Hello All,
We have had quite an increase in people viewing our blog. Thank you all so much for your participation. Please feel free to make comments and ask questions. We are always working on ideas for blogs so please pass along any suggestions for subjects you would like to read about. Today's blog was suggested by a member of our support group. It took several days of research to put it together. We 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.
In one study it was determined that some individuals who
were not helped by or who were worsened by acetazolamide (the main drug used),
responded well to dichlorophenamide another drug.
http://www.ncbi.nlm.nih.gov/pubmed/6855804
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!
http://www.webmd.com/drugs/drug-6755-acetazolamide+oral.aspx?drugid=6755&drugname=acetazolamide+oral&pagenumber=6http://link.springer.com/article/10.1007%2FBF00311396#page-1
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!
http://www.webmd.com/drugs/drug-6755-acetazolamide+oral.aspx?drugid=6755&drugname=acetazolamide+oral&pagenumber=6http://link.springer.com/article/10.1007%2FBF00311396#page-1
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.
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 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), but other medications, namely dichlorophenamide, may help. 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.
That being said, the only drug known to possibly improve
the symptoms of PP including possibly lessening the chances of PMW, dichlorophenamide
which was widely accessible previously, is not registered anymore and only
available for trials bring run by researchers.
The following is the results of a study from 2008:
“Although the carbonic anhydrase inhibitors have been used in the treatment of the primary periodic paralyses (PPs), their efficacy has not been demonstrated in double-blind, placebo-controlled trials. Therefore, we tested the efficacy of dichlorphenamide (DCP; Daranide), a potent carbonic anhydrase inhibitor, in the treatment of episodic weakness in the primary PPs. We performed two multicenter, randomized, double-blind, placebo-controlled crossover trials, one involving 42 subjects with hypokalemic periodic paralysis (HypoPP) and the other involving 31 subjects with potassium-sensitive periodic paralysis (PSPP). In each trial, two 8-week treatment periods were separated by an active washout period of at least 9 weeks. The primary outcome variable in the HypoPP trial was the occurrence of an intolerable increase in attack severity or frequency (end point). The primary outcome variable in the PSPP trial was the number of attacks per week. In the HypoPP trial, there were 13 subjects who exhibited a preference (in terms of the end point) for either DCP or placebo, and 11 of these preferred DCP. In the PSPP trial, DCP significantly reduced attack rates relative to placebo. DCP also significantly reduced attack rates relative to placebo in the HypoPP subjects. We conclude that DCP is effective in the prevention of episodic weakness in both HypoPP and PSPP. 2008”
https://www.medify.com/insights/article/10632100/randomized-trials-of-dichlorphenamide-in-the-periodic-paralyses-working-group-on-periodic-paralysis
It seems odd to me that a drug, which might actually help someone who has Periodic Paralysis is now unavailable to them or is only available for a few people taking part in PP studies and research (as soon as the study is completed the drug is stopped) or those who have enough money to get it through questionable channels. So for some people with PP they are in a "Catch 22" with acetazolamide. They can take acetazolamide and increase the chances of developing PMW or of an increase in the progression of muscle weakness, kidney stones, metabolic acidosis and more as well as growth issues for children. However, they cannot get or take a drug, dichlorophenamide, which may actually help them.
It seems odd to me that a drug, which might actually help someone who has Periodic Paralysis is now unavailable to them or is only available for a few people taking part in PP studies and research (as soon as the study is completed the drug is stopped) or those who have enough money to get it through questionable channels. So for some people with PP they are in a "Catch 22" with acetazolamide. They can take acetazolamide and increase the chances of developing PMW or of an increase in the progression of muscle weakness, kidney stones, metabolic acidosis and more as well as growth issues for children. However, they cannot get or take a drug, dichlorophenamide, which may actually help them.
I personally prefer and must use natural methods. I am unable to tolerate most drugs, including acetazolamide.
The following links provide more information for those
interested in researching dichlorophenamide
tests now going on
rare side effects metabolic
acidosis
prohibits use
not withdrawn for safety
reasons
http://www.scribd.com/doc/2768678/Notice-Human-drugsDrug-products-withdrawn-from-sale-for-reasons-other-than-safety-or-effectiveness8212-Daranide-dichlorphenamide-tablets-50-m
Wall Street Journal
http://www.onlinecanadianpharmacy.com/section/productgroup.aspx?DrugName=Daranide&Generic=no
buy in Cananda
Until later...
HI Susan and everyone.. I want to remind others that some of us who don't to our knowledge have full paralysis attacks also develop permanent muscle weakness.. I may paralyze during sleep but only once in my entire fifty some year life span have I awakened in a fully paralyzed state.. Could this be related to my normokalemic presentation of ats like syndrome? Perhaps, I am not certain. I believe it is this group of persons with ATS that are the most difficult to diagnose and most likely to be diagnosed as conversion disorder, fibromyalgia or some other disorder..We are the group also most likely to lose our children to the state as in the Justina Pelletier case, as we seek answers for our life long illnesses.. It is unconscionable that this is still happening in our modern hi tech country and medical care system but it does,,, these are not rare disorders more likely rarely diagnosed, misdiagnosed or dismissed as unimportant disorders..
ReplyDeleteYes, Karen, not everyone with PP goes into full paralysis during waking hours but they may still develop PMW. I believe, however, knowing the manner the way the PMW develops in people with PP, that many of us are in paralysis during our sleeping hours. The way one wakes up in the morning would be a good indication...the way I described it in the article. I have to admit, I am not sure how the type of PP plays into it at this time...perhaps another blog?? Yes, those if us who are difficult to diagnose or have been misdiagnosed or diagnosed with mental disorders, typically are worse. We can and do develop mitochondrial and autoimmune disorders, due to the lack of proper management and treatment and the improper treatment and drugs we are made to take. I wrote the book to educate about and bring awareness of PP to the world. I hope it is reaching the right people out there to make a difference for us and to help those who are still struggling with their wrong diagnoses and treatment. Thanks Karen for your support.....
ReplyDeleteI did some more research...this is what I found:
ReplyDeleteThe 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/