Serene Forest

Monday, April 6, 2015

Doctors Not Being Held Accountable: The Periodic Paralysis Diagnosis By Guest Blogger Maureen McCutcheon


Hello All,

The topic of seeking a diagnosis and being abused by doctors in the process, appears nearly daily on our PPN Support Group. Almost every new member has a horror story to relate of their past battles and/or their on-going battle as they traverse across and through the 'diagnosis nightmare highway' lasting sometimes decades, and leaving individuals in a 'diagnosis limbo' at the hands of fallible doctors inflicting pain and damage through disrespectful and discriminatory actions and dis-action.

Maureen has written another great article on this topic for us...Thank you.

Doctors Not Being Held Accountable
The Periodic Paralysis Diagnosis

By Guest Blogger Maureen McCutcheon

The Medical Community is aware that tests and imaging and interpretation of tests and imaging, are fallible and have proven to provide incorrect, false, misleading, inaccurate and/or missed data and information.  The medical community is aware of this and  that this may be due to several factors that have proven  the limitations of technical equipment to always provide accurate information.  And people are fallible. People performing the test may not have been conscientious or have insufficient experience or the person (even 'specialists') interpreting the data, misreads or just 'misses' that can result from and in factors such as writing and reading the report, handling/mis-handling either or both the patient and testing equipment, image, or lab product. The condition of the patient can hinder obtaining results/image or variable factors/conditions that can influence the outcome of the image or testing. Or may not able to 'catch' abnormality at time of test, imaging or study.

Though a doctor may say that a test is "not showing anything wrong" or all is "normal," it doesn't mean nothing is wrong. It can mean that the test/imaging that doesn't reveal 'abnormal' that seems 'normal' or 'nothing wrong' isn't revealing the abnormality at that time so result isn't giving accurate info. It may be that test isn't adequate for any number of reasons to reveal and help to identify/recognize what is causing symptoms. Or as we know from experience, the doctor has inadequate or inaccurate information that would to facilitate accurate diagnosis (and doctor doesn't know enough to question or know that they don't have accurate information). This may result in mistaken diagnosis or treatment or no treatment. That may be a form of malpractice more than discrimination. But mal-practice tends to lend itself to being a co-factor/co-condition with discrimination and forms of abuse that could be considered forms of medical abuse that is discriminatory because it tends to be directed towards those who are disabled with rare conditions who physicians discount and use hostile attitudes behavior that they would not or don't use with people/patients who have common, familiar conditions that physicians are comfortable with because they can readily give a diagnosis to.
NOT addressing malpractice. The Human Rights we are addressing as ‘discriminatory’ is behavior expressed by attitudes, actions and being treated differently and denied equal treatment by some in the medical community towards many of those who are disabled by physical incapacitation due to rare and hard to diagnose disorders. (This kind of mistreatment probably is also exhibited towards those with some mental compromise/capacity) There are too many examples of the medical community not being held accountable and allowing themselves/each other to behave in discriminatory manner towards those disabled by rare health conditions. This isn't about having a doctor  'dismiss' person who is having physical symptoms/dysfunction without being able to identify the cause other than to say that since "can't find anything wrong to explain symptoms" (despite significant signs that something is wrong and not normal) or discharging from the ER because in their opinion they feel they've adequately ruled out imminent life-threatening (primarily stroke/heart attack). The form of Human Rights discrimination that we are concerned about is focused on the both the intentional use of behavior/attitudes/actions by some doctors and others in medical community towards those disabled by conditions that due to being rare seem to mistreated and are not being treated with dignity, respect and are given a different quality of CARING being extended to those with what doctors must believe have a 'real' condition because they have a 'real' diagnosis.

Physicians can also too easily use explanation/label from a psychological perspective that they should not be able to use without proving they've ruled out everything else. (How can they with over 6 or 7 thousand rare diseases/disorders?). However, that still should not be reason for a doctor to behave in some of the despicable, horrific behaviors they aren't being held accountable for that is being disrespectful and discriminatory. And it is dishonorable to their profession. 



Until later...

Tuesday, March 24, 2015

375 Members!!!



Congratulations to us all at the Periodic Paralysis Network Support, Education and Advocacy Group!!! We now have over 375 members!!!!!!!! Thank you all for your support!!!

Our PPN Forum:

The following are the services and features of our PPN forum:

PPN Support, Education and Advocacy Group:
https://www.facebook.com/groups/periodicparalysisnetworksupportgroup/

PPN Website: www.periodicparalysisnetwork.com

PPN Books: "Living With Periodic Paralysis: The Mystery Unraveled" and "The Periodic Paralysis Guide And Workbook: Be The Best You Can Be Naturally"
(Found on our website http://www.periodicparalysisnetwork.com/books.htm)

PPN Blog: http://livingwithperiodicparalysis.blogspot.com/

PPN Book Discussion Group:
https://www.facebook.com/groups/periodicparalysisnetwork/

The PPN Learning Center and Workshop:
https://www.facebook.com/groups/1416848568618404/

PPN Genealogy-Genetic Discussion Group:
https://www.facebook.com/groups/580168915344191/

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

PPN Author's Page:
https://www.facebook.com/SusanQKnittleHunterauthor

Email: periodicparalysisnetwork@gmail.com

Please check out our PPN Members World Map: http://www.multiplottr.com/?map_id=55083

Saturday, March 21, 2015

A Poor 'Product' That is Being Called 'Medical Care': By Maureen McCutcheon


A Poor 'Product' That is Being Called 'Medical Care': By Maureen McCutcheon

It has happened again…another misinformed and arrogant neurologist is denying a diagnosis to one of our new members based on old, outdated and/or misinterpreted information. He believes that potassium levels must be 2.1 or lower in order to qualify for a diagnosis of Hypokalemic Periodic Paralysis. This is so ludicrous that it is laughable!!!!

