The Toaster, Routines and Inspra

Our Guest Author today, is "L", a member of our Listserv. Thanks "L" for letting us share your insights!

As I was sitting on the floor with my toaster I realized where
I had gone wrong, and that I had some things that might be
helpful to share with you all if I could ever get up off the
floor and get my head together again.

Well here I am a couple of days later.

I'm one of us folks who has episodes on a daily basis. Years
ago they were so severe and so uncontrolled that as I've said
before I was wheel-chair bound, or using a walker. Gradually
even before diagnosis I/we found things that helped, both with
meds but also thru diet and routines.

By the time I was diagnosed by Dr. Stephen Lewis, my routines
had helped a lot. The diagnosis and meds helped a lot more and
altho I continue to have daily episodes they are
well-controlled and short-lived. My routines keep my life
running smoothly. I have them set up so that I can do them
at home, during our frequent travels, when brain fogged
(or "Blonde"as we say) or not. I even coach students (for free)
who have always had trouble with school in the Art of the Routine,
and their grades and lifestyle improve dramatically.

Then came the latest change in my meds to Inspra. Inspra for me
has been a beautiful, wonderful thing. I feel ten years old again.
I have SO much energy. Yes, sorry, that was a shout. I am so excited.

And in my excitement I gradually lost track of my routines.
(I was cured!!!) For a day or two I didn't need this one or that
one, so it slipped. I got a little sloppy with eating times. I got
a little lazy with timing my meds, with having foods pre-made
and ready in the fridge, with going to sleep and getting up times,
with pre-made decisions on routine matters.

So when things got a little crazy here, and the weather hit, and
I had no early breakfast pre-made and was dragging the toaster
out of the cupboard to make ... what ... an english muffin for
a hypo??? ... and was too weak to walk to the counter with it .....
... well ... that's when I realized that ...

... Meds are really only a part of our solution.
Our routines and attitude play a really big role too.

So there's my thoughts while I'm getting back on track.
With humor and humility. And toast crumbs.

A Call to Action!

HR 801, the Fair Copyright in Research Works Act

Sharon Terry of the Genetic Alliance talking about why open access to medical information is vitally important to patients.



We must oppose Bill HR 801, the Fair Copyright in Research Works Act. Rep. Conyers wants to restrict the distribution of articles to the publications and their websites. His argument is that the information is still freely available through the library, and that the publishers are suffering because of the free distribution of the material.

I'm sure that I don't have to tell you that the average public library doesn't have a subscription to Nucleic Acids Research, nor would it be appropriate: it's expensive, and few patrons would benefit from it. Even the average college library is hard-pressed to maintain a good collection of science journals. Most people don't live near a medical research library, so it's difficult for them to do research about their condition if they want to.

The argument that the publishers are suffering is a poor one. Scientific journals should not be profit-driven: they aren't "People" or "Time". Most of them don't have advertising. All of them recoup their publishing costs with page charges which are paid by the authors. When those page charges are paid from a grant from a publicly funded agency such as the NIH or NIA, the taxpayer has a right to be able to have easy access to the results and conclusions of the research.

Briefly, we can summarize by saying, "If the Internet was invented for something, surely this free exchange of information is it." Let's at least keep the level of transparency the NIH has put in place, and let's look for more ways to make government and the research it funds even more transparent.

So if you live in the US, take a moment to email or call your Representative. Go to
www.house.gov/writerep/ to find how to contact your Representative.

When you make contact, it's especially helpful to mention your situation: you don't have a lot of medical knowledge, you have an obscure disease, and it's difficult to get good information on it. The representatives will be hearing from a lot of scientist types about this, so it will be really good to have them hear from "Joe and Jane Public".

Please take a moment to write. Thank you.

Time Marches Forward

It's been a very long time, a year in fact, between blog posts! I will not belabor reasons, but it's been a busy year involving broken bones, surgery, and a move. All in all, to quote Lady Bracknell who said in The Importance of Being Earnest, "A life full of incident!" Despite it all we prosper and enjoy ourselves.

