Serene Forest

Wednesday, August 26, 2015

The Truth About The “New” Drug For Periodic Paralysis: It Is Not New



The Truth About The “New” Drug For Periodic Paralysis: 

It Is Not New And It Is Not For Everyone

  A member of our Periodic Paralysis Network Support, Education, Advocacy Group asked me today for my personal opinion of the “new” drug approved by the FDA, which is now available to treat “Periodic Paralysis.” I myself have received no fewer than fifty Google alerts about this new “wonder drug” touted to treat Hypokalemic Periodic Paralysis and Hyperkalemic Periodic Paralysis. I have seen no articles discussing the truth about this drug in relationship to what Periodic Paralysis really is and how the paralysis and other symptoms in individuals are created with this condition. My reply is as follows addressing these issues.


I want to begin by saying we at PPN are a patient advocacy group as well as a support group and educational group. It is our desire and duty to provide information to keep each of our members informed, safe and to be the "best each person can be." For that reason I am passing along this information.

Periodic Paralysis is a mineral metabolic disorder (ion channelopathy). Until this “new” drug, there were NO drugs recommended or approved by the FDA for the treatment of Periodic Paralysis. The ones typically used, diamox/acetazolamide, are strictly "off-label" (made and used for other conditions). These drugs were and are unsafe for most individuals with Periodic Paralysis due to serious, sometimes harmful side effects, short term and long term. They are carbonic anhydrase inhibitors and are sulfa-based diuretics.

The “new” drug, now approved by the FDA to treat Periodic Paralysis, dichlorphen-amide, which will also be known as keveyis, is basically another carbonic anhydrase inhibitor (a diuretic) and it is sulfa-based. It is exactly the same as diamox (acetazolamide) so if someone has a problem with those drugs they most likely cannot take the new drug.

Regarding the condition of Periodic Paralysis itself, some things need to be explained in order to understand how this drug may or may not work. There are several forms of Periodic Paralysis and many genetic mutations for each form. The different types cause the paralytic episodes, muscle weakness and other symptoms in varying ways. Potassium shifts in the body abnormally either in low, high or within normal ranges caused by many triggers. This happens as follows depending on the various forms.
  • Paralysis results from potassium moving from the blood into muscle cells in an abnormal way,
  • Paralysis results from problems with the way the body controls sodium and potassium levels in cells.
  • Paralysis results when the channel does not open properly and potassium cannot leave the cell.
  • Paralysis results when potassium shifts within normal ranges.
  • The sodium channels close much too slowly and the sodium, potassium, chloride and water continue to flow into the muscles.
  • Or the normal pores in the muscle cell walls just do not work correctly.
Knowing this information, my question is as follows, “How can one drug possibly treat and correct all of these possible malfunctions?”

Another serious issue is related to the lack of knowledge that many individuals with Periodic Paralysis must deal with regarding the diagnosis of the condition.
More than one half of all of the individuals with the various forms of Periodic Paralysis will not know what mutation they have due to poor, limited, narrow or biased DNA/genetic testing or because many of the mutations have yet to be discovered and research in this area has been halted. Without knowledge of which form a person has, taking these drugs is very risky.

Now I will discuss what this drug entails’


What is a carbonic anhydrase inhibitor?


A carbonic anhydrase inhibitor is a diuretic. It works by causing “increased excretion of bicarbonate with accompanying sodium, potassium and water, resulting in an increased flow of alkaline urine. They inhibit transport of bicarbonate into the interstitium from the proximal convoluted tubule. Therefore less sodium is reabsorbed, causing greater sodium, bicarbonate and water loss in the urine.”

http://www.drugs.com/drug-class/carbonic-anhydrase-inhibitors.html


From the pharmaceutical company itself about dichlorphenamide/keveyis:

Keveyis is not for everyone. Do not take Keveyis if you:

Are on a high-dose aspirin regimen
Are allergic to sulfa-based drugs
Have liver, kidney, or certain lung conditions
Are pregnant, planning to become pregnant, or nursing
Are under 18 years old”

“Taking Keveyis may cause a drop in the amount of potassium (an electrolyte) in your body, which can lead to heart problems. Ask your doctor if you need to eat foods that contain high amounts of potassium while taking Keveyis.” (This means it creates Hypokalemia (low potassium levels), exactly the reason most people are taking it.)

Your body may produce too much acid or may not be able to remove enough acid from body fluids while taking Keveyis. Your doctor will run tests on a regular basis to check for signs of acid buildup and may reduce your dose or stop your treatment with Keveyis.” (This means it can cause the development of metabolic acidosis, which nearly everyone with a form of Periodic Paralysis is prone to and develops due to the nature of Periodic Paralysis. It is a mineral metabolic disorder.)

”Keveyis may also increase the risk of falls, especially in elderly patients and patients taking high doses of Keveyis. Use caution when driving, operating machinery, or performing any other hazardous activities while taking Keveyis, as this medication may cause drowsiness.” (Most individuals with Periodic Paralysis already have issues with balance, weakness and possibly falling.)

Unfortunately, these are some of the same issues as diamox/acetazolamide!