In response, Maureen, one of our members wrote the following:

"We are not expecting doctors to KNOW everything. There is too much to know and more to know than they or anyone can even wonder about or what it is they do not know TO KNOW... BUT, it is not unrealistic to expect that they will not act like they know everything! And that they will be willing to admit they cannot KNOW everything (especially when there are over six or seven thousand known rare disorders) and over and over in text books I have read the words, "we don't understand,", or "we don't know," or "we can't explain why this is, but ... ")

I have read comments like this in textbooks about conditions as common and well-researched as diabetes and other well-known conditions that are not rare. I wish they would just to be willing to use the ability they must have to learn, that they have proven they have, by going through medical school and all the rigors and requirements to become a physician. I wish they would use that ability to think, learn and attain knowledge ( if the ability to think has not been beaten out of them by the current push to use 'pathway' thinking and cookie-cutter 'follow recipe' protocols that can have a place as a FOUNDATION to start and incorporate in the search for a diagnosis, but not to end there) then dr's need to stop following the recipe of 'standard protocol' (which includes the easy of the use of 'psychological' and labels like somatorform, conversion, etc.).

Standard of care and protocol has a place in medical care. It has some value. But people ARE NOT factory made machines. When the protocol is hindering rather than facilitating the real purpose of the physician (which is to seek accurate diagnosis so can provide care that is best fit for the patient), that ties their hands and makes them afraid to 'change the recipe', that facilitates and forces doctors to use a 'bad' recipe and actually encourage them to 'discard/ 'throw out' the patient rather than throw out the 'bad recipe'.

A chef will throw out a bad recipe. A quality chef will be able to tell the recipe will be bad even before starting it, so they will not even waste their time and ingredients. Doctors need to be able to be like the chef who knows to throw out the 'bad recipe' or decide that if they are required to use it, that they will improve upon it in a 'creative' way. But doctors seem to be hindered, even 'punished' for trying to 'change the recipe', even if it results in a poor 'product' that is being called 'medical care'..."

Thank you Maureen!!

Until later…

Wednesday, March 18, 2015

How To Get A Periodic Paralysis Diagnosis: “Hit Them Between The Eyes With The Facts”





How To Get A Periodic Paralysis Diagnosis: “Hit Them Between The Eyes With The Facts”

I realized last night that I have not written a blog on this very important subject. Getting a diagnosis is one of the most discussed subjects on our PPNI Support, Education and Advocacy Group. I have written many articles about the diagnosis and the many and complicated issues related to it, but I have neglected to write one with the actual plan or steps one can follow. I have however, written two books in which I cover this subject at length. They are "Living With Periodic Paralysis: The Mystery Unraveled"  and "The Periodic Paralysis Guide And Workbook: Be The Best You Can Be Naturally." In fact the second book was, in part, actually created to be used as a tool to aid in getting a diagnosis.

Written in the “Introduction” of the front matter of the book is the following, which includes the “plan” for seeking and obtaining a diagnosis:

”It is a user-friendly guide, a set of plans, instructions and ideas for aiding individuals with all forms of Periodic Paralysis, to better manage their symptoms in natural and common sense methods. It is a workbook with a set of tools such as charts, forms and even a medical journal, with clear instructions for completing and using them. Each is designed to be individualized and may be utilized for the various plans or sections of the book. The charts and forms may be scanned and used separately or the pages may simply be completed in the book itself for keeping all medical information in one place. Easily transportable, this book is also a handbook and can accompany each visit to doctor appointments, the ER or the hospital. This guide may stand-alone or may be used in conjunction with the first book about Periodic Paralysis, living with Periodic Paralysis: The Mystery Unraveled. This guide, workbook and handbook is designed to assist individuals with all forms of Periodic Paralysis to ‘be the best they can be naturally.’”

This is the Outline for the Plan:
(From the book)

Getting a Clinical Diagnosis
The Plan

Process of elimination
Gather the facts
Lab work
Periods of paralysis, documented
EKGs
Oximeter recordings
Genetic testing?
Previous medical records
Chart the triggers
Document potassium use
Gather and chart family information

Introduction:

There are two methods used for diagnosis of the various types of Periodic Paralysis; one is through a genetic test, which studies one's DNA. If a mutation is found the person receives a "genetic" diagnosis. If a person is diagnosed based on their symptoms, once all else has been ruled out, it is called a "clinical" diagnosis. Too many doctors will not give a clinical diagnosis once everything else has been ruled out and are eager to diagnose a person, especially women, with a mental disorder such as "conversion disorder" and then prescribe a psychotropic medication which makes the symptoms worse or can actually kill someone with certain forms of PP.

