Monday, October 10, 2011

The Beginning...The 7 Month Journey to a Real Diagnosis

The Itch...  It was approximately seven months before Pemphigus Vulgaris became part of my every sentence, everyday.   This is when I first felt it... an itch.    It was during an overseas business trip and I was under the impression that the hotel room sheets were bad... or something.  Haha...   Little did I know then that my autoimmune system was quite excitedly creating antibodies that were attacking specific proteins in the skin and breaking the bond between skin cells -- thus the creation of a blister... or as I thought.... "Hmmm... I have a rash".

Do I have an Allergic Reaction? -- The First Non-Professional Diagnosis...  By March of 2011, I realized that I was getting "rashes".   This is how they worked.  I would first feel a tingle under the skin.  So, I could tell that I was about to experience the rash.  Next within hours I would see a rash.  Sometimes it was a straight line and other times it was a big round rash.   My thinking was that I was probably having an Allergic reaction to something... and let it go at that. At this point, rashes would appear every 4 to 5 days.

My Gums Bleed...  In April... the rashes were coming and going... and I was not paying any attention to them especially since It did not bother me too much.   By now, they would appear every 3 to 4 days.  Again, I did not really think too much about it.   But, when I visited my dental hygienist for a regular cleanup, I had several of my gums bleed.  Again... Did not think too much about it.  Rashes would again go away within 1-2 hours.

A Dark "Spot" on my Tongue... In May, 2011, I felt something on my tongue... like a rash or like a "bump" and when I investigated... it was like a dark spot on my tongue...  it was a little scarry but after it went away in a few hours, I never thought about it again.

"Rashes" More Frequent ...  By early June, 2011, my rashes were coming on a daily basis.... I was quite sure that I was having an on-going allergic reaction.  However... that did not explain my gums now getting worse and worse. 

A Visit to My Periodontist...  When my gums were bleeding really bad, I decided to visit my Periodontist... and initial assessment was that perhaps I was having stress related shingles as I mentioned to my Dr about the rashes as well.  After examining me, he said... my gums were kinda peculiar in that it appeared that my autoimmune system was attacking them... because they were somewhat flabby... not typical of standard gum disease.   He gave me some antifulgal medication and after that did not work, he highly recommended that we did a biopsy.   Yes!  He felt that it was some kind of Autoimmune problem....  Yes! he called it!

By late June, I was now experiencing lesions or redness in my throat and the inner walls of my mouth.  And they hurt when I ate...  When the redness was too much to bare, I decided to visit the Urgent Care and after a bunch of different test... I was told that I proabably had a bad case of sore throat!  Haha!  Nice diagnosis Urgent Care Dr!   Perhaps you should have spent more than 5 minutes with me.

Primary Care Physician Doing Their Thing...  By July 1, 2011, the lesions in my mouth were coming more and more regularly but not regular enough for me to show my Primary Care Dr when I visited him.  So, once again, we did blood work and a bunch of other Allergen tests.... Wullah!  They found it!  I am simply allergic to California Grass, Dust Mite, Cats, Dogs and a bunch of other environmental allergens....   What?  I have been an avid gardner all my life... and I never had lesions in my mouth!!!   Oh well... no worries... the Dr prescribed me the over the counter Allegra -- once a day... and I should be just dandy!

Hello!  I can't Eat!!!   By August 5, 2011, which also happens to be my Birthday, I was having a hard time eating anything solid... It was painful.... Of course the Allegra was not doing the job!   My rashes would come and go every day now... but again I was easily able to handle them... It was the lesions in my mouth that I was having a hard time with!   So... Once again... I insisted to see another Physician and this time... he referred me over to an ENT Specialist just to check out my mouth.

My ENT - My BIG Break!  On September 2, 2011.... I was seen by an Ear Throat Nose Specialist....  and one look and this Physician immediately knew that we had to take a biopsy....  He agreed with the initial assessment of my Periodontist... that it appeared to be something along the lines of an Autoimmune Disease...

