When to Call For an Ambulance

Hello All,

I continue to do well and have been able to be more active after stopping my supplements. I believe they were making my system more acidic. I have now become more balanced. It is still very clear, however, that no matter how much better I feel; I still have exercise intolerance and permanent muscle weakness. I must continue to use oxygen, eat a pH balanced diet, stay hydrated, get enough sleep, avoid my triggers, use my power wheelchair, and rest as much as possible (all day). I must continue to “walk the tightrope”.

This morning I received a link to a group who is writing a book about “harm in the Emergency Room” (ER). They are collecting as many stories as possible of people who have received mistreatment in the Emergency room. They hope to stop it. If anyone would like to share his or her story you may go to:

http://www.stoperabuse.org/

I decided that today should be the day I share my experiences in the ER. I will do this in three parts over the next three days. Today I will discuss when to go to the ER. Tomorrow I will discuss avoiding the pitfalls in the ER and the third day I will tell of my experiences in the ER.

When to call an ambulance or go to the Emergency Room if you have Periodic Paralysis

After studying Periodic Paralysis and Andersen-Tawil Syndrome, I have learned that an ambulance does not need to be called every time I become paralyzed. When I have an episode of paralysis, I will usually be fine in a few hours. However, if I have trouble with my breathing, my heart or with choking or swallowing, then an ambulance may be necessary.

I want to add here, that an 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.

The following is written about Periodic Paralysis in relationship to the need to call for an ambulance or to go to the emergency room:

Go to the emergency room or call the local emergency number (such as 911) if you faint or have difficulty breathing, speaking, or swallowing. These are emergency symptoms.

http://www.nlm.nih.gov/medlineplus/ency/article/000312.htm

In more serious cases, the swallowing or breathing muscles may be involved.

http://www.activeforever.com/a-642-familial-periodic-paralysis.aspx

When dealing with hyperkalemia, high levels of potassium, the following may be helpful. I have been told, if the level is 6.0 or more than emergency treatment may be necessary:

The following is a guideline for hyperkalemia:

Hyperkalemia is a medical condition of elevated bloodstream levels of potassium. While the normal blood potassium level is 3.5-5.0 mEq/L, mild hyperkalemia can be between 5.1-6.0, moderate between 6.1-7.0, and severe above 7.0. In an extreme case, hyperkalemia can be an emergency as the condition may lead to fatal effects.
http://hyperkalemia.net/

Go to the emergency room or call the local emergency number (such as 911) if you have symptoms of hyperkalemia. Emergency symptoms include:

Absent or weak heartbeat

Changes in breathing pattern

Loss of consciousness

Nausea

Weakness

http://www.nlm.nih.gov/medlineplus/ency/article/001179.htm

More serious symptoms of hyperkalemia include slow heartbeat and weak pulse. Severe hyperkalemia can result in fatal cardiac standstill (heart stoppage). http://www.medicinenet.com/hyperkalemia/page2.htm

Mild hyperventilation is a symptom of hyperkalemia due to metabolic acidosis. http://www.ehow.com/facts_5446075_hyperkalemia-symptoms.html

Symptoms of hyperkalemia include abnormalities in the behavior of the heart. Heart abnormalities of mild hyperkalemia (5.0 to 6.5 mM potassium) can be detected by an electrocardiogram (ECG or EKG). With severe hyperkalemia (over 8.0 mM potassium), the heart may beat at a dangerously rapid rate (fibrillation) or stop beating entirely (cardiac arrest). Patients with moderate or severe hyperkalemia may also develop nervous symptoms such as tingling of the skin, numbness of the hands or feet, weakness, or a flaccid paralysis, which is characteristic of both hyperkalemia and hypokalemia (low plasma potassium). http://www.healthline.com/galecontent/hyperkalemia#ixzz1Imk6qHHA
 

When dealing with hypokalemia, low levels of potassium, the following may be helpful.

Mild hypokalemia is often without symptoms, although it may cause a small elevation of blood pressure,^[2] and can occasionally provoke cardiac arrhythmias. Moderate hypokalemia, with serum potassium concentrations of 2.5-3 mEq/L, may cause muscular weakness, myalgia, and muscle cramps (owing to disturbed function of the skeletal muscles), and constipation (from disturbed function of smooth muscles). With more severe hypokalemia, flaccid paralysis and hyporeflexia may result. There are reports of rhabdomyolysis occurring with profound hypokalemia with serum potassium levels less than 2 mEq/L. Respiratory depression from severe impairment of skeletal muscle function is found in many patients.

