Living with the Burden of Wegener’s Granulomatosis (Part 2)

How do you fight an illness that makes your own body destroy itself? This is my journal about living with a rare autoimmune disorder.

© Brian Mann

I am My Own Enemy to Fight

If cancer is a school bully that you need to stand up to, then autoimmune disorder is like your buddy who’s doing dirty things behind your back. There’s no reason for her to hurt you, but she does nasty things by tricking some of your own cells into mistakenly attacking others until you get sicker and sicker.

There’s no true enemy, no malignancy to rally against. In a way, you’re what’s wrong with you. There’s no one else to blame but you. Autoimmune disorder is just like committing suicide, killing yourself slowly but surely. And you can NEVER fully recover.

In 2012, I was diagnosed with a rare, incurable autoimmune disorder called Wegener’s Granulomatosis, which inflames the walls of the blood vessels, depriving the organs of blood and leading to the destruction of tissues and cartilage (in my case, it attacks my sinuses). I was 35 years old then.

No one knows what causes Wegener’s, which is in the same family as lupus and multiple sclerosis, or how to cure it. So, doctors treat it like a form of cancer, with a combination of steroids and chemotherapy, hoping to force the disease into a long-lasting remission. And there’s no permanent cure to it.

Still, I’m grateful that it’s not cancer. At least, I can live my life as normal, taking care of my family, working and blogging in my free time. I don’t need an oxygen tank to breathe, or a wheelchair to move around. Just a bunch of pills and routine blood test to keep me alive.

However, 2017 has got off with a rocky start for me. Just after New Year, I was down with a nasty flu that could have triggered a flare. I had sudden heavy nose bleeding. That weekend, my nose bled more than 10 times, and each time, it took ~10 minutes to stop. It was MASSIVE bleeding, and if I were to quantify it, I lost about 1/3 cup of blood each time.

What worries me most is NOT the amount of blood loss, but the LOCATION of the bleeding.

I’ve always suffer from nose bleeding due to crusting inside my nose (left side). Nasal crusting is part of the Wegener’s package. It’s just like a scab that formed on your wound when you’ve a fall. If you tear the scab, it will lead to bleeding. Nasal crusting is similar to scab, except that it’s inside the nose and persistent (very irritating too!).

This time, the nose bleeding came from the back part of my right nose (not due to crusting) and the volume is heavier than before – that’s what worries me. What could it be this time?

Living with the Burden of Wegener’s Granulomatosis (Part 1)

How do you fight an illness that makes your own body destroy itself? This is my journal about living with a rare autoimmune disorder.

autoimmune-disorder-blood-testThe long list of blood test I just had at SGH

Seeing Life Differently Now

It’s been more than a year since I last talked about my autoimmune disorder. If you’re wondering whether I’ve fully recovered from it, the answer is NO. For a Wegener’s granulomatosis patient, chances for a complete recovery is very, very low. Unfortunately, Wegener’s is also a disease in which relapses frequently occur.

Therefore, I’m not hoping that I can ever fully recover, and I’ve resigned to fate that I’ll live with this disease for the rest of my life. =(

I was diagnosed with Wegener’s granulomatosis in year 2012. In Wegener’s, an abnormal type of circulating antibody called antineutrophil cytoplasmic antibodies (ANCAs) attacks small and medium-sized blood vessels. Injury to blood vessels affects several organs, including the lungs, kidneys and upper respiratory tract. If left untreated, there is a risk of kidney failure and death.

According to the Vasculitis Foundation, Kansas City, USA, this disease affects about 1 in 30,000 people. The cause is unknown, although it tends to strike between the ages of 30 and 50. The disease can damage vital organs and severely shorten one’s lifespan if the patient is diagnosed at the later stage of the disease.

I’m truly thankful that I was diagnosed early, before any lung or kidney involvement were detected. Since then, I’ve been seeing a private consultant rheumatologist at Gleneagles Hospital. I’ve been taking oral medication for the past 4 years, and my condition is under-control.

