Serene Forest

Wednesday, February 26, 2014


Hello All,

Yesterday someone asked me a question about the urgency to urinate after or as you are coming out of an episode of weakness or paralysis. The answer to this question is yes, this is a very common part of the process. This is due to the body retaining fluids during an episode.

This person also mentioned a problem with urine after an episode being dark brown and having an odd smell. Now this is not normal and the sign of a serious condition. In case others of you may have this happening I wanted to share my answer with you.



It is common for one to not have an urge to urinate until after the episode and when coming out of the weakness or paralysis. This is because the fluids in the body are elsewhere being retained during the episode.

 I always have an urge to urinate quite badly but it is always difficult for me to move or walk so my husband must get me there by way of my wheelchair and help me onto the toilet. This is the most embarrassing thing for me, though we have been married for nearly 34 years.

 The thing that concerns me is the color you mention. That is a sign of a serious condition called Rhabdomyolysis, or muscle wasting. Do you take statins? Statins are a main cause of this. If not then it is even more serious. You should have it checked out right away.

Those with the genetic mutations found in
CACNA1S, forms of Hypokalemic Periodic Paralysis are more susceptible to it."
According to Wikipedia:

is a condition in which damaged skeletal muscle tissue breaks down rapidly. Breakdown products of damaged muscle cells are released into the bloodstream; some of these, such as the protein myoglobin, are harmful to the kidneys and may lead to kidney failure. The severity of the symptoms, which may include muscle pains, vomiting and confusion, depends on the extent of muscle damage and whether kidney failure develops.

Release of the components of muscle tissue into the bloodstream causes disturbances in electrolytes, which can lead to nausea, vomiting, confusion, coma or abnormal heart rate and rhythm. The urine may be dark, often described as "tea-colored", due to the presence of myoglobin. Damage to the kidneys may give rise to decreased or absent urine production, usually 12 to 24 hours after the initial muscle damage...

...Causes: ...hypokalemia (low potassium levels)". Rhabdomyolysis - Wikipedia, the free


Until later...

Sunday, February 23, 2014

Over 150 Members Now!!!

I wanted to let everyone know that the Periodic Paralysis Network (PPN) now has over 150 members!!!! Congratulations to us all!!!! Thank you all for your support!!!!

Metabolic Acidosis and pH Balance

Hello All, 

Yesterday I had a very rough day. I am, however, doing better today. I did end up in paralysis and after coming out of it I was very weak and well you know the rest of that story! I did discover that I was in metabolic acidosis and had very low potassium so treated it with my potassium bicarbonate and sodium bicarbonate.


For awhile, I have been lazy and not used my pH meter. It is a little difficult to use, especially when I am not feeling well and on the edge of paralysis. So I discovered that Calvin had ordered the strips quite awhile back and did not realize we had them. I accidentally found them the other day.

When I woke up yesterday after 9 hours of sleep, a very unusual thing for me, and I felt really strange. I took my vitals and used the pH testing strip. I almost fainted when it was bright yellow and at 5.5 or lower. I knew immediately what to do but I went into paralysis anyway. After coming out of it, I continued to take more potassium and bicarbonate through the day and ate nothing that had any acidity whatsoever. By bedtime I was back in a better pH range and it held through the night.

So, my advice to all of you is to get some of those strips and also monitor your pH balance. PP is a mineral metabolic disorder which makes us prone to metabolic acidosis. When trying to figure out our triggers, the pH balance must be considered.

That is why we recommend the pH balance diet. It makes a huge difference if I follow it faithfully. I went from four or five full-body, severe episodes, lasting several hours each, a day to one or two a month which are less severe and lasted a shorter time.

If I had not realized the pH balance issue yesterday, with the levels I was at, I could have died. This is a very important issue for all of us. 

This is a good link about the pH balanced diet:
Thanks for your continued support.

Until later...