The
truth about the genetic mutations for Periodic Paralysis and Andersen-Tawil Syndrome:

More than 40% of all individuals with Periodic Paralysis have a form, which has not yet been discovered and must therefore, be diagnosed clinically, based on symptoms and characteristics.

The other issue is that up to 50% of those with PP have potassium shifting in normal ranges, rather than low or high, which causes their symptoms. Without a test proving low or high potassium, some uniformed doctors refuse to diagnose although everything else is ruled out and there are clearly periods of muscle weakness and/or paralysis.
It is my belief that too much emphasis is placed upon the genetic testing, because only about one half us will have a genetic mutation if we are tested genetically. Diagnosis needs to be based on symptoms and characteristic once all else is ruled out.

Getting a clinical diagnosis, based on our symptoms, is the most important step we can take. Once it is on paper, we are at least believed for emergency care issues, hospitalizations, possible medications, school issues for children, insurance, validation, vindication, disability and much more.

It is an individual decision to do genetic testing once one has a clinical diagnosis and best made knowing all of the facts.

The Plan

Before I had a diagnosis and I was very ill, Calvin was beside himself with worry because of the severe paralytic episodes I was experiencing four and five times a day and every night. I was having difficulty breathing during them. He met with a technician for medical equipment when we were trying to get oxygen and he told her the story. She told him the best way to get the help we needed and a diagnosis was to, “Hit them (doctors) between the eyes with the facts.” That is just what we did!! Based on that idea I have created the plan for others to use to gain a diagnosis. It is presented in an outline format to make it easy to follow and to use as a checklist.
~~~~~~~~~~~~~~~~

You must gather all of the facts:
Ø       It is important for everything else to be ruled out. Your PCP and a neurologist do these, because most of the symptoms resemble neuromuscular disorders. The tests ruling everything else out might include but are not limited to:
§         Lab work of all types,
·         Blood
·         Urine
§         MRI's,
·         Brain
·         Spine
§         Spinal taps,
§         X-rays
§         EEGs
§         EMGs
§         EKGs
§         CMAPs
§         Muscle biopsy
(Some of the tests above may show changes that can be markers for PP).
v      Most doctors diagnosing PP want lab work showing either:
Ø       Paralysis during shifting in normal ranges
Ø       Paralysis during shifting in low potassium and/or,
Ø       Paralysis during shifting high potassium
§         This is done by obtaining serum potassium levels
·         May need to be done several times until a baseline is established
·         Then during episodes every 5 to 10 minutes...not just one blood draw...there is no way to see the shifting otherwise.
·         It may be necessary for hospitalization in order to do this while in the paralysis.
·         May need to be done for more than 24 hours until each is documented, during the episodes.
·         If the shifting is in the normal ranges, it may never show up during tests, unless it is done every few minutes.
¨        50% of PP patients may not have potassium shifting out of normal ranges
¨       ATS patients may shift all three ways.
Ø       However, the latest information for diagnosing PP based on potassium levels in blood serum is as follows:
§         The potassium in the blood does not always shift above or below normal ranges in 50% of patients experiencing muscle weakness or paralysis or it shifts so quickly that it cannot be measured. Doctors need to diagnose a patient with Periodic Paralysis based on the patient’s symptoms, specifically, heart symptoms on an EKG and the muscle strength or weakness and history of episodes of paralysis.
Ø       *** The information above needs to be shared with your doctors. Most do not understand these concepts. ***
v      There needs to be periods of paralysis, either total or partial, which can be documented (or progressive, gradual, fixed muscle weakness, all other things ruled out).
Ø       Videotaping is the best way to do this.
Ø       It may be necessary for hospitalization in order for doctors to see an individual while in the paralysis.

Warning!
Under no circumstances should an individual provoke his or her symptoms or an episode of paralysis by omitting medication or ingesting foods or perform activities, which are known triggers. This is a very serious thing to do and can lead to death. Please read Chapter Twenty-Nine for more information on this subject.

v      ECGs or EKGs consistent with "ion channelopathy", Periodic Paralysis or Andersen Tawil Syndrome. For specifics see Heart Issues in Chapter Nineteen.
Ø       Needs to be done while in the paralysis so it may need to be done for more than 24 hours until each is documented, during the episodes.
§         Holter Heart Monitors are the best method.
v      Oximeter (oxygen) recordings
Ø       Indicating, levels dropping during paralysis.
Ø       If in an advanced case of PP, it may show hypoventilation.
v      Genetic testing is available
Ø       Not necessary, but helpful for treatment and prognosis
Ø       See below for Specifics for Genetic Diagnosing of PP
§         30% of all people with PP do not have identified mutations.
Ø       See below for Specifics for Genetic Diagnosing of ATS
§         30% - 40 % of all people with ATS do not have identified mutations.
v      Gather all previous medical records.
Ø       Be sure to ask for all doctors' records from each appointment you attend.
Ø       Get all lab records, x-rays, hospitalizations, etc.
v      Chart the triggers for the episodes.
Ø       See “Triggers” in Chapter Seventeen and “Discovering Your Triggers” in Chapter Twenty-Two”
Ø       Documenting an increase of episodes after eating carbohydrates or red meat, after exercising or after taking certain medications is important for being able to control the episodes and letting the doctor see what the triggers are for a diagnosis.
v      Documenting a reduction of episodes when using potassium is good. This can indicate the loss of potassium after shifting and may indicate low potassium levels.
v      Gather a team of doctors. (Knowledgeable about PP or willing to learn)
Ø       PCP
Ø       Neurologist
Ø       Electrocardiologist
Ø       Nephrologist
Ø       Endocrinologist
Ø       Counselor or therapist
Ø       Others as needed for symptoms
Ø       MDA doctors if possible
v      Gather as much medical information as possible from family members who may have symptoms similar to Periodic Paralysis. It has a hereditary component.
Ø       If one suspects Andersen-Tawil Syndrome, gather as much medical information as possible from family members and note the characteristics/symptoms. Create a family flowchart with this information. Adding pictures can be helpful in demonstrating the characteristics.


