Tuesday, December 31, 2013

A fantastic article to kick off 2014

I think this is the perfect article to wrap up 2013.

May we all have a healthier and happier year ahead.

Monday, December 9, 2013

Education, Understanding, and the Battle of Senses

HKPP is an ion channelopathy and a systemic disease, and it is very complex. Not only has it resulted in kidney disorders, skeletal weakness, reproductive system failure, endocrine problems, and muscular dystrophy, I am also hypersensitive to lights, smells, touch, certain tastes, and certain sounds. Once it became more severe and I realized that I have sensory problems, my life started to make so much more sense. I can remember walking into class growing up and instantly feeling simultaneously drained, nervous, and agitated. I even escaped to the office or restroom sometimes because I just had to get out of the room, and I couldn't explain why, but I HAD to because I was in pain and felt like I was going to collapse. Unfortunately, this was never addressed on a professional level in school, but there were plenty of mean and ignorant people in my life who said I was crazy, faking, seeking attention, and never took me seriously.

I found the same thing happening in college when my health began to plummet again. I definitely don't regret going to college, but I regret spending extracurricular time there, because it did more harm than good. Hindsight is 20/20, as we all know. I also finally figured out that public places like Walmart were a big trigger thanks to the crowd and lights. I shop at smaller grocery stores now (except the occasional Publix run, but I try to go during non-peak hours when few are there...also, thank God for power chairs). I wear sunglasses a lot now, including indoors, and it has made a big difference in how well I cope with sensory overstimulation. I spend a lot of time alone now in a quiet, dimly-lit room, which might sound sad, but I'm going to do whatever helps. People may think I'm weird, crazy, or a loner, but there are reasons for everything I do and I'm finally learning that the criticism of others is their inadequacy, not mine.

This is a big world, and there are an awful lot of people in it that we must coexist with. It should be everyone's duty to learn to do so with respect and grace. Education and understanding are the only way we will progress as the human race. Why wouldn't we strive for that? Is it not our mission to grow and become better with each passing day? We owe it to ourselves, those around us, and future generations.

Monday, November 18, 2013

Letter from Germany

I received a letter from Dr. Lehmann-Horn stating that they have received my blood and was able to extract my DNA. They issued a "family code" and that is the code I will use when I send them email or medical records. They want as many records as I can get my hands on. I will attempt to make the trip to Jackson soon (my next appointment isn't until March), if I can't get copies of my records via mail or internet. Sadly, I was told that some of the records were lost, but they should certainly have something on me.

Will keep everyone posted.


Sunday, November 10, 2013

2013 PPA Conference Links


Here are the videos and powerpoint presentations from this year's conference.

Thanks so much to the PPA for all they do.


Wednesday, October 23, 2013

I came, I saw, I went to the conference

The Periodic Paralysis Conference was on the 11th-13th of this month. I had a bit of trouble as I was ill with digestive issues the entire time, even throwing up in the car on the way there. I had a hard time hiding the fact that I was sick and miserable throughout this thing, but I survived.

I also ate anyway. The food was good. And I caught this amazing sunrise:

It was nice to meet several people from the online support groups. I loved meeting friends from Ohio, Colorado, North Carolina, New York, The Netherlands, and Ukraine. Alexander's wife was there and she spoke about the Periodic Paralysis community that has formed in her part of the world as a result of Alexander's work. I'm so happy that I was able to talk with her in person, it was the highlight for me. I admire her strength and her willingness to speak out about this condition.

Several physicians, including the top researchers in the world, were in attendance. I learned from all of them, and I appreciate the time and effort they put into this event.

I didn't quite make it through Saturday, and had to bail about 20 minutes early. I rested for a couple hours, and was back up and around for a small dinner.

Sunday, I could have easily shown up in my pajamas...ha. I was very tired. My tolerance of the lights and sounds and walking through the massive hotel and just sitting up in general was nearly to the limit. I was trying hard to take in the information that was being presented, but it's all a blur. I can only hope to God I didn't come across as obnoxious when I opened my mouth. That would certainly never be my intention, and I appreciate those who were patient with me.

I had bloodwork, and I look forward to hearing from the doctors in Germany in a few months. I am also sending off my 23andme kit soon. I'll report back when I get the results.

Road trips are very difficult for me these days, and being sick with my stomach on top of that really took a lot out of me. I wish I had felt better and connected with people more, but I considered myself lucky to be there to begin with. I was on my own at the hotel, and I didn't want to press my luck. Last thing I needed was for my muscles to melt down. They held up about as well as I was expecting, maybe a little better. I'm grateful.

I've been a bit down since arriving home. I returned to some drama, which definitely hasn't helped. I am hoping my circumstances will change for the better in the near future.

I will compile the notes I took at the conference (it wasn't much...too fast-paced) and provide some links to presentations as soon as possible. See you then.