However, we continue to have mail from patient after patient who suspects that they might have periodic paralysis, but can find no diagnosis. It's a rare group of disorders. It's only common sense that medical schools spend time training students in diseases they will encounter frequently, like high blood pressure and cancer, not disorders they will probably never encounter in their entire practice.

I can say from many years of experience the most common response from any physician when you tell them that you have periodic paralysis is, "That's a new one on me. I've never heard of it before." Little surprise then that it is difficult for a patient to obtain a diagnosis.

There are numerous conditions (some of them very common) which cause serum potassium levels to fluctuate to a point which weakens or paralyzes a patient. So how do you tell the difference between paralysis or weakness caused by an ion channelopathy and any of the other numerous disorders, conditions and reactions which might produce the same symptoms?

There are ways to sort out the problem but they involve needles, blood, urine, some exceedingly unpleasant tests and a certain amount of knowledge and determination on the part of both patient and doctor. In other words the answers do not generally come easily.

For this reason we've put together a manual of sorts, a how-to-book which leads the reader through the process of diagnosis. It's a complex read for anyone unfamiliar with the concepts, but understanding the complexity of the problem is a good beginning. It encourages some compassion for the physician who is out of his or her depth. You can download the booklet here. It's in pdf format, so you will need an Adobe reader. If you don't have one there's a link on the page.

We continue to advise people who contact us that we cannot and do not diagnose patients. But what we can do is share knowledge, so you know what you need to do to begin the process with your physician.

Take a Step Up!

I get them in my e-mail all the time. "Inspirational" pieces, jokes, goofy stuff, forwarded through an endless string of victims. The boys and I refer to them as the "Halo Polishing and Dead Puppy" mail. Most of the time I hit delete as soon as I see what it is. But this one said something to me, mostly because what I see from day to day while dealing with patients.

The chronically ill are no different than anyone. Everyone has some kind of stressor to deal with. In this world it's not what you have to deal with, but how you deal with it. You see smiles and dancing in refugee camps, and you see the worst kind of misery among people who the world sees as blessed.

One day a farmer's donkey fell down into a well. The animal cried piteously for hours as the farmer tried to figure out what to do. Finally, he decided the animal was old, and he'd been planning to fill in the well anyway; it just wasn't worth the time and effort to retrieve the donkey.

He invited all his neighbors to come over and help. They each grabbed a shovel and began to shovel dirt into the well. At first, as the dirt rained down on him, the donkey cried and screamed and begged for mercy. Then, to everyone's amazement he quieted down.

A few shovel loads later, the farmer looked down the well. He was astonished at what he saw. With each shovel of dirt that hit his back, the donkey was doing something amazing. He would shake it off and take a step up.

As the farmer's neighbors continued to shovel dirt on top of the animal, he would shake it off and take a step up. Pretty soon, everyone was amazed as the donkey stepped up over the edge of the well and trotted away!

Life is going to shovel dirt on you, all kinds of dirt. The trick to getting out of the well is to shake it off and take a step up. Each of our troubles is a stepping stone. We can get out of the deepest wells by shaking off the self-pity, the defeatist attitude, the self-righteous anger and take a step up.

There are five simple rules:

Give more!
Free your heart from hatred - Forgive!
Free your mind from worries - Most never happen!
Live simply and appreciate what you have!
Expect less!

NOW ............

Enough of that . . . The donkey later came back, and bit the farmer who had tried to bury him. The gash from the bite got infected and the farmer eventually died in agony from septic shock.

MORAL FROM TODAY'S LESSON:

When you do something wrong, and try to cover your ass, it always comes back to bite you. That's Karma for you. This applies to the strong as well as the weak, the healthy and the ill, the rich and powerful as well as the poor and defenceless.

