Sunday, August 21, 2016

Keveyis article that I may or may not have already shared

http://www.biopharmadive.com/news/Taro-keveyis-free-rare-disease-drug/418564/

I know I've mentioned it here before, but here's a short article about ‪Keveyis‬ being given to patients free of charge after lack of profit. One reason for the marketing downfall is that several of us with Periodic Paralysis were unable to tolerate the drug's potassium-wasting potency, which caused us to get worse instead of better. In my case, I'm already on very high amounts of prescription potassium, but it wasn't enough to supplement the loss that occurred on the drug. Anybody with severe Periodic Paralysis will tell you that even the smallest fluctuation of potassium in the bloodstream can affect us. If prescribing physicians aren't familiar enough with voltage-gate ion channelopathies to know how they work, don't expect them to understand how the drug works and how to compensate for the potentially catastrophic side effects. Doctors hear the word "Hypokalemic" and look up dichlorphenamide and think we're bonkers because they don't understand the condition nor the the way the drug manipulates the kidneys and muscle channels to prevent depolarization. A little education can go a long way, but when it comes to our rare disease, a great deal of (continuing) education and experimentation is required. Unfortunately, most doctors aren't up for that, and patients suffer as a result.

While I figure the change from over $100 a pill to free is a write-off for Taro, it's one that is helping patients who are benefitting from the drug. There aren't many, but the ones whose lives have improved are worth it. And for that, we sincerely thank them for their contribution. It's not something to be taken for granted in a country full of capitalist asshats like Martin Shkreli.

I'm still so disappointed that Keveyis didn't work out for me. But knowing my turbulent history with Periodic Paralysis, it wasn't the least bit surprising.

Monday, August 15, 2016

August 11 test results

I picked up my test results from the clinic today.

Phosphorus is still too low, potassium needs significant improvement, and kidney function has slipped again. In better news, after heavy supplementation (over 150,000 IU a week), my Vitamin D is finally in low-normal range for the first time in my adult life.

I will continue all scripts, my K+ dosage has been increased again and is now 200mEq a day, and I will have to keep searching for any possible way to bring phosphorus back into normal range.

I have a CT and Barium swallow test at the crack of dawn Wednesday.


Thursday, August 11, 2016

Internal Medicine August 2016

I spent the afternoon at internal medicine clinic. They administered emergency potassium because it was 3.7 and I could barely lift my body. (Feel free to visit previous blog entries if that number makes you skeptical, as I've already explained why the 3s are a very bad place for me.)

I had the usual panels of blood work (Stat K+, BMP, Mag, Phos, Vit D), and based on results vs medication doses, we agreed again that my body isn't absorbing my diet or supplements well at all. No solution known.

New GI and CT scans are being scheduled, assuming insurance approves, to monitor my choking and swallowing issues and double-check my head since the migraines and auras are so severe.

I met the on-staff social worker for the first time, who is getting me back in the door with social services, as well as arranging free transportation to future doc appointments that are scheduled in my county. That'll be interesting.

The social worker and Dr. B both assured me not to worry about my impending SS Disability review. I don't know why it has worried me so much, but it has, and I'm relieved to know they have my back. My disability is dead obvious, but my anxiety loves to play worst case scenarios over and over in my head. I'm sure social services will probably offer therapy, and I'll accept. I think everybody should seek counseling at some point in their life, even if only to confirm that it's unnecessary. I dare say nearly everyone could benefit from it. At any rate, I am not considered a severe case, according to the social worker, but I certainly have enough on my plate to qualify for services. We'll see what happens.

I'll post my test results when I know them, and I'm scheduled to go back in two weeks for follow-up tests.

Thursday, July 21, 2016

It's Complicated.

















If you don't have Facebook, you're probably confused about the above photo. It's OK.

I have a difficult time eating like a regular person, at least not without big consequences. MD, CKD, GERD, and Gastroparesis do not make my relationship with food easy. I dare say it's an unconquerable battle. There's the fact that I have to avoid carbs, yet eat them to prevent Hypoglycemia. There's the sodium that I have to limit greatly without becoming Hyponatremic on three diuretics. There's the issue of wanting to eat more vegetables, but they contribute to kidney stones (of which I've had at least 40 in my lifetime, including surgery). I also have Dysguesia, which makes vegetables taste terrible to begin with, but that's another story. There are also the nightshade vegetables, which contain much needed Potassium, but by golly they also contain an ingredient that inhibits neuromuscular transmission. Epic fail. Fruit contains fructose, which triggers HKPP just as badly as glucose does, and artificial sweeteners are even worse than that - they've nearly put me in the hospital with serious paralysis. Thanks for that no-win situation, universe. Caffeine both helps and hurts, depending on what organ we're talking about. My Magnesium fluctuates just as badly as Potassium does, resulting in a wide variety of ugly symptoms that qualify as TMI (I could write a thousand words on Magnesium alone). My Phosphorus and Vitamin D are chronically low, with no resolution via diet nor supplementation - I've confirmed that nothing works, and nobody knows why. My Muscular Dystrophy diet is high protein and fat, and I've gained nearly 30 pounds as a result. I'm now borderline obese, and doctors are bringing it up. Some days, I'm too sick to eat anything substantial, so I drink protein drinks. My kidney disease and heart disease calls for - guess what - low protein and fat! That's the exact opposite of my MD diet - lucky me! I was referred to a Dietician concerning low electrolytes, who gave me quite possibly the worst advice a Dietician could ever give anybody - eat more chocolate and processed meats, and drink Coke. That was a WTF moment if I've ever had one, and a fast track to HKPP. My doctors keep telling me to eat things that I cannot safely eat, and take drugs that I cannot safely take, because they are dumbfounded by the situation and can't keep up with all of my triggers and side effects (not a real complaint, because I don't know anybody who could remember this much insanity unless they were living the life themselves). Not knowing or understanding isn't the issue, by the way. It's pretending to know and understand, or being condescending and criticizing in spite of the lack of knowledge, that is the problem. Doctors across the globe need to learn to say "I don't know, but I'll listen and try to help." I digress.

Let me get back on track. Food! It must be so nice to be able to eat and drink whatever you want without acute, life-altering side effects. If there is a heaven (I continue to believe there is, personally), let's just say I'll enjoy feasting. Because what I'm dealing with on Earth is a whole lot of BS.

