Thursday, April 25, 2013

Action Potential and Periodic Paralysis Videos

Familial Periodic Paralysis is classified as a voltage-gate ion channelopathy, which means that ion channels do not open and close properly. This results in failure of the muscles' action potential when potassium leaks in and out of the cells inconsistently. Above is an animated video that shows what the process is supposed to be.

Below is a video produced by the Periodic Paralysis Association, which shows and explains the malfunction. Thanks to Dr. Jacob Levitt, Linda Feld, my friend Wade, and all who were involved in the making of this video.

Monday, April 22, 2013

A Request, Apology, and Thanks

With Hypokalemic Periodic Paralysis, salt, sugar, and many other common substances are potentially dangerous. Since there is no way to avoid them completely, life is a roller coaster and every day is a challenge to find the right balance, which is constantly changing. The best HKPP patients can do is avoid these triggers whenever possible, and face them when we have no choice. The list is wouldn't believe how extensive it is. It would be fantastic to be "normal" enough to not have to nit-pick about every little detail of every little thing I come into contact with, but most who live with this disease are required to treat triggers as allergies for the sake of survival and quality of life.

That said, when someone presents a suggestion to me (product, advice, etc) and my response isn't what you wanted it to be, I assure you there is a valid reason for it. It is not meant to offend you nor make excuses, and I ask that you try to respect me enough to take my word for it. You can say you're knowledgeable about the condition and you can think you're more intelligent than me...that's perfectly fine...but the fact of the matter is that I know my body more than anyone else, and I'm not going to allow something or someone to do me harm no matter how precious and pure the intentions are. I welcome all input, suggestions, and questions...don't ever hesitate to make them known. I am a nice person, and I don't bite. I promise to respond in truth, with respect, even if it's in disagreement, and thank you for your consideration. Because I sincerely appreciate it.

I chose a long time ago to make my journey public because I need to bring awareness to this rare disease...certainly not just for my sake, but for others too. People have found information about HKPP and learned about the condition because of my public rambling coming up in searches. People have thanked me for my honesty because it makes them feel less alone. People have asked me to continue to share my struggle because it encourages them to pray and be grateful for their health. If anything I say helps even one person, I dare to say the rants are worth it.

I'll be as mindful as my tired brain can be and do my best to spare everyone any major drama or TMI, but otherwise...this is my life right now. Take me or leave me. If you are a new reader, I welcome you and I apologize if I have scared or upset you with my posts. I don't talk about these things to create anxiety in people, but I have friends who love me and truly want to know what's going on. Some even take time out of their day to pray for me, and that matters. I personally think it keeps me alive.

To those who care and have been here for me, thank you will never be enough. I do not take you for granted, even when I'm silent. What I would give to be able to repay the kindness...words cannot express. Thanks for putting up with me.

(Originally posted on Facebook)

Saturday, April 20, 2013

April MDA and Renal Update, Fighting, and Gratefulness

God, how depressing is this blog? Ha. I just reviewed the last entry and it made me laugh a little bit, honestly. I probably shouldn't post anything on the internet when I'm feeling so badly, but I've always been one to keep it real.

Hard to believe I've been blogging online for over 10 years now. It has been a lonely road with this disease, but I feel worlds better since finding others who know what I'm going through. Having a mother who has the condition but refuses to acknowledge it is not easy. Had she been willing to pursue diagnosis and treatment, perhaps we could have dealt with this together. She has made it clear, though, that she wants to be left alone. It's her choice, and there is nothing I can do to change her mind. I just hate to see her (and her husband) with reduced quality of life as a result.

I'm forever grateful to have my aunt and uncle. They stepped in when the rest of the world stepped out. I would have never made my way to the docs in Jackson had my aunt and uncle not decided to help. Thank you thank you thank you.

I'm grateful for the support groups on Facebook and the PPA/PPI Listserv. What I have learned there has saved my sanity, if not my life. In spite of the occasional drama that rears its inevitable head, it is imperative that these support systems remain. I hope the powers-that-be realize how much they are needed.

I also can't say enough about the doctors in Jackson. I become discouraged often between visits, but I am always encouraged again after I've talked to them. Having the MDA team and Dr. Fulop on my side is a blessing that I do not take for granted. Dr. F continues to deal with my ups and downs with the utmost kindness. I always arrive to the office feeling sad, and end up laughing about something by the time I leave. God knows I find humor wherever I can; sometimes I think it's the only thing that keeps me going. I wish I could clone my doctors and send them to every hospital in the world.

My heart breaks when I think about my friends with HKPP who still can't find someone who listens or takes the condition seriously. I lived that nightmare for 20 years, and I am a broken person for it...albeit, stubborn and perseverant. When things became much more serious in recent years, I launched into "sink or swim" mode and became what my friend Katherine now calls her "snarky, loving, tender, angry soul sister". That cracks me up and makes me a bit sad all at the same time. God knows I've never wanted to be the fighting type, but I've been forced to be and it's a big reason why I'm still here. I've had some terrifying times...moments when I didn't know if I would see tomorrow. I've learned that living this way can alter one's perspective about life. I am an old soul. I'm very tired, I get cranky, maybe even angry, yes; but I'm also quite passionate, empathetic, and grateful as a result. I pray with all my heart that the latter triumphs over the former. I want everyone with this condition to never give up on finding the medical attention you need. Get angry and fight if you have to, but whatever the case, hang on to hope and don't surrender. If I can do it, I know you can too.

My appointments in Jackson went well, with the exception of my legs paralyzing half way through the day. I was in a wheelchair by the end of lunchtime (lunch was at Babalu, by the way, which was amazing). I met the CNP at the MDA clinic, as Drs Veda and Witt were out of the office. Carolyn was great. We discussed my current condition and medications, and she ordered A1C and vitamin blood work. We agreed to put me back on Vitamin D 50,000 IU weekly, as I seem to be deficient otherwise. I'm still waiting on B12 results, but my A1C already came back and it was fantastic at 5.7. This was a bit of a surprise, as I have not only gained 15 lbs over the past 2 years, diabetes runs heavily on all sides of my family. Praise the Lord, I beat the odds again. I met with Dr. Fulop and one of his students in the late afternoon. I told them about the acetazolamide, and there was no hesitation that I should remain on whatever dose I can tolerate. I admit I have mixed feelings about staying on it because, God help me, I'm such a miserable person on this drug. Nevertheless, I agree wholeheartedly that I should continue with the regimen due to the slight reduction of paralytic attacks. We also discussed Inspra and agreed to double it. I'll let everyone know how that goes. I return to Jackson in July.

I think I forgot to tell Dr. F about Dr. B, who has rewritten some of the scripts (perhaps he realized this, when I didn't ask for refills) so Medicaid would cooperate. They have a problem with the fact that Dr. F is in Mississippi, even though the hospital itself accepts Alabama Medicaid. Frustrating situation. Hopefully it will all work itself out. My next appointment will be at Dr. B's office in a few weeks, where we'll discuss the latest news from Jackson and take a look at labs.

I was going to tell my doctors about Alexander's death, but I knew I couldn't get through it without sobbing, so I didn't. Maybe next time I'll be able to talk about him.

I still haven't caught up on the blog here, and I apologize. I am going to try to remedy this soon. Thanks for reading my ramblings, and don't hesitate to ask questions if you have them. I realize I have the vast majority of my readers on Facebook and elsewhere, but by all means comment here if you prefer.

Quite a few articles coming soon.