Serene Forest
Thursday, February 27, 2014
Wednesday, February 26, 2014
Rhabdomyolysis
Hello All,
Yesterday someone asked me a question about the urgency to urinate after or as you are coming out of an episode of weakness or paralysis. The answer to this question is yes, this is a very common part of the process. This is due to the body retaining fluids during an episode.
This person also mentioned a problem with urine after an episode being dark brown and having an odd smell. Now this is not normal and the sign of a serious condition. In case others of you may have this happening I wanted to share my answer with you.
"Hello,
It is common for one to not have an urge to urinate
until after the episode and when coming out of the weakness or paralysis. This
is because the fluids in the body are elsewhere being retained during the
episode.
I always have an urge to urinate quite badly but it is always difficult for me to move or walk so my husband must get me there by way of my wheelchair and help me onto the toilet. This is the most embarrassing thing for me, though we have been married for nearly 34 years.
The thing that concerns me is the color you mention. That is a sign of a serious condition called Rhabdomyolysis, or muscle wasting. Do you take statins? Statins are a main cause of this. If not then it is even more serious. You should have it checked out right away.
Those with the genetic mutations found in CACNA1S, forms of Hypokalemic Periodic Paralysis are more susceptible to it."
According to Wikipedia:
"Rhabdomyolysisis a condition in which damaged skeletal muscle tissue breaks down rapidly. Breakdown products of damaged muscle cells are released into the bloodstream; some of these, such as the protein myoglobin, are harmful to the kidneys and may lead to kidney failure. The severity of the symptoms, which may include muscle pains, vomiting and confusion, depends on the extent of muscle damage and whether kidney failure develops.
...Release of the components of muscle tissue into the bloodstream causes disturbances in electrolytes, which can lead to nausea, vomiting, confusion, coma or abnormal heart rate and rhythm. The urine may be dark, often described as "tea-colored", due to the presence of myoglobin. Damage to the kidneys may give rise to decreased or absent urine production, usually 12 to 24 hours after the initial muscle damage...
...Causes: ...hypokalemia (low potassium levels)".
http://en.wikipedia.org/wiki/Rhabdomyolysis Rhabdomyolysis - Wikipedia, the free
encyclopediaen.wikipedia.org
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1126495/
"Rhabdomyolysisis a condition in which damaged skeletal muscle tissue breaks down rapidly. Breakdown products of damaged muscle cells are released into the bloodstream; some of these, such as the protein myoglobin, are harmful to the kidneys and may lead to kidney failure. The severity of the symptoms, which may include muscle pains, vomiting and confusion, depends on the extent of muscle damage and whether kidney failure develops.
...Release of the components of muscle tissue into the bloodstream causes disturbances in electrolytes, which can lead to nausea, vomiting, confusion, coma or abnormal heart rate and rhythm. The urine may be dark, often described as "tea-colored", due to the presence of myoglobin. Damage to the kidneys may give rise to decreased or absent urine production, usually 12 to 24 hours after the initial muscle damage...
...Causes: ...hypokalemia (low potassium levels)".
http://en.wikipedia.org/wiki/Rhabdomyolysis Rhabdomyolysis - Wikipedia, the free
encyclopediaen.wikipedia.org
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1126495/
Until later...
Sunday, February 23, 2014
Over 150 Members Now!!!
I wanted to let everyone know
that the Periodic Paralysis Network (PPN) now has over 150 members!!!!
Congratulations to us all!!!! Thank you all for your support!!!!
Metabolic Acidosis and pH Balance
Hello All,
Yesterday I had a very rough day. I am, however, doing better today. I did end up in paralysis and after coming out of it I was very weak and well you know the rest of that story! I did discover that I was in metabolic acidosis and had very low potassium so treated it with my potassium bicarbonate and sodium bicarbonate.
For awhile, I have been lazy and not used my pH meter. It is a little difficult to use, especially when I am not feeling well and on the edge of paralysis. So I discovered that Calvin had ordered the strips quite awhile back and did not realize we had them. I accidentally found them the other day.
When I woke up yesterday after 9 hours of sleep, a very unusual thing for me, and I felt really strange. I took my vitals and used the pH testing strip. I almost fainted when it was bright yellow and at 5.5 or lower. I knew immediately what to do but I went into paralysis anyway. After coming out of it, I continued to take more potassium and bicarbonate through the day and ate nothing that had any acidity whatsoever. By bedtime I was back in a better pH range and it held through the night.
So, my advice to all of you is to get some of those strips and also monitor your pH balance. PP is a mineral metabolic disorder which makes us prone to metabolic acidosis. When trying to figure out our triggers, the pH balance must be considered.
That is why we recommend the pH balance diet. It makes a huge difference if I follow it faithfully. I went from four or five full-body, severe episodes, lasting several hours each, a day to one or two a month which are less severe and lasted a shorter time.
If I had not realized the pH balance issue yesterday, with the levels I was at, I could have died. This is a very important issue for all of us.
Yesterday I had a very rough day. I am, however, doing better today. I did end up in paralysis and after coming out of it I was very weak and well you know the rest of that story! I did discover that I was in metabolic acidosis and had very low potassium so treated it with my potassium bicarbonate and sodium bicarbonate.