It could always be worse. And I'm thankful it isn't. I'm human. I get frustrated, especially when every single day is difficult, and there is no reprieve. I don't want my daily life to be consumed by what I put into my body, nor the atmosphere that I surround myself with, but I have no choice but to obsess over it if I intend to survive and have any quality of life. I'll elaborate on the atmosphere part in another blog (same bat time...same bat channel).

At least I haven't lost my stupid sense of humor.

I can't cook all the time due to muscle weakness, but I'll leave you with pictures of some of the foods I eat on a fairly regular basis. Protein galore. Enjoy.

Yellow Split Pea Soup with
extra crispy bacon

Turkey Vegetable Soup
I make so much soup.

Lots of Taco Tuesday happening,
albeit, without nightshade veggies.

Occasionally, I honor my grandmother.
It's a comfort food. I miss her.

The most incredible veggie frittata ever.
(Prior to nightshade elimination.)

French Lentils

Veggie Beef Soup

Shrimp Burrito
(Also prior to eliminating nightshades.
I don't buy flour tortillas anymore either.
P.S. - This is subject to change. LOL)

The beginnings of hummus.

Citric Acid is essential if you have kidney stones.

Kale and Collards
So bitter, but I'm trying.

A simple Hawaiian pulled pork roast
This fed six people, with leftovers.

New obsession: Turkey Tenderloin

As seen here.

And here.

No caption necessary.

I just discovered avocado smoothies
next door at the donut shop of all places.
It was great. Super high protein.

If you don't have access to this
Alabama goat cheese, I am so sorry.
It'll knock your socks off. Swoon.

Mediterranean Lentil Soup
I make this regularly.

Beeeeautiful.

I still have symptoms, even though I constantly analyze whether or not I should eat something. The thing is, I never know when even some "good" meals are going to affect me. It could be a matter of a pinch too much salt, or the carb load was too high that day, or eating slightly too much in one sitting, or not eating enough, or it may contain tryptophan, or I've had too much fiber - the list of possibilities goes on and on. As usual, I can only do the best I can.

One thing is for sure: being a foodie with systemic disease is like being in an intimate relationship - it's complicated.

Monday, July 18, 2016

Banana Advice

Thanks to Geoff Lehman for the meme.

Please cease and desist this terrible advice to HKPP patients. :) I would personally have to eat 26 bananas a day, and not only would my body not absorb or magically utilize the potassium properly, I would die from the carb load. We don't care that bananas have a little bit of potassium in them. You don't understand Primary Periodic Paralysis.

Stop the madness. Thank you. :)

Saturday, July 9, 2016

Medical Equipment Drama

Indy 500 (my rolling walker) and I have visited a large local med equipment company a couple of times. They may be really nice folks, but they are the absolute worst salespeople I've ever met. Every time I have gone there for walker or wheelchair discussions, they have said things so ridiculous and aggravating, I've wanted to scream. Just a few examples:

"It doesn't matter what kind of seat is on the walker, you're not supposed to be sitting on it anyway."

"That one only holds up to 200 pounds." (Side note: I'm not 200 lbs)

"The whole chair is 129 pounds. It's not THAT heavy."

"You don't need a wheelchair right now. You don't want to become dependent on it."

"You look like you're doing fine."

These people have tested my patience to its maximum level.

At any rate, they informed me that a power chair lift cannot legally be installed on a Buick because it sits too low to the ground. They said some people have been brave/crazy enough to rig a lawn mower trailer on the back of their cars for this purpose, but that is not an option for me for several reasons. Mostly, I wouldn't do it because power chairs are a thousand bucks, and I'm not "rigging" a thing. It will be done right, or it will not be done at all. But I also live in public housing with one parking space, and a trailer would block incoming traffic. This is not a possibility.

A truck or SUV with a proper hitch is the only way to legally do this, so that is what I'm facing. An approved vehicle first (unknown cost), then a chair and lift (which amounts to about $1950, not counting tax, accessories, or lift installation).

In summary, I'll have to accept my limitations on the walker unless my circumstances (finances, living arrangements) somehow change.

Saturday, June 18, 2016

HKPP Episode, and Nightshades

I had a big episode last night after eating mashed potatoes. Not only is it a simple carbohydrate, it is also a nightshade vegetable. I decided to research nightshades due to the fact that people have said they can cause muscle problems, and what I learned was very disturbing.

They're cholinesterase inhibitors, which can cause everything from inflammation to dementia. Other cholinesterase inhibitors include pesticides (HUGE dangerous HKPP trigger in me...one of the worst!) and even some teas. If pesticides wipe me out (and believe me, they do with a vengeance), it makes sense that nightshades would do the same.

List of nightshades and other risky produce:

tomatoes
­ potatoes (not including sweet potatoes or yams)
­ eggplant (also called aubergine)                                      
­ peppers (all varieties except white and black peppercorns)
­ goji berries
huckleberries
blueberries
­ gooseberries
cherries                  
­ tobacco
­ paprika

Beware of spice blends, which usually contain red pepper and/or paprika.

There are a few other no-no plants out there, like Petunia and Belladonna. Feel free to Google if you want to look into it further. The lists are all over the net.

I've concluded that I need to address the following:

1. A trial of nightshade elimination, perhaps for several months to see what happens. I can give up potatoes and berries. It's the tomatoes and peppers that will prove to be a significant challenge.
2. Switch exclusively to organic produce to avoid pesticides, which can seep into the vegetable or fruit, making rinsing kind of pointless. Of course it depends on what you're talking about. For example, strawberries are on the danger list, but onions are considered safe due to their skin and protective layers.
3. I already know to avoid carbs, but I can't eliminate them altogether because my blood sugar drops (which causes serious problems in itself). I limit them already, but the mashed potatoes were an obvious mistake. Noted.

This isn't going to be easy, considering my diet is already strict, but I will make it happen if it will help. I hope I never forget how miserable I've been since last night...the pain has been unbearable, I've barely been able to move my legs or sit up due to severe weakness, and it has been difficult to breathe. Typing isn't exactly a walk in the park either, but I felt this was too important not to blog about as soon as possible. (My arms are furious, though.)