The only safe course is to exhibit compassion for everyone, including yourself. When you fail you use the experience to understand how others can still be good human beings and yet fail. When you react angrily, you can use the experience to understand how others, still good human beings, can be pushed to react with anger. Each mistake you make can teach you compassion because you can see that you and all of humanity are very much alike. If we want to see more compassion exhibited by those in our lives, we must begin by showing them what compassion looks like.

Naming the Zebra

There was once a man who had never seen a zebra. Perhaps he'd heard mention of zebras, but he'd certainly never encountered one. He was familiar only with horses. He didn't know horses well, but he had a checklist he could refer to, and that gave him confidence that he was able to identify a horse, should one come into view.

One day a zebra came along. The poor man had no idea what this striped creature was, but it had some of the characteristics on the horse list, so he wondered if the creature was a horse wearing pajamas?

He pulled out his checklist and saw that, like the horse, the puzzling beast had four legs, hooves, a long head and large teeth, ears that stand, a swishy tail, etc. The only difference, as far as he can tell without close examination, was that it had stripes. His checklist said nothing about stripes. However, if you ignored the stripes his checklist identified the animal as a horse.

So he skinned the zebra.

In 1998 we did a survey of 64 patients who were diagnosed with periodic paralysis. This survey revealed that 92% of female participants and 72% of male participants had been initially misdiagnosed. Of these 41.5% had their symptoms attributed to erroneous physical causes and 40% had their symptoms attributed to psychological origin, usually somatization or conversion disorder.

Women definitely find it more difficult to get a diagnosis. Even females with brothers and fathers who had been diagnosed and were being treated for periodic paralysis had their symptoms attributed to psychological factors or described as 'emotional in origin'.

In the last couple of weeks we've had several patients seek treatment for a paralytic episode, either in hospital or in a hospital emergency department. In each case the patient (even though previously diagnosed with periodic paralysis) was told that they had conversion or somatization disorder, without reference to the diagnosis, the information the patients carried/Medic Alert bracelet, without lab tests, examination, and certainly without a psychiatric consult.

It's impressive to find so many interns, residents and hospitalists with the depth of knowledge in psychiatry which allows them to make a diagnosis on the spot which requires an in-depth investigation by a full-time psychiatrist.

But what is this somatization or conversion disorder, and why are so many of our patients told they have it time after time after time? According to psychiatrist Shaun Kerry's article on somatization disorder it is an uncommon disease seen most often in young women. A typical patient will be a female, in her early 20's, complaining of depression, anxiety, gastrointestinal problems, genitourinary problems, menstrual irregularities, joint pains, panic attacks, shortness of breath, numbness, tingling of the fingertips and lightheadedness. She frequently comes to the emergency room because of a panic attack precipitated by a break up with her boyfriend.

A "primary male" usually plays a large role in the patient's life - much more so than normal.  This might be a husband or boyfriend, but can be a father or son.  There is enormous dependency on this figure.  If the relationship is disrupted, the patient gets much worse and the symptoms flare up.

"...In fact," Dr. Kerry says, "the diagnosis (of somatization) is usually missed because the patients are more concerned with their social problems than with their (physical) symptoms. I have never seen a case of SD in which there was not a significant personality disorder.  A personality disorder is a marked, persistent aberration in thinking and/or feeling that causes the individual to have difficulty in relating to others and adapting to his environment.  Generally, the personality disorder is more disabling than the physical symptoms."

In his article Dr. Kerry remarks that in the course of his career he has seen 1000 patients with somatization disorder. He notes that one of these 1000 patients had hysterical paralysis of the limbs. He says; "The illness has quite narrow criteria.  The problem is that many doctors broaden the definition... To make matters worse, some of the doctors who do this write scholarly books to support their position.  Generally, the books are very intellectual but not very mindful."   

He continues, "The doctor must ask very specific questions in order to make the diagnosis.  In addition to applying the DSM IV criteria, (DSM-IV states that for a symptom to qualify for somatization disorder, it has to be without medical explanation)... 

