Balance and Periodic Paralysis

Balance and Periodic Paralysis

Hello All,

I constantly discuss staying ‘totally balanced’ in all ways in order to manage our symptoms…but what does that mean exactly?? The following is my explanation.

Periodic Paralysis is a Mineral Metabolic Disorder, which is also known as an ion channelopathy. This means that the levels of the minerals/electrolytes/ions in the blood can become abnormal or can fluctuate in error depending on several issues. There will be either not enough minerals in the blood or too many minerals in the blood. Many things we call ‘triggers’ can cause this for us including, but not limited to, drugs, IV’s anesthesia, some foods (junk food, processed foods-anything not natural), food fillers and dyes, exercise, exertion, temperature fluctuations, heat, cold, stress, sleep, sodium, sugar, carbohydrates.

For those of us with varying forms of Periodic Paralysis, when a trigger is introduced in our bodies, the minerals shift and it causes many symptoms, most notably, periods of paralysis. These can be either partial or full-body. Therefore it is important for us to stay balanced.

First, this means we must keep the electrolytes/minerals in balance. These include:

potassium (K+) Use potassium meter to monitor levels.

magnesium (Mg++)

sodium (Na+)

chloride (Cl-)

calcium (Ca++)

bicarbonate (HCO3-)

phosphate (HPO4–)

Second, other things that need to be well balanced:

Sugar/glucose: Use glucose meter (blood), follow a diabetic diet and eat smaller amounts more often to prevent sugar highs and lows.

pH/acid/alkaline: Use pH strips (urine and saliva), follow a pH or alkaline balanced diet.

Hydration/dehydration: Drink plenty of water, but not too much. Low or high levels of water/fluid affect mineral levels.

.

Salt/sodium: Salt can be a trigger, use low sodium foods with care, the salt in those foods is replaced with potassium for flavor so will increase potassium levels.

Body temperature: Use a thermometer. Fever can create symptoms. Heat and cold also causes symptoms.

Vitamins: Any vitamin imbalance can create symptoms.

Exercise/exertion: Too much exertion or exercise sets our symptoms into motion, it is important to know your own limits.

Blood pressure: Monitor with a blood pressure wrist cuff.  Blood pressure needs to be kept at normal levels. This can be done with diet and staying balanced.

Oxygen levels: Monitor with finger pulse oximeters, below 95% the cells begin to be starved causing damage. If blood pressure is up or down, oxygen may be needed to avoid damage. Low oxygen levels prevent the cells from working properly and it affects the brain, heart and energy levels.

Heartbeat: Monitor with finger pulse oximeters and blood pressure wrist cuff. If heart rate is too slow the heart has to work harder and the brain and other organs are not getting the oxygen they need and if the heart is beating too fast the organs and other tissue is being deprived of oxygen.

Carbohydrates: Too many carbohydrates in a meal affect HypoKPP, but HyperKPP needs more carbohydrates in the diet.

Sleep: A lack of sleep causes chemical imbalances in the body and affects insulin levels, among other issues.

Stress (good or bad): This causes adrenaline to rise, which lowers potassium levels and also affects insulin levels.

Drugs/medications/over-the-counter: Avoid at all cost. These will cause serious imbalances in many different ways, depending on their composition and our form of Periodic Paralysis and co-existing conditions.

If we can keep our bodies in balance, we can minimize our symptoms and improve our quality of life. It is a constant battle and not easy but the results are well worth it. We can be the best we can be, naturally, by following the above ideas. We equate it to constantly walking a tightrope.

This form helps to monitor the above issues in order to stay in balance.

Until later.....

Pain and Periodic Paralysis

Unfortunately, many doctors have the misconception, based on outdated and archaic information and data, that pain is a not a symptom and does not exist in patients who have Periodic Paralysis. This is a serious issue because these doctors refuse to recognize Periodic Paralysis and refuse to diagnose individuals who desperately need to be diagnosed because they experience pain.

