Monday, July 31, 2017

Strongbridge Biopharma Conference Call Re: Keveyis

The link below offers people the opportunity to listen to a conference call at Strongbridge Biopharma in reference to the drug dichlorphenamide (KEVEYIS). If you didn't hear, Strongbridge acquired Keveyis from Taro Pharmaceuticals about six months ago. I haven't talked about it since my posts in 2016, but I've been following along quietly.

I will try to remember to listen in, and share anything of relevance here.

I've fumbled miserably on emails, and I apologize. I've been absent from the support groups even longer. I get tired, and go through periods of time when I simply cannot stand to talk about it anymore. If only it were possible to take a vacation from the disease itself.

At any rate, if you want to leave comments or questions, feel free. I'll do what I can, even if that means directing you to someone else. Sleep deprivation has turned me into an exhausted, absent-minded mess, but I'm still here...sort of.

Talk to you soon.


I'm back. I was going to compile a detailed summary of the conference call, but I ran across this fantastic breakdown from Yahoo Finance. It is more than sufficient.

Tuesday, July 25, 2017

"You Don't Look Sick"

To be honest, I think some people mean it as a compliment. This comic, of course, refers to skepticism. Don't expect HKPP patients to take it kindly. It's nice to be cautious about saying things that can be taken out of context. It may help to realize that people who are chronically ill have been through a lot and that we may feel like you're being rude even if it's not your intention.

I wouldn't feel the need to say this if it wasn't a real issue.

"You don't look sick."
"Never heard of it. Is it REAL?"
"Well what are you doing to fix it?"
"You're going to have to try harder."
"You need to try. You need to really try."
"It sounds like you're making excuses."
"Just trying to help. I wouldn't just GIVE UP."
And the ever-popular "think positive" and "pray more".

I've heard every bit of it, and even more nonsense than that.

A good rule of thumb is that if you're not sure whether or not you should say something, it's probably best not to say it. But let's face it - many of these folks are too stuck on themselves to believe they could be wrong, and those kind of people love going around trying to fix or degrade others. Patients, don't be afraid to stand up for yourself. Sometimes, that's the only way to be respected.

Wednesday, June 28, 2017

Internal Medicine June 2017

I arrived to Internal Medicine clinic feeling like death. I had the usual labs, plus a few extra. I'm waiting on the extra results, but my stat labs looked good with the exception of my chloride levels being too high. That is undoubtedly due to being on so much potassium chloride. Dr. B didn't seem concerned about it; he was just glad to see everything else in normal range again, including my kidney function which has improved significantly on my current medication regimen. He noted amiloride, and I agree it is the drug that brought my filter rate out of the danger zone.

Much of my family is diabetic, but my blood sugar is a champ in spite of being overweight. I haven't reached the point of "obese" and hopefully won't, but it is a constant worry with failing muscles and a protein/fat diet. It is more frustrating than I can express that I cannot exercise without catastrophe. My doctor is less worried about my weight than I am, and he circled my blood sugar as if to say "look at this fabulous number", so it was good news overall.

The two biggest problems right now is the muscular dystrophy and my incredibly antagonistic digestive system. I'm a wreck head to toe with weakness and severe pain. My bowels are at a halt most days. I can't lie down without choking. I feel like I'm on fire, and I feel like I'm smothering. My acid reflux disease is uncontrolled even with the most aggressive prescription-strength medications - I have tried nearly all of them. Dr. B has tested me for digestive cancer in the past, and he said he will probably do another h.pylori urea breathing test in a couple of weeks. Erosive esophagitis and gastroparesis can be serious in a variety of ways. We decided to try Protonix and Amitiza again to see if things improve. If I don't see results in two weeks, he wants me back in the lab. So far, the meds aren't working.

