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.


Tuesday, April 21, 2015

Another short sleep update

My experience with pressurized oxygen is going like this:

Confession: I am known to remove any article on my body in my sleep if it is making me uncomfortable...sock, shirt, whatever (go ahead, laugh. You have my permission). That said, the fact that I keep waking up within two hours with the mask in my hand instead of on my face is not a surprise. It IS a problem, needless to say. I did go to the doctor and have the pressure increased, and I had to reduce the humidity significantly, but I'm not sleeping through the night any better than I was. Dr. H and I are both disappointed, but hoping I'll get used to it over time.

One thing I will also mention is that I had a paralytic episode last night and was having trouble breathing. I put on the cpap and it seemed to help a little. Take that info for what it's worth.

MDA and Renal clinics were rescheduled to June 2nd.

(edit: renal clinic was rescheduled again to June 10th)

(edit: renal clinic was rescheduled yet again to late August)

Friday, March 27, 2015

Sleep Woes

The CPAP has been overwhelmingly unsuccessful thus far. At best, it will prevent death by sleep apnea because the oxygen is being pushed into my nose, but I'm sleeping no more than I have been. In fact, I have times when I give up and take the mask off my face because I can't stand it anymore.

I've heard from plenty of people that this is common. A friend from overseas who has Periodic Paralysis and is also on a CPAP suggested I hang in there and give myself time to adjust to it, even if that means months down the road. I have no intention of quitting the thing, but it's not helping me to fall asleep or stay asleep, and I feel defeated.

I'm tired of feeling less than human. I feel like I don't have an ounce of strength or sanity left at times, yet I surprise myself by making it through another day. I don't know how I'm doing it. I'm absolutely convinced that when I do keel over someday, it will be from sleep deprivation, not muscular dystrophy.

Edit Note 3/30: I saw my doctor today. The study results showed that my breathing was compromised 79 times in the 3 hours I was asleep. We're disappointed that I'm not sleeping better on the cpap so far, but we agreed to increase the pressure to see if that will help.

Wednesday, March 4, 2015

CPAP

Really appreciate the folks who donated this equipment to me. I have no idea what I would have done otherwise.

I'll give a review of my sleeping habits in a few weeks.

Thursday, February 26, 2015

Sleep Study

I had two long-overdue pulmonary sleep studies in January and February. It confirmed what my doctors and I already believed - I tested positive for apnea. I slept very little the first night, which is the norm for me. I was diagnosed with insomnia and sleep deprivation with sleep apnea. Interestingly, I wasn't diagnosed with restless legs to my knowledge, although I haven't followed up with the doc to discuss everything in detail yet, so it's still a possibility. The second night, I was on oxygen and it seemed to help me breathe better. You're sort of forced to breathe whether you feel like you can take the breath or not...you don't get a choice on pressurized oxygen, which is the point. I didn't fall asleep any faster or sleep too much longer (5 hours the 2nd night verses 3 the 1st), but it's certainly a start. Something tells me I'll start sleeping a little better once I'm used to the mask and not hooked up to 25 electrodes like a science experiment, assuming I'm not getting up every other hour to go to the restroom like I often do thanks to my meds. We'll see. I'll meet with a CPAP company next week to discuss equipment.

Sorry for my hiatus, by the way. A great deal has happened over the last few months. Had another death in the family. I've moved to another city, still trying to get settled. Respiratory problems with the new place that had to be addressed. Fought the flu and a sinus infection...still can't seem to shake the annoying cough. Weaker than usual. Very stressful time, but I'm alive and kicking. Thanks for the emails checking on me. I really appreciate your concern.

I have no internet at the new place, and won't for a while longer. The offer happened very quickly, and while I am so relieved to finally be in my own apartment, it's unfortunately not in the right state where medical care is concerned. It will have to do for now, and I've decided to stay on other waiting lists in hopes of being where I need to be to get the best possible care for this condition. Perhaps Medicaid will someday be allowed to cross state lines, but I'm not holding my breath. Pun intended.

Next appointments are MDA and Renal clinics in April.