If you have HKPP and you have eliminated nightshades, I'd love to hear from you about your experience. ugottafriend4life@gmail.com

I'm off to drag myself to the kitchen for more potassium. Thank God my apartment is small and I have a rolling walker. I have been having an incredibly difficult time lately, and this setback has been no help. I missed a family reunion today and I've missed many other events over the last few months thanks to a decline in my condition. A power chair is on my wish list for Santa this year. It's time.

Wednesday, May 18, 2016

Internal Medicine vs Renal Clinic Labs (alternate title: My Body Is An Idiot), & PTSD

These labs were taken only 7 days apart.
Labs are from the same company in 2 different clinics in 2 different states.

Renal Clinic 5/5: Creatinine was normal, WBC was high, sodium was low, potassium was 3.9, phosphorus was low.

Internal Medicine 5/12: Creatinine was high (indicating kidney failure), WBC was normal, sodium was normal, potassium was 4.2, phosphorus was even lower in spite of starting on a supplement.

The potassium doesn't surprise me at all. I purposely took 40 mEq potassium chloride right before my lab draw to see what it was doing. 4.2 is normal, but I function best in the 5s. Unfortunately, I haven't seen the 5 range in years thanks to the progression of my condition.

I upped the sodium a little in my diet, and it worked. It's very easy to go too high or too low where sodium is concerned, so it's a constant roller coaster.

White blood count is back to normal. Most likely, they were slightly elevated (barely, not a big deal) due to a mild UTI that has since resolved. (I'm still sick with my sinuses, but my white blood cells apparently don't care). This is something I keep an eye on for the sole reason that a doctor almost diagnosed me with Leukemia when I was 20. I still remember how scared he was that it was Cancer, and how relieved he was to tell me it wasn't.

Creatinine...I don't even give a damn anymore. If it's going to fluctuate like that every few days, forget it. It's not even worth documenting anymore.

Phosphorus - I went to GNC and bought a supplement, and my levels went DOWN? How's that for some stupid BS. My body is an idiot.

Ok, breathe. Let's take an intermission from this rant fest to give a round of applause to my Magnesium level, which is holding on for dear life in excellent normal range. Good job, Mag.

I had a post-traumatic stress event a few days ago. I won't go into detail publicly, but I'm just mentioning that it happened. This is not quite the same thing as a panic attack, I've learned, as PTSD episodes are the result of something that has happened in the past, as opposed to a panic attack which is defined as having no known cause. It took me by complete surprise, and I'm beyond disappointed that it occurred, but all I can do is continue to be real about my life and do the best I can. I am unable to be medicated due to the serious HKPP symptoms anti-anxiety meds cause, so I'm up a creek where treatment is concerned. I applied for counseling in 2014, back when I had a social worker, but I never received a response to my application. I figure if they're that busy or careless, it's not worth it. Maybe I'll look for other options.

I think we're all a little broken inside. Some, a lot more than others.

C'est la vie, mes amis.

Friday, May 13, 2016

Renal and Internal Medicine Clinics May 2016


I'm not able to sit up and type very well, but I'll do my best here:

I had renal clinic on the 5th. My electrolytes are still lower than they need to be, in spite of heavy supplementation, so things remain concerning on that front. Believe me, they could be a lot worse, but they also need to be a lot better if I'm going to function at my best (which is well below average to begin with). The problem with ion channelopathies is that lab numbers aren't an accurate reflection of how I should be feeling. My potassium can be 3.5 and I'm fully paralyzed, whereas a normal person with potassium level of 3.5 is ok. That really complicates things, and it's hard to explain to a doctor that life isn't that great when my potassium level is 3.8 or my magnesium is 1.5 or my sodium is 134 or my phosphorus is 2.4. The numbers sound acceptable for the average person, but I'm in agony, unable to drive or perform basic household tasks, and barely able to use my limbs. There is nothing standard about this disease.

My renal doctor and I discussed the failure of Keveyis. We are both disappointed that it didn't work out, but I was quick to let her know that A)I'm not the only one who reacted badly to it and B)There are people who are doing much better on the drug. It really boils down to the individual's body and its ability to tolerate sulfonamide diuretics. We already know I barely tolerate Diamox, so with Keveyis being more potent, the risk for me was high. Still worth trying, obviously, as treating this disease involves a lot of trial and error. That's simply the nature of this rare and unpredictable condition.

My kidney function is otherwise stable, and she is "thrilled". She said it's incredible how well I'm managing all of this. I told her I didn't feel like I was handling it (or anything else) well at all, but she disagreed. She said anyone else wouldn't be able to take all of this in, proactively fight with so much knowledge, or know what to do to cope with such a serious disease. Dr. M is a very optimistic person, and I appreciate that.

I also appreciate a friend driving me to and from Jackson. I was ill and would not have been able to make it on my own, so I'm super grateful for the help.

I had internal medicine on the 12th. We discussed my Vitamin D, and I'm waiting for labs to come back to see if I am finally within normal range. Vitamin D toxicity is possible with the average person, and can be detrimental. That doesn't appear to be something I'll ever have to worry about, since 100,000 IU a week still didn't bring me up to par. I'm on 150,000 IU a week now, and have been for a few months. I'll update when I have the results, but Dr. B said this is something I will probably be fighting with for the rest of my life.

In addition to needing follow-up labs (will update on those Monday), I arrived absolutely miserable with upper respiratory mess and urinary tract pain. I even saw blood at one point, which was alarming. I had a mild UTI at renal clinic the week prior, but as of yesterday, it had cleared up. We're pretty sure my UT issues are kidney stones. I told him I've passed over 40 (I stopped counting at 40) and he sympathized. He checked out my vitals, breathing, coughing, and we agreed to keep me away from antibiotics unless I get worse. Antibiotics inhibit neuromuscular transmission, and in my case, always result in life-altering weakness and paralytic episodes. The last time I was on antibiotics, my stomach paralyzed and I couldn't keep anything down. I ended up in the ER twice, and needed daily assistance from relatives for almost two months. Needless to say, we're avoiding the blasted antibiotics if at all possible. He's hoping prescription strength sinus and allergy meds will get the junk out of my head and throat.

