Wednesday, October 24, 2012

New Article: The Impact of Permanent Muscle Weakness


http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3476862/

Here is a fantastic new study that went on within the support group I am a member of. I was too young to participate, but it is worth reading if you have Periodic Paralysis, no matter what age you are.

My only major concern with the information I read here is the usual...that serum potassium levels are not always "abnormal" 
when experiencing an episode. My episodes and those of many with HKPP occur well within normal range. During most of my recent hospital stay in telemetry, my potassium was in the 4s but I was severely weak and could not swallow for three days due to a paralytic episode. The abnormal shift of potassium through the muscle membrane can be minimal and still do tremendous and potentially life-threatening harm. This confusion is why I was ignored and untreated/undertreated for so many years, and quite possibly why I am myopathic at a young age.

I would like to get some input from those who reported that irbesartan helps HyperKPP. I have HypoKPP and I've been on it for nearly nine years now. At one point, my doctor switched me to a different medication, and my condition declined significantly. I got back on it as soon as I figured out that it was a factor. I find it very strange that irbesartan potassium would help someone with HyperKPP. Would it then hurt someone with HypoKPP? I can't wrap my brain around that, so I would love to find more information.

Thanks always to Dr. Lehmann-Horn and his entire team for the continuous work they put into Periodic Paralysis research.

Saturday, October 20, 2012

Social Security Update: Approved


This is cross-posted from my personal blog. I was going to edit it so it didn't sound so despairing, but it is what it is and I really have nothing to lose in sharing how I feel about it. I'm angry and it shows in this entry. Fair warning. My disclaimer, however, is that this is only my story...it is not intended to discourage HKPP patients from applying for assistance. Everybody's abilities and circumstances vary, as I'm sure you are already aware.

My recent visit to the Social Security office was successful. With detailed documentation, and my visibly obvious condition, I was declared disabled effective immediately. Once SSA got back on track and processed my application properly, it was an open and shut case just as one of my doctors said it would be. I have Medicaid now and will give a review here once I use it.

That's the good news.

The bad news...very bad news...is that they have only awarded me $65 a month. They calculated this based on my current living arrangements (rent free, because hello...I'm broke). Forced to quit all of my jobs and drop out of college thanks to a debilitating disease that I was born with, and I get a whopping sixty-five bucks a month to live on.

I don't even know what to say to that. Doomed? That's a pretty accurate word. Once my aunt and uncle step out of the picture as caregivers, I assume based on the lack of consideration I have received since this began over 20 years ago that I will have no other relatives to turn to.

So what's next? Only God knows. All I know is that I didn't ask for this BS. Never have and never will. I have fought people tooth and nail to be believed in order to receive the medical help I needed to keep this disease at bay for as long as possible. Yet, here I am not even 35 years old at what feels like the near-end of the rope. I have battled my own body since childhood with one goal in mind - to be completely independent. I despise with every fiber of my being a life of relying on others to meet my needs, and at the present time there is no hope in sight of that ever changing. I will never accept it.

That is all I have to say right now. Again, I am simply sharing my personal journey and it is not meant to be a doomsday speech to anyone with this condition.

I will be back to explain, in detail, my social security application process in hopes that it will help others to get the assistance they are seeking in a timely manner.

Wednesday, October 10, 2012

What Are Channelopathies?


Familial Hypokalemic Periodic Paralysis is classified as a channelopathy.

Courtesy of WiseGeek.com:
"Channelopathies are diseases of the ion channels, structures allowing for the passage of ions across the membranes of individual cells. These conditions can also involve the regulatory proteins responsible for moderating the behavior of ion channels. In both cases, the channels do not function properly, causing disease in the patient. The nature of the disease can vary considerably with hundreds of different kinds of ion channels in the body, and treatment options are dependent on the channels involved and how the channelopathy manifests.
There are two types of channelopathies: congenital and acquired. Congenital conditions are genetic in nature and can be inherited or the result of spontaneous mutations. Acquired conditions, most commonly associated with autoimmune disease, occur later in life when the body begins attacking its own ion channels, incorrectly identifying them as foreign proteins. Channelopathies can cause both neurological and muscular disorders depending on the nature of the disease and the channels involved.
Migraines, some forms of epilepsy, cystic fibrosis, and myasthenia gravis, among many other conditions, can be associated with channelopathies. Typically, the patient experiences attacks in the form of paroxysms leading to temporary paralysis or other impairments. These attacks can grow more severe over time as the ion channels experience increasing levels of damage and become more dysfunctional. Patients may need mobility aids and other forms of support, along with medications to manage some symptoms.
Study into channelopathies is a complex and rich field of research in the sciences, especially since many animals develop similar conditions across species. Researchers are interested in identifying the origins and potential treatments of known channelopathies with the goal of assisting people who have these conditions. It is usually not possible to effect a cure, but patients may be able to manage the disease and its symptoms with medical support, and this can make it easier to live with a channelopathy, extending a patient's lifespan and keeping people more comfortable.
In families with a history of channelopathy, it can be advisable to discuss the situation with a genetic counselor before having children. The counselor can provide information about the likelihood of passing the conditions on to children and can also provide information about management of channelopathies to help people prepare in the event that they have children with diseases involving the ion channels. Genetic counseling can also be an opportunity to discuss other concerns with family history, such as histories of cancer and certain kinds of mental illness."

No references given on the site, but the information seems to be valid. I copied and pasted here so you didn't have to deal with the excessive ads and lame generic clipart. You're welcome. :-)



Wednesday, October 3, 2012

HKPP FAQ

This is not a real update. I just wanted to share the link to HKPP.org's FAQ.

http://hkpp.org/patients/hypokpp-FAQ

That site has been a life-saver, literally. I am forever grateful to PPI and PPA for the work they have done and continue to do to bring correct information about this disease to the general public.

I have it bookmarked on my desktop, so that when someone asks what I have, I am ready to share. Sad to say, some people are more likely to consider a random website they've never heard of than the person they're questioning. I think most of us have been there, haven't we? I know I have.

Feel free to print and share my article as well, if you feel that it may be helpful.

It's a shame that we find ourselves in defense mode so often. The information is out there now. There is really no excuse for ignorant mistreatment anymore. We're not the lazy ones...the skeptics who ignore the facts are. I intend to make that truth known until my last breath.

This snarky post has been brought to you by Kelli.