Friday, July 13, 2012

Chronic pain and sodium channels

I found this article to be worth sharing. I know many of us with Periodic Paralysis deal with pain 24/7, while it doesn't seem to be an issue for others. I can't help but wonder if all of us with PP who have severe, chronic pain happen to have the SCN4A or other sodium channel mutation.

As I've said in the past, I don't know what mine is yet, but I hope to be one of the lucky ones to find out someday. I feel it's relevant to note that I have monitored my sodium intake over the past few years (I didn't know sodium was an issue before 2009), and I found that not only does sodium cause me to become weaker due to potassium shifting, but it also greatly increases my chronic pain level. I think it is also important to mention that sodium (glucose as well) causes potassium to shift even in someone who does not have Periodic Paralysis. For that reason, it has crossed my mind many times that chronic pain and chronic fatigue syndromes could be related to sodium.

I will share more at a later date, but wanted to go ahead and give you this article today.

http://www.molecularpain.com/content/4/1/37


Talk to you soon. I think I responded to everyone who emailed me over the past few weeks. If I didn't, please feel free to shoot me another email and fuss. I'll get right on a reply.

Thanks,
Kelli

Thursday, July 12, 2012

FDA Safety and Innovation Act Passes In Washington

My email inbox has positively exploded with press releases about the FDA Safety and Innovation Act, which was signed by the President on July 10th. This law is vital to people like me who have rare, life-threatening conditions that are unusually difficult to treat. There are no words for how disheartening it is to know what you need to sustain your life and have it denied to you because it's not formulary, conventional, or convenient. I appreciate all who brought this Act to pass, because it may give me and others a better chance of survival.

---

FOR IMMEDIATE RELEASE - Obama Signs FDA User Fee Legislation Bringing Hope to Rare Disease Patients

EveryLife Foundation for Rare Diseases Applauds Congress for Including Provision to Empower the FDA to Accelerate Approval of Lifesaving Treatments

July 10, 2012, Washington, DC – Yesterday President Obama signed into law The Food and Drug Administration Safety and Innovation Act (FDASIA), S. 3187, bipartisan legislation that will spur the development of lifesaving treatments for 30 million Americans suffering from rare diseases.

“We are thrilled the language to improve access to the FDA’s Accelerated Approval pathway for rare diseases has been included in FDASIA,” said Emil Kakkis, MD, President, EveryLife Foundation for Rare Diseases. “We wish to thank Representatives Cliff Stearns (R-FL) and Ed Towns (D-NY) for being champions for the rare disease community.”

Stearns and Towns first introduced Unlocking Lifesaving Treatments for Rare Diseases Act (ULTRA) to empower FDA to use all the science available for allowing surrogate endpoints in clinical trials for rare diseases to determine whether a drug is working, significantly decreasing the development time and cost. Stearns and Towns later introduced Faster Access to Specialized Treatments (FAST) Act that improved Accelerated Approval for life-threatening diseases while maintaining high safety and efficacy standards.

FDA’s Accelerated Approval has been successful in getting treatments approved for cancer and AIDS patients, but has been essentially unavailable for rare disease treatments. There are currently fewer than 400 FDA-approved treatments for nearly 7000 rare diseases. Investment and interest in development will surge for rare diseases if there is access to the Accelerated Approval pathway.

“We would not have been successful if it were not for the great work of Energy and Commerce Chairman Fred Upton (R-MI), Biotechnology Industry Organization (BIO), and more than 300 patient organizations that advocated for improving the FDA’s regulatory process,” added Kakkis.

FDASIA is the culmination of more than a year of negotiations between industry and FDA and includes the reauthorization of the drug and device user fees.

The Foundation will host its fourth Rare Disease Workshop on “Developing Guidance and Policy Recommendations for Accelerated Approval in Rare Diseases” on November 15th in Washington, D.C. FDA, NIH, industry and academic scientists are invited to participate.

The EveryLife Foundation for Rare Diseases is dedicated to accelerating biotech innovation for rare disease treatments through science-driven public policy. We can do more with the science we already have and bring life saving treatments to millions of people suffering from rare diseases.

------------------------------------
Contact: Julia Jenkins, Director of Public & Government Relations Email: jjenkins@EveryLifeFoundation.org Phone: 415-884-0223
www.EveryLifeFoundation.org

Tuesday, July 10, 2012

The Recent Hospital Stay

I'm finally getting around to talking about my recent experience in the hospital. This is going to be extremely long-winded, but I want to make everything known in case it is helpful to others with HKPP.