Unfortunately, these meds are kicking my butt also. Insert sad trombone here. I can't take OTC sinus and allergy meds without HKPP symptoms, so you can imagine how well prescription strength is going. But the reality is, if I don't get rid of this mess, I'll end up on both these meds AND antibiotics, which would be potentially catastrophic. Yay systemic disease! Thanks for making my life impossible, jerkface.

So I'm laid up in bed, at least for a few days. I'm really hoping this won't linger for weeks this time, so wish me luck.

Saturday, May 7, 2016

Updated Article Link

I finally uploaded my article, The Battle Against Periodic Paralysis, here to the blog. This solves the issue of my article frequently disappearing from various freelance and pdf sites.

The Battle Against Periodic Paralysis

It is also updated in my sidebar. Feel free to share.

I had renal clinic this week, and will have internal medicine next week. I've decided to wait until after that appointment before I give a medical update.

I appreciate my readers. Thanks again for riding this roller coaster with me.

-Kelli

Wednesday, May 4, 2016

Thoughts on Uninformed Judgment of the Handicapped



People who don't understand muscular dystrophy or other disabilities have a hard time wrapping their head around the fact that some patients are capable of independent life, including driving, if provided the resources and support to do so. The uneducated public would look at this man and listen to him speak and assume he's too handicapped, perhaps even mentally impaired, but they would be absolutely wrong.

Muscular Dystrophy can greatly affect cognition (we call it brain fog), just the same as Diabetes can, but the diseases have no effect on overall IQ.

Years ago, I had an acquaintance who responded to the greeting of someone in a wheelchair very awkwardly at a public event. The acquaintance runs a non-profit organization, but said she didn't know whether to acknowledge the lady, citing that she assumed the lady was intellectually disabled because of how she looked. As a disabled person myself, I was pretty horrified by that reaction, and it turned out the lady was independent with a non-profit business of her own!

The point is don't make uninformed assumptions about people who are different from you. The best approach when meeting someone like Ira (or me) is to assume we have the capacity to be standard members of society. If the person turns out to not have that capacity, educate yourself and act accordingly, but at least you didn't treat a perfectly capable individual like they were inadequate. How insulting that would be, and it's something they would never forget.

Thanks for reading.

Tuesday, May 3, 2016

Good News for Periodic Paralysis Patients on Keveyis

Taro contacted me yesterday concerning changes to their sale of Keveyis (dichlorphenamide), and asked that I share with my readers. I am pasting part of the email here. As you may know, when Keveyis was first released to the market, it was listed at over $150 per pill, with patient assistance available to those who qualified. They are now stating that they plan to offer Keveyis to all patients who need it regardless of ability to pay. Please share with everyone you know who is affected by the distribution of Keveyis for Periodic Paralysis. This is good news, and we owe many thanks to Taro for making this drug available at no charge.

---

Taro Pharmaceuticals announced earlier today that it will change its approach to providing Keveyis (dichlorphenamide) to patients.

In the near future Taro will make Keveyis available at no cost to distributors for fulfillment. We are implementing a number of changes to make this possible, including absorbing all costs associated with manufacturing the medicine and halting of all commercial sales and promotional activity. Our press release is available at www.taro.com , please feel free to share it with your social media networks.

Taro brought Keveyis to market for the treatment of periodic paralysis because we thought it was the right thing to do, and we still do. As a voice for the periodic paralysis community, we want to reassure you that Keveyis will remain available. In the near-term, patients will continue to receive their medication through Diplomat as we evaluate best options moving forward.

While there are business-driven reasons for this decision, it remains consistent with our desire to ensure anyone with a prescription has access to the medicine regardless of insurance status or ability to pay.

You and your readers may have questions related to this new structure. I encourage anyone with questions to send them to PeriodicParalysis@taro.com. We will answer every question possible, but please understand that some questions will require more time to answer than others.

---End

Sunday, May 1, 2016

My article on Periodic Paralysis (which keeps disappearing)

I can't keep that article (in the sidebar) online to save my life, can I? Things keep happening to the links. Annoyed is the word of the day.

I've decided to put the entire article directly here on my blog. I'll get to work on that. Just letting my readers know that this will finally be resolved soon.

Thanks!

Wednesday, April 6, 2016

Updates coming soon

I realize I haven't posted in a while. I'm just letting you all know that I'm going to work on the page soon, including the sidebar and medical updates. For now, I'm dealing with a lot of muscle weakness, but trying my best to stay busy in spite of it. Talk to you soon.

Thursday, February 25, 2016

Internal medicine clinic 2/23/16

Internal medicine was this past Tuesday. I had the usual blood work, including Vitamin D, Magnesium, and the basic metabolic panel. I'll go down there and look at results tomorrow. Dr. B doesn't deal with my kidneys and HKPP, but tries to manage anything else that comes along, including some of the symptoms associated with these conditions. This includes my ridiculous digestive system and my heart, which loves to race and palpitate over nothing. It gets especially fussy when I try to do anything productive, like laundry or dishes or walking to the mailbox. And if I'm startled in any way, forget it. I have to lie down.

Conclusion: My heart is a jerk. I'm back on a monitor for a little while, to take a look at things. Gone are the days of the Holter. This monitor hangs out on my torso and communicates wirelessly with an app on a cell device.




The electrodes are standard, and they still suck. My skin hates electrodes, so I'll be bringing out the Vaseline or Neosporin or whatever to deal with that. Not a real complaint. It could be worse.

We also discussed several of my medications. I'm no longer on esomeprazole (NEXIUM) due to its utter failure. Not only did it not work, it greatly aggravated my Gastroparesis and caused significant magnesium absorption issues (as PPIs are known to do), which brought my digestion to a halt as well as triggered new bouts of myotonia (think charlie horses that are prolonged and so severe that they require medical attention...yeah). So this just hasn't been going well.

Once I stopped the med, my acid reflux returned with a vengeance, worse than ever, and I began choking again. This has been in the news quite a bit lately, and to my dismay, I am one of the statistics of which they speak. So it's out, as are the two drugs I took prior to this one, omeprazole (PRILOSEC) and pantoprazole (PROTONIX). I'm now eating calcium carbonate tablets and propping up on four pillows until something else can be figured out.

In spite of stopping the PPI, my digestive system has still not recovered, although it is finally seeing a little bit of improvement now that I've increased my magnesium supplementation. I hope the increase will be temporary, since it's making my stomach and back ache like there's no tomorrow. It's always something.