Sunday, February 16, 2014

Periodic Paralysis and Anesthesia

 Hello All, 

This is an article written and created with a compilation of information, excerpts, references and links related to the problems and issues of the use of anesthesia with individuals with the various forms of Periodic Paralysis. There is quite a bit of technical information, which can be shared with your doctor, anesthesiologist or dentist (though most of them should know this information).

  Periodic Paralysis and Anesthesia

 Article and Compilation

The Periodic Paralysis Network (PPN) February 2014

Periodic Paralysis (PP) is a mineral metabolic disorder, also known as an ion channelopathy, which is a dysfunction of the ion channels. There are four types of PP, Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis, Normokalemic Periodic Paralysis and Andersen-Tawil Syndrome. Ion channels transport the electrolytes, such as sodium and potassium through the cells. This transport is faulty in individuals with ion channel dysfunction and extreme care must be used when anesthesia is going to be utilized. This is due to the possibility of developing serious symptoms such as breathing issues or failure, arrhythmia, blood pressure issues, choking, muscle weakness or paralysis, longer recovery after surgery, malignant hyperthermia or death. Managing the use of anesthesia in individuals with Periodic Paralysis is mostly aimed at preventing attacks of paralysis or the other symptoms during or after surgery. The manner in which the situation is handled for the individual depends on which form of Periodic Paralysis is involved. 1, 2, 3, 4, 5

Hypokalemic Periodic Paralysis and Anesthesia

For individuals with Hypokalemic Periodic Paralysis, anesthesia is a known trigger for paralytic episodes. According to research, in order to successfully manage the patient there is need for an evaluation before surgery, avoidance of known triggers, careful monitoring during the surgery and immediate and proper treatment if an issue arises.
According to the National Journal of Maxillofacial Surgery, “pre-operative stress along with necessary fasting and administration of dextrose containing fluids” precipitates attacks. “The guidelines for care include control of plasma potassium, avoidance of large glucose and salt loads, maintenance of body temperature, acid-base balance, and careful use of neuromuscular blocking agents. Good pre-medication to allay anxiety, avoidance of stress and adequate analgesia is vital in preventing an attack. Fluctuations in electrolytes, infection, and pain can lead to paralysis in the post-operative period. Hypokalemia manifests earlier than paralysis and so its correction can prevent paralysis. Dextrose containing solutions administered during surgery should be avoided...” 6, 7, 8

This information was found in Miller’s Anesthesia written by Ronald D. Miller:

”Management of HypoPP patients should focus on avoiding triggers and medications causing shift of potassium. General anesthesia, postoperative stress, glucose-containing intravenous solutions, and long-acting neuromuscular blockers are associated with postoperative paralytic events.138 Epidural analgesia has been shown to reduce both pain-related hyperventilation and serum catecholamines, thereby minimizing changes in serum potassium levels.138” 4

Hyperkalemic Periodic Paralysis and Anesthesia

Nothing was written about the use of anesthesia and Hyperkalemic Periodic Paralysis before 2002. Early research concluded that anesthesia might be used without complications if the potassium levels were with-in normal levels prior to surgery, if the carbohydrate levels were up, if anesthetic drugs, which released potassium, were not used and if normal body temperature levels were maintained.

 This information was found in Miller’s Anesthesia written by Ronald D. Miller:

