Sunday, November 23, 2014

Sleep Consult

I finally finally (did I mention FINALLY) met with a pulmonary sleep physician a few days ago. He was great. We discussed my history and my condition (he knew it had to do with potassium, but didn't realize the primary form involved a myopathy or that the MDA was involved). He said he'll read up on it, and I offered to give him more information when I see him again.

A bit of back history on me: I had two sleep studies at age 20. I was found to have restless legs and borderline sleep apnea. It wasn't enough to officially diagnose me with sleep apnea, but they said I was holding my breath for no reason and questioned why. It annoyed me because it's not like I do it on purpose or something... hello. At any rate, they were sleep psychiatrists and wanted to talk psychiatry and give me klonopin, both of which I declined. The hospital eventually shut down, and my records were lost (probably in Hurricane Katrina, if nothing else).

I would have been willing to see a sleep psychiatrist again, but I'm happy that he ended up being a sleep pulmonologist instead. With my breathing issues and muscle disease, that is the right place to be in my opinion.

The doctor was very confident that my condition has worsened since age 20, and diagnosed me with obstructive sleep apnea. He wants to confirm it via sleep studies, of course, which is what I was expecting. Their schedule is incredibly backed up, so I'll return for study one on January 30th. The second will take place a week later.

He said he has no doubt I am not breathing well at night, considering that I choke and occasionally snore. He also diagnosed me with sleep deprivation (glad that's finally documented) and said he hopes he'll be able to help me with that via oxygen. The studies will tell him what kind I need. I already have a CPAP machine at home in the closet that was donated to me, I just couldn't get it calibrated without the studies, so here I am. He is hoping that the CPAP will be the right thing for me so I don't run into any trouble with getting other equipment (financially speaking). 

Overall, it was a solid consultation. He was very nice, eager to answer any questions, and seemed positive and helpful. We'll see what happens come January. I'll keep you posted.

Sunday, November 2, 2014

June's Hospital Stay

I realize how far behind I am on things. This has been a tough year, and blogging took a bit of a back burner. Thanks for bearing with me on this. I want to talk about my experience in the hospital back in June. It was different from the usual hospital stay. I often leave a hospital feeling the same or worse than when I went in, and regret it. Surprisingly, I left feeling so much better than when I went in this time, in spite of several medication errors. The reason for that boils down to compassion and understanding. Here's the rundown:

I went to the emergency room in meltdown mode. I was in tears from the pain in my head and lack of sleep. I've had a headache, sometimes to the point of migraine, for over a year and I couldn't take it anymore. I was also afraid, very stressed out, and upset, as I felt my independence was being threatened. I told them if things were going to stay this way, I wanted off this planet. The nurse knew I didn't really mean it, and said they would try to figure out how to get my pain under control. I asked for a medical doctor, but also asked to speak to a psychiatrist and a social worker. I wanted a medical doctor, social worker, and a psychiatrist to collaborate and come up with a plan to deal with my pain, stress, and help me to achieve as much independence as possible. I told them I didn't care how this had to happen - I was willing to be admitted to any floor to be taken seriously. They were reluctant, but ultimately did exactly what I asked. I was admitted to the geriatric wing of the psychiatric floor, as that is where the three doctors I requested reside. I was more than fine with that.

I went through the usual motions that every patient on the floor goes through: they checked my body for injuries to ensure that I wasn't abusing myself or being abused. They asked me a lot of questions. I gave them my medical history and told them everything I could possibly think of concerning my emotional state. I brought up how differently I am acting since my condition declined and my medications were heavily increased. I ranted about acetazolamide, mostly, but we all agreed that I can't stop taking it because of how well it's working to prevent paralytic attacks (which I still have, certainly, but I'm not staying in a near-constant state of paralysis like I once was). I even mentioned my long marathons of lamenting on Twitter when I'm upset or stressed. I told every doctor who entered the room how unhappy I was with the fact that I've started cursing. I didn't curse growing up, even as a teen, or even when I was married to a sociopath. But now, the words come out of my mouth before I can even think about it, and it disturbs me. The head psychiatrist actually chuckled a bit, and then he said "You are acting completely normal. You're living with a no-win situation, and any normal person would be reacting like this." In total, I met with a medical doctor, three psychiatrists, one group therapist, and one social worker. The diagnosis was the same across the board: Muscular Dystrophy, and anxiety and depression that is based on my current circumstances. My problem is, I quote, "medical, not mental". I'm being too hard on myself, and perhaps others have been too hard on me as well, my social worker suggested. I'm paraphrasing, because she was surprisingly blunt about it, if not harsh. I was told that I was "very intelligent with no psychosis", and that my story is somehow "intriguing and inspiring". Okay, if you say so. Thank you very much.