For awhile, I have been lazy and not used my pH meter. It is a little difficult to use, especially when I am not feeling well and on the edge of paralysis. So I discovered that Calvin had ordered the strips quite awhile back and did not realize we had them. I accidentally found them the other day.
When I woke up yesterday after 9 hours of sleep, a very unusual thing for me, and I felt really strange. I took my vitals and used the pH testing strip. I almost fainted when it was bright yellow and at 5.5 or lower. I knew immediately what to do but I went into paralysis anyway. After coming out of it, I continued to take more potassium and bicarbonate through the day and ate nothing that had any acidity whatsoever. By bedtime I was back in a better pH range and it held through the night.
So, my advice to all of you is to get some of those strips and also monitor your pH balance. PP is a mineral metabolic disorder which makes us prone to metabolic acidosis. When trying to figure out our triggers, the pH balance must be considered.
That is why we recommend the pH balance diet. It makes a huge difference if I follow it faithfully. I went from four or five full-body, severe episodes, lasting several hours each, a day to one or two a month which are less severe and lasted a shorter time.
If I had not realized the pH balance issue yesterday, with the levels I was at, I could have died. This is a very important issue for all of us.
This is a good link about the pH balanced diet:http://www.rense.com/1.mpicons/acidalka.htm
Thanks for your continued support.
Until later...
Until later...
Sunday, February 16, 2014
Periodic Paralysis and Anesthesia
Hello All,
This is an article written and created with a compilation
of information, excerpts, references and links related to the problems and
issues of the use of anesthesia with individuals with the various forms of
Periodic Paralysis. There is quite a bit of technical information, which can be
shared with your doctor, anesthesiologist or dentist (though most of them
should know this information).
Periodic Paralysis and Anesthesia
Article and Compilation
by
The
Periodic Paralysis Network (PPN) February 2014
Periodic
Paralysis (PP) is a mineral metabolic disorder, also known as an ion
channelopathy, which is a dysfunction of the ion channels. There are four types
of PP, Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis,
Normokalemic Periodic Paralysis and Andersen-Tawil Syndrome. Ion channels
transport the electrolytes, such as sodium and potassium through the cells.
This transport is faulty in individuals with ion channel dysfunction and
extreme care must be used when anesthesia is going to be utilized. This is due
to the possibility of developing serious symptoms such as breathing issues or
failure, arrhythmia, blood pressure issues, choking, muscle weakness or
paralysis, longer recovery after surgery, malignant hyperthermia or death.
Managing the use of anesthesia in individuals with Periodic Paralysis is mostly
aimed at preventing attacks of paralysis or the other symptoms during or after
surgery. The manner in which the situation is handled for the individual
depends on which form of Periodic Paralysis is involved. 1, 2, 3, 4, 5
Hypokalemic Periodic Paralysis and Anesthesia
For
individuals with Hypokalemic Periodic Paralysis, anesthesia is a known trigger
for paralytic episodes. According to research, in order to successfully manage
the patient there is need for an evaluation before surgery, avoidance of known triggers,
careful monitoring during the surgery and immediate and proper treatment if an
issue arises.
According to the National Journal of Maxillofacial Surgery, “pre-operative stress along with necessary fasting and administration of dextrose containing fluids” precipitates attacks. “The guidelines for care include control of plasma potassium, avoidance of large glucose and salt loads, maintenance of body temperature, acid-base balance, and careful use of neuromuscular blocking agents. Good pre-medication to allay anxiety, avoidance of stress and adequate analgesia is vital in preventing an attack. Fluctuations in electrolytes, infection, and pain can lead to paralysis in the post-operative period. Hypokalemia manifests earlier than paralysis and so its correction can prevent paralysis. Dextrose containing solutions administered during surgery should be avoided...” 6, 7, 8
This information was found in Miller’s Anesthesia written by Ronald D. Miller:
”Management of HypoPP patients should focus on avoiding triggers and medications causing shift of potassium. General anesthesia, postoperative stress, glucose-containing intravenous solutions, and long-acting neuromuscular blockers are associated with postoperative paralytic events.138 Epidural analgesia has been shown to reduce both pain-related hyperventilation and serum catecholamines, thereby minimizing changes in serum potassium levels.138” 4
Hyperkalemic
Periodic Paralysis and Anesthesia
Nothing was written about the use of anesthesia and Hyperkalemic Periodic Paralysis before 2002. Early research concluded that anesthesia might be used without complications if the potassium levels were with-in normal levels prior to surgery, if the carbohydrate levels were up, if anesthetic drugs, which released potassium, were not used and if normal body temperature levels were maintained.
