Colonoscopy

At 9am this morning I was wheeled via stretcher to the procedure room for a colonoscopy.
At 41 years young I had my first colonoscopy 3 years ago because my father struggled with Cancer that began in the colon.

I am glad I went 3 years ago... they found and removed an abnormal area.
I didn't need to return for 3 years!
Now...after today... I am cleared for 5 more years!!!
The Colonoscopy is a great Cancer prevention/ early detection tool that I promote Whole-Heartedly!!

But...
It is a little harder for people with AIP.
My first experience was awful! I was absolutely miserable and stayed unwell over a week!
But...
I didn't know that I had Acute Porphyria back then!

My AIP Colonoscopy Protocol:
1. Schedule the procedure first thing Friday morning (if possible)
2. Take Thursday and Friday off from work (and make sure the weekend is clear of obligations)
3. If I still had a menstrual cycle I would try to schedule the procedure the week or two after menstruation ends
4. I use a "Dulcolax" and "Mirilax" colon "cleanse" process outlined by my Gasteroenterologist
5. The day before the procedure I am only allowed "clear" liquids...so I drank GREAT quantities of blue "Gatorade" to keep my carbohydrates from dipping too much... this also helps in avoiding dehydration
6. I thoroughly went over my AIP diagnosis with the GI doctor and the Anesthesiologist... giving them both printed literature I ordered from "The American Porphyria Foundation"
7. I determined ahead of time which medications I would be receiving and made sure that they were AIP "SAFE"
8. The nurse started an IV infusion of dextrose as soon as I arrived
9. I packed a small meal for after the procedure
10. I went home and rested for the remainder of the day

Look Good = Feel Good

My Porphyria activity/ severity can be clearly gauged by how dark my under eye circles become.
I honestly look like Old "Uncle Fester" from the "Addams Family"!!!
If I am super sick...well it just doesn't matter how I look.
But...
If I can manage to remain actively engaged in life I do my very best to do so.
I try to invest in my appearance... I want to do the best with what I have... It keeps self esteem at a positive end of the spectrum and can lift spirits too!
So...
The dark circles are a real problem!
Hiding them with traditional makeup is impossible...I have tried them all,
I did some research and found a "medical make-up" called "Dermablend"...
I bought the "Quick-Fix Concealer" made for "severe skin flaws"...
It works very well and I don't see "Uncle Fester" when I look in the mirror anymore!!!

Filling In The Gaps

It has been over a month now since my last entry.
Life has been pretty challenging between then and now, but not all bad.
My Porphyria calmed a bit and we traveled to see friends and family...
Being with the ones you love is like food for the soul!

Then after returning home a respiratory virus took over our home and local community.
The curious connection I noted was that as I "cranked up" the juicer with fruits and vegetables I was able to "crank up" my immune system!
I never got the full-blown illness everyone else did, which was great!

However...

As my immune system was going "full tilt" my Auto-Immune Thyroiditis became a big swollen issue of dysfunction... super tired...super weak...severe headaches...no endurance...significant neck/ throat discomfort...then after about a week of this my Liver began to feel irritable and swollen...I began retaining fluid...especially in the abdominal area... Thankfully, I never developed any Porphyria issues this episode.

It appears that when my immune system is turned up...it protects me from illness, but attacks my Thyroid too.
Then my Thyroid in its dysfunctional state forms an "unhealthy relationship" with my already burdened Liver causing increased malfunction.
The "Up Side" to all of this is that as my immune system gears down after the threat of illness is removed... my Thyroid reduces in size and improves functioning.
As the Thyroid improves...so does its "relationship" with my liver...
then my Liver begins to return to baseline... the fluid stops accumulating... and I can re-engage in a semi-normal life...smile!
Some might ask...why didn't you go to the doctor???
I have... thus the complete Thyroid "testing / work-up"..several times.

Diagnosis:
Chronic Auto-Immune Thyroiditis with benign nodules...
intermittent episodes of Hypo-Thyroidism and even occasional Thyroid Storm if manipulated/ palpated (physically touched)

Treatment:
Tried Synthroid...did not tolerate... migraine headaches, increased "storming" (firing erratically... too high...then too low)and a whole-body sensation of being "squeezed"... these issues persisted for about three weeks after discontinuing the medication.

Final Medical Advice:
"Learn to cope with the episodes and monitor Thyroid/ nodules with regular testing"

So...I have been coping with these intermittent episodes for four years now.
My liver function has only been impacted during these "Thyroid Episodes" for about a year now.

My new proactive plan:
I found a Hepatologist and have an initial appointment next month.
I found a Thyroid Specialist and am in the process of setting up a new patient appointment.
I joined a Long Term Clinical Study: Acute Porphyria... my initial appointment is in about three weeks.

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.