The Connection Between Mitochondria and Autoimmune Disease in Periodic Paralysis

The Connection Between Mitochondria and Autoimmune Disease

I wrote about the connection between mitochondrial issues and autoimmune dysfunction in the new book, “The Periodic Paralysis Guide And Workbook: Be All You Can Be Naturally.” I did not go into the research and the information that led me to my findings. Although I had written about this for the members in our PPNI Support Group, I felt it was important to add that information to our blog for others to read about and understand.

I am now posting what I wrote last year.

November 3, 2013

Yesterday I spent a great deal of time researching the connections between Periodic Paralysis and lactic acidosis. I did this because of several reasons, but mostly due to the results of a set of tests done a few years ago, that no doctor seems to know what it means or what to do about it. 

           

I posted these a few weeks ago but I am posting it again in order to further explain what I discovered yesterday which may be a major break through for us all !

The following is the results of the Amino Acid Plasma Quantitative Test :

Aspartic Acid (<2, 0-6) and 3 Methylhistidine (<;6, 0-64) were low and Proline Plasma (501, 110-360), Alanine Plasma (744, 230-510), Valine (331, 150-310) Plasma, Tyrosine Plasma (107, 45-74), Homcysteine Plasma (11.9, 4.0-12.0), and Pyruvic Acid (0.146, 0.030-0.107) were all high. Lactic Acid was high also (1.8, .5-1.6)

"The high Alanine Plasma indicates: Primary or Secondary Lactic Acidosis or Hyperammonemic Syndrome. Clinical findings may be episodic. Further workup may be warranted."

"Hyperprolinemia is consistent with Mitochondrial Dysfunction or may indicate Type 1 or Type 2 Hyperprolinemia. Further analysis may be warranted."

"Recommend measurement of blood lactate and repeat of the Plasma Amino Acid Analysis."

These test results indicate "primary or secondary lactic acidosis" and Mitochondrial Dysfunction.

My real eye opener came when I read this:

"Metabolic dysregulation can also cause mitochondrial dysfunction. Vitamins, minerals, and other metabolites act as necessary co-factors..." So, wanting to understand how I could have Periodic Paralysis, over 10 autoimmune condition diagnoses AND Mitochondrial Dysfunction, I set out to find some answers. The discovery I made was unbelievable. Karen Carr has mentioned mitochondrial issues on this board for as long as I can remember. I have read through the complicated articles, etc. and I never quite understood the connection. I will try to put this in an easy to understand format, though it may be a little difficult to follow.

First of all...a biology lesson: There are many mitochondria in a cell. Mitochondria can became damaged for many reasons and then mutate or change. When this happens we can then have "Mitochondrial Dysfunction", leading to things such as lactic acidosis.

There are of course, Mitochondrial Disorders that can be present at birth also which I knew. I had no idea that we can "develop" them.

“…..for the synthesis and function of mitochondrial enzymes and other compounds that support mitochondrial function (see Table 4 ), and diets deficient in micronutrients can accelerate mitochondrial decay and contribute to neurodegeneration (Ames, 2004)"

However, since symptoms vary from case to case, age of onset, and rate of progression, mitochondrial dysfunction can be difficult to diagnose when it first appears. According to Cohen, who wrote a July 2001 article in the Cleveland Clinic Journal of Medicine, “The early phase can be mild and may not resemble any known mitochondrial disease. In addition, symptoms such as fatigue, muscle pain, shortness of breath, and abdominal pain can easily be mistaken for collagen vascular disease, chronic fatigue syndrome, fibromyalgia, or psychosomatic illness”

http://www.nbihealth.com/publications/Mitochondrial_dysfunction.pdf

Periodic Paralysis is a 4th Class Metabolic Dysregulation (Disorder)!!!!!! Periodic Paralysis can cause Mitochondrial Dysfunction!!!!!!!!! Obviously the potassium shifting can cause the damage to the mitochondria in the cells!!!!!! We already know there seems to be an issue with autoimmune issues....Here is the connection!!!!

