Saturday, January 19, 2019

Things Disabled People Know

An important conversation happened on Twitter overnight at the hashtag #ThingsDisabledPeopleKnow.

I highly recommend spending some time there.

Wednesday, January 2, 2019

These books are important.

This has been my life since childhood.

I was neglected, falsely accused, mocked, shunned, misdiagnosed, abused, and refused treatment because I was a woman who hadn't achieved my doctors' sexist ideals.

I suffered greatly. The damage was done and is now irreversible.

This is my story and countless others'.

People love to victim-blame and make ableist statements in an attempt to discount someone's injustice, especially in regards to their health, safety, and quality of life. The fact of the matter is, this is my truth, and nothing you say will make it not true. My one life, the only one I will ever have, has been permanently impaired by a patriarchal society.

You don't get to tell me how to feel about that, no matter who you are.

I hope women will continue to speak out. It may be too late for many of us, but, God willing, future generations of women will benefit from our horror stories as some doctors learn how to treat them with the respect and care they deserve.

Friday, October 12, 2018

MDA clinic 10/2/18

On October 2nd, I was back in Jackson for Muscular Dystrophy clinic. They had gone through some changes since I was last there, including losing the director, Dr. V, and my nurse practitioner, Cookie, who was leaving that very week. Both of these people were a very important part of my story and my medical history, and it has been unnerving to lose them. Speaking of medical history, an MDA rep came into the room and informed me that I was no longer in their system, as my files had been inadvertently purged...for the second time.

TO ME!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

Breathe, Kelli. Don't yell at anybody. Just breathe and remind them how vital documentation is since I am owned by Social Security and will end up broke, homeless, and/or dead if they don't keep detailed paragraphs about my disability readily available for the rest of my stupid life. SO STOP THROWING IT AWAY!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

So we had to start over. I met a nice new nurse practitioner, Beverly. Needless to say, there was a lot to talk about. She allowed me to address everything on my list while she wrote notes. She brought up the free genetic testing through Invitae, and said she would talk to someone about pursuing it. It's free, and I'm poor, so we agreed that it would be crazy not to make it happen.

I informed her of the newly-found gene, P1158S, and the fact that I have both paralytic episodes and myotonic episodes, as that particular gene mutation refers to. There's no guarantee it's the one, and there's no guarantee that I'll find my gene mutation to begin with. A high percentage of Periodic Paralysis patients aren't genetically confirmed, but prove the condition, as I have for years. At any rate, I hope I can be tested for this one, specifically, someday.

I talked about my episodes, and the Gastroparesis that has absolutely wrecked my body and my diet. I also brought up tinnitus, migraines, chronic pain, sleep disorders, balance problems, heart palpitations and dizziness over the least little activity, the fact that I can't frequently handle basic tasks like grocery shopping anymore, and the possibility of Dysautonomia and/or Ehlers-Danlos, since these are common accompanying diagnoses with Primary Periodic Paralysis (a LOT of people in my support group have all of the above conditions). She wrote it all down, but I don't know if it will be pursued. I'm uninsured, so they avoid labs and diagnostics at this particular clinic.

I made sure they knew about the conference that happened in Dublin this year, as well as renal clinic's interest and possible conference or interview or whatever it is that Dr. C was planning to do to, in his words, bring awareness. He's busy, and I'm not going to bother him about it. He's welcome to contact me whenever he wants to. I'm just thankful doctors are listening instead of being jerks, but with or without me, any attention Primary Periodic Paralysis receives is a good thing. I'm just another face in a crowd of hundreds, possibly thousands, who are living this nightmare.

I'll return in one year to continue documentation.

Friday, August 31, 2018

Another Periodic Paralysis Patient Lost

HKPP support group member Kendra Rowan has died at age 33. She was a highly gifted artist. This is such an unfair disease and the loss of this young woman is more devastating than I can express.

I can only hope my obituary is half as nice as this one when it's my turn to go. As it states, Kendra was a volunteer at Common Art for the Homeless in Boston, MA. In lieu of flowers, the family suggests donations be made in Kendra's name to Common Art for the Homeless. Send checks to: Common Cathedral, 15 Newbury Street, Boston MA 02116 (write "Common Art" in the note section). You may also donate online at Where it says "Add special instructions to the seller", write: "Direct to Common Art".

I'm so angry I can't stop crying.

I'm taking a break. I don't want to talk about this anymore.