The most common characteristic of the somatoform disorder is the appearance of physical symptoms or complaints which have no organic basis. Such dysfunctional symptoms tend to range from sensory or motor disability to hypersensitivity to pain. Four major somatoform disorders exist: conversion disorder (also known as hysteria), hypochondriasis, somatization disorder, and somatoform pain disorder. Somatization disorder is also known as Briquet's Syndrome.

Starting before age thirty, the patient has had many physical complaints occurring over several years and has sought treatment for these symptoms, or they have materially impaired social, work or personal functioning. The patient has at some time experienced a total of at least eight symptoms which have no organic medical basis from the following list.


PAIN SYMPTOMS:
(4 or more) related to different sites, such as head, abdomen, back, joints, extremities, chest or rectum, or related to body functions such as menstruation, sexual intercourse or urination.



GASTROINTESTINAL SYMPTOMS:
(2 or more, excluding pain) such as nausea, bloating, vomiting (not during pregnancy), diarrhea, intolerance of several foods.




SEXUAL SYMPTOMS:
(at least 1, excluding pain) including indifference to sex and others...




PSEUDONEUROLOGICAL SYMPTOMS:
(at least 1) including impaired balance or coordination, weak or paralyzed muscles, lump in throat or trouble swallowing, loss of voice, retention of urine, hallucinations, numbness (to touch or pain), double vision, blindness, deafness, seizures, amnesia or other dissociative symptoms, loss of consciousness (other than with fainting). None  of these is limited to pain.

For each of the above symptoms, one of these conditions must be met:


1) Physical or laboratory investigation determines that the symptom cannot be fully explained by a medical condition or substance use, including medications and drugs of abuse, or:



2) If the patient does have a general medical condition, the impairment or complaints exceed what you would expect, based on history, laboratory findings or physical examination.

3) The patient doesn't consciously feign the symptoms for material gain (Malingering) or to occupy the sick role (Factitious Disorder).

Herein lies the rub. If the physician does no tests to determine the cause of a symptom there is NO OBJECTIVE PROOF of a medical disorder. This doesn't mean objective proof cannot be found, just that no one has made any effort to find it.

The problem is that the symptoms on the list which relate to muscle weakness or paralysis are part of the recognized clinical picture of the PPs. As long as no one does an appropriate medical work-up a patient with periodic paralysis could very easily look like a patient with somatization disorder. A zebra does resemble a horse, if you use only the "four legs, hooves, long head, standing ears" criteria and ignore the stripes.

But how does one explain that patients are still labeled with somatization even after diagnosis? But how does our man with the list look at a zebra who walks in with a sign around its neck that says:Equus zebra and think, "horse in pajamas"? It's puzzling.

Is it arrogance, mistrust of another's diagnostic skills, an unwillingness to deal with something unfamiliar? Who knows? Most diagnosed PP patients must deal with a new physician at some point in their lives. It is often during a crisis, an episode of paralysis during which the patient is isolated from family by the hospital staff and left unable to speak for themselves.

A physician looks into a cubicle and sees a paralyzed patient who isn't having a stroke and has no head/spinal trauma. Lacking the ability to tell the difference between the horse and the zebra, the common sense to read the medic alert bracelet and the initiative to do appropriate testing, he immediately concludes, "Somatization Disorder". The zebra is skinned, and called a horse, despite its stripes and the sign around its neck which says, Equus zebra.

Where the Web and the Ion Channel Meet

This is Deborah Cavel-Greant. Welcome to the first podcast from the Periodic Paralysis International Blog, "At the Front Lines with Periodic Paralysis."

In the past 15 years technology has transformed the experience of chronic illness. In the late 1980s, when I first encountered the patient self-advocacy work of the late Dr. Tom Ferguson, the patient was still, for the most part, an uninformed, passive recipient of medical care. Decisions were made at a level somewhere above the patient's head. Patients rarely participated in the process of health care, and most assumed that was the way it should be.