I personally cannot take it... keveyis is sulfa based (I cannot take sulfa-based drugs) and diamox/acetazolamide nearly killed me for that reason and others.

Even for individuals who take diamox/acetazolamide and it helps them reduce episodes, most still have symptoms, and side effects; both short term and long term. These effects can be very serious and even deadly. It can cause liver disease and kidney stones as well as metabolic acidosis, very serious conditions.

In my personal opinion, I do not suggest that anyone take either of them. That being said, however, some people do fairly well but not without new problems. This is something each person must decide for himself or herself. One will not know unless he or she tries it. But like I said, some of the effects are over time and at quite a cost.

 One of our members developed kidney stones, and then had to have surgery and it nearly killed her due to the anesthesia and other issues. She cannot do it again and they did not get all of the stones. She is in constant pain and will never be as well as he was before taking the drug.

I am so sorry to be so biased...but I want everyone to be safe...and I do not believe these drugs to be safe for anyone of us with Periodic Paralysis.

The truth is that dichlorphenamide, which will also be known as keveyis, is another carbonic anhydrase inhibitor, a diuretic that is a sulfa-based drug. It is the same as
diamox/acetazolamide; it is nothing new or different. It is just more of the same.

But
to be fair, some people do well on these medications and we are not telling anyone to stop taking them if that is the case.

I did find this in one study:
“It was determined that some individuals became who became unresponsive or worsened by acetazolamide, responded favorably to dichlorphenamide.”
http://www.ncbi.nlm.nih.gov/pubmed/6855804

However, as previously discussed, others do not do well and/or they may develop side effects from these drugs, which can be harmful and can become life threatening. If that is the case, there is more information here for you to consider. If you have been given a prescription for one of them, you may want to read the information here before you start it, so you will know what to expect or what to look for, in order to be safe.

I hope this is helpful and if you decide to take it please do so carefully and monitor yourself very closely.

I have included many links to articles and studies regarding these drugs:

The following is information about the side effects of dichlorphenamide (keveyis):

http://www.webmd.com/drugs/2/drug-8770/dichlorphenamide-oral/details/list-sideeffects

www.businesswire.com/news/home/20150810005655/en/FDA-Approves-Taro’s-Keveyis™-dichlorphenamide-50-mg#.VdZBTvRS6ih

The following is some information about diamox/acetazolamide use in individuals with Periodic Paralysis:

For those with Periodic Paralysis, some forms of Hypokalemic Periodic Paralysis do not respond to diamox/acetazolamide and some cause paralysis and/or worse symptoms:

 A study from 2001 relates two different types in particular but I am sure more have been discovered by now: CACNL1A3=Hypokalemic Periodic Paralysis-1 and SCN4A=
Hypokalemic Periodic Paralysis-2, each with several mutations...and of course there are the ones that have not been found yet.

When someone is diagnosed with Hypokalemic Periodic Paralysis, clinically, (based on their symptoms); one must be very careful. One form does not respond to diamox/acetazolamide, and it causes more paralysis or more serious symptoms. So, d diamox/acetazolamide 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 study:

“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.”

As written above, about half of us do not have a genetic diagnosis and do not know what form of Periodic Paralysis we actually have. If we are going to take these medications, we must be very careful and closely monitor our symptoms and the side effects.


Connection between liver disease and carbonic anhydrase inhibitors:

It has been linked to liver disease ranging from acute hepatitis to severe injury with acute liver failure or prolonged jaundice.


Articles and information about issues related to acetazolamide and diamox. 

Contraindications for oral diamox

More information about diamox:

Side effects for children:

Some things to consider before putting your child on diamox/acetazolamide: Many people do fine on it but there can be some serious side effects to watch for including kidney stones, liver dysfunction, osteoporosis and metabolic acidosis. It should not to be used for children under 12 because it can affect growth. It is a sulfa drug...some people cannot take sulfa drugs.

"
This medication should not be used in children less than 12 because it may affect normal growth."
 http://www.webmd.com/drugs/2/drug-6753/diamox-oral/details#precautions

I understand that in some cases it may be the best of two evils...a very tough decision...but you must be aware of the possibilities...

https://www.riverpharmacy.ca/faq/are-there-any-side-effects-to-acetazolamide-diamox-avva


PPN Blog Articles written about the effects of drugs/medications/pharmaceuticals/anesthesia in Periodic Paralysis

http://livingwithperiodicparalysis.blogspot.com/2013/11/pharmaceuticals-are-not-answer-for-some.html
http://livingwithperiodicparalysis.blogspot.com/2013/12/triggers-december-1-2013.html
http://livingwithperiodicparalysis.blogspot.com/2013/12/idiosyncratic-and-paradoxical-reactions.html
http://livingwithperiodicparalysis.blogspot.com/2013/12/why-people-with-some-forms-of-periodic.html
http://livingwithperiodicparalysis.blogspot.com/2014/01/permanent-muscle-weakness-in-periodic.html
http://livingwithperiodicparalysis.blogspot.com/2014/02/some-forms-of-pp-worsened-by-diamox.html
http://livingwithperiodicparalysis.blogspot.com/2014/02/rhabdomyolysis.html
http://livingwithperiodicparalysis.blogspot.com/2014/02/periodic-paralysis-and-anesthesia.html
http://livingwithperiodicparalysis.blogspot.com/2014/06/beware-of-off-label-drugs.html


Until later...

