Wednesday, January 3, 2018

Chapter 5: Almost Did Not Make It

About 10 days before Thanksgiving in 2011, I ended up in the ER at UC Irvine Medical Center and this time, it was bad enough that they admitted me.   I was put on high dosages of Steroids in addition to a cocktail of other medications.   Best of all, I was also put on high dosages of morphine.   After an initial dosage of 250Mg of steroids, I was taking 160 Mg everyday at the hospital.   I was also getting IVIG treatment.   UCI Medical Center is a teaching university so I was pretty popular.   Most physicians had not seen PV and so everyone was visiting me.  And I was so high on the meds, that I welcomed it.   I was not sleeping much at all - after all I had a business to run.  I was not sleeping for more than 3 hours a day.  Just could not sleep. I was on watch 24 hours... as the concern was that my throat would swell up and cause suffocation.   I felt like the darn PV was in my throat so it probably was.

I still clearly remember how I had 2 laptops and 1 samsung tablet and my smartphone beside me at all times.  The nurses, the physician and the staff was just amazing.  My family was amazing.  They endured all of this with me, supported me and suffered through my maniac mental conditions.   I do remember my three kiddos visiting me - it was very hard for them - the oldest was 11, next was 6 and the youngest was only three.  They put on a brave front but I could tell that they were terrified.  My mom and my sister comforted them while they looked around my surroundings - medical equipment with beeps and cables - me laying on the bed - hair all wild and my face was still pretty messed up - hard to describe but let’s just say zombie like. 

Yes, the combination of steroids, morphine and other pain medication and the other meds for my PV was clearly having a HUGE impact on my MENTAL HEALTH.   I did not trust anyone including family members.  

My condition did not improve after the first 24 hours.  I was not able to eat - it was all Ensure.  100% I was having seven 320 Calorie version a day.  

By the 2nd and 3rd days, there was some improvement on my face, my throat but my anal area was still crazy painful.... yes, I would scream in the middle of the night and the bathroom was always full of blood and no poop.   I was pooping blood!  My hemoglobin levels were too low... like 7-8 and the physicians wanted to do a blood transfusion... and I said NO!  

COLONOSCOPY time!   What????  Hmmm.... I don't know about this crap!  I don't think so!!!! is what I said... of course my physicians convinced me that they wanted to make sure I did not have any other potential cancerous conditions up my butt!  Hmm.... fine... but you will have to put me under!

The next day, I am wheeled off to the "procedure room" ....  it was kinda funny because since the room was other end of the hospital, they had to get an Ambulance to transport me.   Haha.... I thought that was a blast!  Wheeeeee.....  yes, I was still high and guess what?  for my book, I will have pictures!  :)   

Mr CEO, Mr. CEO..... Mr.CEO!   Wake up!!!!  Wake up!!!!
That's what I heard and when I opened my eye, there were 4-5 nurses trying to wake me up.  I suppose my body was happy to sleep and did not want to wake up for more than 3 hours.  

Good news!  No cancer found!  Check!

Now what? Can I just go home already????  I guess not - :(

Happy New Year - 2018!

Happy New Year - it’s 2018 - perhaps it’s time for my journey to continue where I left off - yet again after a long pause - don’t worry - I shall explain a little later when I catch up why these long pauses.

Saturday, May 6, 2017

Chapter 4: Pemphigus Vulgaris Strikes Back!

November 3 2011:  By now the IVIG (Introvenous immune globulin) should have fully deployed its armies of immune globulins or antibodies.  The goal of administering IVIG is to stop the immune system from producing autoantibodies and to bind the rogue autoantibodies to remove them from circulation...  I like the plan.... so that is the goal..... But somehow.... Not sure if that worked in my case!   All we know is that some 7-8 days after I had it....  I started experiencing blisters on my body!   Huh? What?  I am on super high Prednisone.... how can I get skin blisters? (which i have seen pictures on the net -- Not fun stuff)

Okay.... so I am now experiencing tiny 1/8th of a penny size blisters all over my neck, nose, back and on my legs..... okay fine it was all over.   My Pemphigus Vulgaris just made a come back!  Unbelievable.... and I headed again for the Emergency Room at UCIrvine Medical Center.  After a high dosage of methyl prednisolone of 250mg (yes it was), I started feeling a little better and was out of ER after 6 hours.

