Serene Forest

Thursday, December 3, 2015

450 Members!!!!


Congratulations to us at the Periodic Paralysis Network Support, Education and Advocacy Group...We now have over 450 members!!!!
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" and "A Bill Of Rights For Periodic Paralysis Patients" the first in our ***A.S.E.A.Series!!!
( Found on our website http://www.periodicparalysisnetwork.com/books.htm ) (also on CreateSpace and Amazon.com)
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
Video about Periodic Paralysis:
https://www.youtube.com/watch?v=YSRVOkdFRQc
Thank you all for your support........

Thursday, November 12, 2015

Happy Second Anniversary For Our PPN Blog!!

Hello All,

Today is the second year anniversary of the day I created our Periodic Paralysis Network Blog and posted our first Blog Article!!!!

We now have over 118 Blog Articles about Periodic Paralysis and as of today, we have had 37,000 page views!!!!! This is exciting news!! It is wonderful to know that more and more people are learning about Periodic Paralysis.

Thank you all for your support!!



http://livingwithperiodicparalysis.blogspot.com

Tuesday, October 20, 2015

Permanent Muscle Weakness (Revised)


Hello All,

I have combined two previous blog articles into one with some revisions and updating. The subject of Permanent Muscle Weakness came up again…so I thought I should repost. It is a serious subject for most of us. It took several days of research to put this all together originally. I hope it will be informative and helpful to you.

Permanent Muscle Weakness in Periodic Paralysis



Many members of our Periodic Paralysis Network Support and Education Group discuss muscle weakness. It is very common to wake up in the morning unable to move, get out of bed or walk. As time passes, individuals are able to finally to move, drag themselves out of bed and walk with assistance, though they are very weak. As the day progresses, they either get a little better or remain in the weakened state. What most of them do not know or understand is, that they are in paralysis much of the night, most every night. Damage to the muscles is the result of this, thus creating gradual muscle weakness and over time Permanent Muscle Weakness (PMW) results.

I did some research on PMW related to Periodic Paralysis (PP) and discovered some interesting information. The damage done to the muscles is written about much less often than the episodes of partial or full paralysis in articles or studies about Periodic Paralysis. The information available, however, indicates that PMW is seen in all forms of PP, Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis or Andersen-Tawil Syndrome. Progressive muscle damage is also seen in all forms and it is irreparable. It cannot be reversed http://mda.org/disease/inherited-and-endocrine-myopathies/periodic-paralysesirreperable.

Acetazolamide is a drug that must be used with extreme caution. It can cause kidney stones, metabolic acidosis, low potassium levels, affects growth in children and has caused death.  The most important issue is that it can actually cause paralysis thus creating more permanent muscle weakness!

In another study it was concluded that Hypokalemic Periodic Paralysis is a myopathy (muscle disease, however, it is actually a mineral metabolic disorder, which affects the muscles) with permanent muscle weakness of late onset in all the patients. This study though older and only a few participants were used, indicated that most everyone with any form of Periodic Paralysis would develop permanent muscle weakness as they age.
http://www.ncbi.nlm.nih.gov/pubmed/2276049http://www.ncbi.nlm.nih.gov/pubmed/2276049

In the last study, there is correlation between PMW and abnormal muscle biopsies. This means that a muscle biopsy, completed for people with PP, may show abnormalities and damage in the muscle fibers namely changes in size and shape, vacuoles, splitting of the fibers, tubular aggreagates, increased glycogen (fat).

This overlooked subject is extremely important and needs to be addressed. Each paralytic episode causes more muscle damage so it is necessary to do everything possible to stop the episodes.

We know that avoiding the things that cause the episodes is the most important issue. So it is important to know what a person’s triggers are and avoid them. Other treatment may include following a pH balanced diet, using supplements, avoiding exerting oneself and more. Some individuals may use drugs, but they must be used with caution and as discussed earlier.

So the conclusions seem to be that all individuals with Periodic Paralysis will have some form of Permanent Muscle Weakness. We can also conclude that acetazolamide (diamox) seems to make it worse...thus this could be a problem for younger people with PP who take acetazolamide and begin to develop PMW (as well as kidney stones and metabolic acidosis and growth issues with children).

