My Story: How I Got My Diagnosis Of Periodic Paralysis

Hello All,

      

 How I Got My Diagnosis Of Periodic Paralysis

I am 74 years old and was diagnosed with an extremely rare, hereditary, debilitating, genetic disorder called Periodic Paralysis on February 7, 2011 at the age of 62. The form I have is a variant of Andersen-Tawil Syndrome (ATS). I was misdiagnosed for over 50 years. How could such a thing happen in today’s world with all of the modern medicine and technology? The following is my story, information about this cruel disease and how and why I control the symptoms.

I have had episodes of partial and total full-body paralysis for much of my life. Due to a series of misdiagnoses and mistreatment with improper medications, I have become totally and permanently disabled with weak muscles throughout my body including those involved with my vision, digestion, breathing and my heart. I must be on oxygen constantly and cannot exert myself in any way. I have had a heart loop monitor inserted in my chest to monitor the tachycardia and arrhythmia, which include life-threatening long QT interval heartbeats. I sit in a recliner for most of my day and can walk (with a walker) only from one room to another or stay on my feet only short periods of time to do simple tasks like brushing my teeth. I must use a motorized wheelchair if I leave home or need to go any distance. If I did not have the help of my husband, I would have to live in an assisted living program.

Through the past years of my physical decline, I have had to give up my career as a special education teacher, my hobbies to include hiking, walking, swimming, exercising, fishing, camping, traveling, shopping, cooking and baking. I had to sell, and move away from, a beautiful home in the mountains of Utah.  I can no longer drive. I have lost many friends, because I could not keep up with them or entertain any longer. I have lost contact with family members who did not understand or did not want to watch my decline or who thought I was a hypochondriac. I have lost the connection I once had with my grandchildren because I can no longer keep up with them or continue a meaningful relationship with them. The relationship with my husband has changed from husband and wife to caregiver and patient. Most of the over 30 doctors I had seen in those 6 years have treated me poorly and as if I were mentally ill.

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

One Sunday morning after recovering from yet another full body paralytic episode, I searched once again on my computer for “periods of paralysis”. I was shocked and dismayed to read about a disease, which actually had all of the components many of my family members and I had been experiencing for so many years. Once I realized what I actually had, the struggle became even more difficult trying to convince my doctors. By this point, everything else had been ruled out, but no one wanted to diagnose me. I heard I was “too old” to have it. I was ignored. I was dismissed and told to go have a “good time” as long as I was in Portland, after driving 250 miles for the results of a muscle biopsy (The test did show myopathy (muscle disease), change in shape and size of muscle fiber but I was told it was normal). I was given lidocaine after telling my primary care physician (PCP) I could not have it during a mole biopsy. It caused an episode of paralysis but I was treated as if I were a naughty child behaving badly. I was left alone in the room in paralysis. I was in metabolic acidosis, twice in front of my PCP and sent home rather than to the hospital. My heart was in tachycardia and I could not breathe. After discovering that I was having long QT interval heartbeats on a Holter monitor (a marker for ATS), this was dismissed by my PCP, even after being told it meant I could go into cardiac arrest at any given moment. After two months, I had to request a referral to an electrocardiologist. The referral took two more weeks to get from my PCP and the insurance company.

During this time, I continued to decline as I had more and more severe total paralytic episodes. I had tachycardia and palpitations of my heart and I was having difficulty breathing. Sometimes my breathing would actually stop for a few seconds at a time. It felt like an elephant sitting on my chest. It was very frightening. Soon the difficulty of taking breaths in and out began to happen when I was not in paralysis. I found it more and more difficult to breathe. Every time I stood up, ate a meal or exerted myself in anyway, the breathing got worse and my heart would speed up until it was beating 130 to 140 beats per minute, even while I was eating.

 My husband became so concerned with the lack of caring being displayed by my PCP and our insurance company, that he walked into a medical supply company and told them what was happening and asked if they could help me to get oxygen because I could not breathe. After speaking with him for a few minutes, the manager told my husband that she would give all of the information he had carried in with him, to one of the technicians and that they would see what they could do for us. She told my husband that they find it is best to get all the information together and then, “Hit them (doctors) between the eyes with the facts”.

