy is produced in our bodies. ³

To clarify, even further, Periodic Paralysis is a channelopathy which is a mineral metabolic disorder. Metabolism disorders involving minerals are conditions in which there is either not enough or an overabundance of minerals in an individual’s blood. Minerals have many functions in metabolism and the functions of the human body. They are important in bone and muscle building and growth. Organs, cells and tissues need minerals in order to function properly. So, a dysfunction of minerals in the body affects many processes and functions. Potassium, the main mineral involved in Periodic Paralysis, is involved in making proteins from the amino acids and plays a role in carbohydrate metabolism, so a dysfunction involving it, can affect more than just muscles. ^{1, 2}

 

1. Wikipedia. (February 2013) Ion Channel. Retrieved from:  

http://en.wikipedia.org/wiki/Ion_channel

2. Wikipedia. (December 2012). Channelopathy. Retrieved from: 

http://en.wikipedia.org/wiki/Channelopathy

3. WikiDoc. (August 2012). Metabolic disorder. Retrieved from: 

http://www.wikidoc.org/index.php/Metabolic_disorder

More information:Oxford Journals. (2002). Ion Channel Diseases. Retrieved from:

 http://hmg.oxfordjournals.org/content/11/20/2435.full

Added 1/25/2017

***An Introduction to Metabolic Disorder (Mineral metabolic disorder= Channelopathy)

http://www.encognitive.com/node/1181

A fourth class, the channelopathies (some of which cause periodic paralysis and/or malignant hyperthermia) could be considered to be metabolic disorders as well, though they are not always classified as such. These disorders affect the ion channels in the cell and organelle membranes, resulting in improper or inefficient transfer of ions through the membranes.


***Mineral Metabolism Disorders
http://careers.maimonidesmed.org/Main/AdamMultimediaEncyclopedia/Mineral-metabolism-disorders-1007271.aspx

Definition

Mineral metabolism disorders are abnormal levels of minerals -- either too much or too little -- in the blood.
Minerals are very important for the human body. They have various roles in metabolism and body functions. They are essential for the proper function of cells, tissues, and organs.
Some minerals, such as iron, make up part of many proteins and enzymes in the body. Others, such as potassium, help to produce proteins from amino acids and are involved in carbohydrate metabolism. Minerals also play a role in the building of muscle and bone and are important for normal body growth.
Metabolism refers to all the physical and chemical processes in the body that create and use energy, such as:

• Breathing
• Circulating blood
• Digesting food and nutrients
• Eliminating waste through urine and feces
• Regulating temperature

Causes

Disorders of mineral metabolism are sometimes passed from parents to their children through genes. Other medical conditions, such as starvation, diarrhea, or alcoholism, can cause mineral metabolism problems.

Disorders

Minerals that play a large role in the body include:

• Calcium
• Magnesium
• Phosphorus
• Potassium
• Selenium
• Sodium

Disorders in which mineral metabolism problems often occur include:

• Disorders of phosphorus metabolism:
• Hypophosphatemia
• Osteomalacia
• Rickets
• Rhabdomyolysis
• Hyperparathyroidism
• Hypoparathyroidism
• Disorders of potassium metabolism:
• Bartter syndrome
• Periodic paralysis with hypokalemia***
• Hypokalemic periodic paralysis***
• Hyperaldosteronism - primary and secondary
• Cushing’s disease
• Proximal renal tubular acidosis
• Distal renal tubular acidosis
• Fanconi’s syndrome
• Addison’s disease
• Kidney disease
• Disorders of iron metabolism:
• Hemochromatosis
• Cirrhosis
• Disorders of copper metabolism:
• Wilson’s disease
• Menkes syndrome
• Disorders of calcium metabolism:
• Hyperparathyroidism
• Nephrocalcinosis
• Pseudohypoparathyroidism
• Hypercalcemia
• Osteoporosis
• Movement - unpredictable or jerky
• Kidney stones
• Milk-alkali syndrome
• Paget’s disease
• Multiple endocrine neoplasia (MEN 1)
• Osteomalacia
• Rickets
• Disorders of sodium metabolism:
• Dilutional hyponatremia (SIADH)
• Hypernatremia
• Disorders of magnesium metabolism:
• Hypomagnesemia
• Hypermagnesemia
• Disorders of selenium metabolism
• Selenium deficiency
• Selenium excess
  