Monday, August 20, 2018

Losses in the Periodic Paralysis community

I learned last night that a member of the Periodic Paralysis community, Jodie Butler, passed away. He was only 53. I met him at the Periodic Paralysis International Conference in 2013, and he was frequently involved in bringing awareness.

No one knows the exact details surrounding his passing, but those of us who witnessed them at the conference knew how severe his episodes were. This disease sucks, and many patients still aren't being taken seriously enough concerning their symptoms, some of which are life-threatening. Most of the time, emergency rooms and physician offices who aren't well-informed of this condition are not safe places for patients. At one point, I know Jodie was trying to raise money to go to Germany to undergo further testing that could have resulted in more treatment and awareness for not only himself, but all of us. The German doctor/researcher, Frank Lehmann-Horn, sadly died a few months ago, and now Jodie has died. Unbelievable.

Dr. Lehmann-Horn's tribute can be found on the Periodic Paralysis International website. I talked about it on social media, but my blog was shut down at the time, so I didn't mention it here. He was extremely important to us, and we owe it to him that we know as much as we do about the Periodic Paralyses and its treatments.

As for Jodie, I hardly know what to say. He was a force to be reckoned with. I think many who are battling this disease are. Some of us don't want to be fighters, but we have to be.

I hope the afterlife exists, and welcomes with kindness all who have suffered in this cruel world.

Friday, August 17, 2018

Internal Medicine Aug 2018

I saw Dr. B at my local clinic for a follow-up. We discussed gastroparesis, diet, insomnia, sleep deprivation, tinnitus, chronic pain, potassium, and hormones.

My insomnia and sleep deprivation reached a dangerous level once again. I was disoriented, and it was no longer completely safe for me to go anywhere alone. I have felt on the verge of collapse. Tinnitus has also been a big issue. I told him this, and we went over my options. Unfortunately, with Periodic Paralysis, the risk is very high with any sleep aid, anxiety medication, antidepressant, or muscle relaxer. Most of these medications have already been attempted over the last 28 years (yes, even in childhood I was asking for help because of this) and made me too weak and sick to function. Dr. B suggested trying the lowest possible dose of Ativan, beginning with a fraction of a pill, to see if it does anything. In my desperation, I agreed.

I'm breaking a 0.5mg dose in half and taking it at night. I am extremely tired, I'm having more muscle weakness than usual, and the tinnitus is still at full screaming volume. That's the bad news. The good news is that I'm finally getting a few hours of real sleep, and my anxiety has lessened a little. That alone is enough to make me want to keep taking it. At some point, I may have to get off of it. The muscle weakness is a problem, but for now, it's manageable enough.

I requested a hormone panel, and the results were normal. I'm not in menopause, or even pre-menopause. I had to have surgery in 2010 to remove small tumors and nuke my psychotic uterus, and I haven't had a noticeable cycle since, but Dr. B explained that I'm still ovulating and experiencing hormone fluctuations. That explains a few things, as Estrogen is a pretty severe HKPP trigger.

My weight is steady now, and although I'm still having plenty of problems with gastroparesis and intestinal motility, the situation has definitely improved. I haven't been back in the ER and I'm tolerating a little more food again. I'm still trying to figure out what I can eat and what I can't. I can say without a doubt that fiber is out. It is my number one enemy. I occasionally try vegetables to see if I can digest them without a problem, but most of them are a no-go. I don't do well with beef, pork, or chicken. I am currently mostly pescetarian, and it's not a perfect diet, but it's going ok. I am still eating a lot of tomatoes, brothy soups, and soft foods that contain little to no fiber. I've been baking fish, boiling shrimp, and making ceviche. Noodles digest well, but they don't do my HKPP any favors, that's for sure. I still eat them sometimes, and handle the side effects as best as I can. I am supposed to meet a new gastroenterologist next week. I'm on the fence about going, but I probably will.

I'm saving up to drive to Jackson if and when my renal doctor wants to do that HKPP interview he talked about. I appreciate people who want to know about the condition and bring awareness. I spent so many years being disregarded that I am still very defensive about having this condition. I tuned into the live stream of day one of the Periodic Paralysis International Conference today and was instantly upset on behalf of those who stated that they weren't being taken seriously and were suffering from lack of treatment. I don't think I'll ever stop being angry that this happened to me and continues to happen to others. It's disgraceful. It's unacceptable.