They hooked me up with a recording oximeter. It was discovered that my oxygen saturation levels were dropping dangerously low during my episodes of paralysis and it was apparent that they were low every time I exerted myself in any way. The technician took the information to my PCP and she had no choice but to sign a referral for me to get oxygen. At that point, we began to look for another PCP and decided to change insurance companies to avoid the need for referrals.

A month or two before this point, I was in despair over trying to find a doctor who knew about Periodic Paralysis. Then on the evening news, I saw their weekly feature of offering direct calls to doctors with any medical question. I quickly picked up the phone. After a wait of only a few minutes, I was speaking with one of the physicians. I asked her if she had heard of PP or knew of any doctors who might know about it. As luck would have it, she herself had a patient with it. She gave me the name of the neurologist the patient sees.

I went to my PCP with this information and talked her into giving me yet another referral. The neurologist eventually diagnosed me with “probable” Periodic Paralysis. He wrote a letter telling my PCP that I needed to see an electrocardiologist right away. It was several months before I got the referral. He described my heart condition, by that point, as serious with no treatment, but insisted I needed to have a heart monitor implanted. He also set up a renal specialist to help diagnose what he believed was Andersen-Tawil Syndrome based on all the information being presented to him by my PCP, the neurologist and me. I did get the diagnosis while in the hospital for the implant after going into paralysis and being observed by the doctors. The paralytic episode was caused by a mistake. They gave me a saline drip and lidocaine during the procedure. My diagnosis was actually based on an accident.

Since my diagnosis eleven years ago, my husband and I have created and now manage the Periodic Paralysis Network. We are an organization with an online community of over 1,300 people who are affected by Periodic Paralysis. The Periodic Paralysis Network provides a hands-on approach to understanding the disease, getting a proper diagnosis, managing the symptoms, and assisting caregivers and family members. Our focus is on educational resources and self-reliance. Our approach to treatment focuses on the self-monitoring of vitals and the management of symptoms through all-natural methods. We continue to do research and provide the latest information to our members. Everyone is welcome. Members will receive encouragement, support, sympathy, empathy and validation. Members will also gain information and knowledge about all aspects of Periodic Paralysis. Members ask questions and share ideas. We are usually on in real-time, answering questions and providing support as needed for our members.

We have also  written and published the books, Living With Periodic Paralysis: The Mystery Unraveled, The Periodic Paralysis Guide and Workbook: Be All You Can Be Naturally, A Bill Of Rights For Periodic Paralysis Patients and What Is Periodic Paralysis?: A Disease Like No Other. If you wish to know more about Periodic Paralysis, you may visit the Periodic Paralysis  Network.

 

Until Later...

***** I have now been diagnosed genetically with Andersen-Tawil Syndrome (ATS) KCNJ5 and Hyperkalemic Periodic Paralysis (HyperKPP)/Paramyotonia Congenita (PMC) SCN4A

What is Periodic Paralysis?

Hello All,

I am re-posting this article in two parts. It was not actually posted on my blog but I have a link to it in another site. There may be issues with the link so I decided to post it again. This article contains the facts about Periodic Paralysis and the other article, "My Story" is the personal narrative of how I got diagnosed.
                                             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, hereditary disease characterized by episodes of muscular weakness or paralysis, a total lack of muscle tone without the loss of sensation while remaining consciousness. It is passed from either the mother or the father to any of the children, male or female. It is a mineral metabolic disorder also known as an ion channelopathy, a disease 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. Andersen-Tawil Syndrome is discussed in more detail later in this section.

Only about 50% of the above known types of Periodic Paralysis have identified genetic markers. This means they can be diagnosed by DNA testing. The remainders of the cases are diagnosed by the symptoms and characteristics of the patient. This is called being diagnosed clinically. Those who are diagnosed clinically have symptoms and characteristics identical to others who have known genetic codes. One who is diagnosed with ATS clinically is said to have ATS Type 2 to differentiate.

Although Hypo PP, Hyper PP, Normo PP and ATS are forms of periodic paralysis the mechanism, which creates the muscle weakness and paralysis, is different as described above and the symptoms before and accompanying the paralysis vary. Symptoms can range from simple weakness to total body paralysis with life-threatening heart arrhythmia and tachycardia, breathing problems and choking. Death can occur in some rare cases. The episodes may last from a few minutes to several hours or many days. The speed with which the potassium shifts may cause symptoms to occur suddenly and without warning or there may be a gradual progression into the weakness or paralysis.