This information was found in Miller’s Anesthesia written by
Ronald D. Miller:
”Potassium, cholinesterase inhibitors, and depolarizing muscle relaxants will aggravate the myotonia in HyperPP patients.65 Prolonged muscle weaken ss has been reported when succinylcholine is used.141 Although one third of patients had no signs of myotonia,142 masseter spasm and respiratory and skeletal muscles stiffness could still occur during intubation and ventilation.65 Therefore, neostigmine and succinylcholine should be contraindicated in HyperPP patients. Ideally, all patients with HyperPP need to be admitted preoperatively so that proper preoperative fasting can be accompanied by the administration of dextrose-containing potassium-free maintenance fluid.143 Postoperatively, HyperPP patients may remain paralyzed for up to several hours. Preventive measures such as maintaining normal body temperature and low serum potassium levels and avoiding hypoglycemia are helpful in limiting such paralysis.144 Although patients with sodium channel pathology have often been considered to be susceptible to MH, (Malignant Hyperthermia) there is no increased risk for MH in these patients.145 General anesthesia with and without nondepolarizing muscle relaxants has been shown to have satisfactory outcomes.141,143,144,146 Regional techniques may also be appropriate for this patient group.142,146 Abortion of the hyperkalemic attack may be accomplished by administering glucose, insulin, epinephrine, and calcium supplements, or alternatively, glucagon may be used. β-Adrenergic treatment with metaproterenol has also been shown to prevent attacks and facilitate recovery.” 4,9,10,11
Malignant Hyperthermia (MH)
As mentioned previously, individuals with Periodic
Paralysis are at risk for developing malignant hyperthermia during or after
surgery. All forms of Periodic Paralysis are the result of mutations on
Chromosome 17. Malignant hyperthermia is also the result of a mutation on
Chromosome 17, thus creating the potential for those with Periodic Paralysis,
including, Normokalemic Periodic Paralysis, to develop the serious and
life-threatening symptoms involved with the use of anesthesia. 12, 13
The following information about MH is an excerpt from an
article from CINCH (Consortium for the Clinical Investigation of Neurologic
Channelopathies), which may be shared with physicians, dentists and
anesthesiologists:
Malignant
Hyperthermia
“Patients with
ion channel diseases are at increased risk of malignant hyperthermia reactions
with general anesthesia for surgery. In malignant hyperthermia, muscle cells
become overactive (hypermetabolic) in response to the most commonly used drugs
for general anesthesia. With inhaled agents (such as halothane, isoflurane,
enflurane, desflurane, sevoflurane and even agents such as ether, cyclopropane
and methoxyflurane ) the ion channels in the muscle cells open and remain open.
This results in excessive calcium release causing muscles to continuously
contract, producing massive amounts of heat, rapid rise in temperature and
disruption of nearly every organ system in the body. In the past this reaction
was fatal in 70% of cases. However, with the introduction of the medication,
dantrolene, the mortality has now fallen to 5% of cases. It is essential that
anesthetists and surgeons are made aware that a patient has periodic paralysis
or even that a parent suffers with the disorder, if a son or daughter is going
for surgery. The anesthetists and surgeons should be familiar with
neuromuscular disorders and the associated risks. If they are not in a
specialist center, the patient may need to be transferred to such a center or
at the least the patient's neurologist should be contacted prior to surgery.
Prolonged Paralysis
Other medicines used
during general surgery are called depolarizing agents (such as
Succinylcholine). These medicines act by blocking the signal from the nerve
(Acetylcholine) from reaching it's receptor on the muscle. This causes
temporary paralysis in patients during surgery, relaxing the muscles to make
surgery easier. In patients with ion channel disorders, such as periodic
paralysis, this can result in prolonged paralysis.
Normally,
when people are given the drug succinylcholine to relax muscles, a little bit
of potassium from inside the muscle cells leaks out into the bloodstream. This
is because succinylcholine, like acetylcholine, docks on the receptors and
opens some gates in the cell, letting certain molecules flow in and out.
Normally, this poses no problem. In the periodic paralyses, ion channels that
normally open when acetylcholine docks on membrane receptors don't function
normally, and people experience temporary paralysis attacks in response to a
variety of dietary, exercise, stress-related and other environmental changes.
