The beginning of the real battle with Pemphigus Vulgaris by Sabrena

Among the first things I did upon learning my health condition was to read up on as many literatures I could find on Pemphigus Vulgaris. I learned that this autoimmune disorder is the most serious form of the bullous disease affecting the oral mucosa. Luckily, its fatality rate is very low and it is non-transferable to another. The disorder is frequently misdiagnosed as gingivitis, mucous membrane pemphigoid and erosive linchen planus and though it can remain localized in the mouth, it could also extend to the skin, genital and eosophagal mucosa area – I am thankful that mine is still in the mouth.

Pemphigus Vulgaris has the potential to become fatal to its patients due to the overwhelming infection of the sores. Physically, it looks like a flat whitish blister in the mouth, covering the gums and lips. Mine even covered the under the tongue area (the floor of the mouth) and the insides of my cheek. Healing from these blisters take months. The question is, how do these autoantibodies attack in the pemphigus vulgaris patients? From what I gathered, these autoantibodies attack the ‘desmoglein’, a skin cell which acts like a glue, ensuring that each skin cell is attached to each other. Without the desmoglein, the skin cells become separated and the epidermis becomes ‘unglued’. This in return led to the acantholysis phenomenon which means that blisters were formed because the skin has been eroded and become fragile. In short, it is like using a watered-down glue to stick large numbers of paper. Nothing sticks or stays together and with a soft brush of the hand, the paper will start flying everywhere. In the case of the Pemphigus Vulgaris patients, the area affected will bleed.

To treat the condition I was in, I was put under a high dosage of steroids (60mg of prednisolone per day) to control the inflammation and Azathioprine, an immunosuppressive drug, commonly used to prevent organ rejection after an organ transplant and the treatment of a wide range of autoimmune disorders. Azathioprine suppresses the immune system at the bone marrow, which means it would have a dangerously long list of side-effects of long usage. Though the steroids were reduced by 10 to 20mg around every two months, I had a relapse when I was on the last 10mg. This brings me back to the dosage I first started in.

The relapse had affected my physical appearance to a great length. I started having continuous hungry pangs which made me eat and eat and eat. I tried to limit my food intake to vegetables and fruits yet the weight kept piling up and soon I was obese. My puffed-up appearance caused great concern. Though I tried to exercise every day, I was finding it harder and harder to breathe and move. I lost my balance when I did my yoga and I struggled to climb the stairs due to the knee and joint pains. Then, I noticed that my skin changed. Not only do I lack the lustre and glow; small veins started to appear; as if my skin is being stretched and they are painful too. I highlighted this to the dermatologist and he informed me that these are stretch marks. Frankly, I have heard of stretch marks, but to have them all over my thighs and upper arms is plain unacceptable.

Before Pemphigus, I am the one on the left. Mimi is the one on the right, in a dark brown blouse.

This is me after the first treatment. Notice the cheek got chubbier

In addition to this, I had hair in places I never thought possible for a female to have. I tried to laugh it off though. I told my friends that I was turning into a werewolf since hair is growing all over my face and neck. When I inquired about the hair to the doctor, he said that is a rather uncommon reaction to the medications (how quaint...I just have to be different all the way). With a hump growing on my back (another sign of prolonged use of steroids), I seriously felt unattractive. The only one who might take a second glance at me would probably be Quasimodo. Don’t think I should elaborate how these physical changes were affecting my emotional state of mind. I mean, we are born vain, it is one thing to be obese in a ridiculously short period of time, but to be an obese person with hair growing all over the face and a hump to boot...that is too much. This explains why I was grumpy all the time. I hated the stares I got every time I eat and the look my students or anyone for that matter were giving me (both sympathetic and disgusted stares). Sometimes, I felt like yelling at them, asking them to stop looking at me as if I am  freak! But then again, I was a freak. Here is a picture of me (before and after I was diagnosed in 2010).