Abortive attacks may also affect some individuals. Occasionally, the common symptoms may begin but the full attack or paralysis may not occur. The person is left with extreme weakness and other symptoms such as extreme fatigue. This may last for hours, days or weeks.

The following symptoms may accompany the paralytic attacks. Most are based on my own experiences and the experiences of others I have talked with. Some may cross over.

One who suffers with Hypokalemic Periodic Paralysis may experience a variety of symptoms in relationship to their hypokalemic paralytic attacks including but not limited to the following:
Paralysis, total
Muscle weakness
Muscle stiffness
Muscle aches
Muscle cramps
Pins and needles sensation
Pulse issues-fast heart beat
Breathing problems-barely breathing
Hypoventilation
Irritability
Severe thirst
Nausea
Vomiting
Constipation
Excessive urination
Irregular heartbeat
Sweating
Tiredness
Paralysis, partial

One who suffers with Hyperkalemic Periodic Paralysis may experience a variety of symptoms in relationship to their hyperkalemic paralytic attacks including but not limited to the following:
Paralysis, total
Paralysis, partial
Muscle weakness
Muscle cramps
Tightness in legs
Strange feeling in legs
Tingling sensations
Pulse issues - absent, slow, or weak
Heart palpitations
Irregular heartbeat
Breathing problems-fast breathing
Mild hyperventilation
Nausea
Feeling hot
Slurring words
Sleepiness
Muscle twitching

One who suffers with Normokalemic Periodic Paralysis may experience a variety of symptoms including those seen in both the hypokalemic and hyperkalemic paralytic attacks as listed above.

One who suffers with Andersen-Tawil Syndrome may experience a variety of symptoms in relationship to their ATS paralytic episodes including but not limited to any of the symptoms from above depending on whether the attack is hypokalemic, hyperkalemic or normokalemic. They also have long QT interval heartbeats, a life threatening arrhythmia, which is a marker for ATS. Ventricular arrhythmia is common as is fainting.

The periodic muscle weakness or paralysis is triggered by a wide variety of activities such as exercise or sleep; foods such as carbohydrates or meat; conditions such as heat or cold; medications such as antibiotics or muscle relaxers; compounds such as caffeine or salt or simply resting after exercise. Many of the triggers are the same for most people but some of the triggers can be unique to each person or the type of Periodic Paralysis.

It is important to discover one’s triggers because of the need to stop the episodes, if possible, in order to regain some quality of my live and to prevent further damage to the organs as the potassium shifts and depletes in my body. This damage can lead to permanent weakness and disability as well as tachycardia and serious arrhythmia, including long QT intervals, which can lead to cardiac arrest. Avoiding paralysis is absolutely necessary for me, due to these life-threatening conditions.

Every moment of my present life I must control my symptoms. The following plan was created after trial and error in my own quest for treatment and management. I had no doctor assisting me and gleaned as much as possible on the Internet and in discussion with other people who live with Periodic Paralysis and Andersen-Tawil Syndrome. Anyone with Periodic Paralysis may follow this plan to see promising results.

Discover triggers: Simple carbohydrates,
Complex carbohydrates,
Most meat,
Wheat,
Gluten,
Salt,
Sugar,
Caffeine,
Medications including over-the-counter medications,
Exercise,
Exertion,
Rest after exercise,
Sleep, all aspects: falling asleep, during sleep, waking up and napping.
Stress (good or bad),
Dehydration
Msg
Food in general
Large meals
Fatigue
Fasting
And ?  I still have not discovered all of my triggers. No matter how careful I am, I can still go into paralysis without knowing why.