There is a correlation between your PMW and abnormalities in your muscle (muscle fibers). In other words, it is likely that if you had a biopsy, abnormalities will show up, if you have Permanent Muscle Weakness.


Yesterday’s blog brought a few questions:



The first one is about Physical Therapy (PT). Some one asked, “Can PT help the permanent muscle weakness (PMW)?”  My first response was, ”No, PT typically makes it worse.”

The teacher in me made me realize that answer was not sufficient or totally correct. So, I searched through my data collection looking for an article I had saved about that topic.
 
I wrote another post:

I should have said that for ‘most’ of us PT may not be a good idea. I know there are other members in our support group who do work out; weight-lift or exercise and I did during my earlier years. However, I have had to stop PT every time it was prescribed for me. The pain was excruciating and it caused more episodes and more weakness. There may, however, be things like warm water exercises, or massage and more that may be helpful. Each person is different and all you can do is talk to your knowledgeable medical professionals and try what you think may be helpful and work for you.

The following are a few articles, which may be helpful in making your own decision:

The first one is written by a physical therapist:

"In some neuromuscular diseases, such as the metabolic muscle disorders, strength building exercises such as weight training may lead to muscle deterioration. Muscle deterioration can also lead to kidney damage. Individuals with periodic paralysis may experience increased attacks of paralysis."

http://www.bellaonline.com/articles/art182120.asp


This one is about Periodic Paralysis in particular, which is a mineral metabolic disorder. It is an important message about deterioration of muscle from weight training:

"In some neuromuscular diseases, such as the metabolic muscle disorders, strength building exercises such as weight training may lead to muscle deterioration. Muscle deterioration can also lead to kidney damage. Individuals with periodic paralysis may experience increased attacks of paralysis.

Individuals with any type of muscle disorder should avoid exercising to exhaustion. They should stop exercising right away and consult their physician if they experience muscle cramping or paralysis, or cola-colored urine."

http://www.bellaonline.com/articles/art182120.asp

The last article deals with potassium and exertion/exercise:

"Increased Need For Potassium”

Heavy labor, weightlifting, extended labor, excessive sweating, alcohol, with caffeine (diuretic) (e.g. coffee, tea, some sodas) increase the need for potassium. A drop in blood sugar strains adrenal glands and also causes potassium loss.

Many weight lifters buy protein powder to make protein shakes. All that protein is hard on the liver and kidneys. Your body can’t build an ounce of muscle without enough potassium."

http://actualcures.com/weight-loss-diet-important...

The second issue from yesterday’s blog about permanent muscle weakness is that I failed to mention that there is typically three forms in which Periodic Paralysis may manifest related to permanent muscle weakness. That is:

Clinical Diagnosis

“The two distinct forms of muscle involvement observed in hypokalemic periodic paralysis (HOKPP), paralytic episodes and fixed myopathy, may occur separately or together. The pure paralytic episodic form occurs most commonly; the combination of paralytic episodes and a slowly progressive myopathy is less common; the pure myopathic form without paralytic episodes is rare:
  • Paralytic episodes. The primary symptom consists of attacks of reversible flaccid paralysis with a concomitant hypokalemia that usually leads to paraparesis or tetraparesis but spares the respiratory muscles.
  • Myopathic form. The myopathic form results in slowly progressive, fixed muscle weakness that begins as exercise intolerance predominantly of the lower limbs; it usually does not lead to severe disability. This fixed weakness must be distinguished from the reversible weakness that exists between attacks in some affected individuals.”

”The myopathic form develops in approximately 25% of affected individuals and results in a progressive fixed muscle weakness that begins at variable ages as exercise intolerance predominantly in the lower limbs. It occurs independent of paralytic symptoms and may be the sole manifestation of HOKPP.”

http://www.ncbi.nlm.nih.gov/books/NBK1338/
The above article does not mention the third type which is combination of the two; episodes of intermittent muscle weakness or partial or full body paralysis and gradual permanent muscle weakness.

My suspicion is that paralysis may be going on in sleep unaware to the person in the myopathic form.