Last year we created and carried out a series of four surveys attempting to gather as much information as possible in order to create a set of criteria that doctors could use to aid in diagnosing patients. Ninety-five percent of the participants indicated that they experience pain regardless of their diagnoses; Andersen-Tawil Syndrome, Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis, Normokalemic Periodic Paralysis or Paramyotonia Congenita. It was the same whether they were diagnosed genetically, clinically or still seeking a diagnosis.

It was discovered that the pain can be experienced before, at the beginning, during or after episodes or it can be intermittent or chronic (all of the time). The pain was described in many ways such as achy, burning, sharp, constant, tenderness, sudden, cramping, rigidity, contractions, tightening, stiffness, charley horses, growing pains or spasms. It was reported as only involving one limb or body part, partial body, the trunk, several body parts or the entire body.

The pain results from several natural ways depending on the type of Periodic Paralysis or genetic mutation. In some cases it is from the swelling of the muscles when they fill up with fluid as the potassium shifts. Some of the pain is from the rigidity and contracting of the muscles. A third cause can be the shifting of sugar with the potassium. A fourth cause may be from low magnesium. Cold can create rigidity and pain for some. Metabolic acidosis, which can often develop in Periodic Paralysis, causes pain in the bones and chest pain.

A fifth cause and huge issue that can cause us great pain is as follows...
We must never push ourselves when in an episode or coming out of an episode because our muscles are very weak and the connective tissue (ligaments, tendons, cartilage, adipose) and other organs have to take up the work of our failing muscles, which is not their job. This puts great stress on them which can cause damage to them and creates great pain for many us.

Waiting until your body is ready to move, etc. is something that we should/must do...most of us are in paralysis during our sleep and many of us wake up still paralyzed or partially paralyzed, so not pushing ourselves in the morning (or any other time) is important. You will know you are in an episode or coming out of it if in the morning (or any other time) you are weak, having trouble breathing and/or speaking or keeping your eyes open, among other things.

You should also not be moved, if possible in an episode for the same reasons.
***Please Do Not Push Yourself, Or Try To Push Yourself Or Let Others Push You Or Move You When You Are Weak, Having Symptoms, Or In Partial Or Full Paralysis.***

Other conditions or diseases can co-exist causing permanent or intermittent pain such as Ehler-Danlos Syndrome (EDS), arthritis or fibromyalgia. These may be aggravated when an individual with Periodic Paralysis is in an episode or paralysis. Intermittent paralytic episodes can damage organs in the body, including the muscles. For some individuals the pain becomes permanent and may be misdiagnosed as fibromyalgia, or other medical issues like rheumatoid arthritis. Pain may also be caused from other unnatural means. The off-label medications typically prescribed for Periodic Paralysis or other drugs such as statins may create pain.

The best method to avoid the pain during the episodes is to avoid the episodes by avoiding the triggers and maintaining a balance in diet, rest, hydration and other natural methods.

Some people with Periodic Paralysis may be able to take medications to ease the pain but many  people cannot handle medications. It is advised that the pain be treated with natural means such as eating certain foods, herbal teas, heating pads, warm baths, ice packs, relaxation techniques and massages.  Many more ideas can be found on the Internet.

http://www.healthline.com/health/hyperkalemic-periodic-paralysis#Overview1

http://en.wikipedia.org/wiki/Thyrotoxic_periodic_paralysis

http://en.wikipedia.org/wiki/Hypokalemic_periodic_paralysis

http://www.med.nyu.edu/content?ChunkIID=96851

http://www.medscape.com/viewarticle/410649_3

http://www.socialstyrelsen.se/rarediseases/hyperkalemicperiodicparalysisa

http://www.healthline.com/health/hyperkalemic-periodic-paralysis#CausesandRiskFactors2

http://www.webmd.com/drugs/2/drug-6753/diamox-oral/details/list-sideeffects

http://emedicine.medscape.com/article/242975-clinical
http://beyondmeds.com/2012/10/03/natural-pain-relief/



Until later...