Unfortunately, my medication mis-management (in my opinion) caused a major relapse one week before Thanksgiving.   My lesions were all over the place - I was unable to open my eyes, my mouth, my neck and my face was full of lesions.  But worst of all, it was inside my throat, my eyes, my nose and my anal area and going to the bathroom was like someone shoving a screwdriver up my butt and then twisting it (sorry for the graphical description but that's how bad it was).  I would wake up at 3 am trying to go poop and would scream and all my three kids who were under 10 would wake up and be so scared.  I can still see their faces and it makes me sad that they had to see me that way.

Yup... you guessed it ... off I went to UC Irvine Medical Center...

Friday, September 9, 2016

After a Long Pause, I am Back!

Hello Everyone,

I know... I was a no show from this site and it was sudden... and yes... I have not been around for a very long time.  Actually almost 5 years!  YIKES!  Has it really been that long? Wow.    

Needless to say, this has not exactly been a fun ride for me... just like many of you.   For all of you following me and wanting to know my progress,  for all of you that sent me questions that I never answered, for all of you that oh so wanted me to give you my feedback,  and for all of you well wishers who just wanted to wish me good health and know that I was okay and never heard a word,  I am sorry! I am so sorry for not responding... it was not a good feeling not too. 

I am sorry that I abandoned you and I am sorry that I did not respond sooner.  

Well, here is the good news, I am still alive ... but it has certainly been a long road of craziness that I would wish to no other person.   

This is my story and my journey... my journey for a cure...

I am back and I will provide an update in the days to come... I will continue from where I left off and try my best to get this site working for all of us who wish to help one another; all of us who wish to find a cure so that no one has to go through what we have.

This journey continues... stay tuned.

Tuesday, November 8, 2011

Chapter 3: Good Antibodies to the Rescue...

October 28, 2011  I get a call from Cresent Healthcare Inc.  "We are ready to rock-n-roll with IVIG Mr. CEO... Are you?"  No... that is not what they said... but that is what I heard... and I was ready to rock-n-roll... because I was not really liking this skin blister thing.. Until now, I had soft beautiful skin  :)  ... and I was not about to be bombarded with skin blisters.    I mean have you seen the pictures on the Internet on P.V. Skin Blisters... When I saw them, I cried and cried and cried.  So, I am not about to see myself and cry and cry and cry... I am not even going to post is over here!  There are kids reading this blog!!!

Friday morning, October 30, 2011... at 1pm PST, I mossy my way to the Infusion Center -- which happened to be just 3 minutes from my office -- that was convenient.  Hmm...   Okay.  Nice.

You know... the amazing part of this journey is that as bad as P.V. has been to me.. and I remember Dr. Grando's initial words when we were not sure what I had... and he said "Mr. CEO you don't want to have P.V.."...   So, back to my story.  As bad as P.V.has been to me... everything ... I mean everything and everyone involved around me have been the exact opposite.  Super Good.   Its like the ying and yang of life...   The folks at Cresent Healthcare are amazing!  

My Nurse.. was awesome!  So, we hook me up with an IV line... I take some Ibuprofen 400Mg and I also take Loratadine 10 Mg before the IVIG.    It is all precautionary due the the myriads of side effects that can occur... Btw... I pretty much no longer bother with side effect notices anymore (up to a certain extent) because everything under the sun is now going to be my side effect because of the various meds and treatment... but the miracle is -- ZERO SIDE EFFECTS so far!  Wuuu Huuuu!

IVIG Starts
So, I was given Gammagard 20g IVIG.

GAMMAGARD LIQUID is used to treat patients with primary immunodeficiency diseases (PI). There are many forms of PI. The most common types of PI result in an inability to make a very important type of protein called antibodies, which help the body fight off infections from bacteria or viruses. GAMMAGARD LIQUID is made from human plasma that is donated by healthy people. GAMMAGARD LIQUID contains antibodies collected from these healthy people that replace the missing antibodies in PI patients. In my case... kick some butt against my autoantibodies.