”Potassium, cholinesterase inhibitors, and depolarizing muscle relaxants will aggravate the myotonia in HyperPP patients.65 Prolonged muscle weaken ss has been reported when succinylcholine is used.141 Although one third of patients had no signs of myotonia,142 masseter spasm and respiratory and skeletal muscles stiffness could still occur during intubation and ventilation.65 Therefore, neostigmine and succinylcholine should be contraindicated in HyperPP patients. Ideally, all patients with HyperPP need to be admitted preoperatively so that proper preoperative fasting can be accompanied by the administration of dextrose-containing potassium-free maintenance fluid.143 Postoperatively, HyperPP patients may remain paralyzed for up to several hours. Preventive measures such as maintaining normal body temperature and low serum potassium levels and avoiding hypoglycemia are helpful in limiting such paralysis.144 Although patients with sodium channel pathology have often been considered to be susceptible to MH, (Malignant Hyperthermia) there is no increased risk for MH in these patients.145 General anesthesia with and without nondepolarizing muscle relaxants has been shown to have satisfactory outcomes.141,143,144,146 Regional techniques may also be appropriate for this patient group.142,146 Abortion of the hyperkalemic attack may be accomplished by administering glucose, insulin, epinephrine, and calcium supplements, or alternatively, glucagon may be used. β-Adrenergic treatment with metaproterenol has also been shown to prevent attacks and facilitate recovery.” 4,9,10,11

Malignant Hyperthermia (MH)


As mentioned previously, individuals with Periodic Paralysis are at risk for developing malignant hyperthermia during or after surgery. All forms of Periodic Paralysis are the result of mutations on Chromosome 17. Malignant hyperthermia is also the result of a mutation on Chromosome 17, thus creating the potential for those with Periodic Paralysis, including, Normokalemic Periodic Paralysis, to develop the serious and life-threatening symptoms involved with the use of anesthesia. 12, 13
The following information about MH is an excerpt from an article from CINCH (Consortium for the Clinical Investigation of Neurologic Channelopathies), which may be shared with physicians, dentists and anesthesiologists:


Malignant Hyperthermia

“Patients with ion channel diseases are at increased risk of malignant hyperthermia reactions with general anesthesia for surgery. In malignant hyperthermia, muscle cells become overactive (hypermetabolic) in response to the most commonly used drugs for general anesthesia. With inhaled agents (such as halothane, isoflurane, enflurane, desflurane, sevoflurane and even agents such as ether, cyclopropane and methoxyflurane ) the ion channels in the muscle cells open and remain open. This results in excessive calcium release causing muscles to continuously contract, producing massive amounts of heat, rapid rise in temperature and disruption of nearly every organ system in the body. In the past this reaction was fatal in 70% of cases. However, with the introduction of the medication, dantrolene, the mortality has now fallen to 5% of cases. It is essential that anesthetists and surgeons are made aware that a patient has periodic paralysis or even that a parent suffers with the disorder, if a son or daughter is going for surgery. The anesthetists and surgeons should be familiar with neuromuscular disorders and the associated risks. If they are not in a specialist center, the patient may need to be transferred to such a center or at the least the patient's neurologist should be contacted prior to surgery.

Prolonged Paralysis
Other medicines used during general surgery are called depolarizing agents (such as Succinylcholine). These medicines act by blocking the signal from the nerve (Acetylcholine) from reaching it's receptor on the muscle. This causes temporary paralysis in patients during surgery, relaxing the muscles to make surgery easier. In patients with ion channel disorders, such as periodic paralysis, this can result in prolonged paralysis.

Normally, when people are given the drug succinylcholine to relax muscles, a little bit of potassium from inside the muscle cells leaks out into the bloodstream. This is because succinylcholine, like acetylcholine, docks on the receptors and opens some gates in the cell, letting certain molecules flow in and out. Normally, this poses no problem. In the periodic paralyses, ion channels that normally open when acetylcholine docks on membrane receptors don't function normally, and people experience temporary paralysis attacks in response to a variety of dietary, exercise, stress-related and other environmental changes.