"…diets deficient in micronutrients can accelerate mitochondrial decay"

THIS IS WHY THE BALANCED DIET IS SO IMPORTANT TO US AND WE SEE DIFFERENCES WHEN WE CUT OUT THE JUNK AND ADD SUPPLEMENTS!!!!!!!

"…symptoms such as fatigue, muscle pain, shortness of breath, and abdominal pain can easily be mistaken for collagen vascular disease, chronic fatigue syndrome, fibromyalgia, or psychosomatic illness”  (MUSCLE PAIN, SHORTNESS OF BREATH, FIBROMYALGIA, CONVERSION DISORDER??????)

"Mitochondrial dysfunction is at the core of a surprising range of very common illnesses and conditions...Even autoimmune diseases such as multiple sclerosis, Sjogrens syndrome, lupus and rheumatoid arthritis appear to have a mitochondrial basis to illness.

Mitochondrial dysfunction has been associated with a wide range of solid tumors, proposed to be central to the aging process, and found to be a common factor in the toxicity of a variety of physical and chemical agents."

http://www.umdf.org/site/pp.aspx?c=8qKOJ0MvF7LUG&b=7934637

"EVEN AUTOIMMUNE DISEASES"!!!!!!!!!!!!!

"…toxicity of a variety of physical and chemical agents"    (The ISSUES WE HAVE WITH ALLERGIES AND PROBLEMS WITH MEDICATIONS????)

Table 1

Signs, symptoms, and diseases associated with mitochondrial dysfunction

(Cohen and Gold, 2001)

Organ system: Possible symptom or disease

Muscles: Hypotonia, weakness, cramping, muscle pain,

ptosis, opthalmoplegia

Brain: Developmental delay, mental retardation, autism, dementia, seizures, neuropsychiatric disturbances, atypical cerebral palsy, atypical migraines, stroke, and stroke-like events

Nerves: Neuropathic pain and weakness (which may be intermittent), acute and chronic inflammatory demyelinating polyneuropathy, absent deep tendon reflexes, neuropathic
gastrointestinal problems (gastroesophageal reflux, constipation, bowel pseudoobstruction), fainting, absent or excessive sweating, aberrant temperature regulation

Kidneys: Proximal renal tubular dysfunction (Fanconi syndrome); possible loss of protein (amino acids), magnesium, phosphorus, calcium, and other electrolytes

Heart: Cardiac conduction defects {(electrical system)} (heart blocks), cardiomyopathy

Liver: Hypoglycemia, gluconeogenic defects, nonalcoholic

liver failure

Eyes: Optic neuropathy and retinitis pigmentosa

Ears: Sensorineural hearing loss, aminoglycoside sensitivity

Pancreas: Diabetes and exocrine pancreatic failure

Systemic: Failure to gain weight, short stature, fatigue, and respiratory problems including intermittent air hunger

http://www.nbihealth.com/publications/Mitochondrial_dysfunction.pdf

"Exercise intolerance is a well recognized clinical feature of mitochondrial respiratory chain defects due to pathogenic mutations of mitochondrial or nuclear DNA. Severely impaired muscle oxidative phosphorylation results in disabling exercise limitations in which trivial exertion produces muscle fatigue and lactic acidosis. In such patients, low levels of exercise cause prominent tachycardia and dyspnoea (shortness of breath) due to increases in cardiac output and ventilation that exceed the capacity of skeletal muscle to utilize the increase in oxygen delivery mediated by these physiological responses"

".....results in disabling exercise limitations in which trivial exertion produces muscle fatigue and LACTIC ACIDOSIS."

EXERCISE INTOLERANCE and LACTIC ACIDOSIS!!!!! from the Mitochondrial Dysfunction!!!!!

"Oxygen utilization and Lactic Acidosis”

“Latic acidosis has often been used as an indicator of impaired oxidative metabolism and as a clinical marker for mitochondrial disorders…elevated lactate values at rest strengthen the possibility of a mitochondrial disorder."

http://ghr.nlm.nih.gov/mitochondrial-dna

LACTIC ACIDOSIS a cause of low oxygen levels.....

