Serene Forest

Friday, November 21, 2014

Our New Book!!! The Periodic Paralysis Guide And Workbook

Hello All,

We are excited and proud to announce our new book "The Periodic Paralysis Guide And Workbook: Be The Best You Can Be Naturally" is now available in paperback on Create Space.

https://www.createspace.com/4326356?ref=1147694&utm_id=6026






(We are working on the eBook and Kindle versions but they are not yet available.)



Until later...

Thursday, November 20, 2014

"Living with Periodic Paralysis: The Blog" Happy First Anniversary!!


Hello All,

Congratulations to us!!! It has been a year since we created our Blog “Living with Periodic Paralysis: The Blog” and posted our first article on November 11, 2013. Since that time we have written and posted eighty-eight articles and we have had over 19, 520 page views!!!!

Our most popular article is Periodic Paralysis and Metabolic Acidosis with 461 views and a close second is Permanent Muscle Weakness in Periodic Paralysis with 453 views.

The following is the list of our articles:

Tuesday, November 18, 2014

Prognosis



Hello All,

One of our PPNI Support Group members asked about the prognosis for those of us with Periodic Paralysis. I decided the best way to answer her question was to use my original writing from our first book, "living with Periodic Paralysis" before we edited it. This article was changed and streamlined for the book.

At this time, I have lived three years beyond what we ever thought I would. For that I am thankful and owe it to following the plan outlined in our book; the pH balanced diet, discovering my triggers, avoiding exertion, avoiding stress, using oxygen and taking no drugs.
Prognosis

 As I began to research the prognosis for Periodic Paralysis, I found an inadequate amount of information provided, both in the number of articles and the amount written. I decided to include in this chapter the actual passages to prove my point. So, the following snippets of information are all I was able to locate in my research regarding what to expect now that we know we have Periodic Paralysis. Most of the passages are simply written by professional people who have no form of Periodic Paralysis. There are only two to three sentences, except for one, detailing the rest of our lives!!!! I did, however, find one article that gives an honest evaluation in just two sentences. It is the last quote below.


"Prognosis"

While the disability can range from minor, occasional weakness to permanent muscle damage, inability to hold a normal job and use of a powerchair, most people function fairly well with drugs and lifestyle changes. 1

What is the Prognosis?

The prognosis for the familial periodic paralyses varies. Chronic attacks may result in progressive weakness that persists between attacks. Some cases respond well to treatment, which can prevent or reverse progressive muscle weakness. 2
 
Prognosis

The prognosis for periodic paralysis varies. Over activity, bad diet or simply an unfortunate gene mutation can lead to a type of chronic, low level weakness called an "abortive attack," or to permanent muscle damage. Abortive attacks often respond to extra potassium, cutting carbohydrates, getting plenty of rest, increasing doses of medication and gentle daily exercise such as short walks. Permanent muscle weakness is just what it sounds like, permanent, irreparable damage to the muscles. Vacuoles and tubular aggregates form and destroy healthy muscle tissue. This type of damage should show on a muscle biopsy. Not even anabolic steroids can bring these damaged muscles back.

Life span is expected to be normal, but attacks can drop potassium to levels low enough to cause life threatening breathing problems or heart rhythm difficulties. Patients often report muscle pain and cognitive problems during attacks. Migraines occur in up to 50% of all hypokalemic periodic paralysis patients and may include less common symptoms like phantom smells, sensitivity to light and sound or loss of words. Medical literatures states that muscle strength is normal between attacks, but patients tell a different story. "Normal" for them is not exactly the same as "normal" for everyone else.

Because there are dozens of possible gene mutations, some drugs and treatments that work fine for one patient will not work for another. For example, most patients do well on acetazolamide, but some don't. Some patients will do well with extra magnesium (the body's natural ion channel blocker) or fish oil, while these same nutrients will make other patients worse. Patients and care givers should take extreme caution with all new drugs and treatment plans. 3

Outlook (Prognosis)

Hypokalemic periodic paralysis responds well to treatment. Treatment may prevent, and even reverse, progressive muscle weakness. Although muscle strength is initially normal between attacks, repeated attacks may eventually cause worsening and permanent muscle weakness between attacks. 

