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Courage in crisis

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A new non-fiction book by Sequim author Susan Knittle-Hunter may not ever make it onto the bestseller list but she believes its contents are worth more than any accolades.

This summer Hunter and her husband, Calvin, self-published “living with Periodic Paralysis: The Mystery Unraveled,” their account of them diagnosing her baffling metabolic disease, walking the tightrope of managing it and guiding physicians in its diagnosis and treatment.

After a “sickly” childhood and decades of being misdiagnosed, mismanaged and in some cases maltreated by the medical profession, Susan finally found out in 2011 she has one of the rarest of rare genetic diseases — Andersen-Tawil syndrome — a subcategory of periodic paralysis. An estimated 100 people in the world are known to have ATS, with just 2,500 suffering from PP worldwide, but the Hunters suspect there are many more that haven’t been diagnosed or correctly diagnosed.

First, do no harm

In 1990 at the age of 52, Susan had her first episode of full-body paralysis after years of progressive muscle weakness, fainting, severe pain, tachycardia and therapies that only made her condition worse. Her doctors initially thought she’d had a stroke. Her increasing incapacitation forced her to retire from being a teacher and case manager for children and adults with disabilities.

“Doctors treat it as a neurological disease but it’s not,” Hunter said from the confines of her recliner, tethered 24/7 to an oxygen nasal cannula (tube). “It’s a disease unlike any other — it’s in a category of its own and needs to be treated in unconventional ways. Doctors should keep an open mind and think out of the box in recognizing and treating periodic paralysis.”

Although a soft-spoken woman who sometimes has to catch her breath to speak, Susan’s voice becomes edged with steel and she doesn’t mince words when she tells about so-called experts who sent her packing from one specialist to another over several decades, all the while piling on more medications that caused new and worse symptoms, Susan said. It was a toss-up whose attitudes were the worst — the ones who said it was “all in her head,” the ones who said she was faking symptoms or the ones who said there was nothing they could do for her.

Even when the Hunters told medical staff firmly that glucose, saline and lidocaine propelled her into paralysis, the couple was ignored or even deceived, they said.

“For a long time, after a really big episode, I was so weak and I just kept feeling like I was going to die,” Susan, now 65, said.

“For a long time I was very depressed but when I found out about periodic paralysis and could put a name to it, I was angry about how I’d been treated by doctors. I wanted a diagnosis so I could get treatment and I had a new reason to live, but then I was diagnosed and five medications almost killed me. Three or four specialists said, ‘There’s nothing I can do for you.’ I have this fight in me and I know something is wrong.”

Defining periodic paralysis

According to the Hunters’ book, periodic paralysis is “a rare, hereditary disease characterized by episodes of muscular weakness or paralysis without the loss of sensation or consciousness. It is a channelopathy, a disease involving dysfunction of an ion channel for potassium, sodium, chloride and calcium.” The genetic disorder affects the contraction and relaxation of skeletal and cardiac muscle fibers at the cellular level, weakening both permanently.

Susan’s symptoms included pins and needles sensations, not being able to move her legs, foot dragging, shortness of breath and a heart rate ranging from sluggish to racing. Over the course of a decade, she was diagnosed with everything from multiple sclerosis to malingering. At least one physician believed she had a conversion disorder, a psychiatric illness.

With each new diagnosis and rounds of tests and therapies, new symptoms arose and caused her condition to look like yet another disorder. This vicious cycle culminated in her being on 15 medications a day.

“It felt like the beginning of the end so I gradually stopped taking the medications one by one and the different symptoms stopped. If you have the type of periodic paralysis I have, medications will cause weird and odd symptoms that doctors don’t recognize or a paradoxical effect that can put me into paralysis or an arrhythmia,” she said.

Episodes of extreme muscle weakness and partial or total paralysis can last minutes or hours — the longest one Susan has endured was seven hours — and leave her physically and emotionally exhausted. An accompanying heart arrhythmia called long QT syndrome may lead to sudden death and Susan and Calvin live with that knowledge every day. An hour of conversation and she begins to pause in her thoughts and grasp for words. A dose of potassium in solution brings her back to clear thinking within 15 minutes.

Walking the tightrope

“It’s a wonder that I’m even alive,” Susan said, “because when I finally got diagnosed, there was nothing that doctors knew to do. Based on my symptoms, Calvin researched it on the Internet and did several brave things to save my life — he put me on a pH-balanced diet with supplements … and focused on what things caused my metabolic acidosis.”

This serious condition occurs when the body produces too much acid or when the kidneys are not removing enough acid from the body and can lead to coma or death.

The Hunters realized triggers include any kind of exertion, diet, cold and heat, saline and glucose IV solutions, anesthesia (both gaseous and injectable) many medications and unfortunately, sleep.

