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.