This is me, under high dosage of steroids (again) during the relapse. My face felt sooo full and chubby every time I smile.    

xoxo,

Ms. Invictus

A One in a Million Journey – My on-going Battle against Pemphigus Vulgaris by Sabrena

For years I have never bothered whenever I heard of the term ‘autoimmune’ until recently I was diagnosed with one. In layman’s term, autoimmune is a disorder where the body produces autoantibodies due to its inability to recognize which of the body parts (cells/tissues/organs) is its own, causing inflammation and damage to the area or organ concerned. SLE, Lupus, Rheumatoid Arthritis and Diabetes Mellitus type 1 are just some of the examples of autoimmune disorders. This article describes my journey, as an individual diagnosed with an autoimmune disorder so rare, that it happens to 1-5 individuals in every millions of a population, Pemphigus Vulgaris of the Oral Mucosa.

It started in January 2010. Being a health nut, I would get so excited whenever I go to the pharmacy (go figure J). The aisles of health supplements would entice me to purchase as many bottles as possible. I would read the numerous health magazines just to update myself and refresh my memory on various health issues. I led quite an active lifestyle through yoga, jogging and dance. The types of supplements I would always be on the lookout for are the ones that promise to boost my immune system. But then again, life is so full of irony; little did I know that when I popped that tablet of the ‘complete’ and ‘the only’ health supplement I will ever need in my mouth; I triggered a reaction in my body causing it to produce autoantibodies (please note that I could find no literature to confirm this statement as no one really knows how the disorder is triggered. In my case, my disorder developed after I took the health supplement. This is a possibility as it was the only new addition to my diet back then.)

It started with a simple sore throat, which refuses to go away for two weeks. I went to see my GP for treatment and was given some lozenges and antibiotics. Seeing no improvements after a few days, I went to another GP for a second opinion, and was given the same thing. A week later, I started having white patches inside my mouth, spreading rapidly like there is no tomorrow. I requested my case to be referred to a specialist and was sent to a private hospital. Unfortunately, the ENT refuses to take my case seriously and waved it off as a stress-induced ulcer and gave me lozenges and antibiotics; though I have already informed him that none of them worked! At the same time, it was getting harder and harder for me to consume solid food. I was reduced to eating bread, dipped in clear soup and brushing my teeth would lead to excruciating pain. I took the ENT’s meds for a day when my best friend, Mimi insisted that I seek a second opinion.

Off I went to another private hospital, hoping to find an ENT who would examine my condition carefully. The ENT read the referral letter and peered into my mouth to see the seriousness of the condition. He said it was a serious case of a yeast infection, named Candida. Confidently, he gave me a dose of yellow liquid, which surprise..surprise..I happen to be one of the few people in the world to be allergic to it. The ENT did the cotton swab test on me twice to identify the culprit for the white patches and found it to be negative for fungus and bacteria. Though he was puzzled, he assured me that he would help me solve the puzzle. Through him, however, I learned that the infection reacted positively towards prednisolone, a type of Corticosteroid, used to control inflammation. This development gave him the idea to refer me to a dermatologist.

The dermatologist I was introduced to wanted me to undergo a biopsy. Where a sample of cells from my inner mouth was cut to be delivered fresh and analysed by the lab. Apparently, that is one of the best ways to confirm an autoimmune disorder. A few days passed, and I have lost five kilograms due to the lack of sustenance. At this stage, I could no longer brush my teeth. I rinsed my mouth with a mouth wash available in any stores and I could only drink plain water and milk. Even these were difficult to swallow. I reported the pain to the dermatologist and he gave me Diflam, a type of mouth wash which numbs the pain temporarily. He said the one I was using is too strong for my fragile gums and palate. A simple gentle scrape using a tissue would lead to bleeding gums and using the wrong mouth wash, or eating crispy food would only make matters worse. Finally, one morning, I received a phone call from my doctor, who informed me that I was diagnosed with Pemphigus Vulgaris of the Oral Mucosa, a rare autoimmune disorder which is more prone to occur  among women between 40 – 60 years old (by the way, I am in my thirties), and those of Ashkenazi Jewish descents or of Mediterranean origin.

Unfortunately, the news is only the beginning of my long battle with the autoimmune disorder. As I learned the hard way that there is no cure for it and I have to live the rest of my life being dependent on steroids and azathioprine. To find out more about this disorder from the horse’s mouth (that would be me), do wait for my updates from time to time.

XOXO

Ms. Invictus