Control symptoms:
Avoid triggers
Following a proper ph balanced diet, eating from the farm; not the factory
Take no medications including over-the-counter medications
Avoid stress
No exercise
No exertion
Get plenty of rest
Stay well hydrated
Constantly monitor vitals
Take potassium when needed (if low potassium) (If under 3.0 go the the hospital)
Take sugar or glucose tablets as needed (if high potassium) (If over 6.5 go to hospital)
24/7 oxygen

Monitor vitals:
I use several pieces of medical equipment for measuring my vitals. These items are necessary for my caregiver to monitor me while in paralysis or for me to know which direction my potassium shifts for proper treatment.
Cardy meter (potassium reader),
Finger pulse oximeter,
Blood sugar monitor,
Stethoscope,
Wrist blood pressure monitor,
Thermometer and a digital
pH balance reader
Litmus paper


Gather a team of medical professionals:
I have an MD as my primary care provider and a cardiologist. I no longer have a renal specialist or neurologist.

Continue to gather information: I must educate myself, my family, my friends, my neighbors, my community, my doctors, my hospitals, my dentist, my optometrist and my local first responders about every aspect of my condition. Knowing and understanding this disease and syndrome eases my fears and the fears of those around me and assists me with proper management and treatment. Knowing others will be able to aid me during paralysis episodes is essential.

Join Periodic Paralysis social groups: Being part of a Periodic Paralysis community is vital. I have discovered I am not alone. I receive encouragement, support, sympathy and empathy. I gain information and knowledge from others who live with the same enemy daily. I ask questions and share ideas.

The above plan is followed constantly and diligently.  It is a constant “tightrope” I must balance, day-by-day, minute-by-minute; second-by-second. I cannot let up for even a minute or I can go into paralysis and the cycle begins anew.

Prognosis: Most individuals with Hypokalemic Periodic Paralysis are able to control the symptoms and paralytic attacks with one or two forms of potassium and avoiding the things that trigger them. Individuals with Hyperkalemic Periodic Paralysis symptoms and paralytic attacks can control their symptoms and paralytic attacks with a diet high in carbohydrates and sugar and by avoiding the triggers.

Controlling the symptoms and paralytic attacks in people with Andersen-Tawil Syndrome is much more difficult. This is due to the fact that these individuals suffer from paralysis due to potassium levels that can be low, high or in normal ranges. Taking potassium may make the symptoms worse. Also individuals with ATS are usually unable to take any forms of medication. Managing the heart issues by surgery is also a problem because anesthesia can trigger paralysis and deadly arrhythmia. For these individuals, natural methods are the best way to manage the symptoms.

For most people with Periodic Paralysis the weakness and paralysis are intermittent. There is a beginning and end and between the episodes the individual is normal. Some individuals, however, may experience only gradual muscle weakness. Others experience periods of paralysis and gradual, progressive, chronic weakness. For some individuals the weakness can linger or become permanent. Some people will become disabled and require the use of a power wheelchair. Some individuals may become terminal if the disease weakens the breathing muscles.

The Periodic Paralysis Network:

My husband and I have created and now manage the Periodic Paralysis Network. We are an organization with an online community of people who are affected by Periodic Paralysis. The Periodic Paralysis Network provides a hands-on approach to understanding the disease, getting a proper diagnosis, managing the symptoms, and assisting caregivers and family members. Our focus is on educational resources and self-reliance. Our approach to treatment focuses on the self-monitoring of vitals and the management of symptoms through natural methods. We continue to do research and provide the latest information to our members. Everyone is welcome. Members will receive encouragement, support, sympathy, empathy and validation. Members will also gain information and knowledge about all aspects of Periodic Paralysis. Members   ask questions and share ideas. We are usually on in real time, answering questions and providing support as needed for our members.

We have also written and published the following 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."  If you wish to know more about Periodic Paralysis, you may visit the  
Periodic ParalysisNetwork.





Until later...

A Message To Our Non-English Speaking Members

Hello All,

My blog today is for my friends and members from countries outside of the United States. Over the past year or two, I have been in contact with people who have various forms of Periodic Paralysis from many different countries including Mexico, Argentina, Ukraine, Turkey, Finland, Iraq, Pakistan, and many more. I have spent many hours translating back and forth but I have never been sure that my translations were correct. I have never been able to say what needed to be said in the ways it needed to be said. Much was lost in the translation.

I have felt helpless when I received messages and emails asking me for help or advice and I have not been able to provide the information or help that was needed. In some cases, I have been able to arrange for others who speak the language to provide some help, but not the type of help I would like to provide.