Some electrocardiographic (ECG) findings associated with hypokalemia include flattened or inverted T waves, a U wave, ST depression and a wide QT interval.

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

Hypokalemia is defined as a potassium level less than 3.5 mEq/L.

Moderate hypokalemia is a serum level of 2.5-3 mEq/L.

Severe hypokalemia is defined as a level less than 2.5 mEq/L.

http://emedicine.medscape.com/article/767448-overview

Cardiac arrhythmias This phrase is used to collect a group of different conditions where the heart has abnormal electrical activity. It does not mean that the heart beat is irregular, although it can be, it can also be regular. The speed of the heart beat may be either fast or slow as compared to normal. In some cases of cardiac arrhythmias, they can be medical emergencies and a life-threatening situation.

http://www.hypokalemia.net/symptoms.php

If an individual has Andersen-Tawil Syndrome (ATS), they experience episodes of paralysis based on low or high potassium levels or there may be episodes in normal ranges. Those with ATS experience serious ventricular arrhythmia with the possibility of life-threatening long QT interval heartbeats. They must be monitored closely. They may experience any of the above symptoms, especially, trouble with breathing, with the heart or with choking or swallowing. If these occur then an ambulance may be necessary.

However, it is best to be prepared with the emergency chart posted in:

Emergency Instruction Chart November 19, 2012

It is imperative for one with Periodic Paralysis to know the above information and to have this important information written and handy in case an ambulance must be called. I keep this information in a plastic folder along with everything I know is important and that the paramedics and EMTs must know when coming to my aid in an emergency and for the doctors when I get to the hospital. 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.

Tomorrow:  Avoiding the pitfalls of the emergency room 


Until later...

Pharmaceuticals Are Not the Answer For Some Rare Diseases

Hello All,

I had another great day!!!! I was able for the first time in three years to have Calvin drop me off somewhere and be able to drive away. He drove me to the cottage on the beach where my daughter and son-in-law were staying, visited for a while and then drove to town to run some errands. I had my portable oxygen, which lasts for about 4 hours. The cottage had a huge bay window looking out into the Straight of Juan de Fuca. We sat in front of a roaring fireplace and visited as we saw Victoria, Canada across the bay and watched the ships as they passed.

I realized, however, that although I did not have that horrible fog I had been experiencing any longer, I still have issues with exercise intolerance. Just talking for a while and then having a snack proved to be too much, I went into arrhythmia and become weak, but I avoided paralysis. I had to be very careful after that, however.

Calvin told me later, that other than leaving me in the hospital a few times, it was the first time in three years he felt he could leave me like that. He said he felt sad driving away, but he knew I was in good hands and it was good for him and him to do that.

My visitors must leave today, I will miss them very much, but I will get back to my "work" of teaching the world about Periodic Paralysis.

 
Today, I want to share an article I wrote a few months ago. 

Pharmaceuticals Are Not the Answer For Some Rare Diseases

Some rare diseases cannot use pharmaceuticals for treatment. There are other options, but there is no help to obtain them.

I have a disease called Periodic Paralysis. It is a very rare, debilitating, hereditary and difficult to diagnose disease. I was 62 when I was finally diagnosed, 6 years ago. It is a mineral metabolic disorder called an ion channelopathy. It is often misdiagnosed and mistreated, thus causing more damage or possible death to the person with it. There are several forms of it and the type I have is the most rare and the most serious type.

 On a cellular level, triggered by things such as sleep, exercise, sugar, salt, most medications, stress, cold, heat, anesthesia, adrenaline, IVs, etc., potassium wrongly enters the muscles either temporarily weakening or paralyzing the individual. Episodes can be full-body lasting hours or days during which severe life-threatening symptoms may occur. Due to these complications, it is extremely important to avoid the episodes. Permanent muscle weakness may occur over time. If it affects the breathing muscles it can become terminal.

There are no known cures, but there are treatments and drugs for some forms, which can be and are successful for some individuals.

My husband and I are the co-creators and managers of an independent organization, the Periodic Paralysis Network (PPN), which is patient-safety-related due to the serious nature and potential life-threatening symptoms and side effects of this condition if it is not treated correctly. We have a website, a discussion board and support group on Facebook to help others with support and the natural methods (pH balanced diet, supplements, oxygen) we have discovered.  We also provide methods and ideas on how to find doctors, get a diagnosis, get the proper help in the ER, how to discover their triggers, and much more.