A little overview of those of you interested in the difference between antibodies and autoantibodies....

Antibodies and Autoantibodies

Antibodies are produced in a process of evolution that is still a subject of scientific research. Briefly, antibodies are produced by B cells in two ways: (i) randomly, and (ii) in response to a foreign protein or substance within the body. Initially, one B cell produces one specific kind of antibody. In either case, the B cell is allowed to proliferate or is killed off through a process called clonal deletion. Normally, the immune system is able to recognize and ignore the body's own healthy proteins, cells, and tissues, and to not overreact to non-threatening substances in the environment, such as foods. Sometimes, however, the immune system ceases to recognize one or more of the body's normal constituents as "self," leading to production of pathological autoantibodies. These autoantibodies attack the body's own healthy cells, tissues, and/or organs, causing inflammation and damage. It should be noted that autoantibodies may also play a nonpathological role; for instance they may help the body to destroy cancers and to eliminate waste products. The role of autoantibodies in normal immune function is also a subject of scientific research.

Treatment Starts

We started at slow.. and than zoom to 88 units per something (speed) of the Infusion.  The entire process took less than 4 hours.
The plan is that I take 5 days of this treatment and repeat every month for 6 months.  

Once I was hooked up and being infused by someone's plasma... I had weird visions... I could see other people... and other objects... I saw a HUGE MANSION... a swan lake ....  NO!  Just kidding!!!   I just figured that I am getting plasma from some great soul that donated blood... I should see something.. but nope...  nada.  I did feel pretty good though.

The Cresent Healthcre center itself was a great location with alll the amenities for me to work! :)  So, I called my staff.. and we had meetings and discussions while I was being infused.   And the Nurse was so awesome that they accommodated all our needs...  Thank you to my Nurse Renee!

So... this process continued through Tuesday Nov 1st... and everything went well.. I was doing great!
No problems what so ever...


EXCEPT... for my sleeping... I was only sleeping 3-4 hours a day.   I was okay though ... the Steriods were probably giving me all the energy I needed and keeping me up too... I had not taken Lunesta... I am sure Dr. Grando would not be too pleased about this.   But I just did not want to put more meds into my system if it was not necessary. things are looking good.  I am feeling like my PV is under control... I am getting the right meds and the right treatment and so stay the course to onwards and upwards!

Current Cocktail of Treatment :
80 Mg Methylprednisolone;
500Mg Tetracyclin 3 times daily; ,
500Mg Niacinamide 3 times daily
Lunesta 2 Mg - take as necessary 
IVIG 20g Gammagard (with 400Mg Ibuprophen and Loratadine 1-mg ) 5 days every month
2 daily multi-vitaminss; 

To Great Health Cheers!!! :)

Monday, November 7, 2011

Chapter 2: Skin Blisters Kick In...

October 20, 2011 I begin to notice skin blisters.  Until this point, my skin would tingle, I would see a rash (slight bump) and then within an hour or so it would disappear.  But never a skin blister.  But I noticed 3 of them on my chest and my back.   It was extremely small... Like the size of an eraser on the back of a pencil.  But, I accidentally scratched one of them... and auchie!  It was an interesting sting like sensation. now, Dr. CEO (me...hehe) was realizing that apparently my levels of medication were simply not doing the job.  I should NOT be having any skin blisters... I am being treated.  Right??? 

By October 25 when I saw Dr. Grando, I had several of these little puppies tap dancing on my back.  Dr. Grando decided to do the famous Nikolsky's skin test.  Nikolsky's sign is either positive or negative. A positive result means that my skin portion of P.V. is active. People with a positive sign have loose skin that slips free from the underlying layers when rubbed. The area beneath is pink and moist and usually very tender.  Until this point, my it had been negative. 

Well... wullah!  It was Positive!  So, just in life its important to move forward... So was my P.V. -- yup moving forward and getting more invasive by attacking my skin now. 