I have had two small strokes in the past few months and I have become more ill, much weaker and have little energy to do everything I want and need to do. So, I have had to eliminate some of the things I was doing. Translating is something I have had to stop for now. Please do not think I am ignoring you or have forgotten you. I created this blog partly to help with this problem. The blog translates easily into any language. My hope is that all of you in the other countries can now read the important information I needed to translate and pass on to you in order to help you.

Most of you ask for doctors who can diagnose and treat you. This is impossible for me because, even here in the United States, there are very few doctors who can or will help or understand Periodic Paralysis. Not even the specialist will help or understand it on a level that can help us. They are mostly working on research.

I have had one non-English speaking member tell me that until he read my blogs, he had no idea that others had the same symptoms he has and that those symptoms were a part of Periodic Paralysis. I hope that now, our non-English speaking members can know the important things they need to know, in order to understand more about Periodic Paralysis and treat their symptoms.

 
Avoiding our triggers and changing our diet are two of the most important we things we can do to stop our paralysis. Medications can help some people but not everyone can take the medications. Most doctors cannot help us, unless we educate them.

 
Please ask me questions or ask me to explain things you still do not understand. I am here to help.

 
Thank you for your support.

Until later…

Dysautonomia and Periodic Paralysis

Hello All,

I posted on our PPN Support Group a few days ago about experiencing symptoms of severe weakness with pressure on my head, slight headache, dizziness, brain fog, unable to speak at times and heart arrhythmia. This was accompanied by an odd blood pressure reading of both high and low blood pressure at the same time. I usually have very low blood pressure when sitting and then very high when I am standing, moving around or exerting myself in any way.

One of our members suggested that I might have some kind of dysautonomia or orthostatic intolerance. This is seen when blood pressure drops or heart rate spikes from being up right. It is a dysfunction of the autonomic nervous system.

Though not diagnosed with dysautonomia, I assume I have some form. For several years I have been unable to sit up straight for any length of time. I must be in a recliner and my power wheelchair is designed with a reclining seat for that very reason. I must have it partially reclining when in it, otherwise my blood pressure goes up and oxygen goes down and arrhythmia begins. I can only be on my feet or do anything to exert myself for a short while because of this. I am on oxygen 24/7.

Due to the form of PP I have, I can take no medications because most cause my blood pressure to drop. With fluctuating blood pressure and heart rate and long QT heart arrhythmia, medication is not an option. Getting a definite diagnosis would not help me in any way, just confirm what I already suspect.

I am in this condition because I did not know what I had for 62 years and was misdiagnosed and mistreated and given medications I did not need which obviously caused great damage, unfortunately.

It is our HOPE at The Periodic Paralysis Network, that much of this can be avoided for most of you!

Calvin and I created our website, this Support and Educational Group and wrote our book to share with everyone how to avoid becoming as bad as me.

Our focus is on educational resources and self-reliance. Our approach to treatment focuses on the self-monitoring of vitals and the management of symptoms through natural methods such as the elimination of triggers and awareness of proper nutrition and supplementation. This approach evolved from the inability of the medical community to provide appropriate medical diagnosis and treatment. We continue to do research and provide the latest information to our members.

Due to our work, we have been recognized as a patient advocate organization. We are here to advocate for your rights and medical safety as well as providing the information needed to treat your symptoms.

We are now a member of AdvocateDirectory.org

"This list of links and websites is presented solely as a resource for patient advocates and other individuals who seek information on patient safety and quality."

https://www.facebook.com/groups/periodicparalysisnetworksupportgroup/

http://www.advocatedirectory.org/advocate-directory

We are here to help you be the best you can be!!!!!!!

More information about Dysautonomia:

http://www.dysautonomiainternational.org/

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


Until later...

Isolated Diastolic Hypotension and High Pulse Pressure

  

Hello All,

For the past few days, I have been quite ill again with very odd blood pressure readings and in between exceptionally low readings especially the diastolic (lower number). I have had symptoms of severe weakness with pressure on my head, slight headache, dizziness, brain fog, unable to speak at times and arrhythmia. I am much worse standing or moving around.