I woke up at midnight, choking badly. I was having an episode and my esophagus was affected. I managed to cough. Acid came up and went back down. I was unable to swallow, however, and I feel like it went down the wrong track. I gagged and coughed for a long time. I could not gasp for air, but only take small, shallow breaths. I sat up all night, hoping to improve, but I didn't. I could not swallow, take deep breaths, or speak.

If you've been following for the past year elsewhere, you know I have begun having serious episodes that involve my esophagus, and vocal cord paralysis is common for me now. But this time, things were not getting better and I had never been unable to swallow for such a long period of time. I emailed my aunt and uncle to let them know that I would probably have to go to the ER. It turns out their power was out, and they didn't get the email. I would have called, but I had no voice at all...couldn't even whisper. Sent a text message to my brother, and he called them for me.

In the meantime, I got dressed and printed out information from HKPP.org. I also printed out my personal medical information. Around 8am, my uncle took me to Providence Hospital ER. On the way there, I wrote out flash cards and notes for the staff. I had a ton of info...everything I could possibly think of.

When I arrived, the staff tried to force me to speak and wouldn't accept notes. I mouthed to them what was happening, and of course they had no clue what I was saying. So I sat in the ER and waited until it was my turn, still unable to speak, swallow, take full breaths, or walk. I'm not sure how long I waited before being seen...I decided not to be OCD in timing them. :)

The doc was nice, and he read my lips well. Thankfully, he took me seriously and ordered blood work and an X-ray to check my lungs. The X-ray didn't go well because I couldn't take a deep breath and hold it. We tried twice. The portion of my lungs that the tech could make out was clear, so the doc moved on to other tests and (I discovered later) began orders to admit me to the Intensive Care Unit for Hypokalemic crisis and potential Pulmonary Embolism (DVT).

While waiting for stat labs, I was given IV potassium in 1/2 saline. I told them that saline was dangerous and must be avoided, but if Mannitol was not available, diluted saline could be used as a last resort. So, that's what they did.



I warned the doc that my potassium level would be in normal range. I cannot stress this enough, friends, that someone with Familial Hypokalemic Periodic Paralysis has episodes in very normal range. The episodes are not caused by true Hypokalemia, but the actual downward shift...no matter how big or small. I made sure to explain this (the best way someone without a voice could) to the doc. He said "you know more about this than I do, so you tell me what to do". Can I keep him?

In spite of this understanding, the ICU rejected the doc's orders for admission due to the fact that my potassium level was in normal range. So, he ordered the next best thing - Telemetry.

I was admitted to the Telemetry floor, where I was placed in a very defective DVT-prevention bed (more on that later) and hooked up to a bunch of monitors, including a remote controlled monitor that I had to wear on my chest. I was almost immediately given a Lovenox injection in my stomach. They were not taking any chances of me dying of a blood clot, that's for sure. That shot might as well have been an ice pick. Heaven knows I have had a hundred+ needles in my life, so if I say it hurt, believe me...that freakin' thing HURT.

The problem, as those of you with HKPP will already guess, was the potassium. I received another IV that evening and into the night. They got it wrong at first, and were giving me normal saline. I had to ask tell them to switch to 1/2 saline or Mannitol...good thing I double-checked. They changed it out, but unfortunately, that did not help. Would you believe after two potassium IVs, my level did not raise at all? It did not raise...AT...ALL. Everyone, including me, was floored. They nixed all fluids until they could decide what to do.

One thing that slowly began to improve was my voice. I managed to start whispering again on day two.  I was still not able to take a deep breath, but I wasn't in visible distress and remained calm. They checked my oxygen, blood pressure, and heart rate periodically, and the numbers were acceptable.

I tried throughout the day to swallow, to no avail. The doc called in a gastro specialist, who ordered a swallow test. Unfortunately, it wasn't scheduled until the next day, so I was stuck in a defective hospital  bed that continuously (incorrectly) inflated and deflated. I kid you not, it would deflate, then immediately inflate, then deflate again, then inflate again. It never stopped doing this. NEVER. I had also developed a migraine but was given no fluids, food, potassium...for 24 hours. Bad idea. Calm, perseverant Kelli became dehydrated, agitated Kelli. I sent a text to my aunt that I was going to hurt somebody. She sent my uncle up there. In the meantime, a sweet student nurse came in, saw me upset, sitting up in a chair, and asked what was wrong. I unloaded as much as a weak, whispering peacemaker from the deep south could. I told her I had been ignored for 24 hours and I was FED UP. No fluids, no potassium, no food, broken bed, nobody was listening and I was LEAVING. She was like oh no, don't leave. Please don't leave. I told her that if they did not take the information that I brought and follow HKPP protocol, I was going to start making phone calls. She said she would talk to the doctor ASAP and try to find out what was going on. I told her there was a serious failure to communicate and I was mad. She remained kind and calm, helped me shower and dress, and tucked me back into that [insert-swear-word-of-your-choice-here] bed. I still hate that stupid freaking bed. For the love of God almighty, I would have thrown that thing out of my 10th story window if I could have.