Monday, August 10, 2015

A New Drug For Periodic Paralysis? Helpful Or More Of The Same?

Hello All,

There is a big announcement in the world of Periodic Paralysis today!! A drug has been approved by the FDA as the first drug to treat Periodic Paralysis. It is Dichlorphenamide also known as Keveyis. It is a carbonic anhydrase inhibitors (a diuretic) and it is sulfa-based. It is not new to PP and has been used before but was pulled from the market while studies were done over a period of twenty-three years.

Until now a few other drugs have been used but they are all 'off-label' drugs. This means they were created for other conditions. They are forms of diuretics. They work for some people depending on the particular genetic mutation an individual has. They are not without side effects, both short term and long term. For some they can be very harmful, especially if one has an allergy or intolerance to sulfa-based drugs or the wrong genetic mutation.

This announcement is good for some lucky people and just "more of the same" for others of us who cannot tolerate the carbonic anhydrase inhibitors which are sulfa-based.

This drug,
Dichlorphenamide, needs to be used with great care, just as the off-label drugs.

It is nice to see research for treatments of Periodic Paralysis, but it would be nice to see research being done for those of us who cannot handle these drugs. It would be nice to see research outside of the proverbial box for Periodic Paralysis.

Sunday, July 26, 2015

Bill of Rights for Periodic Paralysis Patients




Bill of Rights for Periodic Paralysis Patients

A ‘bill of rights’ may also be called a ‘charter of rights’ or a ‘declaration of rights.’  It is a list of ethical, social or legal principles of entitlements or freedoms or what could be referred to as that which is “owed” to an individual. Bills of rights exist from lists of basic human rights worldwide to lists of a country’s or a state’s citizen’s rights. A declaration of rights has been created for all patient’s in the United States health system and all hospitals have a bill of rights for their patients. Specifically, targeted groups such as stroke victims, individuals with multiple sclerosis and cancer patients have bills of rights. Until now, however, no bill of rights has been created for individuals with Periodic Paralysis. This is a group of very ill people who are under-recognized; under-diagnosed, misdiagnosed, misunderstood and mistreated by the medical professionals with whom they must deal and depend upon for their care. It is time for a Periodic Paralysis Patient’s Bill of Rights.

First it must be understood that there exists basic medical ethics; called a ‘code of ethics,’ which is a set of moral principles. Physicians around the world follow these guidelines found in the Hippocratic oath. They take this sworn pledge before they begin to practice medicine. The following are the principles typically included:

~A doctor must have the best interest of the patient
~A doctor’s treatment must be based on equality and fairness
~A doctor must “do no harm”
~A doctor’s treatment may be accepted or refused by the patient
~A doctor must treat patients with dignity
~A doctor must be honest and truthful

National Patient’s Bills of Rights

In 1998 the US Advisory Commission on Consumer Protection and Quality in the Health Care Industry created and adopted what is known as the Patient’s Bill of Rights, a summary of principles. There are three major goals:

~To assist patients to feel more confidence in the United States health care system

~To stress a strong association and connection between health care providers and their patients

~To stress the importance for patients to stay healthy

Individuals are entitled to eight major components in the National Patient’s Bills of Rights related to insurance plans and hospitals (including emergency care):

A patient is entitled to:

~Accurate and easy-to-understand information about one’s health plan, medical professionals and facilities for health care

~Choice of the best health care provider for a particular medical condition

~Access to services for emergencies

~Being a member of the team for making all individual health care choices

~Respect and considerate treatment, with a lack of discrimination, from all healthcare workers

~Privacy and confidentiality and the right to copy and read all medical records and to change anything that is incorrect or not relevant and to add to records which are not complete

~Complain and make appeals about any medical care provider, regarding waiting time, actions or non-actions and adequacy of services or facilities

The above goals and components have been adopted and are used by Medicare, Medicaid, hospitals, insurance companies, long-term medical facilities and more. They are the basis for targeted medical groups bills of rights also.

Using these principles of basic entitlement combined with the needs of individuals with Periodic Paralysis, the following bill of rights has been created.


Bill of Rights for Periodic Paralysis Patients
Created by Periodic Paralysis Network Inc
Copyright © 2015

General Rights

The right to be the team leader for your medical care, needs and decisions

The right to be treated with respect and dignity by all medical professionals

The right to have your symptoms of muscle weakness and/or paralysis and any other pertinent issues to be taken seriously

The right to be treated as anyone else is treated who has a chronic, disabling medical condition

The right for medical professionals to understand that Periodic Paralysis is a mineral metabolic disorder (channelopathy), not a neurological or muscle disease and should not be treated as such

The right for all medical professionals to understand that Periodic Paralysis is like no other medical condition and needs to be treated like no other medical condition

The right for all medical professionals to understand that Periodic Paralysis exists in several forms and may present with low potassium, high potassium or potassium within normal levels

The right to have all medical professionals to be educated about all aspects of Periodic Paralysis including all forms, the symptoms for each and the treatment for each

