Heavy Metal Cells: Human Bean Bag

Today is my second day in bed, out of work and out of commission.
Two days ago my "Colicky- Cranky" Porphyria began to transition into something a little more...
Now my body feels as if every cell has become a heavy-metal-pellet and I am, in a sense, a giant 10 ton human bean-bag!
Wow what a picture huh!
My limbs feel so heavy to lift, my body feels so weak I drop everything and can only walk short distances without rest... showering leaves me feeling as if I ran a 10k...
I slept almost 24 hours straight and the nausea, eye, ear, limb and joint pain are beginning to subside... Gatorade and Quiet, Dark resting spaces help a lot! Phenergan, Naproxen and Ativan have also helped. I have found that chocolate, peanut butter, oatmeal no-bake cookies are great for calming AIP too!

I saw this flare-up coming... I have been juggling life at top speed...feeling OK...not taking rest periods...becoming increasingly tired...until I became so fatigued that I couldn't even think straight. The world around me sees me doing "it all" and pushes harder...expects more and more... they can't see what happens on the inside...and sometimes they don't understand when an Acute Phase of Porphyria strikes...
They can lack compassion and become frustrated with me...
I can become frustrated with my own body...not performing the way I want it too...
Again...another lesson in letting go of my drive to be in control.
I am NOT in control and I must accept that AIP and I will coexist for the rest of my lifetime...
I have to be kind to myself, listen to my body, nuture myself...stop the negative self talk and educate the world around me... forgiving the ignorant for their unkind harshness.

This is not a new challenge for me... just another learning cycle.

What about enzyme replacement?
Porphozym by Zymenex... it appears to be available in some areas of the world...
When I am feeling a bit better I will research it some more.

As a nurse, I feel that dextrose IVs are helpful and a fairly easy treatment intervention when oral carb-loading isn't working
but...
Heme Infusions via a central line can present a slew of added risks, if the attack is NOT at the life-threatening stage and if the healthcare team has Little to NO experience/knowledge about AIP!

I feel like there is no one to take care of me...and I get scared.
My AIP specialist is several states away and hasn't seen me since my diagnosing visit going on 3 years ago! He is more of a researcher than a direct care provider...he says any follow up questions I have can be handled by phone... I feel unimportant and lost medically...as I stated in a previous post, my hematologist of 4 years doesn't even know that AIP is a disease of the liver...he is too busy to read the educational literature I provide for him.

Thus my quest to create the role of Porphyria Disease Management Nurse...so that others like me might have someone to talk to...a resource...an educator... Diabetics (another type of metabolic disease) have this type of system with the DNE (Diabetic Nurse Educator).

The American Porphyria Association does a terrific job of providing printed educational materials and advocating for Porphyria Sufferers in general... but due to liabilities cannot veer too far into medical advisement...

Well... I am getting tired out and need to rest...more thoughts to come!

Colicky-Cranky Porphyria

Although my official diagnosis is established as Acute Intermittent Porphyria, I propose that my condition is better known by me as...
"Chronic Colicky-Cranky Porphyria"
My "Acute" times are truly "Intermittent"...but long episodes of dis-ease, generalized ill-feeling, mental/emotional fragility, poorly defined but ever present pain, GI dysfunction, nausea, headaches....and such can last days...weeks...even months.

It is as if my internal environment just feels very "Colicky & Cranky".

*Quiet, low stimuli environments really help but in REAL Life with kids, careers and spouses this intervention is hard to arrange.
*Ativan, Naproxen, Phenergan and Ginger help a lot...but can cause significant drowsiness...caution is needed when health care career and child rearing responsibilities are to be considered.
*Prayer and Spirituality are comforting
*Calls to loved ones helps
*Writing/ Creative Expression helps vent some of the "yuckie feeling"
*Exercise as tolerated definitely helps...but too much and everything gets MUCH worse...balance custom to the situation is key.
*Strict adherence to my nutritional plan helps keep extra health stressors at bay so my body can focus on calming it's current challenges
*Extra Sleep!!! Is one of the best treatments I know.
*Being kind to myself...hot cups of tea...pretty flowers...warm fuzzy blankets...

Book Review

The Gerson Therapy: The Proven Nutritional Program for Cancer and Other Illnesses
by Charlotte Gerson and Morton Walker D.P.M.(Oct 1, 2001)
I read this book about 3 months ago...
Pondering the "Gerson Therapy"...
Dr. Max Gerson, long deceased, is said to have studied the impact of food/nutrition on human wellness. In the book Charlotte Gerson (Max's daughter) presents her father's research promoting the use of juiced organic fruits and vegetables in combination with coffee enemas to promote improved immune functioning and in effect improved health. She offers many individual success stories to validate the theory.