Each of the steps outlined above is further discussed and explained in detail in the books.

The bottom line is that the best way to get a diagnosis is to get as much information together as possible to prove your case. Video tape the episodes, keep a journal, put together a notebook with all of the testing results. Include ER visit reports, hospital records, doctors notes, records of your vitals, and more depending on your symptoms and your particular case. Both books have charts, forms and more to use for this purpose, including all the instructions.

Once you have all of the information together, you can:
“HIT THEM BETWEEN THE EYES WITH THE FACTS!”


"Living With Periodic Paralysis: The Mystery Unraveled"
https://www.createspace.com/4111713

"The Periodic Paralysis Guide And Workbook: Be The Best You Can Be Naturally"

Saturday, March 14, 2015

Periodic Paralysis and "Post Traumatic Medical Stress Disorder" (PTMSD)


Periodic Paralysis and "Post Traumatic Medical Stress Disorder"  (PTMSD)  Thank you Tanya!
 
A few weeks ago one of our members was venting, as we all do from time to time and have the right to do,  about being tired of being “sick” and experiencing a myriad of symptoms. Another member, wanting to be helpful, suggested that some of her symptoms sounded like another condition that may be coexisting with Periodic Paralysis and suggested seeing a specialist to seek another diagnosis.  The discussion turned to others saying they were not in the “mood” to be seeing more doctors and trying to get more diagnoses. To that, another member agreed that she too did not want to investigate any more medical issues. She said she had “ Post Traumatic Medical Stress Disorder” and wanted nothing to do with any more doctors.

To this comment I said, “Oh my gosh!  I love that term! I believe most of us with Periodic Paralysis suffer from it. Post Traumatic Medical Stress Disorder PTMSD! I am with you. I have so many medical issues, but I am not willing to follow through with any more doctors and any more testing for any more diagnosing, especially when I cannot take medications or drugs anyway!! It is too stressful and expensive. I follow the "Plan" and am doing as well as I can. I believe I may write a ‘blog' article about PTMSD. It will be our 100th blog article!!”

I finally have a free minute to put the article together. Instead of reinventing the wheel, however, I am going to gather or compile several articles in this blog  that have been written already about this subject. The horrible and despicable manner in which we are treated by doctors, thus creating Post Traumatic Medical Stress Disorder, is one of our main topics. Nearly every new member who joins us has the same story; years of testing and unforgivable treatment by the doctors and many still seeking a diagnosis; in a “Periodic Paralysis Limbo.”

Here are the facts:

Studies indicate that it takes an average of twenty years and is certainly very costly for someone with Periodic Paralysis to get a diagnosis. This is ludicrous and unconscionable. During those years an individual gets worse. They develop exercise intolerance, permanent muscle weakness, heart issues, breathing issues and are harmed from unnecessary medications. Some may even die.  Those twenty years or more consist of seeing doctor after doctor, specialist after specialist, taking test after test, because it is a condition in which a diagnosis is obtained by exclusion; it is diagnosed after everything else is ruled out.

Besides taking a long time it is very, very costly. I myself did not get a diagnosis until the age of 62. Some of our members in their eighth decade are still trying to get a diagnosis. Many die without one, some very early in life, while trying to get a diagnosis. My own great uncle died at the age of 41 during an episode.

Most have been given the diagnosis of “conversion disorder”, a mental condition. This is due to the doctors’ lack of understanding and using old and out-dated information about Periodic Paralysis and basically being too lazy to research any further to help their seriously ill patients.

It seems that doctors in general have learned little about Periodic Paralysis and even less about how to deal with chronically ill patients. They lack what is called "a good bedside manner."

I had recently considered writing another book, this time about the despicable treatment we have received and the horrible things that have been said to us and done to us while we were/are suffering and languishing in episodes of paralysis and weakness. Doing so would be cathartic and healing for us. We would be uncovering the truth of what happens when the examining room door closes or the ER curtain is drawn for privacy, when someone is extremely ill, in paralysis, afraid, vulnerable and unable to defend themselves. Instead I will address it here in another blog.

The arrogant, lazy, preoccupied and unprofessional doctor does not know what to do, so he/she begins to pinch the person, or stick them with a pin to get them to be honest, yells at them, calls them names, tells them to quit “faking”, lies in the hospital or office notes, for instance, “patient WILL NOT raise their leg” rather than “patient CANNOT lift their leg.” We have been ignored like naughty children in time-out, while left alone in paralysis until we stop misbehaving, when we may actually die if not treated for choking, breathing or heart stoppage and/or arrhythmia. They will tell us we are having ‘psuedo’ seizures and give us medications to make us worse or kill us after being told we cannot have drugs. They put us on IV's after telling them we cannot have them. They use lidocaine despite the information in our charts that it causes paralysis for us. They send us home despite still being in paralysis or they put us in the psyche ward of the hospital. They describe and force anti-psychotic drugs, which can kill us. They demand proof of the condition by genetic testing despite the fact that only about 50% of the forms of Periodic Paralysis have actually been discovered through DNA research. They force us into painful EMG muscle testing, over and over again despite the fact that it is not a conclusive diagnostic test. I could go on and on and have in both of our books and in many of our blog articles, which follow in this article.

We demand to be treated properly and with respect just like anyone else who has a devastating and disabling disease. Alas, I do not see that happening anytime soon, however, so most of us will continue to suffer from "Post Traumatic Medical Stress Disorder"  (PTMSD) and refuse to see any doctor despite what we may be suffering or experiencing.  We will refuse to call an ambulance, even though we should be in a hospital, we will suffer at home that, which no one should have to go through without medical assistance. Our family members, who care and believe us, must sit by and watch us knowing what will happen if we go to the hospital or call the doctor. They hope and pray they are caring for us correctly.