After
surgery, patients with periodic paralysis have found they woke up in the
recovery room and couldn't move; they may not get full strength back for hours
or days. The exact cause of the prolonged paralysis with anesthesia in some
people with periodic paralysis isn't known. It could be the stress of the
surgery and/or any of the anesthetic drugs. It is critical for the medical team
to distinguish between a periodic paralysis attack and a malignant hyperthermia
reaction as the treatments are completely different.” 14,
15, 16
Important link for more information related to Malignant Hyperthermia"
Andersen-Tawil
Syndrome and Anesthesia
Some research
indicated that malignant hyperthermia is not usually an issue for individuals
with Andersen-Tawil Syndrome. However, it is an issue because individuals with
ATS have shifting of potassium into both high and low ranges causing symptoms
and paralysis. The other issue with anesthesia use and ATS is a need for
special precautions due to the serious issue of the long QT interval heartbeat,
a diagnostic marker for the condition and Torsades de Pointes another extremely
serious arrhythmia. There are many medications that must be avoided, which are
used routinely in preparation for surgery and during surgery including the
glucose and sodium IVs, as well as most forms of anesthesia.7
General Anesthestic considerations for patients with LQTS Management
From Drexel
University:
Avoid triggers of QT prolongation and Torsadesde
Pointes (TdP)
Provide Peri-op:
Anxiolysis
β-blockade
Analgesia
Maintain:
Normothermia
Normoxia
Euglycemia
Normocarbia
Avoid hemodynamic extremes:
Bradycardia
Tachycardia
Hypotension
Hypertension
Correct serum electrolytes esp:
Potassium
Magnesium
Prophylaxis beneficial even with normal serum
concentrations:
Prevent and treat arrhythmia:
Continue ECG monitoring in more than 1 lead
If ICD/pacemaker, ensure proper functioning
Have defibrillator and temporary pacemaker
available
Consult cardiology as
needed 18
More important information
available for ATS and Anesthesia at:
http://acibadem.dergisi.org/pdf/pdf_AUD_108.pdf
Lidocaine
Topical, regional and local anesthesia may cause potassium to drop in individuals with Hypokalemic Periodic Paralysis, Normokalemic Periodic Paralysis or Andersen-Tawil Syndrome if it contains epinephrine. The most often discussed and utilized local anesthesia is lidocaine. For some individuals it may work well if the epinephrine is removed. For others it may cause hypokalemia or arrhythmia regardless of the epinephrine being removed. For others still, it may not work at all or the usual amount may be needed during a procedure. Lidocaine and other local types of anesthesia need to be used with extreme caution.4
Topical, regional and local anesthesia may cause potassium to drop in individuals with Hypokalemic Periodic Paralysis, Normokalemic Periodic Paralysis or Andersen-Tawil Syndrome if it contains epinephrine. The most often discussed and utilized local anesthesia is lidocaine. For some individuals it may work well if the epinephrine is removed. For others it may cause hypokalemia or arrhythmia regardless of the epinephrine being removed. For others still, it may not work at all or the usual amount may be needed during a procedure. Lidocaine and other local types of anesthesia need to be used with extreme caution.4
In Summary
“Although
neuromuscular diseases are relatively uncommon, patients with these conditions
will present to the operating room and to non-operating room procedure areas
for diagnostic studies, treatment of complications, or surgical management of
related or unrelated disorders. Overall debility, with diminished respiratory
muscle strength and increased sensitivity to neuromuscular blockers (NMBs),
predisposes these patients to postoperative ventilatory failure and pulmonary
aspiration, and may slow their post-procedure recovery because of difficulty
with ambulation and increased risk of falling. A basic understanding of the
major disorders and their potential interaction with anesthetic agents is
necessary to minimize the risk of perioperative morbidity.”19
”The anesthetic management of patients with periodic paralysis first involves knowing the patient's history and their particular disease characteristics. The concurrent diseases must be ruled out (such as Andersen's disease). The primary goal of the anesthetic is to avoid events (perioperatively) that are known to precipitate muscle weakness. Electrolytes should be normalized, hypothermia should be avoided and frequent monitoring of the serum potassium level is indicated. The ECG should be constantly monitored for signs of arrthymias. These patients can be considered at risk of MH, thus avoidance of MH triggers is indicated. Use of nondepolarizing muscle relaxants is thought to be acceptable, although abnormal sensitivity to these agents may be encountered and adequate muscle strength must be assured prior to extubation.” 20
Anyone with
Periodic Paralysis needs to be extremely cautious when planning any surgical
procedures, which may use anesthesia.
References and Links
Periodic
Paralysis general info about care with meds and treatment
Pre-operative
Hypokalemic Periodic Paralysis
Hyperkalemic
Periodic Paralysis
Malignent
Hyperthermia
Andersen-Tawil Syndrome
Conclusion
19.http://accessanesthesiology.mhmedical.com/content.aspx?bookid=564§ionid=42800567&jumpsectionID=42807020
19.http://accessanesthesiology.mhmedical.com/content.aspx?bookid=564§ionid=42800567&jumpsectionID=42807020
Until later...
Added September 4, 2015:PROLONGED PARALYSIS
Other medicines used during general surgery are called depolarizing agents (such as Succinylcholine). These medicines act by blocking the signal from the nerve (Acetylcholine) from reaching it's receptor on the muscle. This causes temporary paralysis in patients during surgery, relaxing the muscles to make surgery easier. In patients with ion channel disorders, such as periodic paralysis, this can result in prolonged paralysis...
..After surgery, patients with periodic paralysis have found they woke up in the recovery room and couldn't move; they may not get full strength back for hours or days. The exact cause of the prolonged paralysis with anesthesia in some people with periodic paralysis isn't known. It could be the stress of the surgery and/or any of the anesthetic drugs. It is critical for the medical team to distinguish between a periodic paralysis attack and a malignant hyperthermia reaction as the treatments are completely different.
https://www.rarediseasesnetwork.org/cinch/learnmore/faqs.htm
http://www.mhaus.org/.../be-prepared/associated-conditions
http://www.ncbi.nlm.nih.gov/pubmed/11870726
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112/
Added February 25, 2017
Other medicines used during general surgery are called depolarizing agents (such as Succinylcholine). These medicines act by blocking the signal from the nerve (Acetylcholine) from reaching it's receptor on the muscle. This causes temporary paralysis in patients during surgery, relaxing the muscles to make surgery easier. In patients with ion channel disorders, such as periodic paralysis, this can result in prolonged paralysis...