Tuesday, October 1, 2013

Family and HKPP

Big news from my corner of the world. As of this week, my mom is finally acknowledging that she could have this condition, and wants to try the diet restrictions to see if her symptoms improve. We also believe she has two cousins with HKPP, and she plans to contact them for more info.

Anybody who knows what I've been through my entire life with this disease, and specifically, what I've been through with my mother, knows how huge this is. I've traced it back to her paternal grandmother, based on stories about her bizarre sickly behavior and random periods of being bedbound having to be waited on because she was weak. Mom's father also displayed symptoms. He would frequently lie on the couch and (quote from a relative) "act like he was dying, just like his crazy mother did". He would insist that he couldn't get up, but later he would be up and around again. Everyone said he and his mother were just lazy and crazy, and he even spent time in a mental hospital. How horribly sad that my grandfather and great-grandmother suffered like this and nobody believed their misery was real.

Waiting now for my mother to contact relatives. I'll keep you posted.

Wednesday, September 18, 2013

Acetazolamide: Why it works


According to the above 1977 study, acetazolamide therapy in Hypokalemic Periodic Paralysis patients resulted in less of a drop in serum potassium during an attack due to reduced glucose and insulin levels than those who did not take acetazolamide. It still drops when an attack is induced, but not as severely. The higher the glucose, the higher the insulin response and the harder potassium shifts as a result. In a nutshell, less insulin response = less potassium shifting. That would explain why there is less paralysis.

It makes sense now. Brilliant.

Saturday, September 14, 2013


My response to the MDA blog: Are You Hurt?

I'm glad Jaclyn is telling her story. The world tends to display ignorance when it comes to sharing space with someone who is different from them. Bringing awareness is important not only for those who are walking this road, but for future generations of people who will also seek the respect and acceptance they deserve.

I couldn't begin to guess how many times I've been approached with questions like "What did you do to have to be using crutches/cane/wheelchair?" or "Uh oh, you're walking with a gimp limp, what happened?" or "What's wrong with you?"

And then there are the people who refer to me as "crippled", or "gimpy", or who poke fun at me. One person looked me up and down one day with a smug look and said "Okay?" like I was some kind of freak. A man and woman actually followed behind me in a mall one day, mocking me under their breath, thinking I couldn't hear them.

I bet they'd raise hell if the tables were turned, but you see, people don't care unless it's happening to them.

I think it's vital to educate the public about rare and debilitating diseases. For that reason, I am very vocal about my condition, and I am more than happy to talk to someone who is genuine and respectfully inquisitive. In fact, I thank them when they ask. There is a big difference between them and the people I referred to above.

Words have the power to help or hurt. I may not always remember the names or faces of the people who say these things, but I certainly remember what they said. We could all stand to turn on our brains before opening our mouths sometimes. The world would be better if we did.

Thursday, August 22, 2013

HKPP Case Study of the Week


I ran across this article by accident. A nurse practitioner diagnosed HKPP in an 18 year old male who arrived in the ER unable to walk. He was lucky (I say that ironically) that his potassium was so low. Otherwise, they may not have known what was going on or believed him at all.

I'm glad the NP acknowledges that it's hard to diagnose HKPP because potassium doesn't always fall below "normal". I am one of those people, and the fact that my potassium doesn't always drastically fall out of "textbook" range is why I was untreated and mistreated for so many years. I am permanently weak at a younger than average age because of this.

I hope people will continue to bring awareness of all of the facets of this rare disease, so everyone who has it will be diagnosed and treated properly.

Saturday, August 3, 2013

Migraine meds a no-go...definitely.

I regret taking Zomig so much right now. After only one pill, I had an episode...slumped over literally pinned to the bed unable to move. Four hours later, was able to get up and do a Frankenstein walk to the restroom (8 feet from my bed), and almost couldn't open my mouth enough to swallow my emergency liquid potassium at bedside. At some point, I said in my mind "I really don't want to die today, God". Dramatic, but whatever. I meant it. I fell asleep for a few hours. I'm still moving in slow-motion and it's been over 9 hours since it started. I feel like I just came out of anesthesia. I feel every muscle in my body and I cringe with every movement, even hurts to breathe. I would have blogged earlier but I couldn't keep my eyes open or type. I still can't open my mouth enough to do more than sip liquid K.

P.S. - And I still have a damn migraine.

Thursday, August 1, 2013

Local clinic update 8-1-13

The clinic had no explanation for the mistake made on my Inspra script (see last medical update if you don't know what I'm talking about), but they called the pharmacy and worked it out. Now I'm back up to 100mg a day, although I am supposed to be on 200. I'll have to wait till I see Dr. F in November, then Dr. B again in December before it will be increased again. At that point, I will be back to where I was supposed to be nearly a year prior. Lord, this has been so frustrating.