Because I showed up on a Friday, I spent a lot longer in there than I would have. The weekend is kind of a dead zone, so I chilled with three doctor consults and nothing else. Monday was the busy day...group therapy and the official consult by the head psychiatrists. The docs wanted me out of there asap, because there was "no reason to be inpatient" and they decided all the medication errors made me "safer at home". I couldn't have agreed more. I was set up with an outpatient follow-up, recommended grief counseling, and assigned an outpatient social worker to address basic personal needs at home and a transition to more independent living. That's it. They had no solution to my pain or my ailments, and I didn't expect them to. But I was relieved that they cared.

For the most part, I was bored out of my mind. No phone, no TV, no music, just the sounds of nurses and geriatric patients shuffling down the hallway, sometimes yelling at each other, sometimes knock-down-drag-out fighting. It was an eye-opening experience. I looked at those patients, down and out and at the mercy of others, wondering what their lives used to be like verses where they are now, and my heart went out to them. Being surrounded by the 50+ crowd, I stuck out like a sore thumb and was often asked what in the world I was doing there. The staff said repeatedly that I belonged on a medical floor, but they said they were happy I chose to come there if I needed a place to feel safe and get some rest. I really appreciated hearing that, especially from a psychiatrist. My room door remained open the entire time. I was allowed to come and go as I wanted (as long as I didn't leave the floor), and I was allowed to use the phone (which I only did once). I eventually asked for a pencil and paper, and it was given to me. I worked on my novel (side note: I'm writing a novel) in between meals and naps. I attended one group therapy session, which I quite enjoyed and everybody knew it. At one point, I found myself advocating for a young lady who was nearly catatonic and was trying to ask for a blanket because she was shivering, but the staff yelled at her to stay in the room. I put on my former hospital administration hat and flat out told them as nicely and professionally as I could to get up and go get her a blanket. They said they would. Apparently, I was considered lively and animated during group therapy (who, little 'ol me? No way! Just kidding. I'm practically a cartoon character, folks). The therapist said she loved talking to me, and she tried to get me transferred to her wing (she was in charge of the addicted, suicidal, anxious, violent patients of various ages...they were all lovely, by the way). However, I was discharged before that happened.

The medication errors...good Lord. I have no doubt my case took up an entire MERT meeting that month (that's Medication Error Report Team, for you non-hospital-admin folks). I used to do those meetings. I was a medical administrator at one time, so I am all too familiar. The staff would not get their shit together (sorry for the language, the emphasis is needed) and continue my home medications. When they finally did, they got them wrong. It was a constant source of debate, and one nurse finally came to my defense. I am so grateful to her...she was wonderful and I wish she had been my nurse every day. She couldn't have done a better job. Her off day was Tuesday, the day I was discharged, and my nurse that day (who was new) got my meds wrong again. I ended up paralyzed as a result. They took it seriously, handled it with as much grace as they could, and I was able to leave about 8 hours later the same day.

I was a happy camper when I left (if interested, you can see how hard I hit the ground running upon my discharge on my other blog). I was exhausted, but feeling so much better emotionally. It did me a world of good to be told that I was "completely normal" and that it was okay to be acting and reacting the way I was in regard to what was happening to me. I am thankful that I found the courage to "call for backup", so to speak, and I definitely don't regret it. A social worker checks on me 2 to 3 times a month, and I am eventually going to start grief counseling if the counseling center that was recommended to me will ever add me to their schedule. I'll keep you posted, I guess.

I'm glad I finally shared this. I hope it helps someone to not be afraid of asking for help when you feel overwhelmed, whether medically or otherwise. Those of us who have been through the nightmare of medical abuse have valid concerns about seeking care, especially emergency care, but remember that not every experience will be the same. My best advice is to keep your cool, but stand your ground. Arrived prepared with solid information about your medical condition, medical history, and home medications. If someone mistreats you, speak out. If you are treated well, don't take it for granted. You and I have a right to live the best, most independent life possible for as long as possible. Be kind to others and give them the benefit of the doubt, but don't forget to do the same for yourself.