..After surgery, patients with periodic paralysis have found they woke up in the recovery room and couldn't move; they may not get full strength back for hours or days. The exact cause of the prolonged paralysis with anesthesia in some people with periodic paralysis isn't known. It could be the stress of the surgery and/or any of the anesthetic drugs. It is critical for the medical team to distinguish between a periodic paralysis attack and a malignant hyperthermia reaction as the treatments are completely different.
https://www.rarediseasesnetwork.org/cinch/learnmore/faqs.htm
http://www.mhaus.org/.../be-prepared/associated-conditions
http://www.ncbi.nlm.nih.gov/pubmed/11870726
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112/
Added February 25, 2017
Advances in Anesthesiology
Volume 2015 (2015), Article ID 562378, 6 pageshttp://dx.doi.org/10.1155/2015/562378
Review Article
Mutations in Sodium Channel Gene SCN9A and the Pain Perception Disorders
https://www.hindawi.com/archive/2015/562378/Friday, February 14, 2014
Some Forms of Periodic Paralysis are Worsened by Diamox
Hello All,
While researching I found out some interesting information about Hypokalemic Periodic Paralysis. A study from 2001 relates two different types: CACNL1A3=Hypokalemic Periodic Paralysis-1 and SCN4A=Hypokalemic Periodic Paralysis-2, each with several mutations.
When someone is diagnosed with Hypokalemic
Periodic Paralysis, clinically, (based on their symptoms), one must be very
careful. One form either does not respond to diamox (acetazolamide), causes
more paralysis or causes serious symptoms. So,
diamox 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
article:
“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.”
http://brain.oxfordjournals.org/content/124/6/1091.full.pdf
Until later….
Thursday, February 13, 2014
No Tourniquet Please!!!
Hello
All,
I wanted to write about an important issue today. It was
called to my attention recently that not every individual with Periodic Paralysis
knows that using a tourniquet when having blood drawn can result in potassium
levels which are higher than they really are. It is important to understand
that improper use of a tourniquet and the clenching of the fist can result in
false lab results for potassium levels. The pressure (too tight) and time (too
long) of the tourniquet can raise the level of potassium as much as 10% to 20%. This difference can be important when making a decision about treatment or trying to get diagnosed. The following articles explain this problem.
"Pseudohyperkalemia is typically caused by hemolysis during
venipuncture (by either excessive vacuum of the blood draw or by a
collection needle that is of too fine a gauge); excessive tourniquet
time or fist clenching during phlebotomy (which presumably leads to
efflux of potassium from the muscle cells into the bloodstream);[4] or
by a delay in the processing of the blood specimen."
http://en.wikipedia.org/wiki/Hyperkalemia#Pseudohyperkalemia
"Pseudohyperkalemia Caused by Fist Clenching during Phlebotomy"
http://www.nejm.org/action/showImage?doi=10.1056/NEJM199005033221806&iid=f001
http://www.nejm.org/doi/full/10.1056/NEJM199005033221806
http://www.ncbi.nlm.nih.gov/pubmed/2325722
http://en.wikipedia.org/wiki/Hyperkalemia#Pseudohyperkalemia
venipuncture (by either excessive vacuum of the blood draw or by a
collection needle that is of too fine a gauge); excessive tourniquet
time or fist clenching during phlebotomy (which presumably leads to
efflux of potassium from the muscle cells into the bloodstream);[4] or
by a delay in the processing of the blood specimen."
http://en.wikipedia.org/wiki/Hyperkalemia#Pseudohyperkalemia
"Pseudohyperkalemia Caused by Fist Clenching during Phlebotomy"
http://www.nejm.org/action/showImage?doi=10.1056/NEJM199005033221806&iid=f001
http://www.nejm.org/doi/full/10.1056/NEJM199005033221806
http://www.ncbi.nlm.nih.gov/pubmed/2325722
http://en.wikipedia.org/wiki/Hyperkalemia#Pseudohyperkalemia
"Excessive tourniquet time, too tight tourniquet or fist clenching
during phlebotomy (which presumably leads to efflux of potassium from
the muscle cells into the bloodstream) are other important cause of
fictitious hyperkalemia."
http://www.ijccm.org/article.asp?issn=0972-5229;year=2007;volume=11;issue=4;spage=215;epage=217;aulast=Sharma
But I guess we should ask first "When have you calibrated pressure
gauge of the tourniquet?" (unless the nurse tend to lose temper...)
http://www.tourniquets.org/use_care.php#calibrating_testing
But I guess we should ask first "When have you calibrated pressure
gauge of the tourniquet?" (unless the nurse tend to lose temper...)
http://www.tourniquets.org/use_care.php#calibrating_testing
It is imperative for
one with Periodic Paralysis to know the above information whenever blood is
drawn. Ask the technician or nurse to draw the blood without a tourniquet. Be sure to have this important information written and handy in case of an emergency or an
ambulance must be called and you are unable to speak. I keep this information in a plastic folder along
with everything I know is important and that the paramedics must know when
coming to my aid in an emergency. I approach it as if I will have no one with
me to explain my needs. I keep it near the door and take it with me when I
leave home.