In other news, Dr. B wants me to try something...anything...to deal with the constant migraines and tremendous lack of sleep. As those with HKPP know, the meds he prescribed for this (migraine med and a tricyclic) probably won't go well and I expect to have to discontinue them quickly. Maybe I'll get lucky, but history proves that these type of meds cause serious HKPP symptoms in me. We'll see. I start them tomorrow night.

He had no answers for my ailing right kidney. No infection shown, but he put his hand on my back and I nearly jumped off the table. No clue what the thing is up to, but it's giving me a lot of grief at the moment. Maybe the meds I start tomorrow will help a bit.

If you're confused, I don't blame you. Here's the rundown:

Dr. F is Nephrology and Internal Medicine. He and the doctors at the MDA clinic are in Jackson, MS. I see them several times a year and consider them my primary physicians.
Dr. B is local. He's Internal Medicine and runs a low-income clinic where I receive medication assistance. I have to take my scripts from Dr. F to Dr. B, who has a program for people like me who can't afford their medications. He has decades of experience and is widely respected. He has read about Periodic Paralysis, but I am the first person he has met with the condition.

All of my docs couldn't be nicer, and I'm grateful. I believe they are doing their best, and that's all I can ask for.

Monday, July 29, 2013

Alexander's Story

The Life and Death of Aleksandr Batutenko

Periodic Paralysis International has published Alexander's story. Side note: I called him Alexander based on the translation I was given when I met him on the PPA listserv, and he never corrected me. That's why I spell it the way I do.

Sorry it took me so long to share the link.

His wife Nadia and their children remain in my thoughts, and I hope to meet them at the Periodic Paralysis International Conference.


Monday, July 15, 2013

Renal Clinic July 2013, and Circumstances

I'm frustrated about my latest appointment. I was doing okay until the nurse discovered that my Inspra script wasn't filled correctly, which means I've been taking much less than I what I was prescribed. I have no idea how it happened, but I'm so mad at myself for not noticing that the dosage was wrong. With a migraine and running on 2 hours sleep, this news derailed me. Dr. F wants me to titrate the Inspra, so the script was rewritten for less than what I would have been on, and Medicaid doesn't pay for it so I have to take it to my local doc office and apply for patient assistance. That will take another several weeks. I'm upset that I'm going to lose another 1/3 year (next appointment in 4 months) of potential quality of life that I could have gained had the script been correct to begin with.

I am so grateful to friends for getting me to the appointment on such short notice. Finding a way to Jackson is getting harder, and it is extra stress that I don't need. I have an ultrasound on the 24th with no transportation as of today.

Dr. F wants me to start using the Cardy meter regularly again, so I'll have to invest in the calibration solutions soon ($88 plus shipping...ouch). My potassium continues to fluctuate daily.

Although my weakness is profound, everyone said I'm doing better where the paralysis is concerned, so I'm trying to slap on a smile. I met a new doc in training (Dr. H), and he was nice. He agreed that I basically have to choose my own poison. I can follow a muscular dystrophy diet or a kidney disease diet. They contradict each other, so it's a no-win situation. He's not familiar with Primary HKPP and I wouldn't have expected him to be. No big deal. He and Dr. F both seem like sweet people, and I'm thankful they are trying.

I wanted to bring up several things, but I didn't go through my list at all. By the time three doctors were in the room, I just wanted to go home. I came across as obnoxious, I think, although that is never my intention. I'm just tired, agitated, in a lot of pain, and understandably afraid of my future as a single, disabled, chronically ill woman with no income and no relief in sight. I'm a level-headed person, but Grace has never been my name, and it probably never will be.

I'm going to talk to Social Security soon about any possibility under the sun of finishing college online through federal financial aid. I expect them to say no, but I want it in writing. To be stuck here unable to care for myself, watching life waste away, relying on people around me for everything is not living. I don't have a prayer if I don't figure out something.

I'll continue open courseware regardless. I've downloaded some courses from MIT and I'm diving back into Psychology, Social Studies, and Art Therapy as soon as possible.

I will never accept the way things are at this moment. I will continue to fight for a life worth living.

Thursday, June 27, 2013

Limbs On Fire

I'm always floored by those who say they don't experience pain with HKPP. I can remember being in elementary school, rubbing and holding my limbs because they were sore. I even remember saying as a kid that something wasn't right about my arms and legs, and I had headaches and stomachaches all the time, but I was written off as exaggerating. You know how it goes at school: Do you have a fever? No. Are you throwing up? Not yet. Nothing wrong with you then, go back to class. Story of my life.

I'm struggling with constant burning pain lately in addition to my usual pain and weakness and side effects from acetazolamide. My senses are screaming, and I pretty much have to stay in a dim, quiet room most of the time just to tolerate life in general. The acetazolamide has warded off some of my daily paralysis and there's no question that I need to stay on it to some extent, but it has been over 6 months now and I'm not seeing an improvement in side effects. I'm on 125mg a day. I've tried to titrate several times but this has been a complete failure. So I'm stuck at 125, which is better than being in paralysis every morning, but Lord help me...I'm having a terrible time with this. Lights, sounds, touch...even clothing gets unbearable. It's a lot like the pain that occurs when coming out of a full body paralytic attack...for those who have been there and done that I think you know what I mean...the hot-cold-burning-stinging-help-me pain. I have lived with chronic pain since HKPP triggered in childhood, but this is worse.