I don't take my doctors for granted. I hope to God they know that.

I'm so damn tired, but I'm getting by, and life is maybe 10% more tolerable with the new medication. Right now, I'll take whatever I can get. I will keep you posted.

Thursday, July 12, 2018

Under Renovation

This blog is currently undergoing reconstruction. 
Thanks for your patience.

Ride For Rare Diseases (Periodic Paralysis Awareness)

Cyclist Gabriel Low is riding his bike across the country to bring awareness of Primary Periodic Paralysis! He can be followed on Facebook and Instagram. There are quite a few news stories about him on those pages, if interested.

I know this condition affects him and his family, and I'm glad to see that he is thriving in spite of it. People like Gabriel are making a difference for those who are battling rare and under-diagnosed diseases such as Primary Periodic Paralysis. I really appreciate his advocacy!

Monday, June 25, 2018

June 2018 Internal Medicine Follow-Up

I saw Dr. B on the 19th. The office failed to put me on the schedule in spite of giving me an appointment card, so I had to show that to be seen. I won't even get started on their issues. I'm grateful for whatever help I can get.

I had the usual blood work, and my potassium level was where it often is - 4.0. As has been the case for years, I remain in a weakened state in spite of normal potassium levels.

We mostly discussed my gastroparesis. I thankfully haven't been in the ER again since May, but still dealing with nausea as well as a lot of pain everywhere including my torso, lower abdomen, backside, bladder, and everything in between if you know what I mean. It all never stops hurting. My body is super irritated. He did check me for bladder infection, and it was negative.

I am also in tachycardia, and experiencing lightheadedness, shortness of breath, and a decline in equilibrium. I had one of these episodes upon entering my room, and they stopped what they were doing to recheck my blood pressure and heart rate. BP was perfect, as it usually is (Irbesartan is the real MVP), and heart rate was 124. Where my digestion is concerned, since ditching the high protein/fat diet, the majority of the paralysis appears to be in my colon at this point. I've learned that this is frequently referred to as Intestinal Motility Disorder. It is probably related to my Muscular Dystrophy, however, I question the possibility of Dysautonomia and POTS and it is something I hope to talk to someone about at some point. My heart rate goes up for no good reason. All I have to do is turn over in bed or get up out of a chair and my HR jumps. I've worn heart monitors several times that confirmed this, but I've never been diagnosed with POTS for some reason. POTS can cause Dysautonomia, and Dysautonomia can cause Gastroparesis. I know Periodic Paralysis patients who have Dysautonomia, so that's notable as well. Dr. B said he wanted to refer me to the local university hospital's gastroenterology department to discuss it further. I'm waiting on that referral now. He decided to give me samples of another medication, Trulance, to try to force my lower digestive tract to do its thing. And boy, is it. Far, far too much. I'm losing weight again in spite of eating, and as I've already mentioned, I'm in constant pain (I already deal with constant pain in my muscles, but this is a different kind of pain).

So now what? Dr. B said if the new medication is a problem, just switch back to the previous one, Linzess. That one, as I wrote in May, is also harsh on my digestive tract, but it's the lesser of the two evils, I think. I'll start back on that one tomorrow. Other than that, I have to take this one day at a time and wait for the referral.

Dr. B wants to see me again in a few weeks to check on me. His office frustrates me to no end with their frequent careless mistakes, but he has a very high volume of patients due to being so fatherly. I think if more physicians treated their patients like friends, the overall morale of medical care in this country would change. Something to think about.

Monday, May 7, 2018

This is getting old fast.

I had to go to the emergency room again after a bad bout of gastroparesis. I'm home and medicating with the phenergan that my local doctor prescribed recently. I guess I should have been taking it to begin with, but it messes with my HKPP so I avoid it unless I'm to the point of unbearable nausea. In this case, it happened too quickly to consider the meds. I vomited so much for so long, I lost my voice. As soon as I was able to balance on my legs again, I went to the dreaded ER. I did end up in a wheelchair shortly after arrival due to weakness and dizziness.

I complained about being cold so often, they pushed a bag of fluids into me and sent me out the door in 2.5 hours. I couldn't leave fast enough. No pillow, no blanket, just my shivering, purging body in a gown hooked to a sodium IV, which did my HKPP no favors. I'm convinced I'll never receive potassium at the ER again. I tell them I have a history of Hypokalemic Periodic Paralysis, but with serum potassium above 3.3, they won't even consider it.