Possible Complications
  • Kidney stones (a side effect of acetazolamide)
  • Heart arrhythmias during attacks
  • Difficulty breathing, speaking, or swallowing during attacks (rare)
  • Progressive muscle weakness 4
Prognosis
While the disability can range from minor, occasional weakness to permanent muscle damage, inability to hold a normal job and use of a powerchair, most people function fairly well with drugs and lifestyle changes. On a lighter note, people with periodic paralysis are sometimes lovingly called Possums because they play dead when startled or stressed.5

Expectations (prognosis)

Chronic attacks may eventually result in progressive muscle weakness that is present even between attacks. Hypokalemic periodic paralysis responds well to treatment. Treatment may prevent, and even reverse, progressive muscle weakness. 6

Previously, primary PPs were regarded benign diseases as frequency and severity of attacks decrease with age (1). However, with advanced age, quite a number of patients develop permanent weakness and muscle degeneration with fat replacement (1). In individual cases, fatal cardiac arrhythmias may be a severe complication of the paralytic attacks…. (1). 7

How Bad Will it Get?
The Truth
When I began to research I wanted to know what to expect. How long will I live? How bad will I get? Can this disease be reversed if I get proper treatment? Will I lose my ability to walk? Will I ever drive again? Will I need to be in an assisted living program? Is there medication to stop the total paralytic episodes? What are my chances of dying from the long QT interval heart beat? Will my breathing continue to get more difficult until I can no longer breathe on my own?  Is there any medication I can take if I get another bladder infection? What happens if I need an operation and can’t use anesthetics? What can I do to stop the pain in my shoulder and back since I cannot take any pain medications?  When I go into cardiac arrest, is it worth trying to save me? Will I end up on dialysis due to kidney failure? Can I travel? What will happen if I end up in the ER again and they cannot help me with any medications?

 The short blurbs above are all I was able to locate. There are no doctors who can tell me. My renal specialist told me that he is unable do anymore for me since the diamox did not work. My neurologist tells me that he does not know what to do for me. My cardiologist says my heart condition, due to the PP, is “not treatable” for me.  I am, “not a candidate for a pacemaker“, “possibly a defibrillator later”. My Primary Care Physician (PCP) will not treat anything that has to do with my disease. Even the MDA doctors I saw did not recognize PP nor did they know how to diagnose it correctly. I was told by one of the MDA Healthcare Coordinators, that they need me to educate the MDA doctors so they will know how to treat me. This is not at all comforting. No one can tell me how to treat my symptoms or what to expect. 

Here is what I do know about myself and can review in answer to the above questions:

I am 66 years old and was diagnosed with Periodic Paralysis (PP) on February 7, 2011 at the age of 63. The kind I have is like Andersen-Tawil Syndrome Type 2. I have had episodes of partial and total paralysis for many years. During the episodes, my potassium shifts are low, high and within the normal ranges. Due to several misdiagnoses and a lack of proper diagnosis and treatment for over 50 years, I have become totally and permanently disabled with weak muscles throughout my body including those involved with my vision, digestion, breathing and my heart. I must be on oxygen constantly and cannot exert myself in any way. The electrical workings of my heart are defective. I have had a heart loop monitor inserted in my chest to monitor the tachycardia and arrhythmia, which include long QT interval beats. I now spend my days in a recliner, unable to walk farther than across a room. I must use a motorized wheelchair for anything farther. If I did not have the help of my husband, I would have to live in an assisted living program. I was misdiagnosed for many years. The medications given to me made me worse.

Through the past years of my physical decline, I have had to give up my career as a special education teacher, my hobbies to include hiking, walking, swimming, exercising, fishing, camping, traveling, shopping, cooking and baking. I had to sell, and move away from, a beautiful home in the mountains of Utah.  I can no longer drive. I have lost many friends, because I could not keep up with them or entertain any longer. I have lost contact with family members who did not understand or did not want to watch my decline or who thought I was a hypochondriac. I have lost the connection I once had with my grandchildren because I can no longer keep up with them or continue a meaningful relationship with them. The relationship with my husband has changed from husband and wife to caregiver and patient. Most of the over 37 doctors I have seen in the past 8 years have treated me poorly and like I was mentally ill.