I guess I'm just lamenting that I'm trading one debilitating symptom (paralysis every morning) for another (24/7 burning pain, dizziness, auras, migraines, hypersensitivity, etc). The triggers still win sometimes and I have paralysis, but it's not as constant as it used to be since starting acetazolamide, and I'm grateful for that. I am. But I'm struggling. My level of weakness has not changed. Still on crutches or a walker, sometimes in a chair. Have I already talked about this? I probably have...apologies for the repetition. I'm loopy and exhausted and I'll probably regret rambling like this tomorrow.

I'm one of those stubborn people who isn't on prescription pain meds. A few years back I was prescribed tramadol on a long term basis. I found that I was in no less pain after a little while, so I ditched it. I found other meds aren't worth the side effects, and I'm pretty stubborn about taking pain meds in general. I'm to the point where I'd consider it again, though. I'm not sure what to do next but I wish to God I could figure all of it out because I'm not cut out for this. Who is?

Tuesday, June 18, 2013

2013 PPA Conference

I'll be attending the Periodic Paralysis Conference
thanks solely to the kindness of others.
So grateful to those who are helping me get there.
I hope to meet many of you in October.

Click here for info on the conference.

Tuesday, June 11, 2013

Family Matters

I've been up all night writing a long-winded email about my family history with this condition. As ugly as the story is, I think it is important to share with those who have (or think they have) the condition. If you are not on the PPA listserv and want a copy of it, feel free to contact me with your email address.

I'm thinking about my older brother and my younger cousins. It is hard not to worry about them where the possibilities of this condition are concerned. It would be a horrible shame if they went through the same horror that I've gone through...being disregarded, written off as crazy, or downright mistreated. The thought is unacceptable. I hope if any symptoms arise, they will take them seriously and seek medical care. It is important that they understand that Familial Hypokalemic Periodic Paralysis is inherited, and they could be carriers of the genetic mutation.

I'm mulling over whether or not to look for a relative. She is trouble with a capital T, to the point that I don't want her to know my phone number or where I live. However, she expressed years ago that she was dealing with weakness and waking up unable to move. I feel it is my duty to give her the information that I have discovered about this condition since the last time we spoke. She has no idea that I believe she has the condition, because I didn't understand it back then. Had I known, I would have said something and things might be different for her today. I guess I have to do this. Wish me luck. It is not going to be an easy task.

Thursday, June 6, 2013

Periodic Paralysis: Kelli's Q&A June 2013

Disclaimer, as seen in my sidebar:

I am not a physician and I have never claimed to be a medical expert. I am someone who has been actively fighting Familial Hypokalemic Periodic Paralysis for many years. This blog is mine alone and does not necessarily reflect the feelings or experiences of other patients. Any direct quotes used here will be properly cited to the best of my knowledge. Thank you.

I felt like doing a little Q&A session. I may do these every once in a while. If you have questions for future sessions, I'm easily accessible (comment, email, Facebook, Twitter, etc).

Q - What is Hypokalemic Periodic Paralysis?

A - Periodic Paralysis is a voltage-gate ion channelopathy caused by a genetic defect in the ion channels. The gates that control shifting of potassium through the muscle membrane do not open and close as they should, causing potassium to leak into or out of the cells inconsistently. This results in malfunction of skeletal muscles.

In Hypokalemic Periodic Paralysis, potassium moves from the bloodstream into the muscle cells and gets trapped there for a period of time. The ratio of potassium in the muscles vs the bloodstream is thrown off, causing the body to weaken and sometimes depolarize, resulting in episodes (also called "attacks") of paralysis. Episodes can involve only one or two parts of the body (localized) or the entire body (generalized). Episodes can be mild or severe, with some resolving quickly, while others linger for extended periods of time. It is chaotic and unpredictable.

The Periodic Paralyses are classified as a rare form of Muscular Dystrophy, as per the Muscular Dystrophy Association.

Feel free to check out my Yahoo article if you like. It goes into much more detail.

The most thorough source of medical-based information on Periodic Paralysis is HKPP.ORG.

Q - Is Periodic Paralysis life-threatening?

A - It can be. Some patients have few attacks and are able to lead productive lives. Others aren't so lucky, with progressive weakness and frequent episodes, some of which are more serious than others. There are patients who have episodes that affect breathing, swallowing, the heartbeat, and other vital processes. Some patients have died from complications. As per Periodic Paralysis International and the Periodic Paralysis Association, new survey data is being considered for the purpose of updating statistics, as it is believed that the current statistics for PP-related fatalities are underestimated.