  >>>>>>>>>>>>>>>>>>>>>>

Other Disorders Involving Metabolism Of Minerals











Public Health Service Publication No. 1693
https://books.google.com/books?id=icIaAAAAMAAJ&pg=PA153&lpg=PA153&dq=mineral+metabolism++disorders+periodic+paralysis&source=bl&ots=b-rjeyEFyj&sig=Dy_C8ZLRQGtzoqLoBRaxW1p9qQI&hl=en&sa=X&ved=0ahUKEwiY0Zy7j97RAhUQzWMKHbxFBDEQ6AEIUTAJ#v=onepage&q=mineral%20metabolism%20%20disorders%20periodic%20paralysis&f=false

Metabolic Disorder
http://www.wikidoc.org/index.php/Metabolic_disorder

Ion Channels list
http://www.nature.com/ncb/journal/v6/n11/fig_tab/ncb1104-1039_T1.html

PP IS A MINERAL METABOLIC DISORDER http://healthmedicinet.com/ency/article/007271.htm
Mineral metabolism disorders - Health Medicine Network healthmedicinet.com/ency/article/007271.htm

Until later...

Periodic Paralysis and Metabolic Acidosis

Hello All,

 

I had a fairly good day yesterday as long as I did not get up. Once up on my feet, I got dizzy and weak. Otherwise, I stayed busy with writing, editing, analysis of survey data and research. I also took some time to make a grocery list in case I was well enough today to go shopping with Calvin.

 

Since I am feeling fairly well this morning, I think I will try to venture out with Calvin. We will go to our organic market to purchase our vegetables and other unprocessed foods for our pH balanced diet.

I have chosen, today, to write about the reason I follow a pH balanced diet; the issue with metabolic acidosis.

 
Periodic Paralysis and Metabolic Acidosis 

 

 

  (http://medipptx.blogspot.com/2010/09/metabolic-acidosis_1754.html)

Periodic Paralysis is a 4^th class mineral metabolic disorder. People with mineral metabolic disorders tend to have chronic metabolic acidosis. Metabolic acidosis is an excess of acidity in the fluids of the body. In chronic metabolic acidosis, osteoporosis and kidney stones may develop.

 

Metabolic acidosis is a pH imbalance (the balance between the acid and alkaline), in which the body accumulates an excess of acid in the body fluids and does not have enough bicarbonate to neutralize the effects of the acid effectively. An individual can develop metabolic acidosis, if the carbon dioxide levels are allowed to rise and remain in the body.

 

We know that metabolic acidosis affects the heart and breathing. It results in potassium shifting out of the cells and into the bloodstream creating hyperkalemia, too much potassium. The combination of metabolic acidosis and hyperkalemia is a serious condition and can be life threatening leading to shock and death. Metabolic acidosis may also occur in low potassium levels.

 

Some of the more common symptoms of metabolic acidosis are muscle weakness, bone and muscle pain, headache, chest pain, tachycardia, heart palpitations, abdominal pain, rapid breathing, shortness of breath, confusion, drowsiness, a lack of energy and paralysis for persons with Periodic Paralysis. If metabolic acidosis becomes severe it can lead to shock (a lack of an appropriate flow of blood in the body) or death. However, the symptoms of metabolic acidosis are sometimes not very obvious or specific, depending on the cause. It should be noted that in some individual’s metabolic acidosis could be mild and ongoing (chronic).

In chronic metabolic acidosis an individual’s bones and kidneys are affected. When potassium shifts in the body, calcium carbonate from the bone is released. This causes a loss of the bone crystals leading to osteoporosis. When the kidneys are affected, this can be seen by the formation of kidney stones.  

Normally our pH level should be 70% alkaline and 30% acidic. If we do not eat a balanced diet and eat more acidic foods, we are too acidic and we can develop metabolic acidosis a serious condition. Through research we found that those of us with PP may have chronic levels of metabolic acidosis. So a balanced pH diet is a very good plan for some of us.

The normal diet in the U.S. is very acidic with meat, fats, dairy, sugar, white flour, and with all the processed foods we eat. This makes many normal people acidic and they become ill with any number of symptoms and illnesses. So imagine what it does to us with Periodic Paralysis?  We develop, metabolic acidosis, which we are already prone to developing.