Peace,
Kelli

Saturday, November 1, 2014

Updated Medication and Supplement List 11/01/14

Hello my friends,

I figure this would be a good time to update my medication and supplement list. I've decided to include all supplements including all PRN medications and the topical gel I use for muscle spasms (it works...I recommend). Each one is hyperlinked to more information. I'll fix it in the sidebar as well.

irbesartan (AVAPRO) - 150mg 1 x daily
eplerenone (INSPRA) - 50mg x 4 daily
amiloride (MIDAMOR) - 5mg 1 x daily
acetaolamide (DIAMOX) - 125mg 1x daily
spironolactone (ALDACTONE) - 25mg 1 x daily
effervescent potassium (EFFER-K) - 25mEq PRN up to 3 a day
Magnesium Citrate Tablets - 200mg x 2 daily
Magnesium Citrate Powder - 1 to 2 teaspoons in water PRN
Magnesium Chloride Gel - Topical PRN for muscle spasms
pantoprazole (PROTONIX) - 40mg x 2 a day
Omega Oil - 1300mg 1 x daily
ubiquinol (COQ10) - 100mg 1 x daily
Vitamin D - 2,000 IU 1 x daily
Sublingual B Complex - 1 dropper daily
Zinc - 50mg 1 x daily when I remember...let's face the facts here.
Liquid L-Carnitine - Couldn't find the one I bought locally online, but I've linked a similar one. It's refrigerated and I don't always remember to take it, but I'm trying.
Hylands Homeopathic Phosphorus - 2 tablets 1 x week
ibuprofen (ADVIL) - 400mg PRN

For the sake of easing any curiosity, I am going to categorize the meds and supplements that I feel are making a difference, verses the meds and supplements that I don't feel I'm responding to below.

Meds that I know without a doubt are helping:

irbesartan (AVAPRO)
eplerenone (INSPRA)
amiloride (MIDAMOR)
acetaolamide (DIAMOX)
potassium chloride (KLOR-CON)
effervescent potassium (EFFER-K)
Magnesium Citrate Tablets
Magnesium Citrate Powder
Magnesium Chloride Gel
pantoprazole (PROTONIX)
tolterodine tartrate (DETROL LA)
Vitamin D
Hylands Homeopathic Phosphorus
ibuprofen (ADVIL)

Meds and supplements that I feel no effects of. Please understand that doesn't mean they're not working or doing their job, I'm just saying I don't feel any noticeable positive difference on it:

spironolactone (ALDACTONE)
Omega Oil
ubiquinol (COQ10)
Sublingual B Complex
Zinc
Liquid L-Carnitine


I will talk about medications that I've tried previously, and their effects, in a different entry. There are many drugs that I have been prescribed for various reasons that caused side effects - some of them serious. Stay tuned to the blog if you wish. I plan to come up with the long list sometime this month.

I'm so tired of pills. Words cannot express. Gotta do what I gotta do, right? Quality of life is the goal...always has been and always will be. Keeping my eye on the prize.

Feel free to let me know if you have any questions.

Contact

I'm slowly getting my blogs back on track. I still have a lot to catch up on here, so bear with me.

The commenting system seems to be acting up, and has been for a while. Feel free to leave a comment anyway, but you can also follow me on Facebook, Twitter, or email ugottafriend4life@gmail.com. If you email me, please be sure to mention HKPP or Periodic Paralysis in the subject line. I get a lot of junk that I ignore due to a previous hacking issue. If you feel I have missed an email from you, don't hesitate to try again. I don't disregard valid messages intentionally.

Have a safe and happy day. Beware of all of that tempting Halloween candy. :)

Sunday, October 26, 2014

A Bottle of Lies

Greetings! Let's talk about Magnesium.

(From the Wikipedia page. Don't worry, the references are there.)

"Magnesium is a vital component of a healthy human diet. Human magnesium deficiency (including conditions that show few overt symptoms) is relatively rare although only 32% of people in the United States meet the RDA-DRI; low levels of magnesium in the body have been associated with the development of a number of human illnesses such as asthma, diabetes, and osteoporosis. Taken in the proper amount, magnesium plays a role in preventing both stroke and heart attack. The symptoms of people with fibromyalgia, migraines, and premenstrual syndrome are less severe, and magnesium can shorten the length of the migraine symptoms.

Intracellular magnesium is correlated with intracellular potassium. Magnesium is absorbed in the gastrointestinal tract, with more absorbed when status is lower. Magnesium competes with calcium in the human body, in this way it actually keeps calcium in check. However, this can cause a calcium deficiency if calcium levels are already low. Low and high protein intake inhibit magnesium absorption, and other factors such as phosphate, phytate, and fat affect absorption. Excess dietary magnesium is excreted in feces, urine, and sweat. Magnesium status may be assessed roughly through serum and erythrocyte Mg concentrations and urinary and fecal excretion, but intravenous magnesium loading tests are likely the most accurate and practical in most people. In these tests, magnesium is injected intravenously; a retention of 20% or more indicates deficiency. Other nutrient deficiencies are identified through biomarkers, but none are established for magnesium.