Q - What is the life expectancy of someone with Periodic Paralysis?

A - There is no life expectancy given, as Periodic Paralysis is much too unpredictable. People have died young and people have died old. There is no way of knowing, so you might as well take one day at a time. Keep Calm & Carry On. Carpe Diem. Insert your favorite philosophical phrase here. Whatever keeps you going.

Q - I don't understand the medical terms. What can an episode be best compared to?

A - The best way I have found to help people understand the term "episode" is to compare it to a seizure. Everybody knows that a seizure involves uncontrolled movement. Muscle contraction fails in an episode of Periodic Paralysis, resulting in uncontrolled NON-movement. If there was such a thing as an "anti-seizure", this would probably be it. That is as simple as I know how to say it, and this explanation has helped some of my friends and family understand what I deal with a little better.

Q - Periodic Paralysis and Social Security?

A - Some people with Periodic Paralysis have been declared disabled by the Social Security Administration. I am one of those people. I will write a separate blog entry explaining the process that I went through in getting approved.

Q - Are Mitochondrial Disease and Periodic Paralysis related?

A - There are only three things that I can say about this:
     1. Mitochondrial Myopathy (MM) is a rare form of Muscular Dystrophy, just as Periodic Paralysis (PP) is, but they're not the same thing.
     2. I know a few people who display symptoms of both MM and PP.
     3. I have not been tested for MM, but I know others with PP who have.

Q - Are Multiple Sclerosis and Periodic Paralysis related?

A - MS and PP are not related. In fact, Multiple Sclerosis and Muscular Dystrophy are not in the same class at all. As I mentioned in a previous blog entry, Multiple Sclerosis is a debilitating disease that involves loss of motor function via damage to the central nervous system (brain, spine, nerves). Muscular Dystrophy, which includes Periodic Paralysis, directly affects the muscles and the patient remains aware of everything that is happening to them. While Periodic Paralysis patients complain of "brain fog", it is a temporary symptom comparable to the confusion and general "out of sorts" feeling that accompanies more common conditions like Diabetes. MS can result in cognitive deterioration, whereas MD has no effect on intelligence. Both are terrible, but not related.

Q - Is there a connection between migraines and Periodic Paralysis?

A - Last I heard, at least half of Periodic Paralysis patients reported migraines (I'm one of them), but migraines are often a condition all on their own so they may or may not be associated with Periodic Paralysis. That said, I guess the answer is yes and no.

Q - Is it possible for my dog/cat/horse/etc to have Periodic Paralysis?

A - Absolutely. I mean, it's not contagious or anything. :-) But yes, animals can suffer from channelopathies. I've heard of horses, especially, having Hyperkalemic Periodic Paralysis.

Q - Can GERD or other digestive problems be associated with Periodic Paralysis?

A - I have GERD, but its relation to my Periodic Paralysis has not been discussed. I do know of many who state that they have been diagnosed with digestive problems including Gastroparesis in addition to Periodic Paralysis. This makes sense as the condition can be caused by weakened stomach muscles or partial paralysis (thus the word 'paresis').

Q - Why on Earth would acetazolamide be used as a treatment for Hypokalemic Periodic Paralysis?

A - Excellent question. I haven't found a straight answer as to why acetazolamide (DIAMOX) helps some patients with Hypokalemic Periodic Paralysis. It is baffling that a potassium-wasting drug could be beneficial to someone who deals with misplaced potassium to begin with. However, it has been proven effective in approximately half the patients who were studied. If you've been following this blog for the last several months, you know I reluctantly started acetazolamide in November. The side effects are kicking my butt, yet I am 100% sure that it is responsible for reducing some of my paralysis. The reason is pure speculation, but the only thing I can imagine is that this drug is pulling trapped potassium out of the muscle cells and back into the bloodstream. It is eventually wasted, but since I am on large amounts of potassium supplementation, I am replenishing the potassium that is being wasted by the drug. It's just crazy enough to make sense, but I'm no expert. I'm only thinking out loud, so to speak, as usual.

Q - What kind of doctor specializes in Periodic Paralysis?

A - Most patients I've spoken to are treated via Neurology, Nephrology, Internal Medicine, or a combination of the three. The Muscular Dystrophy Association has clinics in North America that are designed to diagnose and treat all forms of MD, including Periodic Paralysis. Unfortunately, I've been informed by some patients that not all clinics are fully on the bandwagon where PP is concerned, so experiences vary.

I am one of the lucky ones who is acknowledged by MDA of Mississippi, although it took a very long time and a drastic nosedive in my condition to get to that point (better late than never?), I am grateful to have their support now. I also have a physician in the same hospital who is licensed in Nephrology and Internal Medicine. He is treating my condition aggressively. Nobody has tried as hard as he has to help me regain quality of life, and I don't take this for granted.