After surgery, patients with periodic paralysis have found they woke up in the recovery room and couldn't move; they may not get full strength back for hours or days. The exact cause of the prolonged paralysis with anesthesia in some people with periodic paralysis isn't known. It could be the stress of the surgery and/or any of the anesthetic drugs. It is critical for the medical team to distinguish between a periodic paralysis attack and a malignant hyperthermia reaction as the treatments are completely different.”  14, 15, 16

Important link for more information related to Malignant Hyperthermia"

Andersen-Tawil Syndrome and Anesthesia

Some research indicated that malignant hyperthermia is not usually an issue for individuals with Andersen-Tawil Syndrome. However, it is an issue because individuals with ATS have shifting of potassium into both high and low ranges causing symptoms and paralysis. The other issue with anesthesia use and ATS is a need for special precautions due to the serious issue of the long QT interval heartbeat, a diagnostic marker for the condition and Torsades de Pointes another extremely serious arrhythmia. There are many medications that must be avoided, which are used routinely in preparation for surgery and during surgery including the glucose and sodium IVs, as well as most forms of anesthesia.7

General Anesthestic considerations for patients with LQTS Management

From Drexel University:

Avoid triggers of QT prolongation and Torsadesde Pointes (TdP)
Provide Peri-op:
Avoid hemodynamic extremes:
Correct serum electrolytes esp:
Prophylaxis beneficial even with normal serum concentrations:
Prevent and treat arrhythmia:
Continue ECG monitoring in more than 1 lead
If ICD/pacemaker, ensure proper functioning
Have defibrillator and temporary pacemaker available
Consult cardiology as needed 18

More important information available for ATS and Anesthesia at:

Topical, regional and local anesthesia may cause potassium to drop in individuals with Hypokalemic Periodic Paralysis, Normokalemic Periodic Paralysis or Andersen-Tawil Syndrome if it contains epinephrine. The most often discussed and utilized local anesthesia is lidocaine. For some individuals it may work well if the epinephrine is removed. For others it may cause hypokalemia or arrhythmia regardless of the epinephrine being removed. For others still, it may not work at all or the usual amount may be needed during a procedure. Lidocaine and other local types of anesthesia need to be used with extreme caution.4

In Summary

“Although neuromuscular diseases are relatively uncommon, patients with these conditions will present to the operating room and to non-operating room procedure areas for diagnostic studies, treatment of complications, or surgical management of related or unrelated disorders. Overall debility, with diminished respiratory muscle strength and increased sensitivity to neuromuscular blockers (NMBs), predisposes these patients to postoperative ventilatory failure and pulmonary aspiration, and may slow their post-procedure recovery because of difficulty with ambulation and increased risk of falling. A basic understanding of the major disorders and their potential interaction with anesthetic agents is necessary to minimize the risk of perioperative morbidity.”19

”The anesthetic management of patients with periodic paralysis first involves knowing the patient's history and their particular disease characteristics. The concurrent diseases must be ruled out (such as Andersen's disease). The primary goal of the anesthetic is to avoid events (perioperatively) that are known to precipitate muscle weakness. Electrolytes should be normalized, hypothermia should be avoided and frequent monitoring of the serum potassium level is indicated. The ECG should be constantly monitored for signs of arrthymias. These patients can be considered at risk of MH, thus avoidance of MH triggers is indicated. Use of nondepolarizing muscle relaxants is thought to be acceptable, although abnormal sensitivity to these agents may be encountered and adequate muscle strength must be assured prior to extubation.” 20

Anyone with Periodic Paralysis needs to be extremely cautious when planning any surgical procedures, which may use anesthesia.

References and Links


Periodic Paralysis general info about care with meds and treatment


Hypokalemic Periodic Paralysis

Hyperkalemic Periodic Paralysis

Malignent Hyperthermia

Andersen-Tawil Syndrome


Until later...

Added September 4, 2015:PROLONGED PARALYSIS

Other medicines used during general surgery are called depolarizing agents (such as Succinylcholine). These medicines act by blocking the signal from the nerve (Acetylcholine) from reaching it's receptor on the muscle. This causes temporary paralysis in patients during surgery, relaxing the muscles to make surgery easier. In patients with ion channel disorders, such as periodic paralysis, this can result in prolonged paralysis...