"In such patients, low levels of exercise cause prominent tachycardia and dyspnoea (shortness of breath) due to increases in cardiac output and ventilation that exceed the capacity of skeletal muscle to utilize the increase in oxygen delivery mediated by these physiological responses"...........

So...could this be abortive attacks or what we may be mistaking for PP episodes in normal ranges or it just happens and we wonder why...we cannot figure out a trigger????? We take potassium when we do not need to and it causes side effects, hyperkalemia???

"In conclusion, this study reveals a wide spectrum of oxidative limitations and exercise capacities........It illuminates the relationship between severity of muscle oxidative defects and symptoms of exercise intolerance in MM patients. Furthermore, it provides insight into the regulatory mechanisms responsible for characteristic physiological responses to exercise in these patients,"

http://ghr.nlm.nih.gov/mitochondrial-dna

"oxidative limitations" problems with oxygen...fluctuating oxygen levels....going down upon exertion....... 

Most of the body's cells contain thousands of mitochondria, each with one or more copies of mitochondrial DNA. These cells can have a mix of mitochondria containing mutated and unmutated DNA (heteroplasmy). The severity of many mitochondrial disorders is thought to be associated with the percentage of mitochondria with a particular genetic change.

http://ghr.nlm.nih.gov/mitochondrial-dna

So there may be varying degrees and severity among us and I suspect that, of course, as we age the symptoms worsen such as the permanent muscle weakness, breathing issues, etc....Thus the importance of stopping the attacks with diet, avoiding triggers, etc as soon as possible when we are young...hopefully avoiding the damage to the mitochondria over a long period of time.....

MY THEORY: Periodic Paralysis, a mineral metabolic disorder, can cause damage to the mitochondria in the cells due to the atypical shifting of potassium. This damage to the DNA of the mitochondria, in turn contributes to the development of autoimmune disorders.

The longer a person with Periodic Paralysis goes with out a diagnosis and without proper treatment and continual atypical potassium shifting, the more chance there is for damage to the mitochondria, which then creates autoimmune diseases.

Periodic Paralysis is difficult to diagnose. Mitochondrial dysfunction is difficult to diagnose. Symptoms of both appear similar to "conversion disorders".  More time passes. Autoimmune diseases develop further clouding the picture of the basic disease...Periodic Paralysis. Damage is irreversible and becomes progressive causing permanent muscle weakness, oxygen issues, cardiac issues...etc.

November 5, 2013
Good Morning Everyone!

This is about Exercise Intolerance......

When I wrote about my discovery yesterday, I forgot to mention how our exercise intolerance may play into this. So, today I will discuss that connection with:

"MY THEORY: Periodic Paralysis, a mineral metabolic disorder, can cause damage to the mitochondria in the cells due to the atypical shifting of potassium. This damage to the DNA of the mitochondria, in turn contributes to the development of autoimmune disorders."

This is based on the fact that metabolic disorders and other issues can cause damage to the mitochondria in cells (probably due to the atypical shifting of potassium). The DNA of the mitochondria gets damaged. Damaged mitochondria then may begin to develop autoimmune disorders. This is all factual except what is in the parentheses...I am not sure of the process for the damage.

This is all related to how the complicated oxygen cycle works in our cells and bodies. That being said, this is the part forgot:

"Exercise intolerance is a well recognized clinical feature of mitochondrial respiratory chain defects due to pathogenic mutations of mitochondrial or nuclear DNA. Severely impaired muscle oxidative phosphorylation results in disabling exercise limitations in which trivial exertion produces muscle fatigue and lactic acidosis. In such patients, low levels of exercise cause prominent tachycardia and dyspnoea due to increases in cardiac output and ventilation that exceed the capacity of skeletal muscle to utilize the increase in oxygen delivery mediated by these physiological responses (Haller and Bertocci, 1994). "

This says that exercise intolerance is related to mutations of the mitochondria. The oxygen cycle does not work properly and then we are unable to do anything in which we must exert ourselves, even the smallest amount of exertion causes muscle weakness and lactic acidosis and then tachycardia and shortness of breath set in. This is because the heart must work harder to try to keep up with a lack of oxygen the muscles are receiving and (our body is not expelling the carbon dioxide as it should).