I think it's important to find a physician who, first and foremost, listens intently. If the person you're talking to doesn't believe you, isn't interested in learning about the condition, or sees you as merely a number in general, it's pretty safe to say they are a waste of your time. Periodic Paralysis is complicated and requires a great deal of education, open-mindedness, and teamwork. Sometimes I have to remind myself that doctors are people too, and some of them just get it wrong. But those that get it right are priceless, and thankfully, they do exist. Do yourself a favor and make it your mission to find them. I cannot stress enough to those who are still seeking medical help that you keep looking after the first doctor rejects your case. And the second doctor. And God forbid, the third and fourth and so on. If you have a medical condition that is robbing your life, whether it is Periodic Paralysis or something else, search until you find a physician who listens and takes your condition seriously. They are out there, so please don't give up. Even when the situation seems unbearable, DO NOT GIVE UP.

If someone has questions about life with Periodic Paralysis, you are welcome to ask. Just remember that all responses are from a patient's perspective. Thank you for choosing to learn more about this rare and complex disease.


Wednesday, May 22, 2013

Thankful for laptop computers and internet so I can communicate from my curled up, partially paralyzed position. This is one of those episodes that I am baffled by, as I don't have any idea what brought it on. Periodic Paralysis doesn't follow a textbook or play by any rules. Sometimes things go badly no matter how hard we try.

Edit Note, 12:40AM May 23: I knew if I thought hard enough, I would figure it out. This is a sodium episode. I've eaten caesar salads the last two days, and after researching, I've discovered that on top of the rest of my meals have put me somewhere in the 5000s for sodium mg in a two day period. Much too high for someone with HKPP. Add that to already being very weak from a short road trip, and you have a recipe for disaster. Salad, of all things. I can't win.

Friday, May 17, 2013

Three Years Ago Today

Three years ago today, I was training at the Bob Ross Art Workshop in beautiful New Smyrna Beach, Florida. How quickly things change.

I wasn't well...not by any means. I was on a walking cane, in horrible pain, and sick a lot, but fairly functional and not taking a minute for granted. I made every effort to work part time even though I was on a downhill spiral with permanent weakness due to Periodic Paralysis. At that time, I had no medical help...I appeared somewhat healthy with the exception of the cane, and I couldn't get anyone to listen to me or take my history with the condition seriously even though I had a documented diagnosis.

So, I carried on with life as best as I could. I returned to Florida for training so I could improve my craft and increase business. My students were wonderful, and I desperately didn't want to let them down. I was so happy to be doing what I loved, as seen by the cheesy grin on my face there.

I look at these photos now and wonder how on Earth I did it. It seems so long ago, it's hard to believe that I'm the same person and that is me standing there only 36 months ago. It almost doesn't seem real.

But it was real, and I miss it all so very much.

Thursday, April 25, 2013

Action Potential and Periodic Paralysis Videos

Familial Periodic Paralysis is classified as a voltage-gate ion channelopathy, which means that ion channels do not open and close properly. This results in failure of the muscles' action potential when potassium leaks in and out of the cells inconsistently. Above is an animated video that shows what the process is supposed to be.

Below is a video produced by the Periodic Paralysis Association, which shows and explains the malfunction. Thanks to Dr. Jacob Levitt, Linda Feld, my friend Wade, and all who were involved in the making of this video.

Monday, April 22, 2013

A Request, Apology, and Thanks

With Hypokalemic Periodic Paralysis, salt, sugar, and many other common substances are potentially dangerous. Since there is no way to avoid them completely, life is a roller coaster and every day is a challenge to find the right balance, which is constantly changing. The best HKPP patients can do is avoid these triggers whenever possible, and face them when we have no choice. The list is long...you wouldn't believe how extensive it is. It would be fantastic to be "normal" enough to not have to nit-pick about every little detail of every little thing I come into contact with, but most who live with this disease are required to treat triggers as allergies for the sake of survival and quality of life.

That said, when someone presents a suggestion to me (product, advice, etc) and my response isn't what you wanted it to be, I assure you there is a valid reason for it. It is not meant to offend you nor make excuses, and I ask that you try to respect me enough to take my word for it. You can say you're knowledgeable about the condition and you can think you're more intelligent than me...that's perfectly fine...but the fact of the matter is that I know my body more than anyone else, and I'm not going to allow something or someone to do me harm no matter how precious and pure the intentions are. I welcome all input, suggestions, and questions...don't ever hesitate to make them known. I am a nice person, and I don't bite. I promise to respond in truth, with respect, even if it's in disagreement, and thank you for your consideration. Because I sincerely appreciate it.

I chose a long time ago to make my journey public because I need to bring awareness to this rare disease...certainly not just for my sake, but for others too. People have found information about HKPP and learned about the condition because of my public rambling coming up in searches. People have thanked me for my honesty because it makes them feel less alone. People have asked me to continue to share my struggle because it encourages them to pray and be grateful for their health. If anything I say helps even one person, I dare to say the rants are worth it.