Many patients have difficulty getting diagnosed and treatment in the US and the same problem exists around the world. We have people contact us nearly every day from all over the world, Iran, Ukraine, Turkey, Denmark, Wales, Netherlands, Canada, Finland, Australia, Mexico, to name a few, who are seeking help for themselves, their children and entire families and are unable to find it anywhere.

For some, medication can help but they cannot get medication. Some need a diagnosis but cannot find doctors who know enough to diagnose it. Many, many people are suffering with this disease and getting no help. The quality of their lives is being destroyed and some are even dying, needlessly.

 With this information in mind, a few months ago, in my position as manager of the PPN, I received an email from an organization, which is described as a driving force behind programs that provide services for patients with rare diseases. The email was seeking input about improving the methods and time frame for processing pharmaceuticals for treating rare diseases.

Though I am not a doctor, researcher or physicist and had some difficulty understanding some of what was written; I understood enough to form some comments and then added a few questions regarding these issues. I quickly wrote a response hoping for some information about the possibility of services for “patients” with Periodic Paralysis, a rare disease. I wrote the following letter.

”I understand that this process has been designed to expedite the discovery, research and use of new medications or pharmaceuticals in rare, very rare and extremely rare diseases. This is definitely something I can agree with. There is a need to expedite the process as many of us are dying. I happen to be one of those people due to a lack of treatment. I agree also with the key-considerations of the benefits and risks of a faster time frame because there must be safeguards in place.

That being said, the condition I have is an, as yet, undiscovered variant of Andersen-Tawil Syndrome (ATS), which is probably unique to my family and me. Our DNA is being studied at Baylor, therefore, it an extremely rare form of Periodic Paralysis, which is an inherited ion channelopathy. Part of the problem with expediting the making of medications to treat a condition, is that it is useless for me and others with ATS because most of the patients with ATS, are not able to take any medications or pharmaceuticals because they are actually triggers for our severe symptoms of paralytic episodes, which include partial to total body paralysis, difficulty breathing, low oxygen levels, heart arrhythmia including dangerous/sometimes fatal long QT interval heart beats and torsades de pointe, fluctuating heart rate, fluctuating blood pressure, cessation of breathing, choking, possible cardiac and/or respiratory arrest.

What we need is a way for timely and better diagnosing so treatment can begin early thus avoiding an outcome like mine; getting a diagnosis at the age of 62 for a disease I had my entire life. I was misdiagnosed over and over and treated with pharmaceuticals, which almost killed me and have left me totally disabled and terminal with no treatment as I progressively decline.

At this time, Periodic Paralysis is diagnosed by a process of elimination, which takes years, in my case a lifetime, and either clinically; based on symptoms and characteristics, which can be subtle or absent or genetically; genetic testing which can take years and is only done in a few places in the world. The problem with these options is, first, most doctors and even the specialists do not understand it well enough to diagnose clinically, and second, the genetic testing will only reveal about 50% to 60 % of the overall types of PP/ATS. The remaining 40% to 50% of forms are, as yet, unknown. An individual with an unknown variant has symptoms indistinguishable from those with the known mutations.

We need something better than this. Many people are dying from the improper medications due to misdiagnosis and by provoking their symptoms in an attempt to get a clinical diagnosis.

Genetic research has been in place for over 25 years. Blood samples from all over the world are studied and then in a year, 2 years, 4 years, or one family has been waiting 9 years, an individual will get his letter stating he has no known form of PP. This wrongly sets up the patients to believe that they do not have PP, though as stated above, 40 to 50 % of them have a variant, which has not yet been discovered. In the meantime there is no help and no treatment until or unless a mutation is discovered. To date I know of no known treatment from these studies and no faster or better method for diagnosis.

This has to stop!!! There must be a better way, a set of better guidelines, better education of medical professionals to be able to diagnose this cruel disease in a more timely manner, and perhaps rather than funding for the discovery of only 50% of genetic codes the money can better be used to research for proper diagnosis, treatment and management.

There are doctors who are associated with an organization specifically for researching and treating diseases affecting muscles. Although Periodic Paralysis is not a muscle disease but rather a 4th class mineral metabolic disorder, it is accepted as one of their diseases. These doctors are supposed to know about and treat PP. However, but most of them, (I have been to 4 of them), did not diagnose it in me and in fact set my diagnosing back years, thus contributing to my present condition, totally disabled and terminal. Again, I suggest if these specialists and this organization are receiving funds for diagnosing, treating and research of PP, then that money should be going elsewhere for those of us with PP or for proper education of those doctors to recognize PP/ATS based on present day criteria.