My blood pressure is normally 90/60 at rest but does crazy things when I stand up or exert myself. The other evening I suddenly felt really weird; nauseous and dizzy just sitting in my recliner. I took my B/P and it was 143/32 with arrhythmia. My research into blood pressure readings with "above" normal systolic (top...above 120...normal=120-90) and a "normal or below" normal diastolic (bottom...less than 60...normal=80-60), led me to the discovery that it is called "isolated diastolic hypotension," which is a form of low blood pressure. So 143/32 is a classic reading for “isolated diastolic hypotension”.

It can be caused by:

"Usually it is due to dehydration and electrolyte deficiencies in young adults. Rarely, it is due to leaky aortic valves. Sometimes it is due to certain hormone deficiencies or anemia. You should see your doctor for lab tests and to see if you have leaky valve (murmur on exam) and if further evaluation with echocardiogram is needed."

https://www.healthtap.com/topics/isolated-diastolic-hypotension

So once again I find another oddity not in the literature that can happen to those of us with Periodic Paralysis..."isolated diastolic hypotension". One cause of this condition is “pulse pressure” (low or high potassium which can affect the other electrolytes) and another cause is dehydration. It would seem that we have found another way to recognize shifting potassium.

Besides "isolated diastolic hypotension," I had a large spread between the systolic and diastolic number. Subtracting one from the other, the spread is 111 points. That spread is called the “pulse pressure.” Normal pulse pressure is 40 to 75. At 111, my pulse pressure was high, an indication of high blood pressure.

http://articles.sun-sentinel.com/2010-06-22/health/fl-jjps-pressure-0623-20100622_1_blood-pressure-blood-loss-diastolic

Research indicates:

”Thus, many older patients can have both a high systolic and a low diastolic blood pressure. This condition is known as high pulse pressure. The heightened difference between diastolic and systolic blood pressure is associated with a higher risk of stroke than an elevation of either systolic or diastolic blood pressure alone. Unfortunately, nothing can be done to raise diastolic pressure that does not also raise systolic blood pressure.”

So I can conclude that my blood pressure readings indicated I probably had "isolated diastolic hypotension" with a high "pulse pressure." Because I had low and high blood pressure, I was not sure if I had high or low potassium levels. Using my potassium reader I discovered I had high potassium. Because low blood pressure usually indicates high potassium levels and high blood pressure usually indicates low potassium levels, it would be difficult to know without using a potassium reader.

Knowing I had high potassium helped me to know what to do to treat it. I ate something with some sugar and since I also may have been dehydrated, as suggested in the information I gathered, I increased my liquid intake. I felt better for a little while, but am still struggling. I may add some magnesium supplement back into my diet.

This is the trial and error that I walk, as do others with Periodic Paralysis, that fine line I call "the tightrope."

 
I also must realize that at my age and with these symptoms, this oddity in my blood pressure may be the cause of the two TIAs (small strokes) I have had in recent months and it may be there is nothing I can do about it since I cannot take any medications and even if I could, any that I would take to lower or higher it would be harmful by making my blood pressure even higher or lower.

I have included some good links with information about the above issues:

http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/Understanding-Blood-Pressure-Readings_UCM_301764_Article.jsp

http://www.ncbi.nlm.nih.gov/pubmed/21947466?holding=palvhlib_fft

http://www.uab.edu/news/latest/item/1681-low-diastolic-blood-pressure-linked-to-higher-risk-of-heart-failure?tmpl=component&print=1

http://www.healthcentral.com/high-blood-pressure/c/58370/110775/pulse/

Until later…

What is an Ion Channelopathy?

Hello All,

Periodic Paralysis is a channelopathy and channelopathies are considered a class four metabolic disorder. Periodic Paralysis is a fourth class mineral metabolic disorder. Today's blog is about ion channelopathies.


What is an Ion Channelopathy?

Periodic Paralysis is a rare condition like no other. It is called an ion channelopathy, which is a dysfunction of an ion channel. Ion channelopathies were first recognized in 1971 and Periodic Paralysis was one of the first to be discovered. ^{1, 2}

 Ion channels are like a microscopic tunnel in the cells of muscles. The tunnels are called muscle fibers. Ions, which are molecules or atoms, flow in and out of the muscle cells through membranes or gates. Each of the gates is shaped exactly for the correct ion or molecule to enter. The ions are made up of what we call minerals, electrolytes or proteins. Some of the common ions are potassium, sodium, magnesium, chloride and calcium. They are electrically charged and each has its own size or shape, so to speak. If the gates or membranes are faulty in size or shape, an inefficient or improper flow through the membranes can and does cause muscle weakness and paralysis because they regulate contraction and relaxation of the muscle.