He didn't send the social worker in to ask me questions this time, and I'm glad. I was feeling badly enough as it is, and having a hard time keeping it together through the nurse's repeated mistakes with my pharmacy. Thankfully, I made it out of there without getting too angry. Some days, I feel like I handle things pretty well...above average. Other days, I definitely don't. Not even close. Last time I was in the hospital (2014), the doctor told me I was acting completely normal concerning my circumstances, including my moments of frustration and cursing, so I'm trying not to overanalyze my ability to cope, or lack thereof. It's a 24/7/365 job just to survive and perform basic functions of living. I honestly want no part of it, but I'm here doing the best I can.

I'll update my medication list in the sidebar soon.

Next appointments: Probably testing for h.pylori in two weeks, but otherwise Internal Medicine and Muscular Dystrophy clinic are in October

Edit Note 6/29/17: Send-off tests came back. I need to continue my high dosage of Vitamin D, as 150,000 IU a week is only bringing me into borderline low-normal range. Magnesium and Phosphorus were fine.

Wednesday, March 1, 2017

Back To Back Clinics

On February 20th, I returned to UMMC in Jackson for renal/endocrinology clinic. I am very, very lucky to have doctors who listen, care, and want to learn more about this absurd disease. It is assumed that my current condition is my best case scenario, and they are relieved that things haven't gotten any worse over the last several months. We agreed that the Keveyis fiasco was a bummer. Since my HKPP is familial, they asked about my parents and whether or not I have children. I reminded them that I am the lone ranger in this thing, my parents are deceased, and I don't have kids. As always, they are interested in my story and are happy to know Facebook support groups exist. Documentation is a big concern for me since social security owns me and will occasionally review my case, but the doctors reassured me that they were keeping up with my peril in writing. They said my labs look stable, and the glass is half full in spite of my condition. I'll return in one year unless something changes.

On the 22nd, I returned to Internal Medicine at the low income clinic in my little town. Dr. B is fantastic as always, and is willing to test anything that is even mildly questionable. He knows how frustrated I am over my permanent muscle weakness and that even with my potassium level in normal range, I cannot function well enough to perform basic household tasks at times. We discussed my episodes, and how I handle them without assistance (answer: I take potassium and lie down...all I can do really is ride it out. If I am unable to take potassium, I find the nearest place to lie down and I hope for the best). He was concerned about the fact that I live alone like this, and suggested I check into obtaining a Life Alert necklace. I probably won't be able to afford it, but I'll look it up. He told me to make sure I have my cell phone within reach at all times.

He took a stat panel like renal clinic did, and the numbers matched this time. Everything looks pretty good, thanks to my overwhelming daily regimen, and it is an improvement compared to the last couple of years of labs. He sent some blood off for additional tests, and I'll receive the results on Friday. I'll write a short update at that time.

Renal clinic is across the state line, and because my insurance refuses to acknowledge that, I am forced to pay in full to be seen there. It's very difficult - nearly impossible - to afford that but I didn't find a clinic in my state that would take my case. That's how I ended up having to travel 4 hours to an out-of-state hospital. They practically brought me back from the dead, and want to continue seeing me at least once a year (I want/need to see them as well), so I am going to have to find a way to pay for it. I explained this to Dr. B and said he would do what he could to help concerning labs or whatever it is they want to monitor in me throughout the year in between my visits there. He also has a medication assistance program, and it is the only reason I have access to my vital medications (which would be around $1000 a month otherwise and my social security is far less than that per month). Low income clinics are so, so important; in fact, downright life-saving for people like me. That's why federal funding for these places must be protected. To cut off access to medical care is to literally kill people. I would be one of them, but only one of millions. God help us.

As long as nothing crazier than usual happens, and as long as my sent-off tests come back in good shape, I am done with clinics until June.

Monday, January 23, 2017


The fact that there are people out there who think they have the answer to my ailments, and that I am simply choosing not to comply - as if systemic disease is curable with your magic potions and notions - but for some insane reason I would rather be seriously ill, in severe pain, and debilitated every day of my life - is maddening beyond words.

Go take several seats. You know nothing.