“I usually wake up in paralysis since there’s no way to avoid sleep but I don’t worry about it as much now,” Susan said. “When I’m in total body paralysis the only thing I can do is hear. Before we changed things, I’d have episodes four or five times a day but it happens once or twice a month now.”

Regulating Susan’s potassium levels is a delicate balancing act because her body reacts to hyperkalemia, hypokalemia and normal potassium levels and can swing wildly among the three, sending her into a paralytic state.

“I will look dead or unconscious and Calvin will have to watch my swallowing and keep me elevated because I can choke.”

The couple have learned that “walking the tightrope” means a pH-balanced diet, supplements, avoidance of exertion and stress, continuous oxygen therapy and monitoring her potassium, blood sugar and oxygen levels, body temperature and blood pressure. They’ve assembled a “vitals” kit — and recommend it on their website — consisting of a blood pressure cuff that identifies arrhythmias, a glucometer for blood sugar, a Cardy potassium ion tester which measures potassium levels, an oximeter and a pH meter for acidity/alkalinity, all for about $500.

“We basically had to develop at home these tools and started charting Susan’s patterns,” Cal said. “It takes a lot of uncertainty out of it for people. When you can see actual measurements, you can calm down and not have to call 9-1-1. It’s been for my peace of mind — otherwise I’d be a nervous wreck.”

The human body naturally has a pH balance that’s 70 percent alkaline and 30 percent acid to be in homeostasis and Cal soon realized that his wife’s diet needed to reflect that.

“Cal was frantically trying to find ways to save me and because he knew I had metabolic acidosis, he found an alkaline diet — it was really strict — with fresh vegetables and fruit, no meat and no simple carbs, sugar, salt or caffeine,” Susan said. “He put me on supplemental amino acids and after six months I’d lost 25 pounds, lowered my blood sugar and cholesterol, and went from four or five paralysis episodes daily to one or two a month, except after sleeping.”

Cal added, “Basically we eat raw foods without additives.”

The Hunters moved to rural Sequim last fall for the climate and are pleased to have found a physician willing to listen and learn about periodic paralysis, internist Dr. Samantha Reiter of Sequim Medical Associates.

On their initial visit, Susan said, “She was listening to everything I said and believing me. No specialist could know more about this disease — she’s taken time to study it.”

On a recent visit to the ER at Olympic Medical Center for a suspected mini-stroke, the Hunters said they found everyone from the first responders to the paramedics, nurses and doctors to be very professional, accommodating and caring. “We do not fear the ER any longer – at least on the peninsula,” Susan said.

Helping more than themselves

The couple wrote the book over 2½ years to educate those with the bewildering symptoms of periodic paralysis who have or may not have been diagnosed, with its complex treatment and to educate medical professionals so they may recognize, diagnose and properly treat their patients in a timely manner.

The 430-page book begins with Hunter’s personal story of a history of bizarre symptoms, progressive disability and rising frustration with a plethora of physicians with no answers — or at worse, ridicule; a detailed analysis of what periodic paralysis is and isn’t and its prognosis; managing and treating the disorder; and what to expect psychologically and socially.

“Once we knew what I had, we decided to write the book and started our own website for people with periodic paralysis because there is a real need for it,” Susan said. “So far we have 70 members and get new ones everyday from all over the world. We do the best we can to help others with natural methods of controlling symptoms so people will have less and less severe episodes. If it’s caught early, people would not be as bad off as I am for lack of proper treatment.”

Susan has researched her family medically six generations back and has found signs and symptoms of periodic paralysis in its depth and breadth.

“I know it killed my mother so when I got my diagnosis, I wanted to share and know everything I could. I knew there must be other people like me and I knew they needed help,” she said.

“We’ve never been the kind of people to lay down and get run over. We come back stronger,” Calvin said.

One day at a time

Married 31 years, Susan and Cal know she’s living on borrowed time and both deal with her limitations. Yet, despite their separate and united burdens, it’s easy to see their overwhelming love for each other. Susan writes in the book’s afterword that like in John Denver’s song, some days are diamonds, some days are stones. The diamond days are just having one relatively brief paralysis episode upon waking, going outside in her power wheelchair to enjoy their forested acreage and connecting with other people worldwide with symptoms and/or diagnoses of periodic paralysis via their website at www.periodicparalysisnetwork.com.

What keeps her going?

“I don’t have an answer to that,” Susan said, pausing. “My children and grandchildren (who have some symptoms) and my loving, caring, wonderful husband Calvin, all the members of our board and their children. Before I die, I want doctors to read this book and have periodic paralysis be something everybody knows about.

I don’t want anybody to go through what I have. The book is to provide hope — if you think you can’t do something, be in my shoes. Do the best you can every day. I really didn’t think I’d live to see it published. Don’t give up, have hope and believe in yourself — knowledge is the key.”

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