Until later…


Tuesday, September 29, 2015

Sunday, September 27, 2015

Our Latest Book: "A Bill Of Rights For Periodic Paralysis Patients" the first in our A.S.E.A.Series




The Periodic Paralysis Network is excited to introduce our latest book, "A Bill Of Rights For Periodic Paralysis Patients" the first in our ***A.S.E.A.Series!!!

It is an easy read and a great educational tool for anyone who wants to know more about, or better understand, the rare mineral metabolic disorder, Periodic Paralysis.

The members of the ‘Periodic Paralysis Network Support, Education and Advocacy Group’ inspired "A Bill of Rights For Periodic Paralysis Patients." The members, now over 425 worldwide, share daily their heart-breaking and frustrating experiences. They relate frightening symptoms, years of painful and costly testing, a lack of being believed, abuse by doctors, denial of diagnoses and denial of proper treatment. These courageous and very ill people are under-recognized, under-diagnosed, misdiagnosed, misunderstood and mistreated by the medical professionals with whom they must deal and depend upon for their care. Their general rights; diagnostic rights; the right to proper treatment in the laboratory, by doctors and in the ER; the right to research and awareness and issues related to insurance rights have been and are being violated in many cases. This needs to stop.

For these reasons a bill of rights for individuals with Periodic Paralysis has been created, by the PPN. The information used to outline, describe and explain these concepts and rights are referenced and can be found at the Periodic Paralysis Network, Inc. Website, Blog Articles, Discussion Groups and Books.

***This series, called "The Periodic Paralysis Network A.S.E.A. Series," is designed to bring awareness of Periodic Paralysis to the world; to provide support to individuals with all forms of Periodic Paralysis and their family members; to educate individuals about all aspects of Periodic Paralysis to include medical professionals and to provide advocacy for those with the condition and their family members and caregivers. The PPN was created and exists to provide Awareness, Support, Education and Advocacy to and for all individuals with Periodic Paralysis, their family members and caregivers as well as all medical professionals, thus the acronym A.S.E.A.

Table of Contents

    About A.S.E.A.      vi
    Preface    viii
    Acknowledgements      ix
    Introduction       x
    What is a Bill of Rights?     13
    The General Rights     29
    The Diagnosis of Periodic Paralysis     41
    Treatment in the Laboratory     55
    Treatment by Doctors     63
    Treatment in the ER and Hospital     75
    Research and Awareness     99
    Insurance    105
    Conclusion    109
    Periodic Paralysis Forum    110
    About the Authors    111

A Bill Of Rights For Periodic Paralysis Patients
Amazon:
https://www.amazon.com/Susan-Q-Knittle-Hunter/e/B00HVEBSSQ/ref=dp_byline_cont_pop_book_1

CreateSpace:
https://www.createspace.com/5705192

Thursday, September 17, 2015

"New" Drug For Periodic Paralysis $163.80 For One 50mg Tablet!! (Revision)