..After surgery, patients with periodic paralysis have found they woke up in the recovery room and couldn't move; they may not get full strength back for hours or days. The exact cause of the prolonged paralysis with anesthesia in some people with periodic paralysis isn't known. It could be the stress of the surgery and/or any of the anesthetic drugs. It is critical for the medical team to distinguish between a periodic paralysis attack and a malignant hyperthermia reaction as the treatments are completely different.

Added February 25, 2017

Advances in Anesthesiology
Volume 2015 (2015), Article ID 562378, 6 pages

Review Article

 Mutations in Sodium Channel Gene SCN9A and the Pain Perception Disorders

Friday, February 14, 2014

Some Forms of Periodic Paralysis are Worsened by Diamox

Hello All,

While researching I found out some interesting information about Hypokalemic Periodic Paralysis. A study from 2001 relates two different types: CACNL1A3=Hypokalemic Periodic Paralysis-1 and SCN4A=Hypokalemic Periodic Paralysis-2, each with several mutations.

When someone is diagnosed with Hypokalemic Periodic Paralysis, clinically, (based on their symptoms), one must be very careful. One form either does not respond to diamox (acetazolamide), causes more paralysis or causes serious symptoms. So, diamox should not automatically be given to people clinically diagnosed with Hypokalemic Periodic Paralysis or if it is, it should be used with extreme caution.

From the article:

 “In conclusion the present study demonstrates that genetic characterization of hypoPP patients is important to decipher the clinical and histopathological features of the disease, and to predict the response to therapy. We suggest that mutations in the SCN4A gene should be systematically sought in hypoPP patients suffering from paralytic attacks followed by myalgias or worsened by acetazolamide, and when muscle biopsies reveal the presence of tubular aggregates.”

Until later….

Thursday, February 13, 2014

No Tourniquet Please!!!

Hello All,

I wanted to write about an important issue today. It was called to my attention recently that not every individual with Periodic Paralysis knows that using a tourniquet when having blood drawn can result in potassium levels which are higher than they really are. It is important to understand that improper use of a tourniquet and the clenching of the fist can result in false lab results for potassium levels. The pressure (too tight) and time (too long) of the tourniquet can raise the level of potassium as much as 10% to 20%. This difference can be important when making a decision about treatment or trying to get diagnosed. The following articles explain this problem.

"Pseudohyperkalemia is typically caused by hemolysis during
venipuncture (by either excessive vacuum of the blood draw or by a
collection needle that is of too fine a gauge); excessive tourniquet
time or fist clenching during phlebotomy (which presumably leads to
efflux of potassium from the muscle cells into the bloodstream);[4] or
by a delay in the processing of the blood specimen."

"Pseudohyperkalemia Caused by Fist Clenching during Phlebotomy"

"Excessive tourniquet time, too tight tourniquet or fist clenching
during phlebotomy (which presumably leads to efflux of potassium from
the muscle cells into the bloodstream) are other important cause of
fictitious hyperkalemia.";year=2007;volume=11;issue=4;spage=215;epage=217;aulast=Sharma

But I guess we should ask first "When have you calibrated pressure
gauge of the tourniquet?" (unless the nurse tend to lose temper...)

It is imperative for one with Periodic Paralysis to know the above information whenever blood is drawn. Ask the technician or nurse to draw the blood without a tourniquet. Be sure to have this important information written and handy in case of an emergency or an ambulance must be called and you are unable to speak. I keep this information in a plastic folder along with everything I know is important and that the paramedics must know when coming to my aid in an emergency. I approach it as if I will have no one with me to explain my needs. I keep it near the door and take it with me when I leave home.

Until later…

More links:

Monday, February 10, 2014

Happy Anniversary to US!!!

Hello All,

 Happy Anniversary to us!!!!!