"Post Traumatic Medical Stress Disorder"  does exist for those of us with Periodic Paralysis, we experience it daily as we suffer through our episodes of paralysis and the myriad of other symptoms we experience while we attempt to treat ourselves. We know we have nowhere to go for proper treatment or care.

The following is one of the articles I wrote demonstrating the treatment received from a “specialist” by one of our members and my response to what he said. 

”Why Do You Need A Diagnosis?”

One of our members was at another frustrating appointment with a “specialist” attempting to get a diagnosis and proper treatment for her teenage daughter who has been suffering greatly during severe and painful episodes of paralysis several times a day and at night. The “specialist” in the process of being rude and arrogant, dared to ask the following question,  "Why do you need a diagnosis?” and then made the statement “There is no treatment," not really expecting an answer or reply.  Distressed and dismayed at yet another doctor’s demeaning, dismissive and egotistical attitude and no diagnosis for her ill and suffering daughter, our member related the story to us on our PPN Support and Education Group.

In answer to the doctor’s rhetorical question and ill-informed and unfortunate comment, Calvin responded with the following: "Why do you need a diagnosis?" Answer...”To stop the insanity and abuse they create by mistreating the condition. This phrase should be included as one of the triggers we use to find another doctor.”

He is absolutely correct. The problem is individuals with Periodic Paralysis need a diagnosis. We need it for safety reasons in the doctors’ office, the dentist office, an ambulance ride, the ER, the hospital, for surgeries and in any emergency. Most of us cannot take any drugs or medications due to idiosyncratic, paradoxical and iatrogenic effects nor can we have IV’s because sodium and glucose can shift our potassium even lower and causes other life-threatening symptoms nor can we tolerate anesthesia due to possible malignant hyperthermia and/or more life-threatening arrhythmia, paralysis, possible cessation of breathing and death. We need a diagnosis so we can be safe and free from harm in any and all situations.

The medical professionals in our lives need to know that we have Periodic Paralysis and how to treat us or not treat us, as the case may be. They need to know that when we are in paralysis and struggling with arrhythmia, fluctuating blood pressure and heart rate, chocking, breathing issues and pain, that although we cannot open our eyes or speak, we can hear them. They need to know that we are not faking. We are not making it up. Why would we??? Does anyone really think we want to be totally helpless and struggling for our life????

We also need a diagnosis to stop the constant, expensive and insane cycle of testing and retesting for every condition under the sun for an average of twenty years out of our life. (Some of our members did not get a diagnosis until they were in their eighth decade.) Because genetic testing is costly, narrow, biased and can only diagnose about half of all patients, diagnosing must be done clinically, based on symptoms, once “everything else is ruled out.” REALLY?? How many medical conditions exist in which an individual intermittently has episodes of paralysis??

During the cycle of insanity for a diagnosis, drugs of every type are prescribed which are unnecessary and harmful causing more damage and possible death. New symptoms may develop and then more testing is done and new drugs prescribed. The insanity continues. Then comes the diagnosis of “conversion disorder” or “somatic symptoms”.  Psychotropic drugs are prescribed at this point. More damage is done and possible death may occur.

Without a diagnosis and proper treatment, the individual naturally becomes more ill because organs in the body are being damaged from the potassium shifting, exercise intolerance and gradual permanent muscle weakness sets in, heart problems get more severe, breathing muscles become affected, osteoporosis, kidney stones develop. Metabolic acidosis can kill us. Adaptive equipment like power wheelchairs and oxygen may be necessary. Without a diagnosis the patients will be unable to receive these much needed aides. Without a diagnosis, disability or social security is impossible to receive.

The child, teenager and young adult with Periodic Paralysis will need a diagnosis for appropriate treatment. He or she will need accommodations in school. Teachers and school nurses need to know how to deal with the symptoms and paralysis and understand what is happening and why. There may be a great deal of school missed. Sports and other activities need to be avoided. A special diet must be followed. Wheelchairs or other adaptive equipment may be needed. Without a diagnosis none of this will happen.

For the adult trying to support a family by holding down a job or a career and living with Periodic Paralysis without a diagnosis and proper treatment may lose their job. The years and years of medical testing, misdiagnosis, wrong medications, inappropriate treatment and more can result in financial ruin. This can lead to homes being lost, divorce may ensue, families will fall apart, friends back away, depression sets in and possible suicide may occur. A clear diagnosis and treatment may help others like employers, family members and friends to understand and be willing to help. Disability may be possible.

One of the most important results of a diagnosis for the individual with Periodic Paralysis and his or her family is validation. Validation that the illness does truly exist and that he or she is finally believed. They are vindicated. Vindicated of making it up, faking it or being a hypochondriac. They do not have “conversion disorder.” They have been telling the truth.

And so doctor, “Why do we need a diagnosis?” We need a diagnosis because we want a chance at the quality of life you are experiencing. We need a diagnosis because we want to be treated with dignity and respect. We need a diagnosis because we want to be free of or ease our paralysis, arrhythmia, heart issues, breathing issues and more with the possibility of proper treatment or medication. We need a diagnosis because we would like to use our family's hard earned money to take care of our needs rather than paying for unnecessary testing and harmful drugs. We need a diagnosis so we can obtain adaptive equipment to make our lives and that of or loved ones easier.  We need a diagnosis so we can be safe in emergency situations. We need a diagnosis because we want to live!!!

In conclusion, your rhetorical question and following comment indicate you lack any understanding of the ill patients who come to you for help. You obviously lack understanding, caring, compassion, empathy and sympathy. Your knowledge of Periodic Paralysis is obviously limited and based on archaic information because there is a great deal that can be done to treat our symptoms, once we have a diagnosis. You are obviously not living up to the Hippocratic Oath that I assume you pledged early in your career. You are not doing the job for which you we hired. And you sir are now fired!!!