The right for Social Security Disability to recognize Periodic Paralysis as a chronic, disabling condition

Diagnosis of Periodic Paralysis

The right to a timely diagnosis

The right to a clinical diagnosis based on symptoms as in every other disease and medical condition

The right to Whole Genome DNA Testing paid for by insurance

The right for doctors to understand that only about 50% of Periodic Paralysis genetic mutations have been discovered, so a negative DNA test result does rule out Periodic Paralysis

The right for any type of doctor to diagnose Periodic Paralysis

The right to refuse provocative testing for diagnosing Periodic Paralysis

The right to have a doctor believe a diagnosis of Periodic Paralysis

The right to maintain a diagnosis for Periodic Paralysis once it has been given, no doctor has a right to change or dismiss a diagnosis

The right for doctors to understand that other medical conditions may co-exist with Periodic Paralysis

The right for doctors to understand that potassium may not shift out of normal ranges during paralytic episodes or other symptoms

The right to not be diagnosed with a mental or somatic disorder because a doctor does not understand or believe in Periodic Paralysis

The right to not be diagnosed with Periodic Paralysis because it is considered “too rare”

Treatment in Laboratory

The right to be treated with respect and dignity by lab technicians

The right to have blood drawn with no tourniquets

The right to have blood drawn as quickly as possible, even if it means going out to the car to draw the blood or to a patient’s home

The right to know the potassium level as quickly as possible using an ISTAT or potassium reader

The right for staff to understand that normal potassium levels do not rule out Periodic Paralysis

Treatment by Doctors and Medical Professionals

The right to be treated with respect and dignity by doctors and all medical professionals

The right to have your symptoms of muscle weakness and/or paralysis and other pertinent issues be taken seriously by your doctors

The right to have your symptoms of muscle weakness and/or paralysis and other pertinent issues be thoroughly assessed and treated promptly as needed

The right to dismiss a doctor for inappropriate care, lack of knowledge of Periodic Paralysis and poor attitude

The right to refuse any and all drugs prescribed by a doctor; to include IV’s and anesthesia

The right for clear, concise and truthful medical notes and records to be written by the doctors and other medical professionals

The right to read and receive copies of all medical records and the right to challenge, add to and change their content if not accurate

The right to complain and make appeals about any doctor or medical care provider, regarding waiting time, actions or non-actions and adequacy of services.

The right to be referred to a doctor who understands Periodic Paralysis

Treatment in ER and Hospital

The right to be treated with respect and dignity by doctors and medical professionals

The right to be seen quickly

The right to be comfortable and safe

The right to be accompanied and observed constantly by medical staff when in a paralytic episode

The right to have the doctors and medical professionals listen to the patient (if possible), family members or caregivers on instruction for care

The right to have the doctors and medical professionals to read information provided by the patient, family members or caregivers on how to care for the patient

The right to have all doctors and medical professionals follow the specific instructions provided to include proper positioning, no IV’s, no drugs and more, as necessary

The right to have blood drawn with no tourniquets for potassium levels which may indicate potassium levels that may be low, high or within normal ranges

The right to have blood drawn as quickly as possible preferably using a potassium reader or ISTAT for instant results which may indicate potassium levels that may be low, high or within normal ranges

The right to have the symptoms of muscle weakness and/or paralysis, and other pertinent issues to be thoroughly assessed

The right to have the symptoms of muscle weakness and/or paralysis and others as pertinent to be taken seriously by the doctors and medical care professionals

The right to be observed for choking, heart arrhythmia, fluctuations in blood pressure, and heart rate, breathing issues, low oxygen levels and others as pertinent

The right to not be harmed during paralysis by doctors or other medical professionals attempting to prove “faking” by pinching, scratching, burning or sticking with pins or needles

The right to be treated for choking, heart arrhythmia, fluctuations in blood pressure, and heart rate, breathing issues, low oxygen levels as pertinent

The right to refuse IV’s, drugs, medications and anesthesia, which may be harmful or deadly

The right to refuse provocative testing for the reason of diagnosing Periodic Paralysis

The right to call in a hospitalist, or other specialists who may know about Periodic Paralysis as needed

The right to call in a social worker or patient advocate as needed

The right to dismiss a doctor or other medical professional for inappropriate care, lack of knowledge of Periodic Paralysis and poor attitude

The right for clear, concise and truthful medical notes and records to be written by the doctors and other medical professionals

The right to read and receive copies of all medical records and the right to challenge, add to and change their content if not accurate

The right to complain and make appeals about any doctor or medical care provider, regarding waiting time, actions or non-actions and adequacy of services

Research and Awareness

The right to increasing awareness of Periodic Paralysis among populations, doctors and governments

The right to “put a face” to Periodic Paralysis as in other known diseases and medical conditions

The right to have all medical professionals to be educated about all aspects of Periodic Paralysis including all forms, the symptoms for each and the appropriate treatment for each

The right to funding for research of all aspects of Periodic Paralysis including but not limited to diagnosis and treatment

Insurance:

The right to have all forms of Periodic Paralysis be recognized as disabling conditions by insurance companies