When they released me, I had to sit in the car with the heater on to warm up my muscles until I could walk the short distance from my apartment parking space to my front door. So frustrating. The only reason I go is because I cannot allow myself to become too dehydrated. That's certainly risky for anyone, but can be absolutely detrimental to someone who already has an ion disorder.

Tuesday, April 24, 2018

New-found gene mutation: Hypokalemic Periodic Paralysis with Myotonia

On April 19, an article was published about a new-found gene mutation for a variant of Hypokalemic Periodic Paralysis that is accompanied by Myotonia - P1158S - a sodium channel in the Nav1.4 region. They have confirmed that it is affected by changes in pH that occur in activity (excitability).

I take issue with one sentence:
"A serum potassium concentration less than 3 mM may trigger hypoPP."
That tells me they still don't fully understand their patients. People with Primary Periodic Paralysis do not have to fall out of "normal" range to be symptomatic, or even to have serious paralysis. A lot of people with this condition, myself included, have proven that over and over for decades. If this does not get acknowledged, patients are going to continue to suffer. The notion that serum potassium needs to be out of a specified range for symptoms to arise simply has to change.

If you've been reading my blog long enough, you know this gene mutation is a possibility for me since I have both Periodic Paralysis with permanent muscle weakness and occasional debilitating Myotonia. However, I don't know how my pH changes during physical activity, and I doubt it's worth finding out since my body handles activity so poorly. Excitability is a big problem, and the last thing I need to do is test that and end up fully incapacitated as I've been in the past. I would, of course, agree to genetic testing otherwise.

This is an important reminder that we must keep looking for answers with an open mind for the sake of patients whose lives are affected by strange diseases such as this. Paralysis and Myotonia are opposites, yet this condition is real. There is so much information to take in, but we're not done. Life is not a textbook. There will always be something else to learn. If more physicians were willing to consider that there are variants of what they believe to be the norm, we would be in better shape overall. Huge thanks to the fantastic medical professionals out there who get that.

Thanks for reading.

Saturday, April 14, 2018

Let's Talk About Internal Medicine

Let me start by saying I've taken in 135 calories over a period of 2.5 days. A few minutes ago, I ate two crackers and gagged the entire time trying to chew and swallow them. I've managed one yogurt smoothie, but not my protein drinks. So things are going pretty badly this week, to say the least.

Dr. B is aware of everything that's gone down over the last year with my Gastroparesis, including the fact that my former Gastroenterologist got annoyed over my high risk situation and gave me the boot (something I haven't even bothered to talk about here, and probably won't mention it again). He wasn't particularly nice, and I didn't let the door hit me on the way out. So that's that. I have no Gastro doc. Thankfully, Dr. B knows enough to deal with this himself, and he is trying his absolute best to help. He's great. He always tells me it's good to see me even if I'm cranky. I can talk to him about anything, and he sympathizes. If he doesn't know the answer to something, he goes into the next room and looks it up while I wait. If I need a new drug regimen, he pulls up the info, prints it out, and we both read it to determine if it is ideal and safe for me to take. He trusts my word and my judgment completely, and we figure things out together. We're a team. This is how doctoring is done.

He runs a low-income/charity clinic, this being one of the most impoverished towns on the entire Gulf Coast. He has been a good friend/dad-figure to me for several years now, only he's impossible to access (even by phone) without an appointment due to patient volume. That's the unnerving part, but I get it.

We've tripled my chloride channel activator. If that doesn't work, I'll be upgraded to a guanylate cyclase-C agonist. I'll start with the smallest dose, and if that doesn't work, I'll try the next dose up, and so forth. He gave me four prescriptions in which to do this, and told me to let him know which one I end up going with so he can prescribe it long-term. It is assumed that since I don't have diabetes, my digestive paralysis is the result of muscular dystrophy. So, if one of these things works, it'll probably have to become part of my lifelong regimen.

I am so tired of pills, but so be it. I just hope something works. If this situation doesn't resolve, or at least improve considerably, he wants me to consider a gastric pacemaker. Unfortunately, surgery is extremely risky. People have died under anesthesia with primary periodic paralysis, and I never fail to have complications from anesthesia every time I have a procedure. These complications include not waking up for an extended period of time even when shaken, pinched, yelled at, etc., severe vomiting and dehydration, paralysis for days with residual weakness for weeks, and waking up too soon on the table screaming. Needless to say, I would prefer not to relive any of this. The highest risk, of course, is death by pulmonary failure or malignant hyperthermia. Let's avoid that too, shall we?