As for other meds, I'm still fighting with my insurance about covering them. It amazes me how an insurance establishment thinks they have the right to say that I don't need a drug...like they would have a CLUE. Sometimes I want to say, "Ok, I'll stop taking these pills and go back to work, and YOU can have systemic disease. Deal?" Stop trying to kill me, you dummies.

That's the nice version of what I've really been saying. You're welcome.

I'm nowhere close to getting housing in Mississippi where my renal and muscular dystrophy physicians are, so I am really grateful to Dr. B here at my local clinic in Alabama for being so helpful. He has a medication assistance program that works with my insurance, and this has allowed me to obtain many of the medications that have saved and continue to sustain my life (as well as sort of wreck it, but that's the way the ball bounces when your body attacks itself). This is a no-win situation, and everybody involved knows that, but I'd say most of us are doing all we can do to keep me around. I don't take it for granted.

Next appointments: OBGYN in April, MDA and Renal Clinics in May.

Friday, January 29, 2016

20 Things Not To Say Or Do To Someone In A Wheelchair (Article)

20 Things Not To Say Or Do To Someone In A Wheelchair

I don't necessarily agree with all of the responses, but I can relate to some of them and I really can't blame her for being frustrated. I use a walker, I've been in a wheelchair more times than I can recall, and I've experienced much of this. Listen, we get that you don't get it. You're not in a wheelchair and you don't have a clue what it's like to live in someone else's body, especially an individual whose body happens to be permanently broken. But there is so much education out there, and a little self-awareness can go a long way. A nice rule for humanity as a whole is pretty simple: think about what you're saying before it comes out of your mouth. If you're not sure if it will offend, it's best not to say it. If you're tactless and don't care how your words affect others, you deserve a sarcastic response. If you're making an effort to be respectful, it will show, and most likely, the individual will be willing to have a discussion. I am more than happy to talk about my condition, but only if the asker has approached me respectfully and the subject is appropriate. Skepticism, cynicism, and general asshatery is frowned upon with a vengeance, as you can see by the jaded author of this article.

Some of these things are honestly ridiculous, but they really do happen.

Stealing a handicapped parking place? If you don't have a valid permit in your name or the name of your passenger, you don't have the right. There are NO exceptions. And while I'm at it, handicapped parking spaces are NOT! YOUR! SHOPPING! CART! RETURN!!!!!!!!!!!!!!!!!!!!! If my crippled backside can put my shopping cart where it belongs, your lazy backside can do the same.

Speaking to the person accompanying me, about me, instead of addressing me? You're assuming that I'm deaf or dumb, apparently, and you would be wrong. As for grabbing someone's wheelchair and pushing them without permission, it is never ever ever okay. Never. I don't know why anybody would ever think it is acceptable to do such a thing. Don't be stupid. It is violating. And don't even get me started on the disability memes. I'm sure you already know that I find such things humorless and in terrible taste.

This is a big world and there are an awful lot of people with an awful lot of varying abilities and conditions. Be mindful, otherwise you're, well, mindless. No-brainer, right? Pun intended. The bottom line is that these things matter, so on behalf of those who live with disabilities, thank you for your consideration.

Thursday, January 28, 2016

January Medical Update

I saw my new eye doctor on the 26th. He was outstanding, and I intend to keep him forever. My eyes haven't worsened, thankfully. I'm still bat-blind without lenses of course, but I see pretty well with them (about 20/50). My eye muscles are weak, which could be contributing to not seeing 20/20, or I could have permanent damage from the scratched corneas I obtained about 14 years ago. There's really no way to know for sure, I guess. I have a script for Restasis for chronic dry eye, but I have to wait to find out if it's safe to use (steroids are a dangerous no-no with HKPP, but eye drops may not be an issue...we'll see). I picked out new purple glasses, and I will begin a contact lens trial after my current migraine resolves.

Next up is Internal Medicine clinic, which is in February. I'll have bloodwork to look at my BMP and kidney function again, but I don't anticipate any other drama (famous last words, knowing me). I pushed MDA and Renal clinics to May 3rd through 5th, but as it stands right now, I am neither physically nor financially able to go to Jackson. If this changes by May, I'll go. If not, I'll have to cancel until I see improvement in these areas. I don't have a choice.

Winter is very hard on me, and I look forward to warmer, less painful, more functional days. In spite of how difficult 2016 has been so far, I'm counting my blessings.

Monday, January 25, 2016

I'm not dead.

I know I haven't posted in a while, and I apologize. I've had so much going on, including recovering from a really bad HKPP episode in December. I'll elaborate at another time. Just letting you know I'm still here. I hope everyone has a happy and healthy 2016. Talk to you soon.

Wednesday, November 18, 2015

MDA's NOLA Clinic

Yesterday, I went to my first appointment at MDA of Southeast Louisiana's clinic at LSU New Orleans.

It started off great, but unfortunately ended in absolute shame.

The physician doesn't deserve another minute of attention from me, so I'm sharing the link to my Facebook post about it. https://www.facebook.com/ugottafriend/posts/10153103655125740?pnref=story

I'm returning to Mississippi MDA next year to discuss future services, assuming I am able to make the trip to Jackson. If I can't, I will just have to survive without the MDA.

Never making that mistake again. Periodic Paralysis patients, I advise avoiding NOLA like the plague unless you're only there to eat gumbo.


Phone call with Taro

I spoke with Taro's Health & Safety department concerning the ‪HKPP‬ episode(s) that occurred on ‪‎Keveyis‬. They asked questions and recorded my answers to send to the FDA. Unless anything changes, my use of the drug is permanently discontinued and this will conclude my documentation on it.

I have resumed acetazolamide (DIAMOX) with mixed results, as my body is acting like it had never taken it before, so it is having to adjust to the initial side effects all over again. My PRN fast-acting potassium bicarbonate (EFFER-K) has been increased during this time and is making a difference.

I have other things to share. Talk to you soon.


Sunday, November 1, 2015

My experience with Keveyis for Primary Hypokalemic Periodic Paralysis

I'm here. Sorry for the delay. It's been an interesting couple of weeks.


I received Keveyis within 24 hours of my doctor signing the script for it. The mail-order pharmacy who handles this transaction is very efficient, and also great at communication.