"New" Drug For Periodic Paralysis $163.80 For One 50mg Tablet!!
I have written two blog articles in recent weeks about the drug Dichlorphenamide, also known as Keveyis, made by Sun Pharmaceuticals Industries Ltd (Taro Pharmaceutical). The first article was to announce a new drug, which had been approved by the FDA to treat Periodic Paralysis (PP). The second article was written after the facts were discovered about the new drug. Today, I find it necessary to write a third article since the cost for the drug has been announced and the stock market is showing gains for Sun Pharmaceuticals after the announcement.
I want to explain a few things before I continue, however. The Periodic Paralysis Network (PPN) was created to help individuals who have been diagnosed with Periodic Paralysis or those who are not yet diagnosed but have symptoms of Periodic Paralysis. Due to the nature of a mineral metabolic disorder, most drugs are harmful and even deadly for those with the varying forms of PP. The major way that the PPN has chosen to help others is to share natural methods to manage the symptoms and paralytic episodes. We have grown to be an organization to provide support, education, and advocacy to our members and attempt to bring awareness of PP to the world.
We are members of an international medical advocacy association working to provide safe and appropriate medical care, so as an advocacy group, we feel it is our duty to attempt to keep our members as safe as possible and to advocate for their medical rights. At least fifty percent of our members cannot take the  “new” drug due to terrible short term and long term side effects. Due to the reasons above and for liability reasons, we have chosen to refrain from discussing drugs in our support group and opt to discuss the natural and common sense methods.
When the new drug announcement was disclosed, we wanted to be fair and hoped for the possibility of something “new” and different, so we were happy to discuss it. However, we found out it was the same as the other drug already being used, which causes harm to a portion of our members. At that time, we felt in necessary to warn our members of the facts…a mistake with drugs can kill some of us…so it is a serious issue. In both articles, to be fair, we mentioned that some of us do well on it and we are happy for those members, but regardless of that, nearly one half of those individuals who do get some relief from it, continue to develop side effects, both short term and long term.
When the outrageous cost was announced, as well as the benefit this would have for the Pharmaceutical Company involved, and because we are advocates for ALL individuals with Periodic Paralysis and the members of our forum, we felt it necessary to write about this. I, myself, cannot take it, so the cost does not directly affect me, but some individuals with Periodic Paralysis can and do want to take it and some rightfully, want to try it. We support these individuals but warn them to use caution.
Information has come to me today that indicates the pharmaceutical company is now saying that the drug will be available for everyone, even if he or she cannot afford it. I hope that is the case, we will see in the future if that is true or not.
For those who wish to take the drug but cannot afford it; and for those who need to know or would like to know about the individuals for which the drug was created and for the pharmaceutical company that has seen fit to charge $163.80 for one pill that could change the lives of some very ill individuals, if they could afford it; I have written the following:
(I have edited my original article. This following is the edited version…)
This drug has been touted as a new drug and the first drug approved by the US Food and Drug Administration (FDA) to treat a group of metabolic disorders known as the Periodic Paralyses. Until now, off-label drugs (drugs used for other purposes) had been used with some success for some of the individuals with this disabling and serious medical condition.
For the community of people who suffer the horrible effects of this disorder, also known as an ion channelopathy, this was big newsa new drug AND it is approved by the FDA! This brought rejoicing and hope to the more than (under-estimated) 5,000 individuals in the United States with various forms of Periodic Paralysis (PP).

This excitement was short-lived, however, for more than half of the individuals who are incapacitated from this cruel condition.

 It turns out that Keveyis, also known as Dichlorphenamide, is not new at all. It is the same type of drug as the off-label drugs being used by some individuals. Some are having great success, but not without short and long term side effects. Dichlorphenamide is a carbonic anhydrase inhibitors (a diuretic) and it is sulfa-based. Some individuals with other forms of PP cannot use it without serious side effects. Some have nearly died from it and deaths have been reported from its use.

Another issue is, it is not new to Periodic Paralysis and has been used previously by patients with Periodic Paralysis for many years at a cost of about the same amount as a bottle of aspirin, but was pulled from the market while studies were done over several decades. Before that time some people had success with it, but others had terrible side effects from it.

During those years, many people with PP were involved in studies using the drug. Many of those individuals did well, but when the study was completed, the drug was no longer given to the patients. They had to go without any treatment or take the off-label drug that was not as effective.

 So we have a new drug that is not new at all, which was taken away from individuals who really needed it and were doing well with it. Then we had twenty-three years of studies being done at which time people with PP were using it and feeling some relief from their horrible symptoms and episodes of paralysis and gradual permanent muscle weakness. After that, the drug was taken away and the patients were left to fend for themselves, with the promise that sometime in the future, the drug would be available for everyone!

The drug is touted as being a treatment for
periodic paralysis”…to mean all formsthis is misleading. There are many forms of Periodic Paralysis. This drug can only be used for a few forms, or genetic mutations of this condition. To the remainder of those with the wrong mutations and who cannot take it, there are serious side effects and possible death.

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?

These realizations were very disappointing to many of us. We were hoping for more research and for possibly some new forms of treatment…not drugs, because most of us cannot take any form of drugs.

Then came the day with the announcement...The “new” drug is now available for use in the United States of America. The cost?????? $163.80 for one 50mg tablet!!! Most individuals need more than that a day, so for two tablets a day it is estimated that the cost will be $120,000.00 per year, for each person!!! Imagine a family with several people with the same condition? After all, it is an inherited disorder. Most insurance companies will not pay for this drug.