 
"......in patients .......peak oxygen uptake and mitochondrial capacity for oxidative phosphorylation decreased in proportion to increasing mutation load in muscle. "

"This study is the first to relate the severity of the skeletal muscle oxidative defect to the severity of mismatch between the exercise increase in cardiac output and oxygen uptake."

As we exert ourselves and need more oxygen, we do not get it. The more exertion, the less amount of oxygen.

"This study demonstrates for the first time that exaggerated ventilation relative to oxygen utilization ........ is related to the degree of oxidative impairment. Hyperventilation was more pronounced in patients with more severe oxidative defects, consistent with symptoms of exertional dyspnoea experienced by many patients. The mechanism underlying this hyperventilatory exercise response may relate to excess carbon dioxide production due to lactate buffering, as suggested by the finding of a correspondingly exaggerated respiratory exchange ratio..."

http://brain.oxfordjournals.org/content/126/2/413.long

The more we exert ourselves the more carbon dioxide builds up.

This may be the reason for our exercise intolerance.

This may be why also, when you go to the doctor and they take your blood oxygen level with the finger oximeter it is just fine, because you are sitting and have been for awhile, but you know you are having problems with your breathing, etc they do not think you need oxygen. They need to test you with an overnight or 24 hour recording oximeter. It should show oxygen level decreases during exercise and episodes of paralysis, etc....any exertion.

I am on oxygen 24/7 because my oxygen drops any time I exert myself, even talking on the telephone, going to the bathroom, etc at the same time, my heart rate increases and breathing gets difficult. This is happening even with my oxygen. I can only imagine how bad it would be without my oxygen.

Before I was on it, I could not even sit up to a meal at the table. It was too much exertion. Even now, sitting up straight for any length of time is too taxing...why I have a reclining feature on my power wheelchair.

I hope this information is helpful to you. It explains a lot of what we are all experiencing everyday and not fully understanding it or how it relates to Periodic Paralysis.

I do not know if anyone has put this together like this. I did it quite accidentally. It does seem to make sense. I forgot to mention that the lactic acidosis thing is also something that would be intermittent and may not show up on tests each time. It also needs to be tested using a very rare test: Amino Acid Plasma Quantitative Test it is only interpreted at a few places in the country. One is: Duke Children's Hospital and Health Center in North Carolina

A thought.....If our body is in stress from the lack of appropriate oxygen to the muscles and build up of lactic acidosis and carbon dioxide...would the STRESS be the CAUSE of the paralysis from exercise or exertion and why it happens after we begin to rest after our running around, walking, shopping, cooking dinner, doing dishes, etc?? As we begin to rest, the adrenaline will be released from the stress?? The adrenaline causes paralysis for most of us. 

Until later...

Pharmaceuticals Are Not the Answer For Some Rare Diseases

Hello All,

I had another great day!!!! I was able for the first time in three years to have Calvin drop me off somewhere and be able to drive away. He drove me to the cottage on the beach where my daughter and son-in-law were staying, visited for a while and then drove to town to run some errands. I had my portable oxygen, which lasts for about 4 hours. The cottage had a huge bay window looking out into the Straight of Juan de Fuca. We sat in front of a roaring fireplace and visited as we saw Victoria, Canada across the bay and watched the ships as they passed.

I realized, however, that although I did not have that horrible fog I had been experiencing any longer, I still have issues with exercise intolerance. Just talking for a while and then having a snack proved to be too much, I went into arrhythmia and become weak, but I avoided paralysis. I had to be very careful after that, however.

Calvin told me later, that other than leaving me in the hospital a few times, it was the first time in three years he felt he could leave me like that. He said he felt sad driving away, but he knew I was in good hands and it was good for him and him to do that.

My visitors must leave today, I will miss them very much, but I will get back to my "work" of teaching the world about Periodic Paralysis.

 
Today, I want to share an article I wrote a few months ago. 

Pharmaceuticals Are Not the Answer For Some Rare Diseases

Some rare diseases cannot use pharmaceuticals for treatment. There are other options, but there is no help to obtain them.