~~~~~~~~~~~~~~~

The following are articles written by me and members of our PPN Support Group in the order that they were written, related to the problems with gaining a diagnosis and the mistreatment by medical professions, thus leading to "Post Traumatic Medical Stress Disorder"  (PTMSD)

These do not inlude the daily discussions of our members about this issue….

”Periodic Paralysis And Iatrogenic Illness”
http://livingwithperiodicparalysis.blogspot.com/2013/11/periodic-paralysis-and-iatrogenic.html


”Periodic Paralysis And The ER…The Narrative”
http://livingwithperiodicparalysis.blogspot.com/2013/12/periodic-paralysis-and-erthe-narrative.html


Conversion Disorder vs Periodic Paralysis”

“Finding a Doctor Who Cares”

“My Story: How I Got My Diagnosis Of Periodic Paralysis”

”Genetic Testing Should Be For Any Mutation Close To The Relevant Gene”
http://livingwithperiodicparalysis.blogspot.com/2014/09/genetictesting-should-be-for-any.html

”Why Haven’t They Done Genetic Testing”
http://livingwithperiodicparalysis.blogspot.com/2014/10/why-havent-they-done-genetic-testing.html

”Conversion Disorder Again??  Really?”
http://livingwithperiodicparalysis.blogspot.com/2014/10/conversion-disorder-again-really.html

”This is Not an Acceptable Way To Make  Diagnosis”
http://livingwithperiodicparalysis.blogspot.com/2014/10/this-is-not-acceptable-way-to-make.html


Until later...

Saturday, February 28, 2015

Phishing Scam Using Our Book...Please Beware.....



Hello All,

Well they have done it again!!! That group of con artists is using our book "The Periodic Paralysis Guide and Workbook: Be The Best You Can Be" again in a phishing scam!!! The last time I reported it to the FBI, the Sheriff, Google, etc but never heard a word other than the site was removed...Now I have received two new emails from Google with our book being given away "Free" once again.

We are signed up to Google Alerts to receive anything new posted that has anything to do with Periodic Paralysis, any and all forms of it. If we were not signed up, we would not know about the website and the scam.

This is such an embarrassment to us at the Periodic Paralysis Network, Inc and we hate to see people who are ill with Periodic Paralysis around the world being taken advantage of, being lied to and possibly being stolen from, in our name.

They request your credit card information before sending you the book.....They DO NOT HAVE THE ACTUAL COPY OF THE BOOK IN ANY FORM...but are using the photo of the cover and information from CreateSpace and Amazon. It looks very real. THIS IS NOT US!!!!!! PLEASE DO NOT GIVE THEM YOUR INFORMATION.