The UK recommended daily values for magnesium is 300 mg for men and 270 mg for women. Spices, nuts, cereals, coffee, cocoa, tea, and vegetables are rich sources of magnesium. Green leafy vegetables such as spinach are also rich in magnesium as they contain chlorophyll. Observations of reduced dietary magnesium intake in modern Western countries compared to earlier generations may be related to food refining and modern fertilizers that contain no magnesium.

Numerous pharmaceutical preparations of magnesium, as well as magnesium dietary supplements are available. Magnesium oxide, one of the most common forms in magnesium dietary supplements because it has high magnesium content per weight, has been reported the least bioavailable. Magnesium citrate has been reported as more bioavailable than oxide or amino-acid chelate (glycinate) forms."

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Prior to adding a supplement, I was having frequent muscle spasms and charlie horses. A muscle in my neck pulled so hard one day, I nearly passed out, started vomiting, and ended up in the ER. At times my leg muscles would contract so hard for so long, I couldn't extend my legs to put them on the ground for hours. It was excruciating. Add migraines and lower digestive muscles crawling at a snail's pace and you have yourself a pretty miserable existence. Mag has been a life-changer for me, but it's been a pretty daunting task to find the right kind of supplement and the right dosage to balance out my chaotic potassium shifting and contradictory medications. Magnesium Oxide turned out to be worthless due to low bioavailability, but Citrate in pill and powder form seemed to be effective. Finding the ideal dosage has been the challenge, and I'm still working on it.

My doctor suggested I try Milk of Magnesia instead of Citrate, so I obliged. Unfortunately, I had to give it the boot within a week, as I basically couldn't leave my house thanks to the waves of pain crashing onto the shores of my abdomen like a Category 3 hurricane on Dauphin Island.

I love similes. Don't you?

Humor is the only way, people.


You're a big fat liar, Phillips.


Anyway, back to my Mag Citrate for now. No offense those who suggested it, but if I'm going to feel like I'm in labor, it would be nice to have an actual baby in my arms at the end of the day.

I now return you to your regularly scheduled program. :)

Monday, October 20, 2014

Local Clinic Update 10/20/14

I woke up feeling pretty horrible. Trouble breathing, felt like something was sitting on my chest, and the usual HKPP weakness, but a little worse. I ended up dozing for as long as possible, then forced myself to get up and get rolling to my local doc office. Lots of blood taken, and over a two hour wait, but as usual it was worth it. My doc is great. My strange condition is interesting to him and he seems to enjoy talking to me, to the point that he picks on me like we're friends and we have a laugh. Today in reference to low Vitamin D, he joked that I need to be thrown out into the parking lot with no clothes on to soak up the sun. That produced a belly laugh in me that I've yet to completely shake two hours later. Sometimes, a friendly and upbeat bedside manner can make a huge difference, even in the face of a no-win medical case. Everywhere I turned, I used to deal with skeptics treating me like a lowly idiot who couldn't possibly know what she's talking about. Now I finally feel like I have a collaborative team - listening, learning, and striving to keep me going. Thank God.

I had a lot of labs, and should have the results tomorrow or Wednesday. We're checking vitamins, magnesium, and doing the usual basic metabolic panel. My stat potassium was 4.4, which was an improvement from renal clinic in Jackson. I had taken 200mg Inspra prior to the appointment, so I believe I have that to thank for it. The potassium level goal is still somewhere in the low fives, as that is where I functioned best years ago. Hoping I'll get there again someday, and somehow keep it there.

I spoke with him about the possibility of sleep apnea. He said there is a new clinic in a nearby city, and he believes they take Medicaid. Touchdown! I'm being referred.

I've been having significant issues with my bladder since beginning diuretic therapy, and my doctor sympathizes because he has the same issue (he gave me all the TMI, bless him). He has been trying to get this under control for me since the day I met him. I have samples of a drug called Vesicare to try. So far, anti-spasmatics have resulted in paralysis, but maybe I'll get lucky with this one. I'll give a report in a few days.

I'll return to the clinic next Monday and the following Monday for potassium labs. Otherwise, I guess my next appointment will be with a sleep specialist in a few months.

Reminder


The Battle Against Periodic Paralysis Article - New Location

Someone uploaded my Yahoo article on a PDF site a while back, which turns out to be a good thing since Yahoo shut down Voices and erased all freelance work. I've been wanting to find a new place to submit it for publishing, but this will do for now.

Those who have shared my article over the last 2.5 years, please feel free to replace the bad Yahoo URL with this one. It has been corrected in my sidebar as well. Thanks so much.