I have spent the past several years working diligently to get a diagnosis and treatment for the ailment that cruelly stole the quality of my life. The most difficult part of this, for me, is knowing that I may not have became this seriously ill if just one of the over 30 doctors I had seen in the 6 years in Oregon before my diagnosis and the many years before, would have taken me seriously.

The following quotes taken from the above passages give us a clue of what can happen, but the passages from which I pulled them insinuate it is not “normally” what happens. I am in contact with many people from around the world who would beg to differ with this. The following is more in line with what is happening to people with PP due to lack of diagnosis and treatment.

“Permanent muscle weakness is just what it sounds like, permanent, irreparable damage to the muscles” 8

“Life span is expected to be normal, but attacks can drop potassium to levels low enough to cause life threatening breathing problems or heart rhythm difficulties” 9

“…some drugs and treatments that work fine for one patient will not work for another.”

“…repeated attacks may eventually cause worsening and permanent muscle weakness between attacks” 10

Possible Complications

·    Kidney stones (a side effect of acetazolamide)
·    Heart arrhythmias during attacks
·    Difficulty breathing, speaking, or swallowing during attacks (rare)
·    Progressive muscle weakness” 11
“…permanent muscle damage, inability to hold a normal job and use of a powerchair,” 12

 In my opinion, the truth is, the majority of people with this disease end up like me; very, very ill; but they are mis-diagnosed, under-diagnosed, called mentally ill, or hypochondriacs. They are diagnosed as suffering from conversion disorder, or having pseudo seizures. They are laughed at and scoffed at. They are told they are “too old” or it is not possible because they are “black”. They are given medications that make them worse. Doctors dismiss them and ridicule them and lie about them in the medical records. They do not get the medication or treatments they need. Then they die of things such as, “unknown” muscle wasting disease, accidental drowning in a pool or bathtub, cardiac arrest at age 40, stroke, or failure to thrive. The worse is from suicide; because if your doctors don’t believe you, how can your family members be expected to believe you. They just give up.

Our main reason for creating our website, the Periodic Paralysis Network, and writing this book, is for these people. They need to be diagnosed and get the proper medications and treatment before it is too late. We want to help them. We are hopeful that the doctors who see our website and read this book will become more aware of this disease and gain enough information to begin appropriately diagnosing their patients with Periodic Paralysis before it is too late.

In conclusion, many people with Periodic Paralysis will live normal life spans and their disability will be minor with occasional weakness. Some may actually not even have episodes of paralysis. Others will have moderate disability and receive proper medical treatment and medication. They will respond well, and may even reverse some of their weakness. But, others with Periodic Paralysis will have mild, moderate or severe disability and they will not receive proper diagnoses or treatment. They will become more disabled as the symptoms progressively worsen. They will suffer needlessly and may die due to complications. So, for some individuals Periodic Paralysis is a terminal condition.

Terminal?

Last year I became extremely ill. We had to call our local home health care. We were hoping they could come to the house and evaluate me by doing some lab work to see if I was in metabolic acidosis (to be discussed in the next chapter) or had some other infection of some type. I was hoping they could help Calvin take care of my needs. He is disabled too and cannot lift me. I could do nothing for myself. I could not eat. I slept most of the time and I was in and out of total body paralysis for weeks.

 A nurse and physical therapist came to our home. Upon evaluation it was decided that I did not qualify for their services due to some technicalities with my insurance and Medicare, mostly due to the fact that my symptoms were intermittent. We did get a visit from the nurse a few times over the following weeks but never got blood drawn. With each visit we discussed the ways we could get some services from them, as I needed them. They personally thought we deserved and needed the help from them.

 It was decided that I might qualify for services under the hospice wing of their services. Hospice is care and support to individuals with life-limiting conditions or illness and their families during later stages of the disease. Hospice is chosen and begins when comfort and management is chosen rather than attempting to reverse or cure the condition or disease. This was and is absolutely the case for me. 

 I have a disease with no known cure. There is no treatment I am able to use. I am progressively getting weaker and my breathing gets more difficult. I could die at any minute from arrhythmia. Because I can use no antibiotic, a simple infection can kill me.