Most people who suffer from Periodic Paralysis are disabled and live on Social Security. This drug will never be available for them to use.

Many of those who wanted to try it were going to take a risk just to see if it might help, knowing the probable side effects. But now, this opportunity has been taken from her.

In the past year, I was contacted by three pharmaceutical representatives seeking information about and from individuals with Periodic Paralysis. I was told there was a “new” drug for Periodic Paralysis that was about to be released. They wanted the names of the members of our PPN Support Group so they could be interviewed about this 'up and coming' “new” drug.

I told each one that I could not give out the names of my members and that I wanted to know more about the drug before revealing such information. I did not want to give false hope to our members. I know that most drugs are harmful to us. I did an interview myself with the first team. I personally explained the facts to them about the issues with the use of drugs with a mineral metabolic disorder. I explained that most of us needed other things to help manage our symptoms.

We need potassium readers that cost $350.00 a piece and are not paid for by insurance. We need other medical devices to monitor our vital signs when in paralysis because during episodes of muscle weakness or paralysis, which may last many hours, there may be serious heart arrhythmia, fluctuating heart rate and blood pressure, choking, breathing issues and low oxygen levels. A patient may stop breathing or go into coronary or respiratory arrest or both and therefore, the patient must never be left alone during an episode and all vitals must be constantly monitored. I described the diet we must follow and how we need to avoid triggers, which can trigger the paralytic episodes. These include but are not limited to: most drugs including antibiotics, food fillers and dyes, pesticides, sugar, salt, gluten, stress, anesthesia, IV’s, stress (good and bad), exercise, exertion, carbohydrates and much more. I explained that, for those reasons, we need a special, natural and organic diet, which is very expensive.

I continued to explain that we have to pay for these things out of our own pockets because insurance does not cover them. I explained that if they really wanted to help us they could research ways to help us to get what we need and for insurance to pay for them. I discussed how most doctors, even the “specialists” know little about the condition. We need a way for all medical professionals to be trained properly about Periodic Paralysis for better recognition, diagnosis and treatment in a timely manner (my own diagnosis took over fifty years!) If they want to really help us, these are the things we need…not another drug we cannot take.

This team never did tell me about the drug or its name, so I did not pass along the information to the members of our Support Group since I had nothing to share.

The second representative never did get back with me after I asked him about the drug. He said he was not allowed to tell me about it, yet he wanted the list of names of all of the members of our support group.

The third representative approached me in a different manner and caught me off guard. She praised my writings on our blog and was terribly “moved” when she read my story. She wanted me to do an interview about the new drug and I told her all of the same information. She was allowed to tell me that at the end of the interview that the “new” drug was actually another carbonic anhydrase inhibitors and knew that it could not help me, or many of the others, from the information I had given to her. She seemed sincere and she assured me that they wanted to gain more information to know of other ways to help those of us who cannot take the “new” drug or any drug. I thought and hoped she was sincere. I now doubt it.

With a heavy heart, I would like to say to her and the pharmaceutical company, which she represents, for those individuals with Periodic Paralysis, who either want to take the drug, or at least try it, and for those of us who cannot take it:

ARE YOU KIDDING!!!!!!!! $163.80 FOR A 50mg TABLET!!!! MOST PEOPLE WILL NEED AT LEAST TWO PILLS PER DAY!!! THAT IS $120,000.00 FOR ONE YEAR FOR ONE PATIENT!!!! NO ONE WHO HAS PERIODIC PARALYSIS CAN AFFORD THAT AND IT IS UNLIKELY THAT INSURANCE WOULD PAY FOR IT!!!!!!!!!!!!!!! THIS IS UNCONSCIONABLE!!! THE PHARMACEUTICAL COMPANIES SHOULD NOT BE ABLE TO DO THIS TO THE SICKEST PEOPLE ON EARTH!!! WE ARE ALREADY DOWN!!!!! JUST KICK US SOME MORE!!!!!!!!

