Friday, March 18, 2011
What about enzyme replacement to treat AIP?
"Porphozym by Zymenex"...
I sent an email to one of the top AIP Specialists in our country asking him this question.
He responded within 24 hours!!!
What a thoughtful person.
His reply basically stated that "Porphozym" was found to be ineffective in clinical trials and was therefore never offered as treatment to the general population.
IV Hematin remains the treatment of choice for AIP.
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!!
It is a little harder for people with AIP.
My first experience was awful! I was absolutely miserable and stayed unwell over a week!
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
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.
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!
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!!!
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!
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.
Chronic Auto-Immune Thyroiditis with benign nodules...
intermittent episodes of Hypo-Thyroidism and even occasional Thyroid Storm if manipulated/ palpated (physically touched)
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.