I told him about my doctor in Jackson, and he was really happy to hear that Periodic Paralysis is getting more attention. Dr. B doesn't treat Periodic Paralysis himself, but he does know what it is and he provides all of the scripts I'm on through his medication program. This quite literally saves my life. I don't know what in the world I would do without him, and I frequently worry about what will happen to me when he retires.

I'll follow-up in June unless something happens to bring me in sooner. Until then, all I can do is take on this new treatment plan, stay hydrated, and eat if at all possible. Wish me luck.

Tuesday, April 10, 2018

April 2018 Renal Clinic

My labs look good. My kidney function is fantastic.

I'm having mild kidney stones again, so we briefly discussed that. I was informed by Dr. P that tomatoes contain a lot of oxalate, and that's bad news because I eat a ton of tomatoes. I honestly can't imagine giving up salsa, but I should consider limiting it. I also need to ditch tea once and for all if I expect to keep the stones at a minimum.

Both the phlebotomist and the nurse asked me to uncross my legs. Apparently, I am doing myself no favors regarding circulation and blood pressure by crossing my legs, and I do it constantly. I am going to have to somehow break the habit.

Dr. C was very, very kind. He thanked me for coming up there again to talk about my rare disease. He asked me several questions to determine if I was all right on a personal level, which I appreciated because I believe his concern was genuine.

He also asked a question I dread: Who brought you up here?

OK, I know it's risky, but I always try to drive myself. I cannot cope with burdening someone else with the task. It's a 7 hour round trip, sometimes 8, and that doesn't include the appointment, labs, lunch, or whatever. In other words, it's a long day. Since I ditched Airbnb, I'm no longer staying overnight in Jackson, so I drive straight there and straight home. It is hard. Not the driving itself, but simply sitting upright that long. It's painful, and always results in further muscle weakness that requires recovery time after returning home. I'm not putting anyone else on the roads in danger. I know my body. If I'm too weak (see: February), I reschedule. People may judge me for this decision, but I am hell-bent on being as independent as possible. I know it's just an innocent question, but answering it always unnerves me because I have more issues than Sports Illustrated.


He said he wants to bring awareness of Primary Periodic Paralysis (hooray!) and asked if I would come up there to be interviewed about HKPP in front of a team of doctors. I hesitated only because of my immense social anxiety, which I told him about, but I agreed that it was important. Sooooo he said he's going to call me soon to schedule this thing. I know I need to help bring awareness of this horrendous systemic disease; I just hope to God the nervous gibberish that will inevitably come out of my mouth does someone some good.

I have to say it's touching that my doctor looks beyond my boring test results. I don't take it for granted, and I wish I could teleport all of my HKPP friends to Jackson to see him. I went up there kind of sad, but left in a much better state. Empathy can make all the difference in a patient's life. I wish every doctor in the world understood that. I'm so thankful mine does.

Monday, March 26, 2018

Gastro and MD Update 3/26/18

Zofran is my BFF right now. This has been a rough week, but it feels like my digestive system is trying to wake up. I hope I'm right, but I'm remaining cautious. I eventually want to incorporate baked or braised seafood and chicken in very small amounts, especially in soups, but I don't plan to start that today. I do have a future meal plan lined up for when I am ready to transition.

In related news, my Lose It app informed me this morning that I was awarded the "Lost 10 pounds" badge. Needless to say, I don't recommend illness as a weight loss method.

In Muscular Dystrophy news, I'm still having random spasms, but nothing like the myotonic episode of doom from a few weeks ago. I do remain weak and haven't fully recovered from that, with my head, neck, and shoulders feeling heavier than they used to (and they were heavy before, thanks). I hope that with time, the heaviness will improve. I continue to push myself in ways that single people must: cleaning, grocery shopping, etc. and it is a struggle every time, but I survive and crash into my bed until I can get up again. That's all I can do.

I don't know if I'm going to make it to Jackson in a couple of weeks. Driving isn't really the issue, it's simply being able to sit up that long. Currently, I'm in bed most of the day, and I have to stay near a bathroom. But if I'm feeling better by then, I'll go.

That's all for now, I suppose.

Tuesday, March 20, 2018

Not How I Planned To Spend My Birthday Week

I spent the night under observation after a horrendous day with Gastroparesis. I had a contrast CT, blood work, and referrals back to Primary and Gastro physicians.