More links:
http://www.questdiagnostics.com/dms/Documents/Physician-Newsletter/2011_fall.pdf
http://www.phlebotomy.com/pt-stat/stat0709.html
Monday, February 10, 2014
Happy Anniversary to US!!!
Hello All,
Calvin and Susan Hunter
Happy Anniversary to us!!!!!
I celebrated the third year anniversary
of my clinical diagnosis of Andersen-Tawil Syndrome, on February 7, 2011. That
is the date we consider the beginning of the Periodic Paralysis Network. With a
diagnosis in hand, we knew we needed to tell the world about Periodic Paralysis
and share our story. Over time, we wanted to help others get a diagnosis and
share the "Plan" we created of lifestyle changes and and other things
we discovered that saved my life and lessened the severity and amounts of my
episodes of paralysis. We wanted to create a place that others with Periodic
Paralysis, regardless of the type they had and regardless of they whether they
had a diagnosis or not, could join and receive support, friendship, education
and advocacy and to feel that they were not alone. We have definitely grown
since that day...a website, a support and education group with nearly 150
members, a blog, a board specializing in PP and genealogy, several Facebook
pages, a Pinterest site, a book and book discussion board (and two more being
written), a project to create a better set of criteria for diagnosing PP (in
progress), using a four part comprehensive survey, and the recognition as a
medical advocacy group by the national AdvocateDirectory.org, nominated by WEGO
Health for awards in the categories of Health Activist Hero, Best in Show
Facebook, and Best in Show Blog. We are
very proud of what we have accomplished and what we have become and hope
to continue for a long time to come. Thank you all so much for making this a
reality and thank you for your support!!!!!
Calvin and Susan Hunter
Saturday, February 8, 2014
The Truth About Diagnosing and Treating Periodic Paralysis
Hello All,
This morning one of our new members expressed, sadness and frustration over the fact, that after searching for a diagnosis and proper treatment for her obvious Periodic Paralysis (PP) symptoms, she has lost all confidence in doctors. I responded with what felt like I was writing a, “Yes, Virginia, there is a Santa Claus” letter. In this case it was “Yes, Virginia, there are very few doctors who know about Periodic Paralysis.” The following is what I wrote:
This morning one of our new members expressed, sadness and frustration over the fact, that after searching for a diagnosis and proper treatment for her obvious Periodic Paralysis (PP) symptoms, she has lost all confidence in doctors. I responded with what felt like I was writing a, “Yes, Virginia, there is a Santa Claus” letter. In this case it was “Yes, Virginia, there are very few doctors who know about Periodic Paralysis.” The following is what I wrote:
Dear Virginia,
Most of us who have Periodic Paralysis do not have confidence in doctors or medical professionals anymore. Periodic Paralysis is a mineral metabolic disorder, but neurologists who typically diagnose and treat it, see it as a neurological disorder and try to treat it as such or due to lack of education about it or misinformation about it, they do not recognize it so fail to diagnose it. The misdiagnosis or lack of diagnosis leads to mistreatment with wrong medications or no treatment. Either way, the individual with PP, becomes worse. This is called iatrogenisis, which is harm that is preventable from medical treatment or a lack of treatment.
Periodic Paralysis should be able to be treated by
an endocrinologist, because they are familiar with the thyrotoxic form of PP,
but those who will or know about PP are difficult to find. The so-called
"specialists" are involved in the research end of it but unless you
are genetically diagnosed or absolutely a "perfect fit" for their
studies you are not diagnosed or your diagnosis may taken away. This happened
to one of our members. She was later found to have the genetic mutation!!!!! I
have seen 5 specialists and discussed my case with 3 others. What I am saying
is true. None of them could/would help me.
My own research and experiences have led me to know
the best way to find a doctor to diagnose and treat your symptoms. We created
our website, our support group, our blog and wrote our books to share these
ideas with others.
We find it best to locate a good MD, usually an
internist is best, who is willing to work WITH you and learn WITH you. You do
this by calling the different doctors in your area; a clinic is the best, and
talk to the office managers. Tell them your story. Ask them to talk to the
doctors in their office or clinic about it and one will surely step forward who
wants to work with you. They will call you back with the name of a willing
doctor. You, then, need to take the lead with the information and with your
treatment.
The first place to start is your diet, strictly organic
and as pH balanced as possible. Then discover all of your triggers and avoid
them. Gather as many home medical instruments as you can to monitor and track
your vitals, at the very least a wrist blood pressure cuff, finger oximeter,
and potassium reader if possible.
Gather your own team of specialists as needed
through and with the help of your MD or internist. They will know or can find
physicians who will work with them and you.
IF YOU FEEL YOU MUST (not my advice), experiment
with the medications and different types of potassium available, but be
extremely careful as most have severe side effects and can make you worse. Do
not take them just because everyone else does and the doctors say to do it,
especially if your symptoms are made worse by the drugs, and if you do not have
a genetic mutation found yet. Some forms shift both high and low potassium and
some shift within normal potassium ranges so taking potassium may make it
worse.