The right to have potassium readers deemed as medical devices and paid for by insurance companies

The right to have ISTAT devices for monitoring vitals for all Periodic Paralysis patients and to have them paid for by insurance companies 


The right to have the special natural and organic diet and supplementation that must be followed by patients with Periodic Paralysis to be paid for by insurance companies

Created by Periodic Paralysis Network Inc
Copyright © 2015




Monday, July 13, 2015

Scandinavian Periodic Paralysis Support Group

Hello All,

We are happy to announce that a new Periodic Paralysis Support Group has been created for Scandinavian individuals who speak Swedish, Norwegian or Danish.

https://www.facebook.com/groups/857899550969217/









Wednesday, June 24, 2015

Identify And Eliminate The Triggers Of Periodic Paralysis: Part Two

Hello All,

As I wrote in Part One, nearly everyday, new members join our PPN Support, Education and Advocacy Group. Their most important concerns are about getting treatment to stop or manage their symptoms/paralysis and/or getting a diagnosis. I have already written articles about how to find a good doctor and how to get a diagnosis, which may take many years, but to this point have not written about how to identify and eliminate the triggers that set off our symptoms and paralysis.

As I have written before, there is no magic drug or cure for Periodic Paralysis. There is an off-label drug that only works for a few mutations and even then, there are and can be, serious side effects, long term side effects and most individuals continue to experience paralysis and symptoms. So despite having a diagnosis and possibly taking the off-label drug, most individuals need to know how to manage their symptoms and episodes of paralysis, because most triggers continue to set these things into motion.

For those still waiting for a diagnosis and hoping for drugs/treatment from a “specialist” the diagnosis will probably not bring a successful treatment. So, while waiting for a diagnosis, or if one has a diagnosis and is on the off-label drug and still experiencing symptoms or if someone has a diagnosis but cannot tolerate the off-label drug; the best way to manage the symptoms and paralytic episodes to is identify the causes or triggers for those symptoms and episodes.

This is part two of a Blog Article about how to identify and eliminate triggers.

From our book: The Periodic Paralysis Guide and Workbook:

Identify And Eliminate Triggers
AVOID - CHART - JOURNAL - MONITOR


  • Record symptoms and possible causes
  • Monitor symptoms
  • Keep a journal
The charts and how to use them:





Trigger Chart

Fill in the date.

  • Begin recording your activities, food, drink and medications in the top section. Start at the time you wake up. Include all food eaten in a meal. Record what you are doing, for instance sitting, eating, reading, exercising, walking, cooking, shopping or more.
  • Record how you are feeling, for instance, good, very thirsty, constipated, confusion, sleepy, unsteady, legs are weak, total paralysis or more.
  • Put a check mark in, (or fill in) the box that best describes your condition, for instance normal, weak, more weak, partial paralysis, more partial paralysis, or total paralysis.
  • Continue this through your day, it is not necessary to do it at night, but you may want to include paralysis, numbness, or more, if it is happening at night.
  • The bottom of the chart will become a graph and it will aid in seeing the times of symptoms.

In the completed chart below, we can see that two and one half to three hours after eating breakfast and taking septra, an antibiotic and a calcium tablet, the patient begins to experience weakness and then paralysis. It is a good chance that the medication or something eaten at breakfast was the cause.

If this person eats the same thing everyday and takes the calcium every morning, and the only new thing is the septra it would be safe to assume the antibiotic caused the episode. By four pm the episode has stopped. At five pm muscle weakness begins after an hour of being up and preparing dinner. This may be from exercise intolerance or due to something eaten at lunch.

If someone eats the same lunch everyday but does not always help with dinner, it could be safe to assume the exercise caused the weakness. The same could be true in reverse. At seven pm, overall weakness takes hold for the remainder of the evening. This may be from the food eaten at dinner or a continuation of the weakness from the earlier activity. During the night there are three episodes of total paralysis. Since sleep is a trigger, it may be impossible to stop the episodes throughout the night.



Evaluating the Data

When looking at your completed charts, it is best to first check the periods of paralysis. Then check the activity, medications taken or food eaten two to three hours before that time. Was anything new? Was any activity out of the ordinary?

  • Check period of paralysis or weakness:
    • Check two to three hours before.
      • New medication?
      • New food, drink?
      • New activity?
        • More than usual?
        • Longer than usual?
  • If answer is not clear: Continue to chart for a few days. If there seems to be a pattern:
    • Change only one thing at that time:
      • Remove sugar or
      • Cut medication dose or
      • Stop or reduce the activity
        • Less than usual
        • Shorter than usual
  • Check again after a day or two. If the symptoms of paralysis are reduced or better, you may have found the trigger. If not, add the thing you removed or reduced back into the meal, etc. Then repeat and change something else: 
  • Change only one thing at that time:
    • Remove sugar or
    • Cut medication dose or
    • Stop or reduce the activity
      • Less than usual
      • Shorter than usual
  • Repeat until you find the trigger or triggers.