 We had to discuss this with my Primary Care Physician. After recovering from the severity of the illness and regaining some strength, I researched the issues and wrote a paper arguing the possibility of receiving services through hospice. On my next visit, we discussed these issues. The following is the paper I wrote and presented to her.

Appointment with Dr. J W   Oct 24, 2011
 Issues

1.      No other doctors are caring for me. They say they “Can do no more for me”.
2.      I need a standing order for blood work for metabolic acidosis, lactic acidosis, bladder infections and anion gap, etc.
3.      I do not qualify for home health care but what about home services through Hospice?
4.      I am technically terminally ill.

 Medical issues

1.      I cannot take any meds for my conditions so they are just progressing.
2.      I cannot take antibiotics for infections.
3.      My breathing muscles are weak and getting weaker
4.      I need oxygen for heart issues, blood pressure and breathing.
5.      I have long QT interval heartbeats during paralysis.
6.      My breathing stops during paralysis.
7.      I experience choking during paralysis.
8.      My blood pressure increases during paralysis.
9.      My heart rate increases during paralysis.
10.  After paralysis I need assistance due to total body muscle weakness

Definitions of “Terminal”

"Terminal Condition"—defined as "a condition caused by injury, disease, or illness for which there is no reasonable medical probability of recovery and which, without treatment, can be expected to cause death." 13

Terminal illness is a medical term popularized in the 20th century for an active and progressive disease, which cannot be cured easily by popular medicinal practice. Curative treatment is not viewed as appropriate. 14

The term terminally ill in the context of health care refers to person who is suffering from a serious illness and whose life is not expected to go beyond 24 months at the maximum. 15
 Terminal illness…an advanced stage of a disease with an unfavorable prognosis and no known cure. 16
Terminal illness is a medical term popularized in the 20th century for an active and progressive disease, which cannot be cured easily by popular medicinal practice. Curative treatment is not viewed as appropriate. 17

The term terminally ill in the context of health care refers to person who is suffering from a serious illness and whose life is not expected to go beyond 24 months at the maximum. 18

 Terminal illness is a medical term popularized in the 20th century to describe a disease that cannot be cured or adequately treated and that is reasonably expected to result in the death of the patient within a relatively short period of time. This term is more commonly used for progressive diseases such as cancer or advanced heart disease than for trauma. In popular use, it indicates a disease which will eventually end the life of the sufferer. 19

 I am 66 years old and was diagnosed with Periodic Paralysis (PP) on February 7, 2011. The form I have is Andersen-Tawil Syndrome. I have had episodes of partial and total paralysis for many years. During the episodes, my potassium shifts are low, hypokalemia; high, hyperkalemia and within the normal ranges, normokalemia.

Due to several misdiagnoses and a lack of proper diagnosis and treatment for over 50 years, I have become totally and permanently disabled with weak muscles throughout my body including those involved with my vision, digestion, breathing and my heart. I must be on oxygen constantly and cannot exert myself in any way. The electrical workings of my heart are defective. I have had a heart loop monitor inserted in my chest to monitor the tachycardia and arrhythmia, which include long QT interval beats. I now spend my days in a recliner, unable to walk farther than across a room. I must use a motorized wheelchair for anything farther. If I did not have the help of my husband, I would have to live in an assisted living program. I was misdiagnosed for many years. The medications given to me made me worse.

 Complications and Symptoms of Periodic Paralysis: 

Permanent muscle weakness is just what it sounds like, permanent, irreparable damage to the muscles”

“Life span is expected to be normal, but attacks can drop potassium to levels low enough to cause life threatening breathing problems or heart rhythm difficulties”

“some drugs and treatments that work fine for one patient will not work for another.”

“repeated attacks may eventually cause worsening and permanent muscle weakness between attacks”
 “Possible Complications
Heart arrhythmia during attacks
Difficulty breathing, speaking, or swallowing during attacks (rare)
Progressive muscle weakness”

“…permanent muscle damage, inability to hold a normal job and use of a powerchair,” (These quotes are referenced above)

The Results

The doctor decided that hospice was an option for me. I did indeed fit the definition of being “terminal”. I am in an advanced stage of a disease called Periodic Paralysis, it has an unfavorable prognosis because of lack of treatment and medications and it has no cure. It was decided that when I felt it necessary, I could begin the hospice services. In the meantime, a standing order for blood work was set up and the home health care could draw it when needed. The doctor also agreed, at my request, to sign a “Do Not Resuscitate Document (DNR)”. This means that if my heart stops or I stop breathing, there is to be no CPR or any other type of life-saving measures used.  This document is displayed in plain view when and if an ambulance is called and recorded at the nearby hospitals.