Before I proceed, I want to make it clear that I am sharing only my personal experience with this drug. As a patient with severe Primary Hypokalemic Periodic Paralysis, I knew things could go one of three ways: no change, more stamina, or crash and burn.

I am sad to say I fall into the latter category.

With my first dose, I was flat on my back in an episode. Nothing else caused it. I am 100% sure it was the drug. I opted not to take the second dose on the same day, and instead waited until the next day to try to recover a little before trying again. The next day, weaker than usual, I decided to take half a dose of Keveyis along with my potassium script. I became weaker, although not paralyzed like the day prior, but too weak to function. I spent the day drinking my emergency stash of liquid potassium. Sometime in the middle of the night, I decided to make another effort with both my slow K+ and fast liquid K+ accompanying a fraction of a dose of Keveyis. Things continued to go downhill. I went into tachycardia, and ended up flat on my back again, barely able to make it down the hallway to my bed. At that point, I knew it was time to discontinue the medication until I spoke with my doctor. The side effects of these episodes lingered for much longer than they should have. I remained very weak for 10 days, and during that time, I was too easily affected by triggers around me. Lights, sounds, smells, foods, the least little movement, and adrenaline release from being startled (by a huge bug, if you're curious) were all a bigger problem than usual. All of the above is a clear sign that my potassium was far too low, and it took a great deal of potassium and rest over a period of a week to bring me out of it. I began Keveyis on October 20th. I was up and around and able to drive a little bit again on October 30th.

Why I took more than one dose:

People who don't have this condition may wonder why I made three attempts. The reason for that is simple: the same thing happened to me with Diamox (acetazolamide), and after many adjustments to the medication, I eventually found a (very small) dose that worked. Albeit, the side effects are terrible, but the tiny amount I'm on with accompanying potassium and potassium-sparing diuretics has made a pretty big difference in the quantity and severity of my episodes. I used to paralyze every day, folks. Every. Single. Day. And while I remain very weak (which doctors have said is permanent), my serious, full-blown paralytic episodes have reduced significantly on Diamox. It's miserable, but God bless it for giving me a thread of quality of life again.

Keveyis is a more potent drug, so I knew it would be risky to switch to it, but the hope was that it would work better and give me more quality of life with fewer side effects. When you're weak and crippled in your 30s, live alone, and you're fighting daily to be as independent as possible, you take these kind of risks. I'm sure I don't have to tell you how disappointed I am that things went so badly. It's pretty devastating. Improvement was absolutely possible, but with my body being as weak as it is, and as hypersensitive as my body is to the least little drop in potassium (including in normal range...I have episodes in the 4s all the time), my unfortunate results with this powerful drug is not a surprise.

Why this class of drug is effective in treating Periodic Paralysis:

Keveyis and Diamox are carbonic anhydrase inhibitors. CAIs act as diuretics while also slowing the release of insulin. Both of these actions are important. In HKPP (HypoK, which is me), episodes are triggered by excess sodium. CAIs cause sodium to exit the body, thus preventing those episodes. Episodes are also caused by insulin releasing into the blood stream, pushing glucose and potassium out of the blood stream and into the muscle cells, resulting in depolarization (paralysis). It makes sense to say that the faster and more extreme the release of insulin, the worse the episode could be. Therefore, a drug that slows down the release of insulin is inevitably helpful to someone with HKPP, assuming the patient is taking potassium to keep their blood levels up. Which brings me to the Catch-22 in this thing: in addition to ridding the body of excess sodium, CAIs also waste potassium, which is big trouble for HKPP. Supplementation is necessary in order to prevent a decline, which is what happened to me. I'm already on a TON of potassium, both slow and fast K+, but it wasn't enough to compensate for the fall. I am still waiting for my doctor to respond to my phone call concerning the possibility of higher doses of K+. Most likely, this isn't going to be an option.

I refer to insulin and sodium as bullies on the playground. If the bullies run slower than you, you won't get pushed in the mud. :) Make sense?

On the other hand, if you have HYPP (HyperKPP, meaning episodes caused by too much potassium in the blood), CAIs help because they are potassium-wasting. Someone with HYPP would in turn eat salt and sugar to maintain balance, because they need sodium and insulin working with them to push potassium out of the blood stream. It's a little more straightforward than the HKPP situation, but success isn't guaranteed at any rate.

I hope this helps to explain what Keveyis' job was, and why it didn't work for me.

Again, let me be absolutely clear on this: The drug works for some patients. Not just a little, but very well. I've heard from people who said it's a dream come true and that life is better now. I am positively thrilled for every one of them, and hope they continue to thrive on Keveyis. It is very important that they are able to access this absurdly expensive drug, and I'm glad to know that Taro Pharmaceuticals has such an extensive assistance program designed to help Periodic Paralysis patients obtain the medication. I am relieved on behalf of my friends who suffer with this condition, and I will continue to support them and talk to anyone who needs to know about the possibilities of a better life with Keveyis.

Thanks for reading. Email me if you have any questions, with "Keveyis" in the subject line.

Wednesday, October 21, 2015

Renal and Internal Medicine Clinics (Part 2)




I don't think people understand just how much any kind of physical activity can knock an HKPP patient flat. What's remarkable is at times it is a delayed reaction, so I can get through a day of heavy activity such as traveling to Jackson for renal clinic (an 8 hour round trip), but my worst moments are the following days. If you're familiar with HKPP, you know this trigger is called "rest after exercise", and it's a doozy. It also never helps when it's insanely hot outside and insanely cold inside, which was the case here. Fluctuations in temperature can result in serious paralytic episodes. I know because I've had them. This condition is painful, frustrating, and complicated, and I don't always cope well, but I try. I am very lucky to have some wonderful friends on Facebook and elsewhere who follow my journey and keep me encouraged with their love, prayers, and well wishes. I'm so grateful to them all.

Next renal clinic is in March, if I can find the funds somehow. The hospital has informed me that I am not eligible for financial aid now that I live where I do (recently moved), so I might be in trouble. If I absolutely cannot handle the cost, I will discuss my options with Dr. M. I hope something will work out so I can continue to go to Jackson.