Each one of us is a little different, even in the same family, and we must find what works for us as an individual.
Each one of us is a little different, even in the same family, and we must find what works for us as an individual.
It is hard work to totally manage and direct your
own care, but it seems to be the only way to do it for most of us. Accepting
that fact is also difficult. I was dying until my husband and I took charge of
my care. I went from four or five severe, full-body paralytic episodes a day
lasting for several hours each to one or two attacks a month with much less
severity, except for the episodes I have at night in my sleep. It is still a
game of experimenting at times and trying new things. We are always trying to
balance while “walking the tightrope.”
I wish I could tell everyone there is a "quick
fix," "magical cures," "healing medications," and
"perfect treatments," but I am sorry to say I cannot. The plan we have written about seems to work
the best.
Description and explanation of all of the above
information can be found in articles on our website and on our blog, in
discussion on our support group, as well as in our books, "Living With Periodic
Paralysis: The Mystery Unraveled" "The Periodic Paralysis Guide and Workbook: Be The Best You Can Be Naturally" "A Bill Of Rights For Periodic Paralysis Patients" and "What Is Periodic Paralysis?: A Disease Like No Other."
www.periodicparalysisnetwork.com
http://livingwithperiodicparalysis.blogspot.com/
DISCLAIMER: There are some very good doctors out there who are willing to help. But when someone has been through the wringer, it is best to try a new approach. Over 30 doctors in 6 years and about 6 more since then, gave us a good and real look at what most of us are going through. For those individuals, this may be an approach that will help them and give them some hope.....
Until later...
Friday, February 7, 2014
What is Normokalemic Periodic Paralysis?
Hello All,
What
is Normokalemic Periodic Paralysis?
Before writing
about Normokalemic Periodic Paralysis, I believe it best to review Periodic
Paralysis in general. I have written several articles previously about the different forms of
Periodic Paralysis namely Hypokalemic
Periodic Paralysis, Hyperkalemic Periodic Paralysis and Andersen-Tawil
Syndrome.
What is
Periodic Paralysis?
One of the neurologists who diagnosed me recently said, “Periodic Paralysis is a disease unlike any other. It is not a neuromuscular, mitochondrial or autoimmune disease nor is it a muscle myopathy. It is in a category all its own and needs to be treated in non-conventional ways.” He further stated, “Doctors need to keep an open mind and think ‘outside of the box’ when it comes to diagnosing and treating Periodic Paralysis.”
The following is an overview of the condition gleaned from years of research and my own experience.
Periodic
Paralysis (PP) is an extremely
rare, difficult to diagnose, hereditary, mineral metabolic disorder
characterized by episodes of muscular weakness or paralysis, a total lack of
muscle tone without the loss of sensation while remaining conscious. It is
passed from either the mother or the father to any of the children, male or
female. It is an ion channelopathy, a condition involving dysfunction of an ion
channel for potassium, sodium, chloride or calcium. Ion channels regulate ions
as they flow in and out of the cells.
There are
several known types:
Hypokalemic
Periodic Paralysis (Hypo PP):
Paralysis results from potassium moving from the blood into muscle cells in an
abnormal way. It is associated with low levels of potassium (hypokalemia)
during paralytic episodes.
Hyperkalemic
Periodic Paralysis (Hyper PP):
Paralysis results from problems with the way the body controls sodium and
potassium levels in cells. It is associated with high levels of potassium
(hyperkalemia) during paralytic episodes.
Normokalemic Periodic Paralysis (Normo
PP): Paralysis results from the movement or shifting of potassium within
ranges, which are normal (normokalemia).
Andersen-Tawil
Syndrome (ATS): Paralysis results
when the channel does not open properly; potassium cannot leave the cell. This
disrupts the flow of potassium ions in skeletal and cardiac muscle. During
paralytic episodes, ATS can be associated with low potassium, high potassium or
shifts in the normal (normokalemia) ranges of potassium.
Normokalemic
Periodic Paralysis
The commonly accepted
range for normal potassium in human beings is 3.5 to 5.0 mEq/l (milliequivalents
per liter), but these numbers may vary somewhat among labs. Our bodies work to
naturally maintain a fine balance, which is within that normal range.
Ninety-eight percent of potassium in the body is located within the cells and
the other two percent of potassium is outside of the cells in the blood. Blood
testing in a lab is used to measure the potassium in the body. There are also a
few different types of potassium readers available for purchase and use in the
home.
For individuals with
Periodic Paralysis, the “normal” ranges of potassium may vary significantly
from person to person. Results from a survey revealed some feel well and are at
their best at about 5.0 while others may do best at 3.8 or 4.3. The potassium
for these individuals shifts in several ways depending on the type of PP
causing many symptoms as discussed previously. It may shift higher or lower.
These shifts may be very slight yet cause paralysis as well as other serious
symptoms including but not limited to heart, breathing and blood pressure
issues. The shifting may also happen very quickly and be undetectable. This
shifting is then within the “normal” ranges of potassium, thus the name
“Normokalemic” Periodic Paralysis, although some research indicates it is not
necessarily a distinct or different form of Periodic Paralysis, but rather
Hyperkalemic Periodic Paralysis. However, it appears that 58% of those with all
forms of PP actually have episodes of potassium shifting within normal ranges
according to a recent survey of individuals diagnosed genetically and
clinically.