And so, we now know that Periodic Paralysis can be a terminal condition for some individuals. It is in fact killing me due to a lack of knowledge and proper treatment. I cannot at this time tell anyone else what to expect or how bad it will get. I truly believe, however, that the sooner an individual begins to follow the appropriate plan for their individual needs and condition, they can and will do better and may live a long and healthier life. Although there is no magic cure, there is hope through following a plan of natural and commonsense methods to manage the episodes and improve the symptoms.
  
http://onlinelibrary.wiley.com/doi/10.1111/j.1600-0404.2007.00963.x/full
http://medical-dictionary.thefreedictionary.com/terminal+condition
http://en.wikipedia.org/wiki/Terminal_illness

Until later…

Saturday, November 15, 2014

Bravelets Cause!!

Good morning All,

At Periodic Paralysis Network Inc, due to several requests, we have set up the Bravelets cause!!

We chose the colors of silver, pearl and black. Silver is the color of potassium, pearl is for the color cream, which represents paralysis and black with the pearl covers the zebra theme. Silver and cream are the colors of our Periodic Paralysis Awareness Ribbon.

Each item bought gives $10.00 to the cause and for the awareness of Periodic Paralysis.

Thank you for your support!!

https://www.bravelets.com/bravepage/alone-in-the-dark-periodic-paralysis

Monday, October 27, 2014

300 Members!!!



Congratulations to us all at the PPN Support, Education and Advocacy Group!!! We now have over 300 members!!!!!!!! Thank you all for your support!!!

Our PPN Forum:

Please check out our PPN Members World Map: http://www.multiplottr.com/?map_id=55083
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 Book: "Living With Periodic Paralysis: The Mystery Unraveled"
(Found on our website)
PPN Blog: http://livingwithperiodicparalysis.blogspot.com/
PPN Book Discussion Group:
https://www.facebook.com/groups/periodicparalysisnetwork/
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

Tuesday, October 21, 2014

Periodic Paralysis Awareness GoFundMe

Hello All, 
We, at the Periodic Paralysis Network, Inc, would like to share our Periodic Paralysis Awareness Project. This is 'Invisible Disease Awareness Week' the perfect time to launch our fundraiser. Even if you cannot donate, please read about Periodic Paralysis and share this link with others. We need to share the facts and bring awareness to the world about this rare condition. Thank you.
http://www.gofundme.com/ftnr50

Until later...

Friday, October 17, 2014

"This is Not an Acceptable Way to Make a Diagnosis" Guest Blogger

Hello All,

In response to a post from one of our PPNI Support Group members about the ever controversial 'Conversion Disorder Dilema' another member,our guest writer Maureen McCutcheon, wrote the following:

These doctors know, but hope we don't know, that the number one criteria for diagnosing 'all in the head' (conversion, somatoform, functional neurological disorder, hysteria), is that ALL other possible diagnosis must be RULED OUT. They have to know that they are being lazy and unprofessional AT BEST! They just hope we'll accept their 'god-plex' label that they can get away with writing as the diagnosis into record. It seems that they don't think about or care about the ramifications for the patient. We are seeing them when we are our most vulnerable and unprepared and despite our many previous negative experiences,  we don't have some 'canned' responses of our own to these kind of comments (that he charged and got paid for). I feel a *rant* coming on that I wish I'd have the presence of mind to say to a provider at the time they say or do something like this: “Doctor, my presence here means I'm hiring this hospital and you for services. What you are doing is at best woefully inadequate, if not willfully inept. I want to see the ER director or highest person in charge of ER at this time (trauma nurse coordinator/supervisor or ??). I didn't come her to be treated in this disdainful, arrogant, dismissive way. There's a lot more ways I could spend my time and dime and I’m not here to waste your time or misuse medical resources. If you cared and meant your oath you took as a doctor, let alone the expectation this hospital /facility/ has from staff to implement Patients Rights to be treated with dignity and respect, then you wouldn't be doing this. What you are doing is not something you would want to be treated like or any of your loved ones. HOW WOULD YOU BE FEELING AND RESPONDING in my place if The doctor was treating you or loved one like you are treating me? By telling me that this is 'all in my head' you are revealing enough to me about you to know that I am NOT choosing to hire you as my doctor. You have revealed that you have a pre-c
onceived bias filter through which you perceive me. This is NOT acceptable…. NOT an acceptable way to make a 'diagnosis'.