I'll be as mindful as my tired brain can be and do my best to spare everyone any major drama or TMI, but otherwise...this is my life right now. Take me or leave me. If you are a new reader, I welcome you and I apologize if I have scared or upset you with my posts. I don't talk about these things to create anxiety in people, but I have friends who love me and truly want to know what's going on. Some even take time out of their day to pray for me, and that matters. I personally think it keeps me alive.

To those who care and have been here for me, thank you will never be enough. I do not take you for granted, even when I'm silent. What I would give to be able to repay the kindness...words cannot express. Thanks for putting up with me.

(Originally posted on Facebook)

Saturday, April 20, 2013

April MDA and Renal Update, Fighting, and Gratefulness

God, how depressing is this blog? Ha. I just reviewed the last entry and it made me laugh a little bit, honestly. I probably shouldn't post anything on the internet when I'm feeling so badly, but I've always been one to keep it real.

Hard to believe I've been blogging online for over 10 years now. It has been a lonely road with this disease, but I feel worlds better since finding others who know what I'm going through. Having a mother who has the condition but refuses to acknowledge it is not easy. Had she been willing to pursue diagnosis and treatment, perhaps we could have dealt with this together. She has made it clear, though, that she wants to be left alone. It's her choice, and there is nothing I can do to change her mind. I just hate to see her (and her husband) with reduced quality of life as a result.

I'm forever grateful to have my aunt and uncle. They stepped in when the rest of the world stepped out. I would have never made my way to the docs in Jackson had my aunt and uncle not decided to help. Thank you thank you thank you.

I'm grateful for the support groups on Facebook and the PPA/PPI Listserv. What I have learned there has saved my sanity, if not my life. In spite of the occasional drama that rears its inevitable head, it is imperative that these support systems remain. I hope the powers-that-be realize how much they are needed.

I also can't say enough about the doctors in Jackson. I become discouraged often between visits, but I am always encouraged again after I've talked to them. Having the MDA team and Dr. Fulop on my side is a blessing that I do not take for granted. Dr. F continues to deal with my ups and downs with the utmost kindness. I always arrive to the office feeling sad, and end up laughing about something by the time I leave. God knows I find humor wherever I can; sometimes I think it's the only thing that keeps me going. I wish I could clone my doctors and send them to every hospital in the world.

My heart breaks when I think about my friends with HKPP who still can't find someone who listens or takes the condition seriously. I lived that nightmare for 20 years, and I am a broken person for it...albeit, stubborn and perseverant. When things became much more serious in recent years, I launched into "sink or swim" mode and became what my friend Katherine now calls her "snarky, loving, tender, angry soul sister". That cracks me up and makes me a bit sad all at the same time. God knows I've never wanted to be the fighting type, but I've been forced to be and it's a big reason why I'm still here. I've had some terrifying times...moments when I didn't know if I would see tomorrow. I've learned that living this way can alter one's perspective about life. I am an old soul. I'm very tired, I get cranky, maybe even angry, yes; but I'm also quite passionate, empathetic, and grateful as a result. I pray with all my heart that the latter triumphs over the former. I want everyone with this condition to never give up on finding the medical attention you need. Get angry and fight if you have to, but whatever the case, hang on to hope and don't surrender. If I can do it, I know you can too.

My appointments in Jackson went well, with the exception of my legs paralyzing half way through the day. I was in a wheelchair by the end of lunchtime (lunch was at Babalu, by the way, which was amazing). I met the CNP at the MDA clinic, as Drs Veda and Witt were out of the office. Carolyn was great. We discussed my current condition and medications, and she ordered A1C and vitamin blood work. We agreed to put me back on Vitamin D 50,000 IU weekly, as I seem to be deficient otherwise. I'm still waiting on B12 results, but my A1C already came back and it was fantastic at 5.7. This was a bit of a surprise, as I have not only gained 15 lbs over the past 2 years, diabetes runs heavily on all sides of my family. Praise the Lord, I beat the odds again. I met with Dr. Fulop and one of his students in the late afternoon. I told them about the acetazolamide, and there was no hesitation that I should remain on whatever dose I can tolerate. I admit I have mixed feelings about staying on it because, God help me, I'm such a miserable person on this drug. Nevertheless, I agree wholeheartedly that I should continue with the regimen due to the slight reduction of paralytic attacks. We also discussed Inspra and agreed to double it. I'll let everyone know how that goes. I return to Jackson in July.

I think I forgot to tell Dr. F about Dr. B, who has rewritten some of the scripts (perhaps he realized this, when I didn't ask for refills) so Medicaid would cooperate. They have a problem with the fact that Dr. F is in Mississippi, even though the hospital itself accepts Alabama Medicaid. Frustrating situation. Hopefully it will all work itself out. My next appointment will be at Dr. B's office in a few weeks, where we'll discuss the latest news from Jackson and take a look at labs.