I'm home and pretty much camped out in the bathroom until this purging episode decides to resolve. They gave me some fluids to combat dehydration, and a shot of Bentyl to try to calm my stomach muscles, but this remains an ongoing situation for now. I'll get a three-day script for Zofran filled sometime today, which will hopefully make a difference.

I may have to permanently halt the Muscular Dystrophy diet and switch solely to a Gastro diet to try to manage this, and just hope my HKPP doesn't get worse as a result. As I mentioned a few years ago, the usual prescribed medication, Reglan, is a major no-go due to triggering serious periodic paralysis complete with respiratory distress. So I'm sort of up a creek without a paddle, as I am with a lot of conditions that I live with.

My late MawMaw used to have a fun cup holder that said "THIS IS NOT THE LIFE I ORDERED".

I could not possibly concur more.

Wednesday, February 28, 2018

2018 Rare Disease Day

I have systemic disease: conditions that affect multiple systems of the body.
I'm going to make this easy on myself and share links from Wikipedia.
The references are valid, so don't worry too much about misinformation.

I may have forgotten some things, but I tried to share as much as I could.
I'll reboot the medical blog soon. I get tired of talking about it sometimes.

At any rate, if you have questions or concerns, you may email me.
I won't promise a quick reply, but I'll eventually read and address it.

I've learned you never know what someone is really going through.
We only see the surface most of the time, so it's best not to be judgmental.
Thanks to those who bring awareness, and to those who stand by us through it all.

Sunday, February 18, 2018


My head and shoulders remain heavy, and muscle spasms come and go. Otherwise, I'm doing a little better.

I went across the street to pick up bottled water and a few other things (I almost barfed from the pain), and I've done a little bit of walking around outside (the weather is a beautiful 70 degrees during the day right now, which helps immensely). I'm still living on ibuprofen and the occasional stronger pain med (a PRN prescription that I've had for years and almost never use). I'm eating pretty regularly, but my digestive system is still being a jerk and I'm having to medicate heavily in that department to force things to move in the correct direction. That's not doing my HKPP any favors, but HKPP is most likely the reason it's happening to begin with, and potassium isn't correcting it. I've upped my Magnesium Citrate dosage in hopes of finding some kind of balance. Believe me when I say nobody wants to be on Mag Citrate, but it's a daily supplement for me and has been for a long time. My acid reflux disease, which I've had more than 20 years, is also at its worst thanks to Gastroparesis. I awoke from a dream at 4:00 this morning where I was at the grocery store mulling over which antacid to buy, so even my subconscious is tired of this crap. LOL

I'm so cranky and exhausted from this misery, but trying to move forward and get better.

Sunday, February 11, 2018

Things Are Bad At The Moment

I'm not going to go into major medical detail right now, but I have had a pretty bad episode, and I have been bedridden for several days. The pain was a 10 on the pain scale for a little while, as heard by my screaming and probably scaring the neighbors (hopefully they didn't hear me, but that's unlikely). I had my thumb on the button to call for an ambulance for a few hours, but I didn't go through with it.

I am, however, having to sedate myself to escape the extreme muscle pain I'm in. Most of my episodes are paralytic in nature, but sometimes my body surprises me with an episode of myotonia. This is what happened, which nearly resulted in passing out because of the location of the muscle; a vertical muscle running down the back of my head, neck, and shoulder blade. I haven't been able to turn my head since late night Friday. As of this morning, I can turn it about an inch. I have been successful in shuffling across the hall to the bathroom, and to pour water to take pills. Swallowing pills has been a battle...I'm amazed how challenging that is when you can't move your head at all. At any rate, I eventually get them down. Otherwise, I'm in a crumpled pile of agony on my bed, alternating between lying on an ice pack and a heating pad.

This episode is in addition to the bout of gastroparesis that was already making me sick. Gastroparesis is stomach paralysis, which can be caused by a number of conditions. My digestive system is a huge issue, and sometimes I think it's going to kill me way before anything else does.

The medications I am currently on to deal with all of the above are making my body weaker (as I have complained about in previous posts...most medications aren't very safe for dystrophy patients). I'm being as cautious as I can, and it is a well-proven fact that I am safer at home than in a hospital subjected to protocols that don't acknowledge the risks of my rare disease.