The book felt somewhat like a promotional advertisement for "The Amazing Gerson Therapy", but the actual theory itself is completely believable. We are very much a product of what we put into our bodies... garbage in...well...disease happens!

The one thing that I remain "hung up on" is the use of coffee enemas...
Not sure where I stand on this idea...
Although Charlotte gives a convincing argument for the healthful benefits of this intervention...it seems pretty far off the path of traditional medicine (not that that is always a bad thing).

I plan to investigate this thought a bit more.
I am not sure how an Acute Porphyria liver/GI system would respond???

Taking Action Steps

On December 20Th 2010 I changed gears and decided that I needed to help educate physicians about Acute Porphyria...

This is the letter I wrote and distributed to all 40 Emergency Room Doctors, Physician Assistants and Nurse Practitioners at my Regional Hospital...

Dear Fellow Health Care Provider,
Due to personal experience with Acute Porphyria, I feel a strong motivation to improve Porphyria awareness; therefore improving the quality of life for those touched by it. Please read the attached information and complete the online CEU (http://medscapecme.com/case/acuteporphyrias)(allow about 30 minutes).
I have also put together a more comprehensive information packet and will leave it with the Medical Secretary if you are interested in reading it.
Thank you in advance for your professional investment.
Best Wishes~
T. Suzanne Jaynes RN,BSN
Emergency Department Care Coordinator

Each Health Care Provider (HCP) received a cover letter and pre-printed education materials from the American Porphyria Foundation.

Unfortunately, I have not received any feedback to date.

My next intention is to create similar packets for other HCPs in the community.

Narrative Note

Well my original plan was to make daily AIP Neuro-assessment entries for one full year... but the plan has changed.
I began feeling like the rote nature of the templated daily entries wasn't as expressive as I like to be... I started feeling like documenting was just another daily chore...no depth..boring...
Then I got a call from my father... "The Oncologist said dad had two to four weeks left to live"... that came like a "sucker punch" to the gut!
At first I used denial to cope... then I packed my bags and it was off to New York...where I was blessed to spend the last 17 days of his life helping him navigate the transition from this world to the next. My last (and final) two templated entries were made during the initial days of my stay with dad.

Any "spiritual" uncertainty I felt prior to this experience...
disappeared as I gained insight into death and the dying process.

Dad didn't want to go yet...he was only 59 and wanted more time...
during his last 5 days...dad stayed right on the very edge of this world and the next...as if straddling the two...one leg ice cold...and one leg blazing hot...no food...no water...a body consumed by ammonia and other noxious toxins...yet a strong will to stay connected with the ones he loved... medically his lab values were off the chart... medically there is no comprehensible way that he could remain coherent... he lay in the hospital bed unmoved...breathing in ways that triggered our anxiety...after hours of silent disconnect...dad would "pop-in" at just the right moment...puckering up for a kiss from his loving wife...vocalizing words of endearment in response to personal whispers and gentle touches...then on December 7Th he left this world completely.

For me...
Spirituality, Medicine and Emotion are now "all tangled-up"... in my mind...in my "heart"... in my very "soul".


The Impact of a Porphyria Diagnosis

The very moment that my father's hospital bed was delivered, I received a telephone call... my parents' DNA testing had been completed...the results were in...
Dad had AIP!
So many feelings and thoughts were speeding through my mind that day...
Why hadn't his doctors ever tested him?
He had taken so many, many medications that had made is health status worse and in relation his life shorter (he had seizure disorder due to lesions on his brain)... If he had been diagnosed earlier he would have made different treatment choices and been much healthier.
I was very emotional and my own AIP was in a colicky state.

Today...after digesting it all for a few weeks...
I accept that we are all human, and therefore we are all mortal...
nothing can change that...
The RN in me still feels strongly that physicians have a responsibility to improve their knowledge deficit when it comes to Porphyria...
Porphyria Diagnostic Testing...
Porphyria Medical Case Management...
and Porphyria Treatment.

So no more templated AIP entries... only descriptive entries that use words like brush strokes to paint the picture of my meaning.

Daily Post

 Neuropathies (central, autonomic and peripheral)- ok
 GI- ok
 GU- ok
 Muscular- ok
 Immune- ok
 Cardiac- ok
 Respiratory- ok
 Psychiatric- calm, melancholy
 Sensory- ok
 Pain/Comfort- ok
 Sleep/Rest- fair night's sleep... concerned about dad
 Self-Propelled Disease Management:
- good nutrition

Daily Post

 Neuropathies (central, autonomic and peripheral)- ok
 GI- ok
 GU- ok
 Muscular- ok
 Immune- ok
 Cardiac- ok
 Respiratory- ok
 Psychiatric- calm, melancholy
 Sensory- ok
 Pain/Comfort- ok
 Sleep/Rest- fair night's sleep... concerned about dad... tired
 Self-Propelled Disease Management:
- good nutrition