Some suggestions for how to avoid the triggers once they are found:

  • On feet too long? : Break the activity in several shorter periods on feet.
  • Cannot eat sugar? : Stop and try sweeteners, honey, stevia, etc.
  • Cannot eat certain food? : Find replacement or do without.
  • Cannot take medication? : Cut dosage, stop taking or get a replacement (under supervision of medical professional).
  • Sitting too long? : Get up every hour or less and move around.
  • Sitting too long? : Exercise in chair.
  • Dehydrated? : Drink more water, set timer.
  • Hungry? : Eat several smaller meals.
  • Too hot? : Wear looser, cooler clothes, use neck cooler.
  • Too cold? : Add clothing layers, use a blanket, drink hot drinks.
  • Cannot drink caffeine: Drink decaffeinated drinks.

As the triggers are discovered and eliminated the attacks of paralysis and other associated symptoms will begin to decline. The quality of life will improve. The information will be recorded and can be shared with the doctors and used as a tool during the diagnosing process. Use the following blank page to record your own triggers as you discover them.


Monitoring the Triggers and Symptoms

Once many of the triggers have been established and a diagnosis has been obtained (or not), it will be necessary to continue to observe and monitor the symptoms and the triggers. As we have discovered from my our experience and that of others with Periodic Paralysis, the triggers may change over time or what may have seemed to be the cause, may not actually be the cause. It is important to keep track continually in order to regulate and control the symptoms.

For instance, at first it may appear that sugar eaten in a cookie is the culprit causing attacks of muscle weakness or paralysis. Later it may be discovered that it was actually the white flour or the chocolate in a cookie that may have caused the episode. Accidentally, it may be figured out that a person might be able to eat small amounts of sugar in a gluten-free cookie. Then after eating the gluten free cookie everyday for three days, a paralytic episode occurs. This may be that too much was eaten. So, it may be that small amounts of sugar may be eaten occasionally, just not everyday.

It is a fine line that an individual with Periodic Paralysis must walk everyday. We must move through life as if we are walking a tightrope. It has been said that when we figure out what our triggers are, we must treat them as if they are allergies, something we must avoid. However, it becomes a problem when those things are important elements needed in the body such as salt or sugar.

In discussion with other individuals, we also have discovered that surprising things may actually cause an attack. We may believe we are doing everything correctly and avoiding all triggers and then we suddenly slip into full body paralysis. This happened to me recently. I tripped on my oxygen cord and landed on my knees. I scraped up one of them, but otherwise felt fine. Three hours later I was in full body paralysis.

After discussing this with a friend and wondering what had caused this attack, he reminded me that adrenaline is released when the body is stressed or experiences some trauma. I realized that although I did not notice much when I fell except a small scrape to my knee, my body had recognized it as trauma, released adrenaline and three hours later I was paralyzed and for the following several days.

Now I will remember that if I fall again, no matter how small or uneventful it may seem, I must prepare for the possibility of slipping into paralysis. I will be sure to rest and hope it will neither happen again nor be as serious as the last time. Better yet, I need to be more careful, now that I know if I fall, I will go into paralysis (or break a bone knowing I have severe osteoporosis).

Monitoring or keeping track of triggers is important because it seems as if they are always changing and maybe they are or maybe it is the amount of something we are using, but also it may be that we have misinterpreted one trigger for another. We must use the charts to keep track of symptoms and possible triggers and put them together in a journal. The information in the charts and journal will be recorded and can be shared with the doctors and used as a tool during the diagnosing process or for treatment options.


Until later...




Identify And Eliminate The Triggers Of Periodic Paralysis: Part One



Hello All,

Nearly everyday, new members join our PPN Support, Education and Advocacy Group. Their most important concerns are about getting treatment to stop or manage their symptoms/paralysis and/or getting a diagnosis. I have already written articles about how to find a good doctor and how to get a diagnosis, which may take many years, but to this point have not written about how to identify and eliminate the triggers that set off our symptoms and paralysis.

As I have written previously, there is no magic drug or cure for Periodic Paralysis. There is an off-label drug that only works for a few mutations and even then, there are and can be, serious side effects, long term side effects and most individuals continue to experience paralysis and symptoms. So despite having a diagnosis and possibly taking the off-label drug, most individuals need to know how to manage their symptoms and episodes of paralysis, because most triggers continue to set these things into motion.

For those still waiting for a diagnosis and hoping for drugs/treatment from a “specialist” the diagnosis will probably not bring a successful treatment. So, while waiting for a diagnosis, or if one has a diagnosis and is on the off-label drug and still experiencing symptoms or if someone has a diagnosis but cannot tolerate the off-label drug; the best way to manage the symptoms and paralytic episodes to is identify the causes or triggers for those symptoms and episodes.

This is part one of a Blog Article about how to identify and eliminate triggers.


From our book: The Periodic Paralysis Guide and Workbook:


Identify And Eliminate Triggers
AVOID - CHART - JOURNAL - MONITOR

  • Record symptoms and possible causes
  • Monitor symptoms
  • Keep a journal

This Blog Article...will discuss the elements or experiences, which trigger or activate the potassium to shift causing the attacks of periodic paralysis and how to identify them. There are some common triggers among those with the different forms of Periodic Paralysis and there are some triggers, which are unique for each individual. Triggers can be certain medications, foods, activities, stress or even sleep. It is important to know one’s triggers in order to avoid having episodes or attacks of paralysis due to permanent damage, which may occur to organs in the body.