In summary, some parts of my body are contracting too much, and some aren't contracting enough. If you know anything about that, you know that means absolute disaster. Thanks for thoughts or prayers or whatever it is that you do.

Tuesday, October 24, 2017

October 2017 MD Clinic

It has been a little while since I've blogged. Sorry.

Muscular Dystrophy clinic was this month, but it was mostly uneventful. I did meet with a geneticist for the first time, who agreed to order testing through a temporarily-free program strictly for Periodic Paralysis patients. By the time I got home, I learned that something went wrong with the form, so it wasn't done.

The trip was as exhausting as ever, and I was depressed and anxious to be in so much pain and so tired while traveling. I told the doctor how I was feeling, and she said it was understandable, but it wasn't discussed beyond that. It happened to be the day after the Las Vegas shooting as well, so she didn't think much of the fact that I said I wasn't coping lately. I don't expect a resolution anymore. It's just the way things are.

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. It's actually perfect. He noted amiloride, and I agree getting back on that drug is what brought my filter rate back into normal range.

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 complete 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, as 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 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. 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 nearly $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.

Wednesday, December 21, 2016

I could have sworn I updated this thing.

By golly, I didn't. Sorry about that.

Not a lot has been going on. I was too weak and broke to go to renal clinic, so it was bumped to February. Internal medicine made me furious by once again ignoring my pharmacy's requests, and I had to raise hell. I have an appointment in a few weeks, but I plan to bump it to sometime after renal clinic.

I'm having some symptoms that are TMI, mostly my bladder issues which are getting worse. I hate hate hate the urologist and it's pointless to go when I already know the medication is a no-go (I've already been there and done that with internal medicine). The only option is to reduce my diuretic intake, which results in muscle weakness. So there's no winning.

I'm receiving emails about Keveyis fairly often, mostly from people who had the same experience that I had. There seem to be plenty of us who were not able to tolerate the medication due to potassium shifting. Physicians and pharmacists could stand to consider further research on Periodic Paralysis and the fact that the drug could work if potassium supplementation was sufficient. Some patients simply don't have the physiology to handle Keveyis and its relative Diamox without heavy electrolyte supplementation. Even the amount of potassium I'm on wasn't enough, but I wonder what would have happened if supplementation had been increased while on Keveyis? I'll never know now. What I do know is that I'm never Hyperkalemic, and that has been proven. My levels are average or below on 100+ mEq a day + PRN emergency K, which I have to take often. Food is worthless. When you have a channelopathy, don't expect a "high potassium diet" to make much of a difference, if any. It made no difference at all for me. Nor did a "high phosphorus diet", "high magnesium diet" and so forth. I'm forced to high doses of these supplements in order to function.

I haven't felt like talking about it much lately, but that's what was on my mind off the top of my head. It's been a stressful year, and I suspect the next year and several years following aren't going to be any better thanks to our new fascist regime. Don't even bother emailing me about that statement. I'll ignore you.

All we can do is the best we can with what we're given, but sometimes, we have to fight to get anything at all. Don't hesitate to be your own advocate. Chances are, nobody else is going to do it for you.

May we all survive the next four years, and beyond.

Saturday, October 8, 2016

Quick MDA Clinic Update

I went to MDA clinic in Jackson, MS for the first time this year. We briefly spoke about my experience in New Orleans, and I must have said the word "infuriating" four or five times. They know I'm back in Jackson to stay, and are fine with that.

In a nutshell, my chronic pain was acknowledged, my proximal muscles are continuing to atrophy, and there is nothing they can do to help since pain medications and exercise make me worse instead of better. Orders are to continue seeing my other doctors, take all of my medication, be mindful with my diet, avoid triggers when possible, rest rest rest, and return for documentation in one year.

Renal clinic is November 14th.

Wednesday, September 28, 2016

Internal Medicine and Social Drama

Today, I had my last Internal Medicine clinic until January. It was thankfully undramatic. This has been a long year of tests and symptoms and medication changes, but I'm feeling somewhat optimistic.

My gastric study looked ok, which is good news since the only emptying drug on the market, Reglan, triggers serious paralytic episodes. Feel free to dig through my HKPP blog for that drama. The ending diagnosis is Gastroparesis. I also have GERD, but that has been known for decades.

As for my potassium level, raising my dosage to a whopping 200 mEq max a day helped a great deal. My level rose an entire point to 4.7, which was our exact goal. Yay! Ideally, it would be best for me to be in the low 5s, but I'll take what I can get. Dr. B was super glad about that.