In two week, we got the results of the biopsy on September 14, 2011:

In my follow-up to my ENT, he looked at the results and just from his face, I knew that this was not good.  He immediately put me on 20Mg of Prednisone (steriods) and set me up to see a specialist in Pemphigus Vulgaris at UCI in California in record time!  This ENT Rocks! Thank you God!   In the meantime, my ENT did a second biopsy for IMMUNOFLOURESCENCE for confirmation but it came back NEGATIVE!  What???  This was great!!! I don't have PV... :)    Well.. lets not be too sure... The best thing that my ENT ever did was to get me the GURU in PV in the region if not the Country!   Yes... I can't thank my ENT... DR. BRIAN PAUL BOYNTON enough.   He showed compession, care and a determination to get me to the right specialist that can take care of me immediately!  Thank you ... Thank you... Thank you... Dr. Boynton!

Dare to Eat!
By September 1, 2011, I was on liquid diet... well... on most part but on some exceptions I dared to eat!  Haha... pretty stupid of me.

On a couple of occasions I had some dorritos, french fries, chips and salsa and anything that was spicy and great tasting!  At one point I said... screw the mouth I am going to eat Popcorn!!!   That was really DUMB!  I had to spend more than 1 hour trying to get all the pop-corn remains out of areas in my mouth that I did not even know existed... my mouth was so sore and the walls were so broken up that food had got stuck inside the lesions...

Finally... A Confirmed Diagnosis
On September 20, 2011, I was already on complete liquid for 20+ days and had lost more than 8 pounds.  The pain was too much.  My visit to my a research Dermatologist from University of California, Irvine, was initially not convincing enough to the specialist that I had PV.   But... a simple blood work will do the trick to find out.   Here is what was ordered:

Enzyme-Linked Immunosorbent Assay Indirect Immunofluorescence (Lab Test Code: XPGUS)   and
Enzyme-Linked Immunosorbent Assay (Lab Test Code: XPGOID)

I had been taken off my Prednisone on September 30... to purposely flare up the skin rash so that another Dermatologist -- another wonderful Dr referred to by Dr. Boynton could do a skin biopsy to figure out what was wrong if it was not Pemphigus Vulgaris -- Remember the second mouth biopsy came out negative and my PV Specialist was not convinced I had PV and so I was not about to sit around while the labs were doing their thing.  So, I proceeded to try to figure out if something else was wrong with me.

Going off the Prednisone was NOT good.  Although I had been taking only 20Mg for 2 weeks, in 3 days, my lesions in my mouth got so worse that I could not even swallow any water.   I felt like I had food stuck in my throat or esophagus.  But I knew it was the lession and it was HUGE.   Everytime I swallowed it was terribly painful.   I was rushed to the Emergency Room and admitted.   The ER doctors thru an IV gave me more 200+ Mg of Prednisone over a 12 hour period.  After 12 hours, I began to feel that my lession in my throat was going away and in 1+ day it was gone.   My visit to UCI Medical Center in Orange, California was the best -- Everyone from the ER Docs, the Nurses, Aides and the Inpatient care was just fantastic!  I can't thank them enough!!!

They let me out on October 4... and I went straight to my PV Specialist... who shared the following results:

The Results on October 4, 2011:
by the University of Utah Department of Dermatology

Cell Surface Pemphigus antibodies
IgG: Positive, titer 1: 320 (H) Monkey esophagus substrate
         Positive, titer 1:80 (H) intact human skin substrate

ELISA Testing
IgG desmoglein 1 antibodies: 10 units
IgG desmoglein 3 antibodies: 57 units (yikes!)

Final Diagnosis:  ICD-9-CM Diagnosis Code 694.4  Pemphigus Vulgaris   :(

This is how my journey to PV diagnosis started and ended...  Well... now the journey begins again... with finding a cure.  Yes, a Cure!   What I went through these last 8 months was not exactly fun; not knowing what was wrong with me... and the final diagnosis was no comfort either.  But atleast I know what I have and I have the best people in the world and the best support system made up of great super family, great friends, an amazing staff that works for my high-tech business and most of all -- the best Doctors and Researchers I could have ever asked for!  

I have created this blog... to share all my experiences as I fight this autoimmune disease so that you may find some useful information in my story... my treatment...  my journey... as I refuse to be taken down by PV!