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...


SLEEP

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.

Okay....so 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!!! :)

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.

So...by 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:

• Immune deficiencies such as X-linked agammaglobulinemia, hypogammaglobulinemia (primary immune deficiencies), and acquired compromised immunity conditions (secondary immune deficiencies) featuring low antibody levels.
• Autoimmune diseases e.g. Immune thrombocytopenia ITP and Inflammatory diseases e.g. Kawasaki disease.
• Acute infections.

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!