The Battle Against Periodic Paralysis


Sunday, October 19, 2014

A blog rant, humanity, and the state of medical care

I wanted to share this blog post from a lady who had an unfortunate and very familiar experience with emergency care. Language alert for the easily offended.

http://letsfeelbetter.com/urgent-clinic-doctor-fail-i-dont-even-care-if-you-google-it/

I've been there. I've been in an emergency situation at the mercy of people around me, only to be mocked, mistreated, and misunderstood. It happened for years. I have been told of so many nightmares of people going through this kind of shame in medical care, and while it's nice to know I'm not alone in my battles, it's sad and unacceptable that this is still so common. I'm glad Ilana is telling her story. More patients need to put the truth out there, no matter how ugly that truth may be. Sometimes, shouting is the only way to be heard.

Doctors and Nurses: if you want to do the world a tremendous injustice, go ahead and assume every young person who rolls through your door is a drug seeker. Go ahead and assume they're being overdramatic and that their symptoms are exaggerated. Go ahead and assume that just because you haven't heard of it, or have never treated it, it must not be valid. Go ahead and do harm, if you dare. Just remember this: your paranoid and uninformed assumptions don't make you smarter or more superior. They only make you a closed-minded jackass.

I struggle greatly with the state of the world. I've learned that where there is good, there is bound to be bad. It is inevitable in all of us, as humanity is deeply flawed. The way I see it, if people want to screw up their own existence, that's their business. But when someone else's well-being is at hand, humility and willingness to accept education, ideas, and possibilities go a very long way. It can save lives and livelihoods. I believe it has saved mine.

My heart goes out to every patient who has lived through these scary and frustrating moments. It took a life-threatening decline in my condition for people to finally listen to me and take me seriously, and I can't help but wonder what my life would be like had I received the care I sought unsuccessfully for 20+ years. At any rate, I am very thankful that I have now found a team of physicians who are in my corner and are doing what they can to help. I wouldn't trade them for anything. I only wish to God I had found them sooner.



(cross-posted to my personal blog)

Wednesday, October 8, 2014

Fall MDA and Renal Clinics

Met a new doc at MDA. He was super nice, and seemed to know a good bit about HKPP. He documented my updates: Mom's death, my hospital stay in June (more on that later), and my loss of strength and proximal muscle tone. My arms were very weak, but leg reflexes were slightly more responsive than usual. He reminded me to eat low-carb, recommended vitamin B2 for my migraine, told me to surround myself with all things happy and try to stay distracted from my circumstances. He's one of several docs who have said this over the last 6 months. I'm a realist, but escapism is important when you're trapped in a broken body and stuck with a life and future you cannot control.

My renal doc saw me the same day. We discussed my potassium level, weight gain, breathing and sleeping problems, chronic pain, and Mom's death. He wants me to find a sleep specialist anywhere in Alabama who will take me. He believes I have sleep apnea. We reviewed test results and noted the general wackiness of my kidneys. We had a chat about my latest meds and symptoms, and the fact that I am needing to move back to Mississippi so I can continue to receive care there. He said he will always do whatever he can to accommodate my schedule because he knows how far I'm having to travel right now. This situation is a nightmare, but I always leave feeling a lot better emotionally after talking to him. I'm an introvert with chronic pain and anxiety, but he has so much patience with my moodiness and awkwardness. Best doctor ever.

New meds added to the pile: spironolactone 25mg titration, vitamin B2

Orders: potassium test in a few weeks (to be done in Bayou La Batre)

Appointments: Back to back clinics again - April 14th - and may God bless them in advance as that is the 1st anniversary of Mom's death. Might be a hell of a day.

I believe wholeheartedly that my physicians at UMMC have sustained my life, and I hope to continue to receive care from them for as long as possible.

Wednesday, August 20, 2014

Weakness/Paralysis on Reglan for Gastroparesis

I was diagnosed with a sliding hiatal hernia and Gastroparesis (partial paralysis of the digestive tract), so I started Reglan last night. I was a chicken and broke it into quarters and only took one quarter of a pill. Good thing I did, because I have been unable to get out of bed all day so far from major muscle weakness. Dr. B and I knew it was risky, but we needed to give it a shot. Will DC Reglan immediately and resume Protonix twice a day until further discussion in October.

Reglan is a muscle stimulator, so for the average person, the drug would have been helpful. For one with HypoKPP, anything resulting in muscle excitability is a potential paralytic trigger.

The above diagnoses are contributing to my breathing issues, however, I hope to see a pulmonologist in the near future. My lungs and/or diaphragm may still be compromised, as two of my doctors have noted reduced breathing.

I'll be back with an updated list of my meds, as well as a list of meds I have recently reacted badly to, and a new location for my Periodic Paralysis article (which is gone at the moment, thanks to Yahoo shutting down their freelance site). Talk to you soon.