Tom Ferguson, who trained at Yale University Medical School, began writing about patients as medical "consumers" over 25 years ago. He made the then-astonishing argument that informed self-care and a team approach between patient and physician would result in better health care and produce a richer and more satisfying partnership.

In 1995 the internet was a new tool for journalists, such as myself. One of my hobbies is genealogy and on my family website I included the information that I have Hypokalemic Periodic Paralysis, a genetic disorder inherited through my maternal grandmother's family. I included basic information about the disorder, drawn from medical sources and referenced.

A few months later a woman contacted me to say she had too had HypoKPP, and we began to exchange e-mail. A little later a man with HypoKPP sent me mail, and another and another, until in March 1997 a group of 17 patients organized as the HKPP Listserv.

From the beginning our conversations have been life-changing. We learned that few of us had optimum treatment. Many of us approached our physicians with the medical journal articles we were sharing to ask for improved access to therapy. Our communication has the advantage of being open-ended. We have conducted surveys which suggest that periodic paralysis patients have a greatly increased incidence of migraine, malignant hyperthermia, and joint hypermobility. We are able to compare episode triggers, drug reactions and responses to therapy.

We learn from each other, encourage each other, occasionally cry with each other. Technology has enabled us to become a community where formerly most of us were alone, often discouraged and frequently terrified.

Technology has continued to expand our horizons. We have an active and busy website which offers state of the art medical information on each of the forms of periodic paralysis. Recently we added this blog which is a more personal vehicle for patients than the website can be. Another blog catalogues journal abstracts pertaining to the periodic paralyses and makes them easy to scan.

Adding a podcast is yet one more way to make information about the periodic paralyses easy for both physicians and patients to access and understand. Some people are visual learners, others learn best when they can hear the material. The immediacy of the human voice is also important to patients who may feel isolated and alone.

In future podcasts we will present case studies, talk about diagnostic problems, interview patients, discuss the different forms of periodic paralysis, and perhaps even have guest speakers. A whole new door has opened. Come on in and enjoy the adventure and the excitement of sharing our knowledge and human potential. Together our potential is multiplied. Together we can accomplish a human revolution which benefits patients, physicians and families.

Let me close with a couple of verses from World Poet Laureate Daisaku Ikeda's epic poem "Rainbow Over Niagara;"

One person is not simply one person;
One person's power can reach far beyond that single life.
A human being's unrevealed potential
Is boundless and inexhaustible.

Begrudge your life - and you lose it.
Give your life - and you gain its true power.
A passionately determined person can surpass
Ten thousand.
Never forget this profound principle.

What would you attempt...

if you knew you could not fail?

I saw it on a sign yesterday, and it set me to thinking. There are many things I don't even attempt because I envision so little chance of success. But what would I attempt if I knew I could not fail?

Here is number one on my list of things: I would get to work setting up a clinic where patients who are suspected of having periodic paralysis, and those who do have it, could come for care.

I'd need a couple of doctors to staff it; a neurologist and an electrophysiologist. I need a rehab specialist, perhaps a physiatrist, a physician who specializes in movement, the mechanics of muscle and bone. We'd work closely with a geneticist. Students would be welcomed through fellowships and research grants. Because the patient cohort would be large it would be possible to do studies which would add to knowledge of the PPs.

Recognizing that not all patients are strong enough to travel, our clinic would have the latest in video conference technology, so that our experts could guide any physician with a web connection and a web cam through an exam, and advise them face-to-face on testing procedures, diagnostics and therapies. With that same video equipment we could have scheduled video education sessions with physicians, medical students and with patients.

If I knew I could not fail I'd begin today to make it all come true, and no patient with periodic paralysis would ever be subjected to years of misdiagnosis and accusations that they have conversion disorder, or somatization syndrome. Mothers with affected children would not be labeled hysterical because they asked for medical help for a child too weak to crawl out of bed or attend school.