There are other types of PP in which some drugs are used with good results, for a few, but not without short term and serious long term side effects,  but for the more rare forms, we are out of luck.

My husband and I have written a book about Periodic Paralysis and discuss these issues in it. Living with Periodic Paralysis: The Mystery Unraveled was published last month. This was in part due to a program coordinator for the organization treating muscle diseases telling me that I need to teach their doctors about Periodic Paralysis! We also have a website, the Periodic Paralysis Network and forum for individuals who have PP and most of our members are unable to get a diagnosis, or treatment. They are living in limbo with no help from the medical field. We have created a plan that helps us naturally with diet, avoiding triggers and by monitoring our vitals, especially monitoring our potassium levels.

There is a device, a potassium reader, which we use to help us to know how to treat our symptoms based on the level of potassium in our blood. Unfortunately, this device costs $350.00 and is not a medical device so insurance will not pay for it. Most families do not have that kind of money, thus making monitoring of symptoms very difficult.

Rather than medications, we use those devices to help us relieve our symptoms and stay alive by avoiding paralytic episodes. We could use funding for those devices and working with the FDA and the company that makes the devices can help us to have them deemed as medical devices so insurance will pay for them.  We are not yet incorporated but hope to be soon, with funding from the sale of our book. We hope to be able to raise money to purchase potassium meters for those who need them.

Pharmaceuticals are not the answer for some rare diseases. The above issues are the type of help we need. Do any of our concerns or issues meet your requirements to receive assistance under your “commitment to fulfill unmet need for patients” with rare diseases? I would love to work with you if that is the case.”

I continue to wait for a reply, although today, I received another e-mail from them asking me to write a letter or call my representative in Congress in order to pressure them to pass the now completed Bill.

All the while I am hooked up to an oxygen tank, confined to my bed, recliner or power wheelchair, with my computer in my lap as I continue to work to improve the lives of individuals with all forms of Periodic Paralysis. I am creating a set of better guidelines for diagnosing PP, writing my next book for better education of individuals of with PP and medical professionals, attempting to raise awareness of PP in the world, marketing my book to raise money for potassium readers as I am in and out of paralysis and muscle weakness. You see; pharmaceuticals are not the answer for some rare diseases. Who will help us? Who will take my place when I am gone?

Until later...

Update 12/19/2016:
We now have written and published four books related to Periodic Paralysis.

Episodes of Paralysis

Hello All,

I had another very rough day yesterday and I seem to have settled into an “abortive attack”. So, as I contemplate my choice of topics to write about today, I have decided to write about what we call “episodes” or “attacks” of paralysis

It is a misconception that everyone with any form of Periodic Paralysis becomes totally paralyzed periodically. The truth is, some individuals with Periodic Paralysis have never actually had an episode of paralysis. They may, however, over time, develop permanent muscle weakness and need a wheelchair or scooter.  For most individuals, the episodes or attacks, are periods of time with muscle weakness or partial paralysis, rather than full-body paralysis. Over time, these may progress to full-body paralysis. Some individuals may have full-body episodes from the very beginning.

An individual may be only partially paralyzed. Only his or her legs may be paralyzed or they may only be weak. Episodes may occur as sudden falls or dropping to the floor. Feet or legs or arms may go numb and tingly. Muscles in the calves may become very tight and painful. An episode may include overall weakness with arrhythmia and fluctuating blood pressure. Walking may suddenly become difficult due to a weak foot.

Each of these attacks or episodes usually has a distinct beginning and end. Each episode is intermittent. They come and go. Most people will be normal between them or their muscle strength will improve between them. Many develop permanent muscle weakness over time.

That being said, the causes of these episodes and the reasons to avoid them are important to note here. On a cellular level, episodes are triggered by things such as sleep, exercise, sugar, salt, most medications, stress, cold, heat, anesthesia, adrenaline, IVs, and much more. Potassium wrongly enters the muscles either temporarily weakening or paralyzing the individual. Episodes may last hours or days. For some, dangerous heart arrhythmia, heart rate fluctuation, blood pressure fluctuation, choking, breathing difficulties, cardiac arrest and/or respiratory arrest can also accompany the episodes. Due to these complications, it is extremely important to avoid the episodes.

After years of episodes of intermittent muscle weakness, and the development of permanent muscle weakness including my breathing muscles, I developed frightening full-body episodes of paralysis.