This was obviously not a good sign.  Dr. Grando was not too happy about this.  Now, there was one part of my treatment that had not begun.  And that was IVIG  or otherwise known as Intravenous immunoglobulin/

Intravenous immunoglobulin (IVIG) is a blood product administered intravenously. It contains the pooled IgG (immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months. It is mainly used as treatment in three major categories:
If you would like to learn more about IVIG simply click on this Link ...Intravenous immunoglobulin/

So, Dr. Grando felt that I needed to get on this puppy as quickly as possible and he contacted several of the vendors and the one we finally were able to accept my health care insurance (that is half the battle) was Cresent Healthcare, Inc.  

Treatment... there was a change... as my PV anti antibodies increased their activities... we call in the Calvary! :)   From 60Mg of Prednisone, we now moved on to 80Mg of Methylprednisolone.  Now unlike Prednisone where the liver activates the steroid.. With Methyprednisolone, it is self activated.  So, watch out P.V. here were come with reinforcements! :)

Who needs Sleep!
On the whole... I am still feeling good.  But these new exciting drugs in my system do not come "freee" as my ENT Dr. Boynton had once reminded me.  I was only sleeping 2-3 hours a day which was fine with me... but not for everyone around me as they could not keep up with me.  But... hey, i felt super productive... so I was happy.  I was not really tired too....

But hold on King Musaby!  -- Dr. Grando was not so impressed...  and said "Sundip! you need at least 8 hours of sleep!"  So, lets give you some good stuff... Remember all those nice ads of Lunesta. Well... that is what I got.  Take a couple of those and I should be out for 8 hours.  Okay... I thought. I will do it if I really need it.

So, Treatment at this point:
80 Mg of Methylprednisolone, 500Mgx3 Tetracyclin, 500 x 3 Niacinamide and Lunesta 2 Mg - take as necessary.

Moving on with cocktail of drugs... to great health Cheers!!! :)

Chapter 1: The Start of My Treatment

For an overview of my disease, please click this link... The Beginning...The 7 Month Journey to a Real Diagnosis

On October 11, 2011, I met my Specialist Dr. S. Grando of UCI -- an amazing physician and scientist with tremendous knowledge, insights, and research experience with Pemphigus Vulgaris.  He is really the one who wrote the book on this disease and I know that I am in the best of hands.  This meeting was very different then the last one... It was confirmed that I had P.V. whereas before it was simply strongly suggestive.  He was most gracious... and answered every question that I had.

So, P.V. can be controlled. But its the mechanism of control which utilizes high levels of steroids ifor longs periods of time (2-3 years) is where the problem is.  The side effects of suppressing my auto immune to reduce the flare ups of P.V. will simply make me susceptible to other diseases.  So, the goal is to get of prednisone as quickly as possible.  Here is the best part!  I am going to be on a clinical research trial where there is a great chance to reduce my Prednisone dosage down in a year!  If this works... and the fact that I am perfectly healthy otherwise, I am going to be just fine!

This new clinical research utilizes a well known drug called Sirolimus.  It is used in combination with other medications to prevent rejection of kidney transplants. Sirolimus is in a class of medications called immunosuppressants.   In P.V. patients, apparently it will allow the slow reduction in dosage of Prednisone to nothing.

So, Looks like I am going to be on it... in November, 2011

So far, I am doing great... no side effects at all.  The sores/blisters in my mouth are getting much better.   I am have been on Liquid Diet since Late August and still am.  But I have been told that I should be able to eat solids in 2-3 weeks.  Cant wait! :)

On the whole, my case looks pretty good.  Other than P.V., I am extremely healthy and all my lab results are great!  Heck, even my Cholesterol levels are perfect.  Good thing I did not inherit heart disease which is so prominent in our family among the men.

So, the Treatment right now consists of 60Mg Prednisone, 3 x 500Mg of Tetracyclin and 3 x 500 Mg of Niacinamide daily.  Not too bad... Zero side effects.

Moving Onwards and Upwards to Great Health!

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!