We cannot afford to give away our books for free in that manner. The money from our sales goes back into the organization, as well as the donations from GoFund Me and Bravelets, to pay for our costs and for Periodic Paralysis awareness.

I am not sure how to stop this from happening....I guess I will have to begin the entire process again....I am very frustrated and concerned and sad about this entire situation...

As always...thanks for your support!!!!!

Our books can be purchased through:

Our PPNI Website:
http://www.periodicparalysisnetwork.com/books.htm



Monday, February 23, 2015

Periodic Paralysis and the EMG (CMAP)


Hello All,

A member of our PPN Support, Education and Advocacy Group asked for information about EMG’s, also known as CMAP’s. These tests are often used to diagnose or rule out Periodic Paralysis. Unfrotunately, the results of these may or may not diagnose Periodic Paralysis depending on several factors: if it is done correctly, the type of Periodic Paralysis an individual has, whether they have had an episode recently, whether they are presently in an paralytic episode and more.

The procedure can be painful and uncomfortable for some individuals, but not unbearable. If it is done properly, it can diagnose only a few forms of Periodic Paralysis. A NEGATIVE TEST RESULT WILL NOT MEAN THAT YOU DO NOT HAVE PERIODIC PARALYSIS. Research indictes that some individuals who have been genetically diagnosed have negative results on the test or varying results on sequential testing.

A decrease of more than 40% (of the amplitude) is considered enough to diagnose PP. Any more than that is complicating to explain. If someone has not had an episode of paralysis in the three weeks before the test, it would be expected to be normal. The forms which may be detected using EMG (CMAP) testing are calcium and sodium channelopathies and thyrotoxic periodic paralysis.

So, for a diagnosis to be made from the results of an EMG/CMAP many things need to be considered. The following is more specific information and links compiled, which explain all of the difficult to understand specifics. Some forms of PP can easily be detected and others cannot and that depends on many factors and that also includes if the testing is done correctly. So, a negative result does not mean that some one does not have PP, just that it was not able to be detected.

For Hypokalemic Periodic Paralysis:

"Diagnosis can be achieved through a specialized form of electromyographic (EMG) testing called the long exercise test. This test measures the amplitude of a nerve response (called the Compound Muscle Action Potential or CMAP) for 40 to 50 minutes following a few minutes of exercise. In affected patients, there is a progressive fall in the amplitude of the potential.....Standard EMG testing cannot diagnose a patient unless they are in a full blown attack at the time of testing."
"Electromyogram (EMG). Muscle electrophysiologic testing must be performed during an interictal period. Protocols for implementation [Fournier et al 2004] and interpretation [Tan et al 2011] have been published. EMG testing includes: 

Assessment for myotonic discharges. 
Repeated short exercise tests. 
A long exercise test (the principal discriminating test for primary periodic paralysis).

The diagnosis of primary hypokalemic periodic paralysis relies on:
The absence of myotonic discharges Note: One family with combined heat-induced myotonia and cold-induced hypokalemic periodic paralysis has been described [Sugiura et al 2000].
The presence of a progressive and marked decrease in the amplitude of compound motor action potentials (CMAP) during a long exercise test [McManis et al 1986].

During an attack, EMG findings are not specific; EMG demonstrates a reduced number of motor units and possibly myopathic abnormalities.

Between attacks, EMG may exhibit myopathic abnormalities in individuals with fixed myopathy.

Specific exercise tests can assist with the diagnosis of periodic paralyses and nondystrophic myotonias [Fournier et al 2004]:
Short exercise test (SET). SET consists of recording evoked compound muscle action potential (CMAP) every ten seconds over one minute after a short effort (5-12 seconds) [Streib 1987].
Long exercise test (LET). LET consists of recording evoked CMAP over 30-45 minutes, every one to two minutes and then every five minutes, after a long effort (2-5 minutes, with brief 3- to 4-second rest periods every 15-45 seconds) [McManis et al 1986].
Five patterns (I-V) of abnormal responses to SET and/or LET in periodic paralyses and nondystrophic myotonias have been described [Fournier et al 2004]. Genetically defined periodic paralyses specifically result in: Pattern IV (no or rare myotonic discharges, increase of CMAP on  SET, immediate increase and late marked decrease in LET), more commonly seen in the hyperkalemic type OR Pattern V (no myotonic discharges, normal response to SET, no immediate increase but late marked decrease in LET), more commonly seen in the hypokalemic type.

A false negative normal pattern may be noted in some individuals who have a disease-causing mutation, especially in asymptomatic individuals or those who have not recently had a paralytic attack [Tengan et al 2004].


A decrease of at least 30% in CMAP amplitude and surface on LET is diagnostic for HOKPP. A decrease of less than 30% and greater than 20% is less specific and may indicate a different diagnosis. This decrease corresponds to pattern IV (with initial increment) and pattern V."