My kidneys will continue to be monitored. Most of the time, they're in normal range, but sometimes they slip into mild failure and nobody really knows why. This odd fluctuation has been noted since age 20. I remember my first Nephrologist back then labeling it "kidney frustration" in German.

I declined the social worker for a few reasons. I received many calls, which I missed, and eventually called them back. The lady said that I had accepted services on such-and-such date and that she was trying to set up an appointment to come to my house. I told her that not only did I not accept services yet, I had never even spoken to her before and didn't know who she was. She said it was in her computer that we had talked on that day and I said yes. I told her again that wasn't true. We went back and forth on this error, and she eventually said "sorry" and gave me my case manager's phone number. What case? Exactly.

Anyway, she explained that the case manager's job is to come to my place to talk about their services, ask questions (from a checklist that I hate...I'll address that another day), and check on me regularly. I told her that I would save the lady's number and call her if or when I was ready to discuss. I hung up and called my insurance to make sure they didn't receive a bill for services that I didn't accept or receive. They hadn't, thankfully, but I'm glad I checked. The fact that she said we spoke on that day and that I signed up when I definitely had not done so yet was concerning. I actually still have the missed call and voicemail saved in my phone as proof.

As I said, I told her I would call when I was ready to discuss their services. Within two days, I began receiving calls from the case manager asking if it was ok to come over to my place to ask some questions. I let it go to voicemail every time, and she gave up by the 3rd or 4th call. I concluded once again that people don't listen to me, and they don't respect my boundaries. Story of my life.

I'm not ok with someone knocking on my door unannounced on a weekly basis to ask me generic questions that don't apply to someone with a terminal illness, and that's what was going to happen. I had a social worker in 2014, and that's what she did. I eventually stopped answering the door. I would have done the same this time. I am fine with going somewhere and talking to someone - social worker, therapist, doctor - but I am not ok with them coming here, and especially popping in to check on me uninvited. As an introvert with anxiety, that derails me, and I have no problem admitting that. I spent the first 36 years of my life being denied boundaries. I'm not about to give them up now.

If I extend a specific invitation to you, or if I tell you that you are welcome here, then you definitely are. I don't want any of my friends or family to think otherwise or feel too intimidated to spend time with me. I wouldn't have set up gaming and art tables in my living room if it was a problem. For your consideration: I get very little sleep, and when I do sleep, it's at odd times. Nothing makes me want to melt down more than finally falling asleep, and being awakened by a knock on the door, a ringing phone, or a loud neighbor. One of my biggest issues every day is that I'm so exhausted, I can't seem to process things in a timely manner (brain fog). I draw a blank when dealing with someone. I really don't feel cut out for living, and I need a lot more sleep. I unravel quickly because I'm too tired and I want the world to stop waking me up, judging me, giving me a hard time about petty things like grammar (for the love of god, find something better to do), comparing my problems to theirs or others' (this is not a competition), making assumptions, or trying to fix me, and just be a friend. I'm the first one to say I'm not a pro at managing this turbulent way of life, but I'm trying. I appreciate those who have been patient and kind, and I thank you sincerely for understanding, or at least attempting to.

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. Anyway, 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.

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 dozens of 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.

Saturday, July 9, 2016

Medical Equipment Drama

Indy500 (my rolling walker...yes it has a name) 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.

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, 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.

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 without a paddle 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.


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 participate in independent living, 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 affect cognition (we call it brain fog), just the same as Diabetes or other conditions 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 they have the capacity to hold a normal, intelligent conversation. 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.

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.


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 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 many times, 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 a 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.

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 there to eat gumbo.

Phone call with Taro Pharmaceuticals, and back on Diamox

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 dichlorphenamide (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, higher function, 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. 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 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 acetazolamide (DIAMOX), 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 big difference in the quantity and severity of my episodes. I used to paralyze every day, folks. And while I remain in a state of weakness (which the muscular dystrophy clinic thinks is permanent), my serious, full-blown paralytic episodes have reduced significantly on Diamox. It's a miserable drug, but God bless it for giving me some 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 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. :)

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 clear on this: The drug works for some patients. I've heard from people who said it's a dream come true and that life is better now. I am 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, so I hope insurance coverage or assistance programs are in place for them to do so.

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 (a 7.5-8 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". 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'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.