I have a disease called Periodic Paralysis. It is a very rare, debilitating, hereditary and difficult to diagnose disease. I was 62 when I was finally diagnosed, 6 years ago. It is a mineral metabolic disorder called an ion channelopathy. It is often misdiagnosed and mistreated, thus causing more damage or possible death to the person with it. There are several forms of it and the type I have is the most rare and the most serious type.

 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 during which severe life-threatening symptoms may occur. Due to these complications, it is extremely important to avoid the episodes. Permanent muscle weakness may occur over time. If it affects the breathing muscles it can become terminal.

There are no known cures, but there are treatments and drugs for some forms, which can be and are successful for some individuals.

My husband and I are the co-creators and managers of an independent organization, the Periodic Paralysis Network (PPN), 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, a discussion board and support group on Facebook to help others with support and the natural methods (pH balanced diet, supplements, oxygen) we have discovered.  We also provide methods and ideas on how to find doctors, get a diagnosis, get the proper help in the ER, how to discover their triggers, and much more.

Many patients have difficulty getting diagnosed and treatment in the US and the same problem exists around the world. We have people contact us nearly every day from all over the world, Iran, Ukraine, Turkey, Denmark, Wales, Netherlands, Canada, Finland, Australia, Mexico, to name a few, who are seeking help for themselves, their children and entire families and are unable to find it anywhere.

For some, medication can help but they cannot get medication. Some need a diagnosis but cannot find doctors who know enough to diagnose it. Many, many people are suffering with this disease and getting no help. The quality of their lives is being destroyed and some are even dying, needlessly.

 With this information in mind, a few months ago, in my position as manager of the PPN, I received an email from an organization, which is described as a driving force behind programs that provide services for patients with rare diseases. The email was seeking input about improving the methods and time frame for processing pharmaceuticals for treating rare diseases.

Though I am not a doctor, researcher or physicist and had some difficulty understanding some of what was written; I understood enough to form some comments and then added a few questions regarding these issues. I quickly wrote a response hoping for some information about the possibility of services for “patients” with Periodic Paralysis, a rare disease. I wrote the following letter.

”I understand that this process has been designed to expedite the discovery, research and use of new medications or pharmaceuticals in rare, very rare and extremely rare diseases. This is definitely something I can agree with. There is a need to expedite the process as many of us are dying. I happen to be one of those people due to a lack of treatment. I agree also with the key-considerations of the benefits and risks of a faster time frame because there must be safeguards in place.

That being said, the condition I have is an, as yet, undiscovered variant of Andersen-Tawil Syndrome (ATS), which is probably unique to my family and me. Our DNA is being studied at Baylor, therefore, it an extremely rare form of Periodic Paralysis, which is an inherited ion channelopathy. Part of the problem with expediting the making of medications to treat a condition, is that it is useless for me and others with ATS because most of the patients with ATS, are not able to take any medications or pharmaceuticals because they are actually triggers for our severe symptoms of paralytic episodes, which include partial to total body paralysis, difficulty breathing, low oxygen levels, heart arrhythmia including dangerous/sometimes fatal long QT interval heart beats and torsades de pointe, fluctuating heart rate, fluctuating blood pressure, cessation of breathing, choking, possible cardiac and/or respiratory arrest.

What we need is a way for timely and better diagnosing so treatment can begin early thus avoiding an outcome like mine; getting a diagnosis at the age of 62 for a disease I had my entire life. I was misdiagnosed over and over and treated with pharmaceuticals, which almost killed me and have left me totally disabled and terminal with no treatment as I progressively decline.

At this time, Periodic Paralysis is diagnosed by a process of elimination, which takes years, in my case a lifetime, and either clinically; based on symptoms and characteristics, which can be subtle or absent or genetically; genetic testing which can take years and is only done in a few places in the world. The problem with these options is, first, most doctors and even the specialists do not understand it well enough to diagnose clinically, and second, the genetic testing will only reveal about 50% to 60 % of the overall types of PP/ATS. The remaining 40% to 50% of forms are, as yet, unknown. An individual with an unknown variant has symptoms indistinguishable from those with the known mutations.