The main problem that I face now is frequent nose bleed, and it’s quite severe when I travel due to temperature and humidity. I’ve a very sensitive nose and whenever I sneeze hard, my nose will bleed. Sometimes, the bleeding is quite heavy and hard to stop.

The Ups and Downs of Life, Living With an Autoimmune Disorder

Life© Eutah Mizushima | Unsplash

Imagine having nose block that never, ever goes away. Imagine waking up at night soaking in blood due to nose bleeding. Imagine the hassle of keeping up with your doctors’ appointments (especially if you’ve a full-time job). Imagine using drug to get your life back. Imagine living in uncertainties not knowing how much longer you can live.

If you ask me what it’s like to live with an autoimmune disorder, these are my answers.

For many people, the diagnosis of a rare disease comes out of the blue. In Aug 2012, I went for a sinus surgery and walked out with an autoimmune disorder. Not really, but that’s what it felt like. I didn’t really think there was anything wrong with me except having frequent nose bleeding. I’ve always been a healthy person, and I rarely get sick.

What is Wegener’s Granulomatosis?

I was diagnosed with an autoimmune disorder called Wegener’s Granulomatosis (WG) after two sinus surgeries. Having an autoimmune disorder in general means that your immune system gets confused and starts attacking a part of your own body.

WG is a form of vasculitis (inflammation of blood vessels) that affects small and medium sized vessels in many organs. The inflammation makes it hard for blood to flow. It affects the nose, lungs, kidneys, and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppressant.

Luckily, I was detected early and there was no lung and kidney involvement yet. Even though this disease is chronic (no cure) and the effectiveness of treatment is unpredictable, my rheumatologist assured me that my prognosis is good. He prescribed prednisolone (a type of corticosteroid) and cyclophosphamide (a drug used in treating cancer and also WG). I started right away.

The First Year – still manageable!

The first year was rather smooth sailing. I did everything I could to get healthy and stay positive. I went for specific blood tests every 2 to 3 months to ensure that the disease is under control. Except battling with weight gain (side effects of prednisolone), I lived my life as normal.

The Second Year – the trouble began!

I was down with a lung infection, my airways were congested, and I couldn’t breathe properly. I went to see my rheumatologist, and was told that I might have a relapse. As there were signs of lung involvement, I was referred to a pulmologist for Heart CT Scan and further treatments.

After I recovered from the lung infection, my rheumatologist discussed a new treatment for me. Rituximab is an expensive drug used to treat certain types of cancer, and it’s also an alternative treatment for WG. It’s more effective that steroids and cyclophosphamide as it’s a B-cell targeted therapy. It’s given through intravenous infusions.

The treatment costs about $14,000. Despite the high cost, I decided to go for it. I convinced myself that I can earn back that amount of money – I only have one life and I can’t risk it. I was hospitalized twice for the infusions.

Thankfully, the treatment worked well for me and my disease is under control until now.

By the end of second year, I started suffering from side effects of cyclophosphamide. I had hair loss, night sweats, and my menstruation stopped. It was no joke – waking up in the middle of the night drenched with sweat was very suffering.

I went to see my rheumatologist again and did a hormone test. The results showed that I had early menopause. I was referred to a gynaecologist for hormone replacement therapy (HRT). This was my toughest ordeal. Hearing the word “menopause” when I was not even 40 years old, it broke my heart and my eyes welled up in tears.

I braved myself to undergo HRT. Several weeks later, I felt myself again and I recovered. My menstruation cycle returned to normal.

The Third Year – began to feel good, but still uncertain

The third year was good. My disease is under control and I changed my medication. The new medication does not have side effects (but more costly). I shed all the kilos that I’ve piled up since 2 years ago through a slimming program, and I’ve never felt so good before.

However, my happiness didn’t last very long.

I experienced blurred vision recently, especially after spending long hours working at the computer. I went to see an optometrist, and he suspected that I might have glaucoma after conducting an eye check and went through my medical history.

My rheumatologist suggested that I should see an ophthalmologist for a detailed eye check as WG may potentially affect the eyes too. I was devastated and somewhat angry. ENT, pulmologist, gynaecologist and now, ophthalmologist. What else is new? Why there is no end to my disease? And why me? Life is so unfair!