Sunday, July 6, 2014

Muscle Activity, Sodium Potassium Pump, and Periodic Paralysis

An abstract from the Journal of General Physiology
http://jgp.rupress.org/content/142/4/327.short

During excitation, muscle cells gain Na+ and lose K+, leading to a rise in extracellular K+ ([K+]o), depolarization, and loss of excitability. Recent studies support the idea that these events are important causes of muscle fatigue and that full use of the Na+,K+-ATPase (also known as the Na+,K+ pump) is often essential for adequate clearance of extracellular K+. As a result of their electrogenic action, Na+,K+ pumps also help reverse depolarization arising during excitation, hyperkalemia, and anoxia, or from cell damage resulting from exercise, rhabdomyolysis, or muscle diseases. The ability to evaluate Na+,K+-pump function and the capacity of the Na+,K+ pumps to fill these needs require quantification of the total content of Na+,K+ pumps in skeletal muscle. Inhibition of Na+,K+-pump activity, or a decrease in their content, reduces muscle contractility. Conversely, stimulation of the Na+,K+-pump transport rate or increasing the content of Na+,K+ pumps enhances muscle excitability and contractility. Measurements of [3H]ouabain binding to skeletal muscle in vivo or in vitro have enabled the reproducible quantification of the total content of Na+,K+ pumps in molar units in various animal species, and in both healthy people and individuals with various diseases. In contrast, measurements of 3-O-methylfluorescein phosphatase activity associated with the Na+,K+-ATPase may show inconsistent results. Measurements of Na+ and K+ fluxes in intact isolated muscles show that, after Na+ loading or intense excitation, all the Na+,K+ pumps are functional, allowing calculation of the maximum Na+,K+-pumping capacity, expressed in molar units/g muscle/min. The activity and content of Na+,K+ pumps are regulated by exercise, inactivity, K+ deficiency, fasting, age, and several hormones and pharmaceuticals. Studies on the α-subunit isoforms of the Na+,K+-ATPase have detected a relative increase in their number in response to exercise and the glucocorticoid dexamethasone but have not involved their quantification in molar units. Determination of ATPase activity in homogenates and plasma membranes obtained from muscle has shown ouabain-suppressible stimulatory effects of Na+ and K+.

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The above information is a bit overwhelming in its language, so I'll do my best to explain it. As we know, muscle function is controlled by the sodium potassium pump. During exercise, potassium leaves the muscles, shifting into the bloodstream. Once the exercise ceases, potassium is pumped out of the bloodstream back into the muscle cells. Someone with Hyperkalemic Periodic Paralysis would experience depolarization and loss of muscle function during muscle activity, thus exercise being a trigger of attacks, while someone with Hypokalemic Periodic Paralysis would experience paralysis following muscle activity, thus "rest after exercise" being the trigger.

As Periodic Paralysis experts have noted on many occasions, the functions of the sodium potassium pump isn't felt by people who are well. It is those who have conditions of the muscle - in our case an ion channelopathy - who experience chaos from these shifts as our voltage gate allows incorrect ratios of sodium and potassium to flow in and out of the muscles. To put it bluntly, our bodies short circuit. Depending on the type of Periodic Paralysis, our sodium potassium pump will be either regulated or triggered by "exercise, inactivity, K+ deficiency, fasting, age, and several hormones and pharmaceuticals". There are other regulators and triggers as well, and again, it very much depends on the type of PP.

I believe this explains why physical activity is a trigger (or ultimately leads to a trigger) in all types of Periodic Paralysis. This is my assessment of the article, but if I am completely off-base (see my disclaimer in the sidebar), feel free to share in comments.

Saturday, July 5, 2014

Another HKPP Blog

Ann Latos and I have parallel lives. She has the same form of HKPP that I do, and her mobility came crashing down as quickly as mine did a few years ago. Just as I've experienced for over 20 years now, some of her doctors, family, and friends didn't believe her illness was real.

I hope Ann never stops sharing her journey, because I know how lonely, frustrating, and despairing it is to live with this rare systemic disease. There are others out there suffering more than necessary as Ann and I have because they can't convince people to listen and care. Some patients have even died by the hands and voices of ignorant caregivers. In order to stop these senseless tragedies, all of these people, and their future generations, need us to speak out just as much or more than we need to be heard ourselves.

I don't know about Ann, but those who wish to silence me will be sorely disappointed. I will never stop talking about my life - the good, bad, and ugly. The thanks and positive feedback I have received from around the world triumphs over the false judgment, unsolicited opinions, and criticism that any skeptic has thrown at me. To those people, I simply say "Game Over". You lose. I have no doubt Ann feels the same way now that she has found the physical and emotional support she needs to continue fighting this battle.