We need something better than this. Many people are dying from the improper medications due to misdiagnosis and by provoking their symptoms in an attempt to get a clinical diagnosis.

Genetic research has been in place for over 25 years. Blood samples from all over the world are studied and then in a year, 2 years, 4 years, or one family has been waiting 9 years, an individual will get his letter stating he has no known form of PP. This wrongly sets up the patients to believe that they do not have PP, though as stated above, 40 to 50 % of them have a variant, which has not yet been discovered. In the meantime there is no help and no treatment until or unless a mutation is discovered. To date I know of no known treatment from these studies and no faster or better method for diagnosis.

This has to stop!!! There must be a better way, a set of better guidelines, better education of medical professionals to be able to diagnose this cruel disease in a more timely manner, and perhaps rather than funding for the discovery of only 50% of genetic codes the money can better be used to research for proper diagnosis, treatment and management.

There are doctors who are associated with an organization specifically for researching and treating diseases affecting muscles. Although Periodic Paralysis is not a muscle disease but rather a 4th class mineral metabolic disorder, it is accepted as one of their diseases. These doctors are supposed to know about and treat PP. However, but most of them, (I have been to 4 of them), did not diagnose it in me and in fact set my diagnosing back years, thus contributing to my present condition, totally disabled and terminal. Again, I suggest if these specialists and this organization are receiving funds for diagnosing, treating and research of PP, then that money should be going elsewhere for those of us with PP or for proper education of those doctors to recognize PP/ATS based on present day criteria.

There are other types of PP in which some drugs are used with good results, for a few, but not without short term and serious long term side effects,  but for the more rare forms, we are out of luck.

My husband and I have written a book about Periodic Paralysis and discuss these issues in it. Living with Periodic Paralysis: The Mystery Unraveled was published last month. This was in part due to a program coordinator for the organization treating muscle diseases telling me that I need to teach their doctors about Periodic Paralysis! We also have a website, the Periodic Paralysis Network and forum for individuals who have PP and most of our members are unable to get a diagnosis, or treatment. They are living in limbo with no help from the medical field. We have created a plan that helps us naturally with diet, avoiding triggers and by monitoring our vitals, especially monitoring our potassium levels.

There is a device, a potassium reader, which we use to help us to know how to treat our symptoms based on the level of potassium in our blood. Unfortunately, this device costs $350.00 and is not a medical device so insurance will not pay for it. Most families do not have that kind of money, thus making monitoring of symptoms very difficult.

Rather than medications, we use those devices to help us relieve our symptoms and stay alive by avoiding paralytic episodes. We could use funding for those devices and working with the FDA and the company that makes the devices can help us to have them deemed as medical devices so insurance will pay for them.  We are not yet incorporated but hope to be soon, with funding from the sale of our book. We hope to be able to raise money to purchase potassium meters for those who need them.

Pharmaceuticals are not the answer for some rare diseases. The above issues are the type of help we need. Do any of our concerns or issues meet your requirements to receive assistance under your “commitment to fulfill unmet need for patients” with rare diseases? I would love to work with you if that is the case.”

I continue to wait for a reply, although today, I received another e-mail from them asking me to write a letter or call my representative in Congress in order to pressure them to pass the now completed Bill.

All the while I am hooked up to an oxygen tank, confined to my bed, recliner or power wheelchair, with my computer in my lap as I continue to work to improve the lives of individuals with all forms of Periodic Paralysis. I am creating a set of better guidelines for diagnosing PP, writing my next book for better education of individuals of with PP and medical professionals, attempting to raise awareness of PP in the world, marketing my book to raise money for potassium readers as I am in and out of paralysis and muscle weakness. You see; pharmaceuticals are not the answer for some rare diseases. Who will help us? Who will take my place when I am gone?

Until later...

Update 12/19/2016:
We now have written and published four books related to Periodic Paralysis.