I was going to tell my doctors about Alexander's death, but I knew I couldn't get through it without sobbing, so I didn't. Maybe next time I'll be able to talk about him.

I still haven't caught up on the blog here, and I apologize. I am going to try to remedy this soon. Thanks for reading my ramblings, and don't hesitate to ask questions if you have them. I realize I have the vast majority of my readers on Facebook and elsewhere, but by all means comment here if you prefer.

Quite a few articles coming soon.

Thursday, March 21, 2013

Acetazolamide treatment update (alternate title...March Madness?)

The Score:

Side effects - 99
Kelli - 0

I have been taking acetazolamide (DIAMOX) since mid-November, and I am not willing to sugar-coat how I feel. It has made my life truly miserable. I cannot begin to express how difficult the side effects have been in spite of titration. I have tried so hard to tolerate it, but I am losing the battle.

My side effects include: lethargy, drowsiness (but no restful sleep whatsoever), nausea, dizziness, loss of balance, paresthesias, tinnitus, hearing dysfunction, alteration of taste (dysgeusia), generalized and localized pain (head, bones, muscles, nerves, kidney, urinary tract), kidney stones (I'm on potassium citrate for prevention), hypersensitivity of all things sensory in nature (light, sound, touch, even emotional triggers), occasional delirium due to sleeplessness, and more. Some of these things are new, while others were an issue before the treatment but have been noticeably heightened by the drug.

The problem with getting off of it is that my paralytic episodes will increase again. I proved this by quitting it for several days. I quickly found myself back in a near-constant attack of paralysis, especially in my upper body, as I often was before starting the treatment. I got back on it, and the paralysis lessened again. I still have random episodes, but not like before the acetazolamide. It's hard to explain...all I can say is take my word for it. The lessened paralysis is the only reason I am still taking the drug. It is too important to ignore, and I still hope for a solution to this madness.

Having to choose which misery to live with is a difficult place to be. I will meet with the doc who prescribed it sometime in April to discuss, but he has not returned my phone calls so I am on my own until I meet with him face to face. I'm aware that he doesn't know what to say or do at this point, and neither do I, but we will discuss any further options and ultimately, hopefully make the right decision.

At the age of 35, I should be in the prime of life. Instead, I fight for any life I can get. Nevertheless, I will keep fighting. I know I'm not alone in this. I am grateful to those who are on the front lines with me, as well as all who have offered their thoughts and prayers.

Thursday, February 28, 2013

Rare Disease Day 2013

                                                Show awareness by spreading the word.
                                               Learn something new about a rare disease.
                                             Offer support to someone fighting their battle.

Monday, February 18, 2013

Local Clinic Follow-Up

I finally returned to the local clinic and saw Dr. B, who ordered the heart monitor for me last month. The results were exactly what I was expecting. Yes, I'm having some arrhythmia. No, there's nothing that can be done about it. So that's the end of that. Quick and painless, I suppose. I'm so glad to be off of the monitor because that thing was driving me absolutely batty.

We spent a good bit of time discussing other things. The aggressive treatment I'm on, the side effects, and the fact that I'm needing a local doc to take over my care...mainly script-writing. I'm now on Medicaid, and need a Medicaid-approved physician to issue all prescriptions. Otherwise, I'm seriously up a creek.

I have yet to successfully titrate the acetazolamide to the dose Dr. F wanted me on, but I'm still trying and I'm not going to give up. I am encouraged by the fact that I am no longer waking up in a state of paralysis. Yes, I still have paralytic attacks regularly...some of them serious...but I used to wake up paralyzed every single day. Since getting the acetazolamide in my system, that hasn't happened. That's a good thing, and in spite of the fact that it isn't improving my general weakness, and the side effects are very difficult if not unbearable at times, the conclusion is that I need to stay on this drug. I'm also still on everything else, and I'll update about meds and side effects later.

Dr. B is concerned about my kidneys, so he ordered a work-up and gave me a month of samples of Detrol to deal with some bladder spasms (sneezing has been a bad idea lately...if you get my drift). I've also been dragged against my will into an OBGYN appointment, which I probably won't talk about here because I don't really see the point. You're welcome.

The great news is that he had no problem agreeing to rewrite most of my scripts. They've been turned in, and I will find out tomorrow whether or not Medicaid is going to cover them all. We're talking about nearly $1000 in a month in medication. Pray hard, brothers and sisters.

Wednesday, January 9, 2013

2013 has been "eventful" so far

The King & I

I'm currently on the King of Hearts Express, a loop heart event monitor that records an EKG when I push the button. Tuesday was a rough day. I hit "record" three times. I only have two recordings left, because they only gave me five.

I'll consider a video blog about the monitor, and of course I'll report back when I know the results.