I'm still having a hard time keeping my potassium level in an acceptable range. When a trigger occurs, and it does so often, it isn't always easy to bounce back to my version of "normal". The rest of my numbers look pretty good right now with the exception of Creatinine, which is slightly elevated again, and Vitamin D, which is still too low in spite of being on a whopping 100,000 IU a week. For the love of God, what is it going to take? Dr. B is increasing my dosage to 150,000 IU a week, which is rare and risky, but I've got to get those numbers up somehow. We'll see what happens.

The great news is that I don't have Hashimoto's Thyroiditis. I know several people with HKPP who have it, and it would have explained some of my symptoms, including the low D, but my numbers were normal. Renal clinic in Jackson may have very well done these same tests, but I don't recall, so I wanted to make sure to rule it out. I'm glad I don't have to think about it anymore.

I have a Keveyis update. It will be posted in the next couple of days.

Tuesday, October 13, 2015

Internal Medicine 10/13/15 (part 1)

I went to see Dr. B this afternoon to follow up from the drama that ensued back in June.



I've lost some weight. More to go, but I was surprised and relieved that I had not gained.

My potassium remains on the low-normal side, in spite of my heavy supplementation. It could be worse, but I really do need it to improve somehow.

My creatinine was high once again. It keeps fluctuating between normal and high. We have no idea why this is happening, but it seems to be the nature of my kidneys. Seventeen years ago, I was crippled in the hospital, and the doctor told me that my kidneys are frustrated. In fact, he documented it as whatever "kidney frustration" is in German. What it boils down to is that my kidneys are working harder than average to try to be normal, and they get exhausted sometimes (as does the rest of me). My electrolytes and other substances, such as hormones and blood sugar, also fluctuate quite a bit. I've come to the conclusion that it's all related to the fact that I have a channelopathy, but I'm no medical expert (just an expert patient).

I feel like I'm getting a sinus infection, but I am unable to safely take antibiotics or antihistamines, so I was given samples of Deconex DMX in hopes to keep the issue at bay. I'm pretty miserable at the moment. Wish me luck.

My Vitamin D, which stays consistently low, was checked. The results should be in tomorrow. We talked about the fact that I have a history of pulled and torn cartilage and skeletal muscle, which points to Osteomalacia (adult Rickets, caused by chronically low Vitamin D). I'm on a very, very high amount of prescription D, but he said he'll raise it even more if we have to. My body clearly has some sort of problem with absorption.

We talked about my digestive system, which has always been pretty lousy. I have a hernia, severe acid reflux disease, and Gastroparesis. All of the above are painful and problematic. I'm going to increase my over the counter magnesium intake to see if that makes a difference. Surgery is out of the question thanks to my high risk of death under anesthesia.

Doc and I agreed to a complete thyroid panel, which will look for peroxidase and thyroglobulin antibodies. This test will rule out Hashimoto's and other thyroid conditions once and for all. I'll have results in two days.

Thus the reason why this is part one of two. I'll update with the results later this week.

No progress on obtaining Keveyis. I've left a voice mail for the nurse to follow up.

Friday, September 18, 2015

Obtaining Keveyis for Periodic Paralysis (Video Blog)






Keveyis.com to sign up for updates and print out prescribing info for your doctor if necessary

Keys2Care 1-855-611-3401
Patient assistance - call them no matter what your income is

I will blog my experience in detail on this blog.

P.S. - Beware of volume...I didn't realize I was so loud.

Sunday, September 13, 2015

August clinic and other ramblings (part one?)

This is me after learning that the best doc in the world
has moved back to Hungary.

Sorry for the major delay in blogging. I've had a pretty severe case of writer's block this year, and it doesn't seem to be resolving anytime soon. Sleep deprivation/exhaustion is probably the biggest factor. Perhaps a bit of depression as well, which has been an issue this year. I am rarely lonely, but something about moving to my own place triggered some unexpected feelings of crash and burn. Like I've hit a brick wall. This is it...this is the dead end and I really am going to die alone in poverty. Pffffth...I don't know. I'm just thinking out loud, so to speak, in an attempt to process the last few years (or the last twenty, for that matter) and where it has brought me. I'm dealing with it.

Anyway, enough of that.

Renal clinic was August 27th, finally. My doctor and I had both bumped the appointment so many times, it had been 9 or 10 months since my last visit. I met with a couple of new doctors (one isn't new, only new to me), and it was then that I was informed that Dr. Fulop was leaving the country. My response was "I'm heartbroken!" and then I told them how incredibly helpful and wonderful he has been to me. The man practically brought me back from the dead, and I will always love and appreciate him for all that he has done for me.

My labs looked better than the ones I had at Internal Medicine clinic in June. I'm still having a very difficult time keeping my potassium at a reasonable level for someone with my condition. I need to be in the high 4s, if not low 5s, to function at my best. I was in the 3s at clinic, which is the danger zone. Sounds crazy to a normal person, but for someone with a channelopathy, it can be detrimental. I have been known to fully paralyze with potassium levels in the 3s, to the point of life-threatening. That said, I have some work to do in getting my K up to where it needs to be. As for the rest, my kidneys were back in normal range. They tend to fall into "uh oh" mode on a frequent basis, as this has happened several times over the years - even in my early 20s, if I recall - but they always bounce back somehow. My Internist in June was very concerned with my numbers, but Renal Clinic in August was quite happy with what they were seeing, so that was good news. It's something we'll have to keep a close eye on, but as usual they consider my biggest problem by far to be Muscular Dystrophy. Needless to say, I agree. They scheduled my next clinic for March, and I had a good ugly-cry on the way home over losing the best Nephrologist on planet Earth. God bless Dr. Fulop. I would have given him a hug and wished him well had I been given the opportunity.

Other labs included sodium at borderline low, which is something I have to be pretty cautious about, especially being on so many medications. I've been Hyponatremic before, and it's no better than being Hypokalemic...trust me on that. You don't want it. Yet taking in average amounts of sodium in my diet is too much because it triggers episodes...so go figure. It is very challenging to try to find a balance between not enough sodium and too much. Same with sugar. Both Hypo and Hyper result in serious symptoms. Balance seems to be the name of the game with this condition, and it's a hell of a lot easier said than done, that's for sure. With paralytic triggers all around, it is a constant battle. One I'd really like a vacation from every once in a while.