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"

http://www.mhaus.org/

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://c.ymcdn.com/sites/www.pana.org/resource/resmgr/Docs/Tekwe,_C_Spring2011.pdf

http://www.ncbi.nlm.nih.gov/books/NBK1264/

http://www.researchgate.net/publication/40446152_Resuscitated_sudden_cardiac_death_in_Andersen-Tawil_syndrome

http://www.docstoc.com/docs/44783177/609085---ANESTHETIC-MANAGEMENT-FOR-CESAREAN-DELIVERY-ANDERSON-TAWIL

http://acibadem.dergisi.org/pdf/pdf_AUD_108.pdf

http://translate.google.com/translate?hl=en&sl=tr&u=http://acibadem.dergisi.org/pdf.php3%3Fid%3D108&prev=/search%3Fq%3DAnesthesia%2Band%2BAndersen-Tawil%2BSyndrome%26start%3D30%26client%3Dfirefox-a%26sa%3DN%26rls%3Dorg.mozilla:en-US:official%26channel%3Dsb%26biw%3D1138%26bih%3D508

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

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

1.http://en.wikipedia.org/wiki/Hypokalemic_periodic_paralysis

2.http://en.wikipedia.org/wiki/Ion_channel

3.http://rarediseasesnetwork.epi.usf.edu/cinch/learnmore/faqs.htm

 Pre-operative

4.https://www.inkling.com/read/millers-anesthesia-7th/chapter-37/periodic-paralysis-hyperkalemic

Hypokalemic Periodic Paralysis

5.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3700163/

6.http://www.njms.in/article.asp?issn=0975-5950;year=2012;volume=3;issue=2;spage=220;epage=221;aulast=Abbas

7.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112/

8.https://www.google.com/search?q=Periodic+Paralysis+and+anesthesia&ie=utf-8&oe=utf-8&aq=t&rls=org.mozilla:en-US:official&client=firefox-a&channel=sb#channel=sb&q=Periodic+Paralysis+and+anesthesia&rls=org.mozilla:en-US:official&start=10

Hyperkalemic Periodic Paralysis

9.http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2044.1992.tb02327.x/pdf

10.http://journals.lww.com/anesthesiology/pages/articleviewer.aspx?year=2002&issue=07000&article=00033&type=Fulltext

11.https://www.inkling.com/read/millers-anesthesia-7th/chapter-37/periodic-paralysis-hyperkalemic

Malignent Hyperthermia

12.http://www.mountsinai.org/patient-care/health-library/diseases-and-conditions/periodic-paralysis-syndrome

13.http://www.ncbi.nlm.nih.gov/books/NBK1146/

14.http://en.wikipedia.org/wiki/Malignant_hyperthermia

15.http://www.mhaus.org/

16.http://www.nlm.nih.gov/medlineplus/ency/article/001315.htm

Andersen-Tawil Syndrome

17.http://www.ncbi.nlm.nih.gov/books/NBK1264/

18.http://c.ymcdn.com/sites/www.pana.org/resource/resmgr/Docs/Tekwe,_C_Spring2011.pdf

 

Conclusion
19.http://accessanesthesiology.mhmedical.com/content.aspx?bookid=564&sectionid=42800567&jumpsectionID=42807020

20.http://www.anesthesiaweb.com/protected/archive/ask/archive_ask_specific.shtml

Until later...

More links: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112

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

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/
 

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:

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.

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...

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

http://rarediseases.info.nih.gov/gard/4009/normokalemic-periodic-paralysis/resources/1

http://www.nlm.nih.gov/medlineplus/ency/article/003484.htm

http://www.ncbi.nlm.nih.gov/pubmed/22926674

http://omim.org/entry/170600

http://en.wikipedia.org/wiki/Periodic_paralysis

www.bami.us/Diet/Hyperkalemia.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."

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…‎