I celebrated the third year anniversary of my clinical diagnosis of Andersen-Tawil Syndrome, on February 7, 2011. That is the date we consider the beginning of the Periodic Paralysis Network. With a diagnosis in hand, we knew we needed to tell the world about Periodic Paralysis and share our story. Over time, we wanted to help others get a diagnosis and share the "Plan" we created of lifestyle changes and and other things we discovered that saved my life and lessened the severity and amounts of my episodes of paralysis. We wanted to create a place that others with Periodic Paralysis, regardless of the type they had and regardless of they whether they had a diagnosis or not, could join and receive support, friendship, education and advocacy and to feel that they were not alone. We have definitely grown since that day...a website, a support and education group with nearly 150 members, a blog, a board specializing in PP and genealogy, several Facebook pages, a Pinterest site, a book and book discussion board (and two more being written), a project to create a better set of criteria for diagnosing PP (in progress), using a four part comprehensive survey, and the recognition as a medical advocacy group by the national, nominated by WEGO Health for awards in the categories of Health Activist Hero, Best in Show Facebook, and Best in Show Blog.  We are very proud of what we have accomplished and what we have become and hope to continue for a long time to come. Thank you all so much for making this a reality and thank you for your support!!!!!

Calvin and Susan Hunter

Saturday, February 8, 2014

The Truth About Diagnosing and Treating Periodic Paralysis

Hello All,

This morning one of our new members expressed, sadness and frustration over the fact, that after searching for a diagnosis and proper treatment for her obvious Periodic Paralysis (PP) symptoms, she has lost all confidence in doctors. I responded with what felt like I was writing a, “Yes, Virginia, there is a Santa Claus” letter. In this case it was “Yes, Virginia, there are very few doctors who know about Periodic Paralysis.” The following is what I wrote:

Dear Virginia,
Most of us who have Periodic Paralysis do not have confidence in doctors or medical professionals anymore. Periodic Paralysis is a mineral metabolic disorder, but neurologists who typically diagnose and treat it, see it as a neurological disorder and try to treat it as such or due to lack of education about it or misinformation about it, they do not recognize it so fail to diagnose it. The misdiagnosis or lack of diagnosis leads to mistreatment with wrong medications or no treatment. Either way, the individual with PP, becomes worse. This is called iatrogenisis, which is harm that is preventable from medical treatment or a lack of treatment. 

Periodic Paralysis should be able to be treated by an endocrinologist, because they are familiar with the thyrotoxic form of PP, but those who will or know about PP are difficult to find. The so-called "specialists" are involved in the research end of it but unless you are genetically diagnosed or absolutely a "perfect fit" for their studies you are not diagnosed or your diagnosis may taken away. This happened to one of our members. She was later found to have the genetic mutation!!!!! I have seen 5 specialists and discussed my case with 3 others. What I am saying is true. None of them could/would help me.

My own research and experiences have led me to know the best way to find a doctor to diagnose and treat your symptoms. We created our website, our support group, our blog and wrote our books to share these ideas with others.

We find it best to locate a good MD, usually an internist is best, who is willing to work WITH you and learn WITH you. You do this by calling the different doctors in your area; a clinic is the best, and talk to the office managers. Tell them your story. Ask them to talk to the doctors in their office or clinic about it and one will surely step forward who wants to work with you. They will call you back with the name of a willing doctor. You, then, need to take the lead with the information and with your treatment. 

 The first place to start is your diet, strictly organic and as pH balanced as possible. Then discover all of your triggers and avoid them. Gather as many home medical instruments as you can to monitor and track your vitals, at the very least a wrist blood pressure cuff, finger oximeter, and potassium reader if possible. 

Gather your own team of specialists as needed through and with the help of your MD or internist. They will know or can find physicians who will work with them and you.

IF YOU FEEL YOU MUST (not my advice), experiment with the medications and different types of potassium available, but be extremely careful as most have severe side effects and can make you worse. Do not take them just because everyone else does and the doctors say to do it, especially if your symptoms are made worse by the drugs, and if you do not have a genetic mutation found yet. Some forms shift both high and low potassium and some shift within normal potassium ranges so taking potassium may make it worse.