I appreciate the MDA for sponsoring her blog. Familial Periodic Paralysis is still a widely unknown and misunderstood disease - one of the rarest forms of Muscular Dystrophy in existence. The only way to save lives and give patients a better, more independent future is if we make it known and understood, and that requires awareness. Go Ann!

Wednesday, June 18, 2014

Breathing Update

I have been having severe abdominal pain and breathing problems for quite some time, but it has gotten a lot worse lately. I tested negative for endocrine infection, so something else is going on.

I have been having so much trouble breathing at times, I feel like I'm smothering. I've burst into tears more than once from the pain of simply trying to take a breath, and I often can't finish a sentence or phrase without losing air altogether and feeling faint.

I finally noticed that it is a lot worse after eating or drinking, and I put two and two together. I've had a hiatal hernia for over a decade, and it has most likely become obstructive.

What makes this so difficult is that I cannot safely have surgery. With my HKPP weakness being so severe already, the risk of death under anesthesia seems very high. I have no idea what's about to happen to me.

Now waiting for the GI referral from my Internist. I'll keep you posted.

Wednesday, June 11, 2014

Breathing would be nice

I feel like I'm dying. I'm having way too much trouble breathing lately. I can't finish sentences sometimes without running out of breath, and sometimes it is so painful that I burst into tears. I don't know what to do.

Tuesday, April 15, 2014

Mom

My mother has passed away. My stepfather said that over the weekend, her digestive system came to a halt and she couldn't speak or swallow. She had cancer and went downhill fast, but I'm in shock over how sudden this was. I didn't even get to see her or say goodbye.

With the exception of cousins I've never met, I'm the only one left in the family with evidence of HKPP (so far). Just making sure I document it here.

Tuesday, April 8, 2014

MDA Appointment 4/08

I met with the MDA and the Neurology team about what to do next. Discussed my swallowing issues, shortness of breath, migraines and nausea, the horrible year with paralysis I've had so far, and the level of pain that makes me want to be shot in the head. Told them about the mood swings and meltdowns caused by the acetazolamide. Nothing to be done about it as anti-anxiety meds cause paralysis (already tried them). Also can't get off the acetazolamide without falling back into a nearly constant state of paralysis (tried that too). The only pain med that I am aware of that I can safely take is ibuprofen (which my renal doc forbids, but I'm taking it anyway).

The new doc who spoke with me first was very interested in my genealogy, so I want to dive into my mother's family history and see if I can pinpoint their origin in the UK and France. New doc also mentioned albuterol for the breathing and swallowing issues, and that was shot down because it is a dangerous paralytic trigger. Steroids are potentially deadly with Familial HypoKPP, as are many other medications. It would make things worse instead of better.

I remained a lot more calm than I thought I would. I struggled not to melt down, but I managed not to. I have never acted like that in my life before recently (not even my ex-husband heard me say a cuss word - ever) but I am a freaking disaster on these anti-paralytic meds. They cause some pretty harsh chemical changes in the body, but I unfortunately have no choice but to stay on them if I want to survive and have any quality of life.

HKPP is a no-win situation, and everybody knows that. Angie with Mississippi MDA is great, and she is recruiting Alabama MDA to help me obtain pulmonary/sleep testing as well as a power chair at some point. I have to contact Shae in Birmingham to follow up in a few weeks. In one week, I'll see my local primary physician to bring him up to date, then back to Jackson for Renal Clinic in June.

Tuesday, March 25, 2014

Medical records for Germany

UMMC agreed this week to mail them to me...thank goodness. That will save a little time. In the meantime, I'm gathering the records I already have in my possession from other doctors, as well as my updated history and triggers.

Will keep everyone posted.

Saturday, March 22, 2014

Long Overdue Health Update - 3/22/14

I'm having a hard time blogging, if that wasn't obvious. Things are extremely stressful at the moment, and I'm still trying to recover from the 2014 body infection and paralysis of doom. At any rate, I'm here with a boring update.

Three weeks of fevers, three infections, three antibiotics, loads of cough and cold meds, an ER visit, major paralysis including my digestive tract (lost around 7 pounds), a trip to the docs in Jackson, increased HKPP meds, and I am finally...FINALLY...beginning to snap out of this nightmare. I am back on a walker and even driving a little bit. I managed to attend a meeting today in a nearby city before coming back home and crashing into bed with a migraine and severe weakness. I'm going to have to take my recovery a little easier I guess, but it's hard when I have so much to do. I have been down for literally seven weeks. I was bedbound nearly the entire time, and my aunt has come over each day to help me. She still is, because I'm still unable to do things like laundry or cook a real meal other than using the microwave or toaster oven (which is better than nothing, I realize). She has been a lifesaver, as usual.