 One of the major drugs used for treating Periodic Paralysis is acetazolamide also sold under the name of diamox and keveyis.  It is a carbonic anhydrase inhibitor, which is a diuretic. It removes water through the kidneys. Interestingly, it is used to treat mild metabolic acidosis, however, it actually leads to more metabolic acidosis by speeding up the process. Many individuals taking this drug to treat their symptoms of Periodic Paralysis are unknowingly making themselves worse and causing more damage to their bodies. It also lowers potassium. Many people are also unaware that it is a sulpha-based drug and should not be taken if an allergy exists to sulfa drugs.

 
If one already has Periodic Paralysis and has chronic metabolic acidosis he or she can develop kidney stones and osteoporosis over time. If one already has Periodic Paralysis and has chronic metabolic acidosis and takes diamox, he or she can become more acidic and can acquire full-blown metabolic acidosis which causes more damage and kidney stones and accelerates osteoporosis, more illness, more paralysis from the stress on the body and lowering of potassium and may even cause death. (Please research this well as you make your choices about the drugs you are using. Many, many of us choose not to use them, they cause us serious side effects in the short and long term.)

So it is especially important for individuals with Periodic Paralysis to maintain that 70/30 balance and it can be done with a pH diet. We may eat some of the foods, which are more acidic, just remember to keep the 70/30 balance to avoid or lower metabolic acidosis.

http://www.lumrix.net/medical/diseases/metabolic_disorders.html
http://www.maimonidesmed.org/Main/AdamMultimediaEncyclopedia/Mineral-metabolism-disorders-1007271.aspx
http://en.wikipedia.org/wiki/Metabolism#Minerals_and_cofactors
http://www.encognitive.com/node/1181
https://sites.google.com/site/metaboliccare/metabolicdisorder
http://the-medical-dictionary.com/metabolic_disorder.htm
https://ufhealth.org/mineral-metabolism-disorders

http://en.wikipedia.org/wiki/Acetazolamide

http://www.drugs.com/cdi/acetazolamide.html
https://www.riverpharmacy.ca/faq/are-there-any-side-effects-to-acetazolamide-diamox-avva

http://www.medicinenet.com/acetazolamide-oral/page3.htm

http://www.rxlist.com/diamox-sequels-drug.htm

Until later…

Periodic Paralysis and Balance November 28, 2013

Happy Thanksgiving to our readers in the USA!!!

About two weeks ago, physically, I could not even get out of my recliner and I was in a brain fog, and more. I felt miserable. I mentioned it in some of my previous blogs. I have had varying degrees of this for many, long months and it was getting worse.

I had to evaluate what the trigger was so I could stop it or avoid it. I just could not face one more day of feeling like I was dying. I studied my diet, which is organic and free of additives, pesticides and pH balanced. I monitored my vitals and my potassium and glucose levels, which were normal. I decided the only other thing out of the ordinary was my supplements. I had been taking them for nearly three years. They are pure and free of gluten, sugar, etc with no fillers. I decided to experiment, however, and stopped taking them.

Since that day...I have been able to get out of my recliner and the fog has lifted!!! My world has color again!! No more shades of gray!! I have more energy. Calvin has been shocked at the difference and continues to caution me to not do too much.

I have been able to prepare an entire Thanksgiving dinner!! Each day I made one thing and froze it or packaged it until today. I will be putting my own turkey in the oven in a little while! My daughter and son-in-law arrived last night and I was able to serve them gluten free and low sugar pumpkin pie that I made, as well as, to be able to visit with them without going into paralysis.

I believe that the problem was taking supplements that I did not need any longer. It was throwing my body out of balance. I may need them again in the future, but not now. As my symptoms wax and wane in the future, I will need to evaluate why, but I know now that I should not put anything in my body I do not need. Those of us with Periodic Paralysis must walk that fine line...We must “walk the tightrope”

Periodic Paralysis is an ion channelopathy. Ion channelopathies are mineral metabolic disorders. Periodic Paralysis is a 4^th class mineral metabolic disorder. As such, it appears that anything may affect the balance…we must continually evaluate and re-evaluate each thing we are putting into out body.

Until later…

Walking the Tightrope Using The Plan November 22, 1013

Hello All,

 

 
Several members have been having suffering with ups and downs of their symptoms and also develop new symptoms without knowing what if triggering it. The following is what I wrote this morning.