Each one of us is a little different, even in the same family, and we must find what works for us as an individual.

It is hard work to totally manage and direct your own care, but it seems to be the only way to do it for most of us. Accepting that fact is also difficult. I was dying until my husband and I took charge of my care. I went from four or five severe, full-body paralytic episodes a day lasting for several hours each to one or two attacks a month with much less severity, except for the episodes I have at night in my sleep. It is still a game of experimenting at times and trying new things. We are always trying to balance while “walking the tightrope.”

I wish I could tell everyone there is a "quick fix," "magical cures," "healing medications," and "perfect treatments," but I am sorry to say I cannot.  The plan we have written about seems to work the best.

Description and explanation of all of the above information can be found in articles on our website and on our blog, in discussion on our support group, as well as in our 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" and "What Is Periodic Paralysis?: A Disease Like No Other."

DISCLAIMER: There are some very good doctors out there who are willing to help. But when someone has been through the wringer, it is best to try a new approach. Over 30 doctors in 6 years and about 6 more since then, gave us a good and real look at what most of us are going through. For those individuals, this may be an approach that will help them and give them some hope.....

Until later...

Friday, February 7, 2014

What is Normokalemic Periodic Paralysis?

Hello All,

What is Normokalemic Periodic Paralysis?  

Before writing about Normokalemic Periodic Paralysis, I believe it best to review Periodic Paralysis in general. I have written several articles previously about the different forms of Periodic Paralysis namely Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis and Andersen-Tawil Syndrome.

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, difficult to diagnose, hereditary, mineral metabolic disorder characterized by episodes of muscular weakness or paralysis, a total lack of muscle tone without the loss of sensation while remaining conscious. It is passed from either the mother or the father to any of the children, male or female. It is an ion channelopathy, a condition 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.
There are several known types:

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

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

Normokalemic Periodic Paralysis (Normo PP): Paralysis results from the movement or shifting of potassium within ranges, which are normal (normokalemia).

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 in the normal (normokalemia) ranges of potassium.

Normokalemic Periodic Paralysis

The commonly accepted range for normal potassium in human beings is 3.5 to 5.0 mEq/l (milliequivalents per liter), but these numbers may vary somewhat among labs. Our bodies work to naturally maintain a fine balance, which is within that normal range. Ninety-eight percent of potassium in the body is located within the cells and the other two percent of potassium is outside of the cells in the blood. Blood testing in a lab is used to measure the potassium in the body. There are also a few different types of potassium readers available for purchase and use in the home.

For individuals with Periodic Paralysis, the “normal” ranges of potassium may vary significantly from person to person. Results from a survey revealed some feel well and are at their best at about 5.0 while others may do best at 3.8 or 4.3. The potassium for these individuals shifts in several ways depending on the type of PP causing many symptoms as discussed previously. It may shift higher or lower. These shifts may be very slight yet cause paralysis as well as other serious symptoms including but not limited to heart, breathing and blood pressure issues. The shifting may also happen very quickly and be undetectable. This shifting is then within the “normal” ranges of potassium, thus the name “Normokalemic” Periodic Paralysis, although some research indicates it is not necessarily a distinct or different form of Periodic Paralysis, but rather Hyperkalemic Periodic Paralysis. However, it appears that 58% of those with all forms of PP actually have episodes of potassium shifting within normal ranges according to a recent survey of individuals diagnosed genetically and clinically.

Because most of the emphasis, literature and studies written about Periodic Paralysis are about Hypokalemic Periodic Paralysis (low potassium) and Hyperkalemic Periodic Paralysis (high potassium), the majority of medical professionals do not understand or recognize Normokalemic Periodic Paralysis or the knowledge that the potassium does not have to shift outside of normal ranges or that it may shift too quickly to be detected to create the paralysis or other symptoms which may be serious or life-threatening. It may also shift high or low and return to normal ranges before an individual can be tested in a lab or be seen in the ER.