My aunt and uncle took me back to Jackson for an appointment last week. I was in a wheelchair, and we decided that in order to bring me out of my prolonged paralytic state, potassium and Inspra needed to be increased. It appears as though I need the increased meds just to be able to function for a couple hours a day. The rest of the time, I'm here in bed trying not to lose my mind. Next week, I'll go to the local clinic to check my potassium level, then it's back to Jackson in April. I'll finally pick up all of my medical records at that time and send them to Germany as well.

I'll try to get back on track here soon. Thanks for hanging in there with me.

Friday, March 14, 2014

New York Times Article: Saving A Future For Those With Disabilities

Saving a Future for Those With Disabilities
By THE EDITORIAL BOARD
MARCH 13, 2014

Back in the old days, when Congress did crazy things like pass legislation, a sensible bill like the ABLE Act might have sailed to the president’s desk. But today, even with a long list of positives — it’s a good idea, solves a pressing problem and has lots of bipartisan support — it’s not a slam dunk, because there’s always a way in Washington to squelch good ideas, especially those that involve tweaking the tax code and spending a little money.

The Achieving a Better Life Experience Act would be a boon for people with disabilities, and for their parents and other caregivers. It would allow them to put money into tax-deferred savings plans, like the 529 plans that parents use to save for college, to cover expenses like education, housing, transportation, therapy, rehabilitation and assistive technology. Advocates say the accounts, called 529-ABLE plans, would be easier and less expensive to set up and maintain than the trust funds often used for beneficiaries with disabilities.

Such accounts would go far to ease the worries of parents who have children with disabilities that require intensive, expensive forms of care. They would also be particularly useful in fostering a saver’s independence. One advocate who has lobbied strenuously for the bill, Sara Wolff of Moscow, Pa., makes that case. She works in a law office and volunteers with the local office of the Arc, the national organization for people with intellectual disabilities. Ms. Wolff has Down syndrome and receives Supplemental Security Income benefits, but cannot accumulate more than $2,000 in assets without becoming ineligible for that aid. A 529-ABLE plan would allow her to work and save and keep those benefits.

Questions about the future of a child with a disability can be a deep source of anxiety, if not anguish, for parents who wonder what will happen after they die. As people with disabilities live longer, it’s only sensible to help them secure their futures. At last count the bill had 342 co-sponsors in the House and 66 in the Senate. That is well beyond a critical mass of support; it just needs a vote. Supporters have been trying for seven years to get the ABLE Act passed. Here’s hoping this is its year.

Monday, February 10, 2014

23 and Me

Since I've received questions about 23 and me, I wanted to update that I unfortunately did not order the kit before the FDA shut down the medical risk portion of the testing. I was short on funds, so I lost out.

Sick in bed again with either the flu or bronchitis. Will update the blog soon to bring you up to date. I appreciate all of the messages, even though I haven't responded to everyone yet. I apologize for my current inadequacy.

Talk to you soon.

Friday, January 17, 2014

Rough start to the year...

I apologize for my frequent absences, and I'm sorry to those I have not replied to yet via email. I have to take breaks sometimes, from all of this. I will never get a break from Periodic Paralysis, but I go through times when I have to distract myself in order to keep my sanity. The last month or so has been one of those times.

A sweet friend died unexpectedly on New Years Eve, and I became ill shortly after with an ear infection, fever, chills, and of course HKPP. I've been bedbound ever since. I managed to continue tweeting and facebooking from bed, but haven't done much else.

I've been on an antibiotic, and anyone with HKPP knows how difficult it is to function on one. Antibiotics inhibit neuromuscular transmission, so people with muscle disease are bound to feel negative effects. As of today, I'm up and around a little. On the walker, mostly just making sure I eat and take my meds. Then back to bed.

The doc who saw me said my left lung sounded fine, but that she heard very little coming out of the right one. This may explain why I feel like I'm being stabbed through my chest and back 24/7 and feel like I'm never getting enough air. They confirmed that it wasn't pneumonia, so I've requested a referral to a pulmonologist. Still waiting to hear back about it.

My mother has withdrawn again, refusing to talk to her cousins about HKPP like she said she would, and doesn't want to discuss it. So needless to say, back to the drawing board with the family. And I'm also having issues obtaining medical records to send to Germany. Being a 7 hour round-trip from my hospital is a problem. So is the incompetent medical records staff who ignores messages and loses important paperwork. Whatever. I'll take what I can get and get it to Germany when I can. I just hope the researchers don't give up on me before that happens.

Current Mood


Sorry for being a downer today. The rest of the year will be better. I look forward to it.