Paralyzed patients would not be placed in Emergency Room supply closets or in a ward with drunks, so that they are subjected to sexual assault while paralyzed and vulnerable. Patients would receive test records in reasonable time and have them explained carefully and with compassion. Patients would not risk losing their diagnosis and treatment every time their physician changes. Patients would be treated with dignity.

If I knew I could not fail I would begin to enlist others to help make this dream come true. I would stop saying, "It can't be done," and start saying, "When our clinic opens...."

Today I have decided that I cannot afford to fail. The cost in human potential is too great. Today I will begin to do something - and I cannot fail.

The first step is to enlist the aid of as many as are willing to help. Together we will not fail. Drop me a note in the comments if you are wiling to help in any way. Code your e-mail address by changing the @ sign to the word AT, and we will embark on the road toward building the clinic we need, if we and our children are to reach our full potential.

Who SHOULD Be Ashamed?

This time our Guest Author is a patient diagnosed with Hypokalemic Periodic Paralysis who describes a recent hospital experience. First a little background;

In periodic paralysis the attacks of paralysis are episodic, occasional, intermittent, sporadic. That is; the patient may have "normal" strength at 3:00 pm and by 4:00 pm be unable to move a muscle, speak, swallow or open the eyes. Paralyzed does not mean unconscious, in fact loss of conscious during an episode of PP (other than from respiratory failure or cardiac arrhythmia) disqualifies the diagnosis.

The periodic paralysis patient who is paralyzed at 4:00 pm may have recovered their strength by 6:00 pm, or by noon the next day. The point is that they do soon recover from their episodic attack of paralysis, and return to their normal state or strength (or weakness) as soon as their serum potassium is stabilized at a normal level. This is a concept many medical staff have a hard time with. They equate paralysis with brain or nerve injury. Paralysis which comes and goes is as alien to them as a sixth dimension.
-----

Our Guest writes:

I really needed to read the blog post on shame. While in the hospital this past week, I had "arrows of shame" shot at me by several nurses and nurse aides.

Upon arriving in the C.C.U. I was too weak to get up or even roll over. Gradually over the next 24 hours I got strong enough to use a walker to get myself to the bathroom and a little ways down the hospital corridor. Of course, many nurses, doctors, nurse aides, etc. saw me do this. I was definitely feeling stronger.

The next evening however, the air conditioning on the whole second floor of the hospital went out. It was very hot in my room. I had not eaten anything for hours as I was too nauseous. I thought maybe the A/C was just out in my room; so I took my walker and went into the hallway but it was even hotter in the hall. By this time, I was really feeling weak and went back into my room to lie down.

Heat and hunger are my two biggest triggers. I lay down on my back and put the nurse call-button under my hand. I quickly went into full paralysis; unable to talk or even open my eyes. My head was pulled to the side and backwards, and I was aspirating (choking) on my own saliva. I was able to flex the palm of my hand enough to push the call-button for the nurse. Of course the nurse kept asking "May I help you?" over and over and over on the intercom.

It seemed that it took forever for someone to come to my room to check on me. Since I was hooked up to a cardiac monitor, they knew I wasn't dead. They kept patting my face and saying things like, "Are you mad because we didn't bring your medicine yet?", "Why are you coughing, do you need some cough syrup?", "Your potassium level is 5 so you're o.k. dear,"(My potassium level had been drawn five hours previously). "We checked your blood pressure and it's o.k., so why aren't you talking to us?. "Your blood sugar is good, so there's no reason for you to act this way" etc. etc.

Eventually my nurse raised the head of my bed and I was able to stop choking and coughing. By this time I was drooling. My nurse eventually called my doctor and was told to give me some oral potassium regardless of my last blood levels.

They wanted me to gulp the potassium down but I was still coughing. It was the end of the shift and the nurse wanted to leave. By this time I could open my eyes and talk a little but not move. The nurse then said, "You need to take your bedtime meds now."

I said, "I don't think I can swallow good enough yet".