Until later...

Monday, October 6, 2014

Conversion Disorder Again?? Really??


Hello All,

It appears I need to write one more blog about diagnosing Periodic Paralysis. There are so many aspects to it, that I find I need to write about it often.


One of our members who recently had genetic testing wrote this morning to let us know that the testing had come back negative for Periodic Paralysis and her doctor is now ready to diagnosis her with ‘conversion disorder.’ She also mentioned that the testing was for only two genetic mutations of Periodic Paralysis! This is unbelievable and unconscionable. She has intermittent episodes of muscle weakness associated with documented low potassium levels. It could not be more clear that she has a form of Periodic Paralysis. The following is my response to her post.
Dear Friend, 

I am so sorry that you are now caught in the 'Periodic Paralysis vs Conversion Disorder Dilemma' based on archaic information and inept and arrogant medical professionals.  You are the prime example of what I am continually writing about. The genetic testing done on you was biased and narrow. They only tested for two mutations!!!!!!! The professionals say only about 50% of those tested will have a positive finding because only about 50% of the known genetic mutations have been found. Right now that covers at least five known different mutations, SCN4A, KCNJ18, CACNA1S, KCNJ2, KCNJ5 under which scores of alleles (SNPs) exist and some combinations and interplay of those alleles and mutations on many exons cause Periodic Paralysis. How do they know only 50% or 60% have been found? So many people are told that they do not have PP after such useless testing and are then considered to have 'conversion disorder' that they are not even figured in to the numbers of those who possibly have PP! 

What I believe is, the number of those who are actually diagnosed genetically is much smaller than the overall number of those who actually have a genetic mutation. If you factor in the fact that we do not know how many varying mutations there may be in all of us and those that change with each generation, there is no way to know how many genetic mutations actually exist for Periodic Paralysis. Whole genome testing may be the only way to know or secure a genetic diagnosis if the research is done by someone who willing to look for such rare and unknown mutations.

That being said, I will say it again...."How many diseases or conditions actually exist that can cause intermittent paralysis or muscle weakness based on potassium shifting (even if it is within normal levels)???" Once the tests for those conditions have been completed and those conditions ruled out, then a form of PERIODIC PARALYSIS IS THE MOST LIKELY DIAGNOSIS...NOT MENTAL ILLNESS!!!!! Why does the diagnosis automatically become 'conversion disorder?' Conversion disorder is diagnosed in 1 to 3 in 10,000 and Periodic Paralysis is diagnosed in 1 in 100,000. I cannot help but wonder how many people diagnosed with 'conversion disorder' actually have a form of Periodic Paralysis or anyone of the between 5,000 to 7,000 known rare diseases, also called 'orphan diseases??

I wish I had some way to change the thinking of these doctors, but it seems to be, “I do not know what is wrong and I do not know what to do, so I will call it a mental disorder so I can refer you on to someone else. I do not want to deal with you any longer.”  Instead they should be diagnosing us with Periodic Paralysis based on our symptoms.

Perhaps the following blogs may help you to feel better about yourself and give you some clues on how to find a doctor who does care and wants to help.

Conversion Disorder vs Periodic Paralysis
http://livingwithperiodicparalysis.blogspot.com/2013/12/conversion-disorder-vs-periodic.html

Finding a Doctor Who Cares
http://livingwithperiodicparalysis.blogspot.com/2013/12/finding-doctor-who-cares-december-19.html

http://www.humanillnesses.com/Behavioral-Health-Br-Fe/Conversion-Disorder.html
http://ghr.nlm.nih.gov/condition/hypokalemic-periodic-paralysis
http://en.wikipedia.org/wiki/Rare_disease