Through research, I have discovered that channelopathies are classified as a class four metabolic disorder. Disorders of metabolism are usually inherited and are involved in  chemical and physical processing, which use and make energy in the body. These processes include: breathing, circulation of blood, food and nutrient digestion, elimination of waste through bowel and bladder and temperature regulation.

Unfortunately, ion channelopathies are not usually categorized nor listed in medical writing or studies as metabolic disorders. This poses a problem for recognition, diagnosis and treatment by physicians and other medical professionals. I have found and heard Periodic Paralysis, which is a channelopathy, referred to as, a neuromuscular disease (affecting muscles and/or nerves also known as myoneural), a muscular dystrophy (wasting of muscle and eventual early death), and a disease of the nervous system (nothing to do with the nervous system), therefore, none of these is correct. Periodic Paralysis is a metabolic disorder, a condition which is based in the faulty cellular level of how energy is produced in our bodies. ³

To clarify, even further, Periodic Paralysis is a channelopathy which is a mineral metabolic disorder. Metabolism disorders involving minerals are conditions in which there is either not enough or an overabundance of minerals in an individual’s blood. Minerals have many functions in metabolism and the functions of the human body. They are important in bone and muscle building and growth. Organs, cells and tissues need minerals in order to function properly. So, a dysfunction of minerals in the body affects many processes and functions. Potassium, the main mineral involved in Periodic Paralysis, is involved in making proteins from the amino acids and plays a role in carbohydrate metabolism, so a dysfunction involving it, can affect more than just muscles. ^{1, 2}

 

1. Wikipedia. (February 2013) Ion Channel. Retrieved from:  

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

2. Wikipedia. (December 2012). Channelopathy. Retrieved from: 

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

3. WikiDoc. (August 2012). Metabolic disorder. Retrieved from: 

http://www.wikidoc.org/index.php/Metabolic_disorder

More information:Oxford Journals. (2002). Ion Channel Diseases. Retrieved from:

 http://hmg.oxfordjournals.org/content/11/20/2435.full

Added 1/25/2017

***An Introduction to Metabolic Disorder (Mineral metabolic disorder= Channelopathy)

http://www.encognitive.com/node/1181

A fourth class, the channelopathies (some of which cause periodic paralysis and/or malignant hyperthermia) could be considered to be metabolic disorders as well, though they are not always classified as such. These disorders affect the ion channels in the cell and organelle membranes, resulting in improper or inefficient transfer of ions through the membranes.


***Mineral Metabolism Disorders
http://careers.maimonidesmed.org/Main/AdamMultimediaEncyclopedia/Mineral-metabolism-disorders-1007271.aspx

Definition

Mineral metabolism disorders are abnormal levels of minerals -- either too much or too little -- in the blood.
Minerals are very important for the human body. They have various roles in metabolism and body functions. They are essential for the proper function of cells, tissues, and organs.
Some minerals, such as iron, make up part of many proteins and enzymes in the body. Others, such as potassium, help to produce proteins from amino acids and are involved in carbohydrate metabolism. Minerals also play a role in the building of muscle and bone and are important for normal body growth.
Metabolism refers to all the physical and chemical processes in the body that create and use energy, such as:

• Breathing
• Circulating blood
• Digesting food and nutrients
• Eliminating waste through urine and feces
• Regulating temperature

Causes

Disorders of mineral metabolism are sometimes passed from parents to their children through genes. Other medical conditions, such as starvation, diarrhea, or alcoholism, can cause mineral metabolism problems.