She asked if she could just pour them in my mouth all at once. I said no, just one pill at a time. Next time I will refuse to open my mouth and ignore the nurse urging me to take them because she needs to leave and "can't just leave them here or I'll get into trouble, please just swallow them real quick so I can go...."

She poured a few in at a time. Two different pills got stuck. Well, I didn't die, but I would have strangled the nurse if I could have moved.

Once the potassium [they'd given me] started working, I needed to pee. The nurse aide's reply to me asking for a bed pan was, "There's nothing wrong with you; I saw you walking in the hall a few hours ago. Get up and get it yourself".

I even heard someone in the hall comment, "She's just faking it for attention because she's mad. There's not a thing wrong with her. I heard the doctor tell her yesterday that she needs to get up and walk around more. If she needs to pee bad enough she'll get up by herself. She's just being lazy".

Normally I am a mild-mannered patient person but I blew a gasket. My nurse came in when she heard me yelling at the aide. I was given a bed pan and then just started sobbing. It's a helpless feeling when you are paralyzed and no one believes you or will help you.

It's hard to keep from feeling shame when people throw it in your face. This time I refused to feel shame for something completely beyond my control. After I was released from the hospital I called the Nurse Manger for the C.C.U.

All right now, all of you patients, stand up for yourselves. Don't take this kind of abuse "laying down". A periodic paralysis episode is nothing to be ashamed of. Don't let anyone tell you different!
---

Do You Feel Ashamed to be Sick?

Today we have a guest columnist, writer Rodney Robbins. To say that I am impressed by this young man's philosophy of life and courage in the face of adversity is an understatement. He is an excellent representative of our Listserv members, intelligent, well-read, thoughtful and compassionate. I am always moved by his thoughtful and insightful blog posts and wanted to share them with our audience. Rodney is an author and cartoonist who lives with three chronic illnesses: Periodic Paralysis, Migraines with aura and Celiac Disease.

"It gets a little complicated," says Rodney.

You can read more at Rodney's chronic illness blog, On Life and Living or go to his Rodney's 52 Ways site to check out his career and work-related tips and his latest cartoon tips booklet.

Shame. Do You Feel Ashamed to be Sick?
by Rodney Robbins

I felt a lot of shame when I was too sick to work. I felt a lot of shame when I had to move my wife out of our little starter apartment back into her parent's house. I felt shame to need to see so many doctors and to need so many pills. Having been through all that, I can tell you now, in terms of dealing with chronic illness, shame is a waste.

If I had done something wrong and hurt someone else, maybe shame would be a good way to prod myself into making amends, but I didn't do anything wrong--I just got sick. Maybe shame would be useful if I was continuing to do something to make myself sicker, but I eat pretty well, take my medicine and try to stay active. Honestly, that's about all I can do.

Feeling shame for no reason is a waste. Feeling shame takes time and energy away from improving my health, from spending time with my family, from being a contributing member of society. I've done my best to notice the shame when I feel it, then let it go. If all you've done to get sick is be alive, you are automatically worthy of love and compassion. Like me, you too can learn to let go of the shame.

We don't kill people just because they grow old and sick. Do we? Why should you or I feel shame because we are young and sick? To feel shame is to say, "I believe society should punish people like me, or at least let us rot." Why was I so hard on myself when, at the same time, I felt that seniors, the mentally ill, people who had been injured in an accident or anyone else with a chronic illness deserved quality care? For me, part of it was not having a diagnosis, but a big part of it was not trusting myself enough to accept the obvious fact that I was sick. Part of me must have felt I might really be a faker (after all, that's what so many experts had told me). My thinking was muddled. Maybe yours is too. After all, if everyone else with chronic illness is worthy of compassionate care, so are you!

Shame is an emotion that must be constantly tended. To feel shame, you must hold your feet to the fire, so to speak. As soon as you take your feet off the coals, your injuries start to cool. The scars of shame can last a lifetime, but the damage stops as soon as you stop inflicting it. Sure it would be nice if you and I had all the energy and health in the world. It would be nice if we were immensely rich and lived at the beach too. Why not? If we're dreaming, let's dream big. But we can cool the heat of shame with the cool waters of compassion and forgiveness.