This makes it difficult when an individual is seeking a diagnosis. Neurologists suspect neurological issues and prescribe very harmful medications, which may cause new symptoms or physical therapy, which can be painful and cause episodes of paralysis. Unfortunately, this may then lead to misdiagnoses of pseudo-seizures, conversion disorder, malingering, attention seeking, and/or hypochondria. More inappropriate and harmful medications and treatments are prescribed to treat these issues. The mis-labels follow the patient from doctor to doctor and the individual is never taken seriously.

These same issues are rampant in an ER situation. Potassium in normal ranges, with paralysis and other issues and uninformed medical professionals, can add up to all of the above and the administration of IVs filled with sodium or glucose with a psychotropic drug to treat pseudo seizures. This can lead to more serious symptoms, permanent damage and even death for an individual with Periodic Paralysis.

Another problem resulting from potassium shifting within normal ranges for someone who has a diagnosis of Periodic Paralysis, especially Hypokalemic Periodic Paralysis (low potassium), is the issues of automatically taking a dose of potassium when symptoms begin or being given an IV with potassium in the ER when the potassium never left normal range. This may then cause a shift into high potassium levels and create new or worse symptoms.

For those who have symptoms and paralysis while potassium levels remain within normal ranges, the best way to know how to treat it is to take note of the symptoms and record them. If one is able to record the levels of potassium at home, keeping a running tally when symptoms begin will eventually show either a trend toward high or low potassium or a shifting both ways as is common in ATS. Depending on whether one gets better or worse when taking potassium, may also be a clue of either high or low potassium. Discovering and avoiding the triggers that set it off is recommended.

When in paralysis and accompanying symptoms, there may be actually nothing that can be done except for time to pass and things to return to normal, because the potassium is already in normal range. Remaining calm is also recommended in order to avoid stress which can cause more shifting of potassium. If at previous times, taking potassium helped, then it may be recommended. If the potassium made it worse, it might be wise to avoid it.

An ambulance does not need to be called nor does someone need to be rushed to the ER, doctor or hospital every time they become weak or paralyzed due to potassium shifting. Most individuals will be fine in a few hours. However, if one is having serious difficulty with breathing, heart, choking or swallowing, then an ambulance may be necessary. Otherwise, the only important reason for going to the ER, if a person does not have a diagnosis, is to get the attacks documented, a "paper trail" is often needed. Proof of the episodes must be established.

From Wikipedia:

“Also of note is that potassium levels do not have to range outside of normal limits to cause serious, even life-threatening paralysis. These diseases are not the same as having a very low level of potassium (hypokalemia) or high potassium (hyperkalemia) and must not be treated as such. The total body store of potassium is usually normal; it is just in the wrong place.”

For more information on when to call for emergency care can be found at:

October 1, 2014

I would like to add another important source to this article:

Hypokalemic Periodic Paralysis:
"Serum potassium level decreases during attacks but not necessarily below normal."  "Creatine phosphokinase (CPK) level rises during attacks."

Hyperkalemic Periodic Paralysis:
"Serum potassium level may increase to as high as 5-6 mEq/L. Sometimes, it may be at the upper limit of normal, and it seldom reaches cardiotoxic levels. Serum sodium level may fall as potassium level rises."

Added July 25, 2015

"Normokalaemic periodic paralysis: In these attacks the blood potassium remains normal

In fact, it has recently been discovered that it is not the change in the blood potassium level that is the
primary problem in periodic paralysis. The primary problem in all of these conditions is that the normal pores which exist in the walls of the muscle cells don’t work properly. It does seem that changes in blood potassium levels can further hinder the function of these pores and that is why changes in blood potassium can be relevant. However, other factors separate from blood potassium can also worsen the function of the pores, so a change in blood potassium is not essential."

Until later…‎