Disorders

Minerals that play a large role in the body include:

• Calcium
• Magnesium
• Phosphorus
• Potassium
• Selenium
• Sodium

Disorders in which mineral metabolism problems often occur include:

• Disorders of phosphorus metabolism:
• Hypophosphatemia
• Osteomalacia
• Rickets
• Rhabdomyolysis
• Hyperparathyroidism
• Hypoparathyroidism
• Disorders of potassium metabolism:
• Bartter syndrome
• Periodic paralysis with hypokalemia***
• Hypokalemic periodic paralysis***
• Hyperaldosteronism - primary and secondary
• Cushing’s disease
• Proximal renal tubular acidosis
• Distal renal tubular acidosis
• Fanconi’s syndrome
• Addison’s disease
• Kidney disease
• Disorders of iron metabolism:
• Hemochromatosis
• Cirrhosis
• Disorders of copper metabolism:
• Wilson’s disease
• Menkes syndrome
• Disorders of calcium metabolism:
• Hyperparathyroidism
• Nephrocalcinosis
• Pseudohypoparathyroidism
• Hypercalcemia
• Osteoporosis
• Movement - unpredictable or jerky
• Kidney stones
• Milk-alkali syndrome
• Paget’s disease
• Multiple endocrine neoplasia (MEN 1)
• Osteomalacia
• Rickets
• Disorders of sodium metabolism:
• Dilutional hyponatremia (SIADH)
• Hypernatremia
• Disorders of magnesium metabolism:
• Hypomagnesemia
• Hypermagnesemia
• Disorders of selenium metabolism
• Selenium deficiency
• Selenium excess
  
  >>>>>>>>>>>>>>>>>>>>>>

Other Disorders Involving Metabolism Of Minerals











Public Health Service Publication No. 1693
https://books.google.com/books?id=icIaAAAAMAAJ&pg=PA153&lpg=PA153&dq=mineral+metabolism++disorders+periodic+paralysis&source=bl&ots=b-rjeyEFyj&sig=Dy_C8ZLRQGtzoqLoBRaxW1p9qQI&hl=en&sa=X&ved=0ahUKEwiY0Zy7j97RAhUQzWMKHbxFBDEQ6AEIUTAJ#v=onepage&q=mineral%20metabolism%20%20disorders%20periodic%20paralysis&f=false

Metabolic Disorder
http://www.wikidoc.org/index.php/Metabolic_disorder

Ion Channels list
http://www.nature.com/ncb/journal/v6/n11/fig_tab/ncb1104-1039_T1.html

PP IS A MINERAL METABOLIC DISORDER http://healthmedicinet.com/ency/article/007271.htm
Mineral metabolism disorders - Health Medicine Network healthmedicinet.com/ency/article/007271.htm

Until later...

Permanent Muscle Weakness in Periodic Paralysis...Continued

Hello All,

Yesterday, it came to my attention that some people are having trouble trying to share my blogs on Facebook. For some reason, these posts have been disappearing from their walls. I am sorry and disturbed that this is happening. I have no idea why or by whom they have been removed.

Please let me know if you have any more problems. I am writing these articles to get the word out about PP for all of us, for your families and friends to understand and maybe some medical professionals and doctors to read them and learn also.


Yesterday’s blog brought a few questions:

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

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

 
I wrote another post:

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

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

The first one is written by a physical therapist:

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

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


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

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

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

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

The last article deals with potassium and exertion/exercise:

"Increased Need For Potassium”

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

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

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

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

Clinical Diagnosis

“The two distinct forms of muscle involvement observed in hypokalemic periodic paralysis (HOKPP), paralytic episodes and fixed myopathy, may occur separately or together. The pure paralytic episodic form occurs most commonly; the combination of paralytic episodes and a slowly progressive myopathy is less common; the pure myopathic form without paralytic episodes is rare:

• Paralytic episodes. The primary symptom consists of attacks of reversible flaccid paralysis with a concomitant hypokalemia that usually leads to paraparesis or tetraparesis but spares the respiratory muscles.
• Myopathic form. The myopathic form results in slowly progressive, fixed muscle weakness that begins as exercise intolerance predominantly of the lower limbs; it usually does not lead to severe disability. This fixed weakness must be distinguished from the reversible weakness that exists between attacks in some affected individuals.”

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

http://www.ncbi.nlm.nih.gov/books/NBK1338/

The above article does not mention the third type which is combination of the two; episodes of intermittent muscle weakness or partial or full body paralysis and gradual permanent muscle weakness.

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

Until later…