I started by accepting that I had made the best choices I could have based on the information I had at the time. We can forgive ourselves for being human, for making mistakes, for getting sick. We wouldn't choose this life (probably), but we can embrace it and make it our own. When you are able to see your own live in a broader context of all humanity, you will able to let go of your shame. Then you can start to extend a feeling of compassion and forgiveness first to those you care about, then slowly to those who may have hurt you. Maybe the people who hurt you feel shame too. Maybe they should feel some shame. Maybe they just haven't lived through all that you and I have. Before we can forgive them, we need to forgive ourselves.

Cry if you have to, but you can at least start walking out of the center of the coals and toward the edge of the fire pit. When you get to the edge, step out of the flames-of-shame and onto the cool grass, wet with dew. Cross the grass and put your feet in the cool waters-of-forgiveness. In other words, when you notice that you are feeling shame, go ahead and really feel it, say hello to the shame, embrace it with love, then let it go.

Paralysis and the Ion Channel

It's hard for patients and some doctors to understand that paralysis doesn't always include brain or nerve injury. A diagnosed periodic paralysis patient reported recently that an EMG showed that her motor nerves are in perfect working order, and her medical consultant was at a loss to explain the mechanism of her paralysis. She came back to the Listserv with the question.

Since it's a question we get asked again and again we thought it might be a good subject for a blog post.

There are several different forms of periodic paralysis. Each form is caused by a distinct genetic mutation which affects a voltage gated ion channel. The mutation causes the ion channel to malfunction.

The mutated ion channels which cause weakness in periodic paralysis are located in the skeletal muscle membrane. The muscle membrane is like an envelope, it covers the outside surface of the muscle cell. Skeletal muscles are ones which are attached to bones and are used to move us from place to place, maintain posture, etc.

The ion channels are formed of groups of flexible coiled tubes. These create and surround an opening (or pore) and function by changing the shape of the pore, opening and closing the opening. The pore extends from the outside surface of the membrane to its inside surface.

When the sensor on the ion channel receives the proper signal the coiled tubes change the shape of the opening, allowing ions of potassium, calcium and sodium to pass through. (Some channels only allow one kind of ion to pass through, others are less selective and allow two or more types of ion through.)

The channel remains open only for a fraction of a second, ions flow through, the tube closes and ion flow stops. The ion channel then goes into a "resting" phase, relaxed but ready to respond to a new signal.

Potassium and sodium ions carry an electrical charge. The ratio of charged ions inside and outside the cell determine the "tension" of the muscle membrane.

Think of a trampoline. If the springs are too loose the trampoline is saggy and you don't get any bounce when you jump. If the springs are too tight there's no give and the tramp bounces like the sidewalk. In either case you have to work really hard, and expend a lot of energy, to get any bounce out of the trampoline. The tension has to be just right for the bounce to work.

The muscle membrane is something like that. It works best at a certain tension. The tension is "set" or maintained by the ratio of charged ions inside and outside the muscle cell, and that ratio is controlled by the ion channels.

When an ion channel is defective in PP the function of the channel is altered. It may not open properly, it may stay open too long, or not close all the way, so that ions leak through like water through a drippy tap.

This is important because it allows the ratio of ions in and outside the muscle cell to become unbalanced and alters the tension of the muscle membrane.

People with Ion channelopathy periodic paralysis have a higher muscle membrane tension pretty much all the time. It takes more energy to overcome the resistance of the membrane. The more resistance the weaker you feel. If that resistance gets too high you can't move at all, which of course is *paralysis*.

In PP it's not the absolute value of potassium in the blood that determines a person's weakness, it's that ratio of potassium inside and outside the muscle cell.

But it hasn't anything to do with nerves or nerve signals. The signals arrive and are received, it's the response (or lack thereof) which causes the weakness.