"During an episode of weakness, the compound motor action potential (CMAP) may be reduced, and rarely, absent on motor nerve conduction studies; insertional activity is reduced, fibrillation potentials and positive sharp waves are seen and there is an increased proportion of polyphasic motor unit potentials on needle examination (Engel et al., 1965). Surface and invasive EMG studies document reduced muscle fibre conduction velocity (Links and van der Hoeven, 2000). Interictal clinical electrophysiological testing (Table 2) is helpful in the diagnosis of PP. Changes in CMAP after exercise may correlate with exercise-induced symptoms (McManis et al., 1986) and channel mutation (Fournier et al., 2004). Myotonia on EMG needle examination occurs in ∼75% of individuals with HyperPP and in all patients with PMC; EMG myotonia does not occur in HypoPP. Electrodiagnostic studies at room temperature and after cooling the extremity may be more sensitive for HyperPP and PMC. The long exercise protocol also may detect a delayed decrement in ATS patients (Katz et al., 1999)." (more information at the following link)

http://brain.oxfordjournals.org/content/129/1/8

"EMG: In between attacks, there may be fibrillation
and complex repetitive discharges, increased by
cold and decreased by exercise (in hypokalaemic
periodic paralysis). During attacks, EMG will show
electrical silence, in both hyper- and hypokalaemic
periodic paralysis."
Electrodiagnostic testing is helpful in guiding genetic testing and in distinguishing between PP and other forms of weakness. Some patients with hyperkalemic PP show an initial increment during the
short exercise protocol.14 However, the short exercise test does not have significant diagnostic value in PP as most patients show no abnormalities.14 The LET is more useful in these patients. Some patients with hyperkalemic PP exhibit electrical myotonia; this finding is not described in hypokalemic PP.11,14
Long exercise test
LET is also typically performed stimulating the ulnar nerve at the wrist and recording the ADM motor responses. Supramaximal ADM responses are recorded at baseline, throughout 5 minutes of exercise, and then more than 45 to 60 minutes following exercise; investigators vary on the frequency of recordings following exercise.14,16,19 for 5 minutes with periodic, short-rest periods every 15 seconds. CMAPs are recorded after each minute of exercise. Some investigators then record postexercise CMAPs every minute for 5 minutes followed by every 5 minute CMAPs for 40 to 45 minutes.14 Others recommend postexercise CMAPs every 1 to 2 minutes for 30 to 45 minutes.19  In the initial version of the LET, decrements in amplitude and area were measured from the maximal CMAP obtained during or immediately after exercise.19,20 Subsequent investigators have measured decrement from the baseline 45 minutes.14  Others recommend postexercise CMAPs every 1 to 2 minutes for 30 to 45 minutes.19 In the initial version of the LET, decrements in amplitude and area were measured from the maximal CMAP obtained during or immediately after exercise.19,20 Subsequent investigators have measured decrement from the baseline demonstrated to have a sensitivity of 80% to 100%, which improved to 100% if CMAP area was examined alone.16 Normal controls typically show a small increment in amplitude and area (10%) following exercise and a subsequent decrement of 15% in amplitude and area compared with maximal-exercise CMAP (never more than 30%).19,20 

An abnormal decrease is defined as 40.9% for amplitude and 48% for area (2 SDs).19,20 When recorded after each minute of exercise. Some investigators then record postexercise CMAPs every minute for 5 minutes followed by every 5 minute CMAPs for 40 to 45 minutes.14 Others recommend postexercise CMAPs every 1 to 2 minutes for 30 to 45 minutes.19 In the initial version of the LET, decrements in amplitude and area were measured from the maximal CMAP obtained during or immediately after exercise.19,20 Subsequent investigators have measured decrement from the baseline CMAP obtained before exercise.14 One study shows the methods to be similar, although use of the preexercise baseline may be less sensitive in hyperkalemic PP.16 Among the small cohorts studied, the LET has a sensitivity of 80% to 90% in both hypokalemic PP and hyperkalemic PP.12,14,19,20 In one series of ATS, the LET was demonstrated to have a sensitivity of 80% to 100%, which improved to 100% if CMAP area was examined alone.16
           
Normal controls typically show a small increment in amplitude and area (10%) following exercise and a subsequent decrement of 15% in amplitude and area compared with maximal-exercise CMAP (never more than 30%).19,20 An abnormal decrease is defined as 40.9% for amplitude and 48% for area (2 SDs).19,20 When measured from preexercise baseline, an abnormal decrement in CMAP amplitude is defined as greater than 20% of baseline.14
        
Two typical LET patterns are observed in PP, which may help to distinguish between calcium and sodium channel mutations.14 Patients with a sodium channel mutation often exhibit Fournier pattern IV (Fig. 3) manifest by an increment in CMAP amplitude/area with exercise followed by a decrement in amplitude/area 40% to 80% of baseline (dependent on method); maximal decrement is observed between 30 and 45 minutes postexercise.14,16,20 Fournier pattern V manifest by a maximal decrement in area/amplitude after 20 to 40 minutes is more typical of calcium channel-related PP;
this decrement may or may not be preceded by an initial increment in CMAP amplitude/area.14,16,20 However, there is some cross over between pattern IV and V in hyperkalemic and hypokalemic PP, which may be explained by the proportion of hypokalemic PP patients who have an SCN4A mutation.14 Pattern V is most typical
in ATS.16

https://depts.washington.edu/neurolog/images/emg-resources/Myotonic_Disorders.pdf

Until later...