Last week, I dreaded myself for a detailed eye check at Singapore National Eye Centre. After a series of tests, the ophthalmologist ruled out glaucoma and cataract. For the first time, it was false alarm.

The reasons behind my blurred vision are dry eyes and higher degree of myopia. I was given eye drops and a prescription to change my glasses. It was such a relief after worrying about it for days. And I walked out of SNEC feeling hopeful again!

Pink bullet

This coming September, I’m entering the fourth year. They say what doesn’t kill you makes you stronger. In a way, I agree – it makes me re-evaluate how I was living my life and take serious steps to improving my overall health.

This trial is never-ending to me. Although it hasn’t advanced to a level that cause a major disruption to my life (I pray hard that it will NEVER), I feel frustrated with life sometimes. I kept most of the anger and disappointment to myself because I didn’t want to affect anyone in my family.

Nonetheless, this disease also makes me see things differently now. It’s a liberating experience to say the least. Every day when I wake up, I’m thankful to see the sun rises on another day of my life. To be with my loved ones for another day, I strive to make every day counts. I want to be here for them for as long as I possibly can.

 

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Living with Autoimmune Disorder

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Just a few minutes into my sleep, I sensed a mild smell of blood. Then, I felt a strange ‘liquid’ flowing from my nose into the back of my throat. I immediately woke up and ran to the toilet. I spit out the ‘liquid’. It was blood. Then, my nose started to bleed heavily. It took me almost ten minutes to stop the bleeding.

Well, that’s part and parcel of my life living with an autoimmune disorder. Nose bleeding has become a common affair to me.

I first heard of the term ‘autoimmune disorder’ about 12 years ago. At that time, my brother suffered a sudden and massive hair loss. He was still in his early twenties, so it’s definitely not male pattern hair loss. Later on, we realized that it was a kind of autoimmune disorder that manifests itself by attacking hair follicles.

Thankfully, he recovered with the right medication and his hair grew back normally again.

In August last year, I was diagnosed with Wegener’s Granulomatosis (WG) – a kind of autoimmune disorder. There was a flood of emotions when my rheumatologist broke the news to me. At first -okay, for a while – I was angry and disappointed. This is not the way I want to spend my thirties, after all.

To further explain WG in layman’s terms, it’s a rare disorder in which certain blood vessels become swollen and inflamed. The inflammation makes it hard for blood to flow. It affects the nose, lungs, kidneys, and other organs. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression.

Later on, I found a relief because I was detected early, and there was no lung and renal involvement yet. Still, this disease is chronic (there is no cure), and the effectiveness of treatment is unpredictable.

My first course of treatment started with prednisolone (50mg daily). It’s a steroid that prevents the release of substances in the body that can cause inflammation. I also took cyclophosphamide – it’s a drug that is used primarily for treating cancer, and also suppresses the immune system.

Prednisolone damaged my look, and I’ve been battling with weight gain issues since then. Cyclophosphamide, on the other hand, also caused undesirable side effects such as hair loss, early menopause, etc.

Four months ago when my hair loss became so severe, my rheumatologist decided to change my treatment. I was off cyclophosphamide immediately, and I started taking Myfortic – a new medicine that has been approved for the treatment of WG. There is no severe side effect for Myfortic, but it is VERY expensive.

I didn’t have a choice, though. Money can be earned back, but health is not. And I cannot live with no hair. So, I’ve decided to continue with this expensive treatment with hope that WG will go away soon. My health is my first priority now.

During these 1.5 years, I frequently experience emotional trauma. Feelings of low self-esteem, sadness, lost of hope, and even fear of dying young. I’m not scared of death, actually. But I’m most worried about my son if I had to leave the world in a sudden. Nonetheless, I tried my best not to let these negative feelings take control of my life.

This autoimmune disorder also makes me see things differently now. It’s a liberating experience to say the least. Every day when I wake up, I’m thankful to see the sun rise on another day of my life. To be with my family and loved ones for another day, I live every day like it will be my last.

Live, Love, and Cherish Life. Now and Always!

 

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