One of the most important things a person with Periodic Paralysis can do, no matter which type they have, is to discover what causes, starts or triggers our episodes of muscle weakness or paralysis. There are many triggers that set into motion the partial and total paralysis and other symptoms. It is important to discover these triggers because we need to stop the episodes or attacks, if possible, in order to regain the quality of our lives and to prevent the damage being done to our organs as the potassium shifts and depletes or increases in our bodies.

Potassium shifting can cause a myriad of conditions and the symptoms that accompany them, including permanent tightness of the calf muscles or muscle wasting of arm and leg muscles, restless leg syndrome or even osteoporosis. The breathing and swallowing muscles can weaken over time and be affected during attacks. If this happens it is an extremely serious and emergency situation. Dangerous arrhythmia of the heart can also occur when an individual is in an episode or attack. In the case of people with Andersen-Tawil Syndrome, the paralysis leads to tachycardia and serious arrhythmia, including long QT intervals, which can lead to cardiac arrest. Other complications can include respiratory arrest causing death or aspiration pneumonia after an attack, causing death. Though rare, these can occur with Hypokalemic Periodic Paralysis. Complications of Hyperkalemic Periodic Paralysis include bi-directional heart arrhythmia causing sudden death and permanent weakness of the muscles. Between attacks, the muscles usually return to normal or strengthen, but over time with repeated episodes of paralysis, progressive and permanent weakness of the muscles which can be severe is possible. This includes the heart and breathing muscles. This can lead to heart failure and respiratory failure, thus eventual death. Avoiding paralysis is absolutely necessary, due to these life-threatening effects.

The easiest way to decide what caused an episode or symptoms would be to look at anything new or different; a new type of bread, a new antibiotic, a new activity, a new shampoo, stressful event (good or bad), or a chilled or over heated room. Discovering our triggers requires a little bit of time to follow a simple plan. In a matter of a few days or weeks, it may be possible to draw some useful conclusions about the possible triggers of a paralytic event and the symptom, which can accompany them.

In the fields of medicine and education, it is said, “If you did not write it down, it did not happen.” This can be applied to our method for discovering our triggers. The first thing we must do is to write everything down. Creating a journal is a good way to make sure this is done. I have created a chart, which can be added to a journal to make the process easier (This will be added to part two of this article).

Several necessary components are included on this chart:

A 24-hour time frame
A section to write possible triggers
A section to write down symptoms and vitals one may be experiencing
A section to record the muscle weakness and paralysis
This section becomes a graph of the periods of weakness or paralysis

Once the information has been gathered for a few weeks, it will be easy to see trends or connections of a particular food, medication or activity to muscle weakness or paralysis.
The following are lists of the common possible symptoms of Periodic Paralysis one may experience as well as the paralysis and muscle weakness and the triggers, which may set them into motion.

Understanding and becoming familiar with the symptoms is another important part of completing the chart. As much information that can be added will be helpful. In this section anything may be included from “feeling well,” or “none,” to some of the known symptoms for hyperkalemia and hypokalemia in the following charts.

The following are at-a-glance lists of typical symptoms and triggers for the different forms of Periodic Paralysis and vitals needed to complete the charts.

The Symptoms Typical Of Low Potassium:

  • Episodic muscle weakness
  • Episodic partial paralysis
  • Episodic total paralysis
  • Episodic flaccid paralysis (limp muscles, without tone)
  • Muscle weakness after exercise
  • Muscle weakness
  • Muscle stiffness
  • Muscle aches
  • Muscle cramps
  • Muscle contractions
  • Muscle spasms
  • Muscle tenderness
  • Pins and needles sensation
  • Eyelid myotonia (cannot open eyelid after opening and then closing them)
  • Irritability
  • Severe thirst
  • Abdominal bloating
  • Nausea
  • Vomiting
  • Constipation
  • Excessive urination
  • Sweating
  • Tachycardia
  • Irregular heartbeat
  • Palpitations
  • Dizziness
  • Fainting
  • Breathing problems (barely breathing)
  • Hypo-ventilation
  • Increase in blood pressure
  • Irritability

Vitals To Add To The Chart As Symptoms:

  • Potassium level
  • pH level (saliva)
  • pH level (urine)
  • Glucose (sugar) level
  • Temperature
  • Blood pressure
  • Heart rate
  • Arrhythmia
  • Oxygen

The Symptoms Typical Of High Potassium:


  • Episodic muscle weakness
  • Episodic partial paralysis
  • Episodic total paralysis
  • Muscle contraction or rigidity during an attack
  • Muscle weakness
  • Muscle cramps
  • Muscle stiffness
  • Fasciculation (muscle twitching)
  • Pins and needles sensation
  • Cramping pain
  • Reduced reflexes
  • Muscle contraction involving tongue
  • Slurring of words
  • Tightness in legs
  • Strange feeling in legs
  • Tingling sensations
  • Pulse issues  (absent, slow, or weak)
  • Irregular heart beat
  • Heart palpitations
  • Breathing problems (wheezing, shortness of breath, fast breathing)
  • Mild hyperventilation
  • Decrease in blood pressure 
  •  Nausea
  • Feeling hot
  • Sleepiness  