Day three was underway. The docs were discussing intubation, which was unfathomable to me (and against my Advance Directive, actually...more on that later). I was still being treated for DVT via Lovenox in the stomach. I may have been dying of Hypokalemic Periodic Paralysis right under their noses, but gosh darn it, my blood flow was stellar. :)

I was so over it, and wanted out of there. I asked for water so I could try to swallow again. I managed to get down the tiniest sips imaginable. I still couldn't produce a good swallow, but it was enough to allow trickles of water to go down the right pipe. Coughs and gagging galore, but it was finally happening at a snail's pace. I had the swallow test that evening. By then, I could swallow small-sized sips with accompanying coughs. Although labored and involved a bit of drama, I passed the test. So on the evening of day three, I started drinking a little bit of water, juice, and chicken broth. I was also able to speak with a loud whisper by then, but was still in pretty rough shape otherwise.

My potassium fell (still in normal range) and I began seeing a decline again by the time the staff changed shifts. I was on the edge of full-body paralysis, and getting very concerned because the pharmacy was still refusing to give me anymore potassium. A side note - I used to work in that very same pharmacy. I was in administration, in fact, but I chose not to ask to speak to the pharmacist. I probably should have, but I didn't want to cause trouble. I just wanted them to understand the condition and do the right thing.

Enter my hero - Nurse Cindy!!!

She was new. She read my chart and was enthusiastic and intrigued. She told me to tell her everything about the disease. We talked for a while, and I offered her the stack of papers that I brought. She is the ONLY nurse who accepted them. She took the info, read it, and Googled the condition further. I warned her and the other nurses that I was on the verge of another serious episode and something had to be done. She jumped on it...called the doc at home at 10:30pm and said "we can't let this happen". Since I could somewhat swallow again, the doc ordered the pharmacy to override their system and administer liquid potassium chloride as well as slow KCl. I was given 60 mEq at 11pm and another 40 mEq at 1am. This was repeated in the morning for a total of 200 mEq. I felt like death, people. It was rough.

But it worked. Day four, labs were drawn and my potassium level was 5.1, which was the goal. I could speak a little better, I could breathe, and I could swallow normally again. PRAISE THE LORD AND PASS THE BACON!

I ate every bit of the horrible breakfast I was given, drank more juice than I had consumed in at least two years, and resumed home medications. The doc canceled all further tests and discharged me, although I was given yet another Lovenox injection on the way out. Relentless, blood-thinning fiends.

I could not thank Cindy enough. I don't know what would have happened to me if not for her. The student nurse arrived later, helped me shower and dress again, and we talked for a while. I thanked her for being fabulous. She thought I was funny but I was dead serious. She handled me better than anyone else there.

Funniest moment of the week - the bed of EPIC FAIL. It finally crapped out on me in the middle of the night and deflated completely. Imagine me butt-first in a sink hole, trying to reach the call button, and calmly asking for someone to come rescue me. We all chuckled as it took two and a half people to pull me out. I say two and half because one person was half dealing with the bed and half dealing with me, so it makes sense in my stupid mind.

Oh, for the record...not one person in that hospital acknowledged the medical bracelet I was wearing the entire time I was there. Sigh.

I will follow this ungodly-long entry with a (short!) video blog to recap the medical specifics...what didn't work, what did, dosages, etc. I will also share my concerns for the future where episodes, breathing, swallowing, and Advance Directive are concerned. I know people have been wanting to know the details of my time in the hospital and I'm sorry I'm late in posting about it. I hope all of this is helpful to someone. If you have any questions, comments are open below. Or if you prefer privacy, email me at ugottafriend4life@gmail.com. Everything is confidential, always.

Sunday, July 1, 2012

Medical ID Bracelet

If you have Periodic Paralysis, I recommend a medical bracelet. Mine contains my name, condition, and a webpage URL that leads to detailed medical information.



Webpages are free if they are blog-based, like this one. I use Blogger.

Medical bracelets can be found all over the internet. You can search for one here.

I've been in the hospital. I will update the blog soon with my experience.

I will also respond to everyone's emails and Facebook messages this week.

Thanks for being here.