IF YOU REALLY WANT TO HELP US CREATE A DRUG OR A TREATMENT THAT CAN HELP ALL OF US, AND UNTIL THAT TIME BUY EACH FAMILY A POTASSIUM READER AND /OR AND OR AN ISTAT, AS WELL AS, ALL OF THE MEDICAL EQUIPMENT WE NEED FOR MONITORING DURING AN EPISODE, PAY FOR THE SPECIAL DIET WE MUST EAT, PROVIDE US WITH THE ADAPTIVE EQUIPMENT WE NEED, PAY FOR THE OXYGEN WE MUST USE WHEN OUR BREATHING BECOMES TOO SHALLOW DURING PARALYTIC EPISODES, PAY OUR MEDICAL BILLS AND COPAYS, AND MORE…YOU CAN ALSO PAY FOR TRAINING ALL OF THE DOCTORS AND MEDICAL PROFESSIONALS WHO DO NOT KNOW OR UNDERSTAND PERIODIC PARALYSIS. 

ACCORDING TO THE STOCK MARKET REPORTS WE ARE RECEIVING, ABOUT SUN PHARMACEUTICAL INDUSTRIES LTD, THE INTRODUCTION OF THE NEW DRUG KEVEYIS AND THE AMOUNT THAT EACH PILL WILL COST HAS RAISED THE VALUE OF THE STOCK/COMPANY ON THE STOCK MARKET! THE HEADLINE FROM AN ARTICE TODAY SAYS:

“POSITIVE NEWS FLOW PROPELS SUN PHARM’

THAT MEANS THE CEO’S AND STOCK HOLDERS WILL HAVE SOME EXTRA MONEY THAT THEY CAN DONATE TO THE EXTREMELY ILL AND DISABLED INDIVIDUALS WITH PERIODIC PARALYSIS. AFTER ALL, THEY WILL BE MAKING IT OFF OF THEIR BACKS; THE BACKS OF SOME OF THE MOST ILL AND MISTREATED INDIVIDUALS ON THIS PLANET WITH A DRUG THAT MAKES MANY OF THEM WORSE, HAS HORRIBLE SHORT TERM AND LONG TERM SIDE EFFECTS AND CAN EVEN KILL THEM.

I hope it is true that
Sun Pharmaceuticals Industries Ltd. is going to provide this drug to those who cannot afford, anything else is wrong.....


Links about the drug Keveyis and the pharmaceutical company:


Here is some more information about dichlorphenamide:

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



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


Articles and information about issues related to acetazolamide and diamox. 
Contraindications for oral diamox

More information about diamox:

Side effects for children:

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/2014/06/beware-of-off-label-drugs.html

Monday, September 7, 2015

Over 425 Members Now!!!

Congratulations to us all at the Periodic Paralysis Network Support, Education and Advocacy Group!!! We now have over 425 members!!!!!!!! Thank you all for your support!!!





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"

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

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, 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

Acetazolamide and Kidney Stones

http://epilepsy.med.nyu.edu/treatment/medications/acetazolamide-0#sthash.5ItPjcBR.dpbs 


Carbonic anhydrase inhibitors and the Eyes: 

Carbonic anhydrase inhibitors (CAIs) reduce eye pressure by decreasing the production of intraocular fluid.
http://www.glaucoma.org/treatment/glaucoma-medications-and-their-side-effects.php

...systemic CAIs possess undesired side effects such as numbness and tingling of extremities; metallic taste; depression; fatigue; malaise; weight loss; decreased libido; gastrointestinal irritation; metabolic acidosis; renal calculi (kidney stones) and transient myopia.
http://www.ncbi.nlm.nih.gov/pubmed/17504129

Myopia, also known as near-sightedness and short-sightedness, is a condition of the eye where the light that comes in does not directly focus on the retina but in front of it, causing the image that one sees when looking at a distant object to be out of focus, but in focus when looking at a close object.
(The myopia comes and goes causing dizziness, etc...)
https://en.wikipedia.org/wiki/Myopia

Transient myopia is a side effect of sulfa drugs:
http://onlinelibrary.wiley.com/doi/10.1111/j.1755-3768.1957.tb02240.x/abstract


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



Until later...