The following is my description of what it I experience in an episode. It was written in 2010 for my doctor to help him understand:

”Usually, I wake up in the morning and I am paralyzed. I find I can’t move. I can’t open my eyes. My mouth is open. I can’t breathe through my nose. I have urges to swallow but can’t so there is a choking sound in my throat every few minutes. Sometimes my heart will race or beat irregularly, though usually, there is no problem with my heart. My mouth is very dry. I cannot speak

As I begin to come out of it, my mouth will start to get saliva, my eyes will open but I can only see what is in front of me, since I can’t move my head. Sometimes my eyes will jerk around when I first open them, usually jerking up. My body will sometimes jerk a little. Sometimes there is a big breath my body will take.

Sometimes, I will go back into it. My eyes close, I feel very hot and all the symptoms return. Sometimes there will be a few jerks as I go back into it.

During all of this I am awake and am aware of everything going on around me. Sometimes I begin to cry, due to the frustration, and fear. I can feel the tears running down my cheeks.

If I have these at times other than upon waking, the symptoms are the same.  I get a strange sensation of heat body wide, usually beginning in my back. My eyes will close and then my body goes limp. I may have a few jerks as I am going limp. My mouth will open and I am in it…unable to move, speak or open my eyes.

Sometimes, I don’t go too deep. It is all the same but I am able to open my eyes and can speak a little with a tight tongue and tight lips. My mouth is still open, however. I can’t move my body.

Once one of these begins, it may last from several hours or can be as short as about 10 minutes, if it is a second or third one in a row.

It takes about 15 to 30 minutes to many hours to come out of it enough to move a little and attempt speech, though usually several days to fully recover. I am always left with lingering weakness for many hours that can linger into days. Speaking is difficult. Walking is difficult. My arms and hands come back sooner than my legs. I begin to get feeling back in my body. I can move my lips. I begin to breath thru my nose again. It is difficult to speak or move but it gradually comes back. Speech is very difficult; my lips don’t want to move. My tongue is difficult to move. I will suddenly have an urgency to urinate. If, at this point, I get help to the bathroom, I am like a rag doll, especially my legs. My arms flail, like a child just learning to stand and walk; balancing herself. 

For many hours, I remain too weak to do much of anything but sit up in bed or sit in a recliner. I must use my walker or a wheelchair.

I do not know what brings on or triggers these attacks/episodes (we know now). I know that sleep has something to do with some of them, but not all of them. I know that sometimes, when I wake up during the night with an urgency to urinate, I am coming out of one, because I have all of the symptoms previously discussed. My arms and hands and legs are numb and feeling is just coming back. My mouth is tight and dry. Walking is difficult.” (May 28, 2010)

After that time, my episodes got increasingly worse and have lasted up to 7 hours. I have severe issues with blood pressure fluctuation, heart rate fluctuation, arrhythmia, including long QT interval heartbeats, choking, and breathing difficulties. I must do everything I can to avoid them and now I do have some relief from these major attacks.

 
We have discovered ways to reduce the number and severity of the episodes. We share this with our members and it is included in our books. The following is a quick overview and will be discussed in depth on another day:

We do this by a recommendation of evaluating our medications (most make it worse and create horrible life-threatening complications and even death), knowing if we have high or low potassium issues, monitoring our vitals, eating a diet based on our type of PP, discovering our triggers, getting enough rest, learning to relax, ensuring proper hydration, maintaining a moderate environment, learning our exercise limitations, using possible supplements, obtaining friend and family support, and seeking our own mental well being based on education and understanding of the condition and how it affects us.

Despite doing all of this, we still find ourselves in abortive attacks for periods of time. Abortive attacks are periods of extended time anywhere from hours, days, weeks or months in which some individuals are totally debilitated by extreme muscle weakness without going into full paralysis. The previously described common symptoms may begin but the full attack or total paralysis may not occur. The person is left with severe weakness and other symptoms such as extreme fatigue.  It is difficult to do anything physically and it also can affect cognition abilities, such as memory, thought processing and speech and some become very fragile emotionally. The individual may want to sleep and may have no appetite.
 
In my own case the abortive attacks are totally debilitating and actually worse than the episodes of paralysis. When I am in them, I wish I could slip into paralysis and get it “over with”. I have experienced no worse feelings of weakness and helplessness in my life other than being in a full body state of paralysis.

I have included a slide show of an episode I experienced a few years ago. I also have videos of a few and will share if someone is interested in seeing a full-body episode of Periodic Paralysis.

Until later…