”I am so sorry you are going through this. I have to admit that I too, have been on the same roller coaster for quite awhile, however, in the past few days I made some important discoveries for myself, and maybe for others. This is what I have discovered:

I posted the following yesterday in response to a question about potassium gluconate. It is so simple but had a profound effect on me:

Differences in the three main types of potassium:

Potassium citrate (and potassium bicarbonate) is alkaline, potassium gluconate is neutral PH, potassium chloride is acidic.

Periodic Paralysis is a metabolic disorder and we can have issues with being out of balance metabolically. We can become acidic or alkalinic. I know that I have issues with chronic metabolic acidosis and lactic acidosis. I have been diagnosed with both and keep track using a pH meter. So, if you tend to be acidic (metabolic acidosis or lactic acidosis) potassium citrate (and bicarbonate) might be best. If you tend to be alkalinic (metabolic alkalosis) potassium chloride might be best. If you do not have acidosis or alkalosis than gluconate may be best..."

If we do not maintain that balance we become unsteady on that tightrope we must walk! We have to maintain that balance...it is a constant battle!

So realizing I was out-of-balance, over a period of several weeks, I stopped taking my potassium bicarbonate (and before that I was taking potassium citrate). I was still having issues so a few days ago, I stopped taking my magnesium citrate and calcium citrate and pro-biotics and my V-8 juice in the morning.

For as many days, I have not had the issues I was having...an over-all fogginess, depression, cranky, agitated, severe fatigue and muscle weakness and what I can only describe as a feeling of "uuuuuhhhhhhh"...what I have considered to be an "abortive attack".

My conclusion is this: In our book, Living With Periodic Paralysis:The Mystery Unraveled describe "walking the tightrope"...walking that fine line ...we must stay in perfect balance... and we do that by following the "Plan" or we have symptoms of all kinds depending on what is out of balance...So...I apparently, got into balance doing all I was doing, but by continuing things I no longer needed, I was making myself sick again with too much of it...so I did not need to continue with potassium bicarbonate routinely because by doing so I was becoming hyperkalemic...if I took more potassium thinking I needed it based on my symptoms.,. I was making it worse. I was also probably making myself too alkaline. I stopped it weeks ago but after doing fine for a few days I went downhill again.

After stopping the magnesium citrate and calcium citrate both alkalinic, probiotics and V-8, I have not had any of the usual symptoms, except for the day I went shopping with Calvin. That evening, I had extreme muscle weakness and shakiness. BUT...I did not have the other symptoms mentioned above, I was clear-headed and even-tempered and no "uuuuhhhh" feeling. (Proof of my exercise intolerance or exertion as a trigger. But also proof that exercise and exertion did not necessarily cause my other symptoms.)

My conclusions: I may need potassium at any given time depending on my symptoms, but not necessarily routinely. I may have needed magnesium and calcium for a while, but taking it routinely, may be making me worse. The V8 juice is probably more sodium and potassium than I need right now or it may be just one of them causing the issue. I will test it by introducing just one of them back into my routine at a time, until I feel those symptoms again. Then I will know which was the trigger.

The key for me is moderation in all to maintain that balance. Low salt, not no salt, low sugar, not no sugar (use my glucose meter), potassium if the potassium reader indicates I am low, no potassium bicarbonate unless I am acidic, no potassium chloride unless I am alkalinic (use my pH meter) and of course follow the pH balanced diet.

Will I still have bad days? Yes. Will I have more good days? Yes. Will I have to work at it? Absolutely. Will it be easy? No. Will it be worth it? Yes.

Until later...

Welcome to the world of Periodic Paralysis!

 

Hello All,

Welcome to our new blog!!!! It is our desire to share with the world everything and anything we can about Periodic Paralysis with the hope of educating and supporting others with the condition and educating others who do not have it but would like to know about it, including doctors and medical professionals. I began a blog several years ago and wrote daily for over a year, but it was a closed blog, as is our support group, so only our members could read what I wrote. We have been looking for a way to be able to reach more people with our important information, so decided it was time to create a new blog and open it up to the world!!!! Please feel free to ask questions and share your experiences with us.

We would like to introduce ourselves:

I am Susan Q. Knittle-Hunter and my husband Calvin Hunter. We are passionate about helping others with Periodic Paralysis and sharing information about it. Periodic Paralysis is very rare, hereditary, debilitating, difficult to diagnose, mineral metabolic disorder, also known as an ion channelopathy. I have a form of it and was 62 years old when I was finally diagnosed, 3 years ago, after suffering the effects of it nearly my entire life and nearly dying from the wrong diagnoses and incorrect treatments I received.