Potassium and Potassium Meters November 14, 2013

Hello All,

I made it through another day!! Although I was able to get everything completed and added to the new blog, I ended up having a difficult afternoon. I suddenly had chest pains and overall weakness. I first took my blood pressure and found it was suddenly high even though I was sitting in my recliner and had been for over an hour. My heart rate was up. I checked my blood sugar level and found it was high. These were all signs that my potassium levels were low, so I checked my potassium level with my potassium meter and found I was indeed low. I took some potassium bicarbonate, and within thirty minutes, I began to feel a little better and never went into an episode of paralysis.

However, I never did regain my strength and did not feel strong enough to get up for dinner so Calvin brought it to me. I was thankful it was mostly soft food because I did not have the strength to chew much and because earlier in the day, as I was eating breakfast, my jaw bones near my temples began to have severe pain as I chewed and I was unable to complete my meal. I had soft food for lunch also. I will have to eat soft food again today....

Yesterday, in our educational support group, there was quite a discussion about the purchase of potassium meters and a newer device for measuring our potassium levels in our saliva or blood. They work much like a glucose meter, which measures the level of sugar in our blood. However, the two biggest differences are; the meters are not medical devices and are very expensive. The original device, which is no longer being manufactured, was $250.00 and the new potassium meter is $350.00. Because they are not medical devices, insurance will not pay for them. This is a large issue for those of us with Periodic Paralysis, because potassium shifting is the bottom line of our condition. When it shifts, we become either weak or paralyzed. We need to know if our potassium level is high, low or in normal levels, so we can know how to treat our symptoms.

I wrote the following to the company to explain why and how we need and use potassium meters and why the price needs to be lowered and why it would be a good thing to have it declared a medical device:

"Dear Sirs:

I have a very rare hereditary disease called Periodic Paralysis (PP). The type I have, a variant of Andersen-Tawil Syndrome (ATS) Type 2,  is the most rare type of this condition. It is an ion channelopathy. It affects the body on the cellular level.

Those that have this condition have a problem with potassium entering the muscle cells in error. When this happens the muscles weaken or become paralyzed and it can last from a few minutes to several hours to days at a time. As the muscles are paralyzed, it effects the heart causing life-threatening arrhythmia (including Long QT interval heartbeat), tachycardia or bradycardia, fluctuating blood pressure, low oxygen levels, choking and the possibility cessation of breathing,  cardiac arrest and /or respiratory arrest.

Individuals with PP have a myriad of triggers which can set this into motion: certain foods, stress, exercise, medications, sleep, salt, sugar, wheat, gluten, heat, cold, IVs, anesthesia and more. When we eat or experience a trigger, it causes the potassium in our body to either increase or decrease or just shift in normal ranges. When either of these happen,  the weakness or paralysis and serious symptoms begin. So, we must discover and know what the triggers are in order to avoid them. Sometimes we can do everything right and we can still have episodes of paralysis.

When we begin to feel symptoms, we can use a potassium meter device that can let us know if our potassium is high, low or normal and this allows us to know how to treat it or a caregiver to know how to treat us or whether they need to call for an ambulance. Unfortunately, this device is not a medical device and as long as this device is not a medical device, insurance companies will not pay for it. You now have a new device which is apparently easier to use but costs $350.00. Most families cannot afford this.

If made into a medical device, your sales would increase because they would be paid for by medical insurance and every family with an individual with Periodic Paralysis that needs one could get it. As well, every paramedic, ER, hospital, doctor's office and school nurse could then have one, paid for by the company they work for, and be trained on how to use them so they can know instantly what is happening with the person's potassium levels for quick treatment. Individuals may die while they wait for traditional blood tests to come back from the lab with the results.

I have a website and discussion and support groups to help others with this disease. We have members from all over the world and most of them cannot afford  this device and suffer daily in paralysis and the other life threatening symptoms I discussed. Many of them have low to no quality of life. This device would help by knowing sooner whether they need to take potassium or avoid it or for their caregivers to know if they need to go to the hospital, etc.The quality of life could improve for many individuals with Periodic Paralysis. Sales would increase because there is a world-wide need.

Please visit my website to learn more:    www.periodicparalysisnetwork.com

Thank you"

I am now in contact with employees from this company. They have been very helpful and we are in discussion over these issues. We remain hopeful that changes can be made making this device more affordable and accessible.

Have a good day!

Until later...