More Possible Symptoms For The Chart:
 
  • Headache
  • Chest pain
  • Numbness
  • Unable to walk
  • Agitation
  • Shallow breathing
  • Pain in calves
  • Cramps in legs
  • Restless legs
  • Burning in feet
  • Hyperventilation
  • Feeling cold
  • Clammy
  • Dizzy
  • Shaky
  • Unsteady
  • Rubbery legs
  • Hunger
  • Jerking
  • Awake
  • Confusion
  • Strange feeling in legs
  • Brain fog
  • Sleeplessness
  • Memory problem
  • Depression
  • Weakness
  • Constipation
  • Foot drop
  • Dry mouth
  • Choking
  • Angry



Triggers

The Common Triggers Of Hypokalemic Periodic Paralysis

The triggers usually responsible for causing potassium to shift in Hypokalemic Periodic Paralysis are:

  • Eating a large amount of carbohydrates in a meal
  • Drinking alcohol
  • Ingesting too much salt
  • Stress (good or bad)
  • Excitement
  • Fear
  • Vigorous exercise
  • Resting after exercise
  • Cold
  • Epinephrine/adrenaline
  • Cold
  • Anesthesia

The Common Triggers Of Hyperkalemic Periodic Paralysis

The triggers usually responsible for causing potassium to shift in Hyperkalemic Periodic Paralysis are:

  • Eating a large amount of carbohydrates in a meal
  • Exercise
  • Cold
  • Ingesting too much potassium in food or medications
  • Stress (good or bad)
  • Rest after exercise
  • Fatigue
  • Fasting
  • Cigarette smoke

The Common Triggers Of Paramyotonia Congenita

  • The triggers usually responsible for causing potassium to shift in Paramyotonia Congenita are:
  • Exercise
  • Exertion
  • Repetitious movement
  • Cold
  • Sleeping in


Other Triggers For Periodic Paralysis

Triggers can include:

Diet: Diet can be one of the biggest contributors to episodes of paralysis. The following are some of my offenders or those that have been reported to me or I have found in the research:
  • Simple carbohydrates: sugar, white flour and more
  • Complex carbohydrates: some grains, wheat, rye and more
  • Meat: mostly red meats
  • Salt
  • Caffeine
  • MSG
  • Alcohol
  • Large meals
  • Gluten

Sleep:
All aspects of sleep may set episodes into motion:
  • Falling asleep 
  •  During sleep
  • Waking up

Other:
  • Dehydration
  • Fasting
  • Sitting too long
  • Changes in the weather
  • Fatigue
  • Heat
  • Cold
  • Electromagnetic Force (EMF’s)
  • Menstrual cycle

Exercise:
Some individuals have no problem with exercise but others may not be able to tolerate any type of exercise or very little exercise. This is called “exercise intolerance.” Episodes may develop soon after or the next day.
Rest after exercise: may set an episode into motion.

Unknown: One can follow all the rules and still have episodes for unknown reasons.

Over-the-counter medications:
Most over the counter medications, can set muscle weakness or paralysis into motion for people with Periodic Paralysis. The following is a list of some known offenders.

  • Eye drops
  • Glycerin enemas
  • NSAID’s
  • Cough syrups 
Compounds or Chemicals:
If the following ingredients are in any products you use…you should stop using them until you are sure they are not causing symptoms:
  • Sodium Hydroxide 
  •  Edetate Disodium
  • Stearic Acid

They may be in any of the following:
Lotions, oils, hair dyes or colors, antiperspirants, enemas, suppositories, soaps, shampoos, shaving creams, foams, toothpastes, deodorants, beauty products, skincare products, cosmetic products, bath salts, emollients, ointments, creams, hair sprays, perfumes, colognes, powders, hair gels, oils, tonics, mousse

Drugs:
Many, many drugs can set muscle weakness or paralysis into motion for people with Periodic Paralysis.

If one must take a drug, it is better to begin with ¼ of a normal dose to make sure it will work for you.

  • Saline drips, glucose Infusion: If an IV is needed, mannitol can be used(or diluted solutions in extreme cases)
  • Oral or Intravenous Corticosteroids
  • Muscle relaxers
  • Beta blockers
  • Tranquilizers
  • Pain killers (analgesics)
  • Antihistamines
  • Puffers for asthma
  • Antibiotics
  • Cough syrups
  • Eye drops to dilate eyes
  • Contrast dye for MRI’s
  • Lidocaine 
  •  Anesthetics
  • Epinephrine (Can sometimes help symptoms of Hyperkalemic Periodic Paralysis)
  • Adrenaline (Can sometimes help symptoms of Hyperkalemic Periodic Paralysis)

For more information about the medications or drugs which can cause muscle weakness, muscle paralysis, long QT interval hearts beats (ATS) and torsades de pointes (ATS), please go to:

http://www.periodicparalysisnetwork.com/pdf/What%20are%20the%20Periodic%20Paralysis%20Triggers.pdf

Until later...