Calvin Hunter and I are the founders and creators of an on-line independent organization, the Periodic Paralysis Network, a private educational, support and advocacy organization, which is patient-safety-related due to the serious nature and potential life-threatening symptoms and side effects of this condition if it is not treated correctly.  We have a website with a forum containing 3 distinct discussion groups and we have written and published two books, the only two books written about Periodic Paralysis...Living With Periodic Paralysis: The Mystery Unraveled, which is an extension of our website and a workbook...The Periodic Paralysis Guide and Workbook: Be The Best You Can Be Naturally. We work towards the improvement of the quality and safety of patients from all over the world with the various forms of Periodic Paralysis. Our focus is on educational resources to build self-reliance and self-empowerment and to prevent possible harm from improper treatment. Our approach to treatment focuses on the self-monitoring of vitals and the management of symptoms through natural methods. We also offer strategies to understanding the disease, getting a proper diagnosis, managing the symptoms, and assisting caregivers and family members. We have members from all over the world, (Iran, Ukraine, Turkey, Denmark, Wales, Netherlands, Canada, Finland, Australia, and more) who are seeking help for themselves, their children and entire families and are unable to find it anywhere. We provide ideas on how to find doctors, get a diagnosis, get the proper help in the ER, how to discover their triggers and much more.

Periodic Paralysis is often misdiagnosed and mistreated, thus causing more damage or possible death to the person with it. There are several types: Hypokalemic Periodic Paralysis (low potassium), Hyperkalemic Periodic Paralysis (high potassium) and the type I have, a variant of Andersen-Tawil Syndrome (ATS), is the most rare and the most serious type. Long QT interval heart beats can accompany the episodes. On a cellular level, triggered by things such as sleep, exercise, sugar, salt, most medications, stress, cold, heat, anesthesia, adrenaline, IVs, etc., potassium wrongly enters the muscles either temporarily weakening or paralyzing the individual. Episodes can be full body lasting hours or days. Permanent muscle weakness may occur over time. If it affects the breathing muscles it can become terminal. Dangerous heart arrhythmia, heart rate fluctuation, blood pressure fluctuation, choking, breathing difficulties, cardiac arrest and/or respiratory arrest can also accompany the episodes. Due to these complications, it is extremely important to avoid the episodes. There are no known cures, but there are treatments/drugs for some forms which can be and are successful for some individuals.

The type I have, Andersen-Tawil Syndrome (ATS), however, has no traditional medications, which can alleviate the symptoms, but by avoiding the triggers and by using some natural methods, the number of paralytic episodes can be reduced and the severity of the episodes can be lessened. Due to many wrong diagnoses and improper medications, I am severely disabled in a power wheelchair and on oxygen therapy 24/7. I was having episodes 4 to 5 times a day but now, with the natural methods have been able to reduce the number to about 1 or 2 a month during my waking hours (sleep is my biggest trigger so I continue to have them every night as I sleep) with less severity and for shorter periods of time. Calvin's tireless research discovered these methods and saved my life.

I earned B.S. degrees in Psychology and Special Education at the University of Utah and spent many years as a teacher and case manager working with children and adults with disabilities. Calvin earned B.S. degrees in Behavioral Science and Psychology at Westminster College and the University of Utah. He also holds a M.Ed. degree in Special Education and M.S. degree in Information Technology from the University of Utah and Capella University. Calvin worked in a variety of fields including teaching, corrections and case management.

We are now retired and enjoy the peace and beauty of the forest in our new home on the Olympic Peninsula in Washington. We live with two spoiled cats and plenty of wildlife. We are presently writing our next books and continue to work daily to help others with PP. In his spare time, Calvin enjoys working with wood and tends our organic garden while I enjoy genealogy research and reading historical romance novels. We have three children and five grandchildren.

Please join us often for a new lesson or story related to Periodic Paralysis. Please feel free to ask questions and share your experiences with us.

If you have Periodic Paralysis or suspect you have it and would like to join us on our education and support group,  please use the following link:

https://www.facebook.com/groups/periodicparalysisnetworksupportgroup/

For more information please visit our:

Website: www.periodicparalysisnetwork.com

Website Facebook Page: https://www.facebook.com/PeriodicParalysisNetwork


Until later....