My Vitamin D wasn't checked. That will be done in October when I return to Internal Medicine Clinic. I did see Dr. B recently for a quick vitals check (heart rate, BP, blood sugar) and all were great. He joked that I was his best patient, ha! I'm a wreck and he knows it. He's funny, though.

I'm having a terrible time with sleep deprivation and chronic pain. I don't even have words for this right now. I'll talk about it in another post. I have more to share, and I will do that soon (really...I will. I promise).

Wednesday, August 26, 2015

I Have Systemic Disease - An Open Letter of Awareness



I have systemic disease. That means every system of my body is broken in some way. I live with muscular dystrophy, chronic kidney disease, migraines, obstructive sleep apnea, sleep deprivation, musculoskeletal disease, depression, chronic fatigue, severe chronic pain that I never escape, and more.

It's terminal and irreversible, no matter what you think.
I'm not lazy or crazy, no matter what you think.
It has no effect on intelligence, no matter what you think.
I may be tired, but I'm not dumb, no matter what you think.

The next person to tell me to try harder or be positive, call me names, judge my life choices, or look at me like I'm some kind of freak may never fully recover from my reply. That sounds harsh, but I have to do whatever is necessary to protect myself. It took over 30 years to grow a backbone, and nobody's words, actions, or assumptions will take it away from me. I'm still a pushover at times, but I'm figuring this out as best as I can. I'm practically Bambi at birth, both physically and otherwise, awkwardly trying to keep my legs underneath me. It may seem ridiculous to you, but I'm going to stand even if I'm the only one standing.

I thrive in solitude, but I also admit that it is very hard doing this alone, and I am forever grateful to those who are standing with me. Not just today, or this week, or when I'm having a decent day, or when I'm agreeable. The turbulent times of unbearable pain, exhaustion, loneliness, grieving, and anger are when the strength, care, and grace of others are needed most, and I am so thankful for those who rise to the challenge. You don't have to, but you choose to, and I don't take it for granted.

We have awareness days and awareness weeks and awareness ribbons and awareness marathons, and that's all nice. But at the end of the day when the awareness ends, the battle still rages every minute of every day, forever. Those who suffer from chronic illness didn't sign up for this war. There is no pay and there are no benefits. There are no coffee breaks, no vacations, no "back to normal". This is normal. Sorry for the inconvenience, healthy folk, but this is it. No matter what you think. But in spite of our unfortunate high maintenance, I believe most of us don't expect miracles, want special treatment, or demand the unreasonable. I believe most of us only ask for the same kindness, consideration, and respect that anyone else does. Our bodies may have changed, but our value hasn't changed. Last time I checked, broken crayons still color the same.

I wanted to share all of this today in hopes of encouraging the cheering section to keep cheering, the caregivers to keep caring, and most of all, the warriors to keep fighting. Together, we can do this thing.

I love you all, even when I'm cranky. I love you all even when you're cranky too. Now let's group hug and kick this day's ass.



Thursday, August 13, 2015

Big News for HKPP Patients: FDA Approves dichlorphenamide (KEVEYIS) for treatment of Primary Periodic Paralyses

I am so behind on everything, I am just now getting to this news:

Earlier this week, the FDA approved carbonic anhydrase inhibitor dichlorphenamide (KEVEYIS) for the treatment of all forms of Primary (Familial) Periodic Paralysis.

Thanks to the Muscular Dystrophy Association​, other Periodic Paralysis researchers, and study participants who made this possible.

Drug website is here: http://www.keveyis.com/

Drug info is here: http://sacdzn.com/clients/cambridge/taro/FINAL%20Approved%20Keveyis%20PI_%208.7.15.pdf

MDA Press Release is here: https://www.mda.org/media/press-releases/fda-approves-drug-primary-hyperkalemic-and-hypokalemic-periodic-paralysis

As for me, I am already on a different carbonic anhydrase inhibitor, acetazolamide (DIAMOX), but I will be discussing Keveyis with my doctors in a few weeks. My hope is that insurance will cover it and it will give me better function with fewer side effects.

I'll keep you posted.

Wednesday, June 3, 2015

June 2015 MDA Clinic Update, Courtesy of Twitter

For those who are unaware, I go to an MDA clinic twice a year for assessment of my Familial Periodic Paralysis. The team is very nice and they have learned a lot about the disease due to my extensive history and information that is thankfully now easily accessible online. It has been a long road, but they are trying and so am I. I'm grateful they are there for me. I know others haven't been as lucky. If you are still looking for help and are able to travel, you can find them at MDA of Mississippi at University of Mississippi Medical Center. Prepare to prove you have the disease via repeated consults, tests, and/or medication which may or may not be risky.

I documented my clinic appointment on Twitter yesterday:



2011 was a horrendous year that I still can't believe I survived.
2012 landed me in Telemetry unable to swallow, so it wasn't much better.


Possibly due to the heat + driving 4 hours to clinic.
Will keep an eye on things.



The CPAP is a fail, by the way. I'll explain later.




My right leg was bent at 36 degrees for over a year.
It's almost straight now (around 4 degrees) thanks to a Dynasplint.
I still have permanent damage, including nerve damage to my 
quads and a wide area surrounding my patella. Yet...




I wish I could make people understand just how laboring it is to take a full breath.
This sucks. I have no other way to put it. It's painful and difficult and it sucks.

I'm on a high-risk cocktail of medications
that come with their own delightful side effects.
The delightful part is sarcasm, if you weren't sure.
I hate all these damn pills, but they're the reason I'm alive.


Translation: Dr. F at Renal Clinic who is prescribing the meds
is doing everything possible. They are keeping tabs on my treatment 
there and offering whatever support they can think of beyond that.

Due to the fact that it is difficult for me to 
make the commute to Jackson 4 times a year, 
I asked to be transferred to MDA 
in Louisiana, which is half the distance. 
I will still go to Renal Clinic in Jackson.

This is a joke.

The hashtag - also a joke.



The next day...







My day did not go well, and I ended up 
back in bed sicker and weaker than I started.
Major fail.


I don't always flood Twitter with HKPP updates, but feel free to follow there if you like. I do bring awareness at times and it would be nice to have other folks there who get it. I appreciate those who already follow quietly. You know who you are.

Sadly, Renal Clinic is going to have to be rescheduled due to my car trouble. I'll update when I manage to finally make it back to Jackson.