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.

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.