Because most of the
emphasis, literature and studies written about Periodic Paralysis are about
Hypokalemic Periodic Paralysis (low potassium) and Hyperkalemic Periodic
Paralysis (high potassium), the majority of medical professionals do not
understand or recognize Normokalemic Periodic Paralysis or the knowledge that
the potassium does not have to shift outside of normal ranges or that it may
shift too quickly to be detected to create the paralysis or other symptoms
which may be serious or life-threatening. It may also shift high or low and
return to normal ranges before an individual can be tested in a lab or be seen
in the ER.
This makes it difficult
when an individual is seeking a diagnosis. Neurologists suspect neurological
issues and prescribe very harmful medications, which may cause new symptoms or
physical therapy, which can be painful and cause episodes of paralysis.
Unfortunately, this may then lead to misdiagnoses of pseudo-seizures,
conversion disorder, malingering, attention seeking, and/or hypochondria. More
inappropriate and harmful medications and treatments are prescribed to treat
these issues. The mis-labels follow the patient from doctor to doctor and the
individual is never taken seriously.
These same issues are
rampant in an ER situation. Potassium in normal ranges, with paralysis and
other issues and uninformed medical professionals, can add up to all of the
above and the administration of IVs filled with sodium or glucose with a
psychotropic drug to treat pseudo seizures. This can lead to more serious
symptoms, permanent damage and even death for an individual with Periodic
Paralysis.
Another problem
resulting from potassium shifting within normal ranges for someone who has a
diagnosis of Periodic Paralysis, especially Hypokalemic Periodic Paralysis (low
potassium), is the issues of automatically taking a dose of potassium when
symptoms begin or being given an IV with potassium in the ER when the potassium
never left normal range. This may then cause a shift into high potassium levels
and create new or worse symptoms.
For those who have
symptoms and paralysis while potassium levels remain within normal ranges, the
best way to know how to treat it is to take note of the symptoms and record them.
If one is able to record the levels of potassium at home, keeping a running
tally when symptoms begin will eventually show either a trend toward high or
low potassium or a shifting both ways as is common in ATS. Depending on whether
one gets better or worse when taking potassium, may also be a clue of either
high or low potassium. Discovering and avoiding the triggers that set it off is
recommended.
When in paralysis and
accompanying symptoms, there may be actually nothing that can be done except
for time to pass and things to return to normal, because the potassium is
already in normal range. Remaining calm is also recommended in order to avoid
stress which can cause more shifting of potassium. If at previous times, taking
potassium helped, then it may be recommended. If the potassium made it worse,
it might be wise to avoid it.
An ambulance does not need to be called nor does someone need to be
rushed to the ER, doctor or hospital every time they become weak or paralyzed
due to potassium shifting. Most individuals will be fine in a few hours.
However, if one is having serious difficulty with breathing, heart, choking or
swallowing, then an ambulance may be necessary. Otherwise, the only important
reason for going to the ER, if a person does not have a diagnosis, is to get
the attacks documented, a "paper trail" is often needed. Proof of the
episodes must be established.
From Wikipedia:
“Also
of note is that potassium levels do not have to range outside of normal limits
to cause serious, even life-threatening paralysis. These diseases are not the
same as having a very low level of potassium (hypokalemia)
or high potassium (hyperkalemia) and must not be treated as such.
The total body store of potassium is usually normal; it is just in the wrong
place.”
For more information on when to call for emergency care can be found at:
http://livingwithperiodicparalysis.blogspot.com/2013/12/when-to-call-for-ambulance-december-3.html
October 1, 2014
I would like to add another important source to this article:
Hypokalemic Periodic Paralysis:
"Serum potassium level decreases during attacks but not necessarily below normal." "Creatine phosphokinase (CPK) level rises during attacks."
I would like to add another important source to this article:
Hypokalemic Periodic Paralysis:
"Serum potassium level decreases during attacks but not necessarily below normal." "Creatine phosphokinase (CPK) level rises during attacks."
Hyperkalemic Periodic Paralysis:"Serum potassium level may increase to as high as 5-6 mEq/L. Sometimes, it may be at the upper limit of normal, and it seldom reaches cardiotoxic levels. Serum sodium level may fall as potassium level rises."
http://emedicine.medscape.com/article/1171678-workup
Added July 25, 2015
"Normokalaemic periodic paralysis: In these attacks the blood potassium remains normal
In fact, it has recently been discovered that it is not the change in the blood potassium level that is the
primary problem in periodic paralysis. The primary problem in all of these conditions is that the normal pores which exist in the walls of the muscle cells don’t work properly. It does seem that changes in blood potassium levels can further hinder the function of these pores and that is why changes in blood potassium can be relevant. However, other factors separate from blood potassium can also worsen the function of the pores, so a change in blood potassium is not essential."
http://www.musculardystrophyuk.org/app/uploads/2015/02/periodic-paralyses.pdf
Until later…
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