Red and the Big Bad Wolf

The story of me – Red living with Myasthenia Gravis or the 'Big Bad Wolf' as I prefer to call it

After being diagnosed with MG (Myasthenia Gravis) and Fibromyalgia I didn’t know that there was one more ‘final’ diagnosis waiting for me. And I refer to it as ‘final’ in apostrophes as I don’t know if one day there will be more. The reality is that when you have one autoimmune disease your are likely to get more autoimmune diseases or other systemic diseases.

I was doing fairly well managing my MG and Fibromyalgia and I was well into 2015. I had noticed that my eye sight wasn’t great, which I thought was due to the MG. I also noticed that my mouth was always very dry, as well as my skin. I didn’t think much of it as I was on so many medications.

I decided to see Dr. P (the Wonderful!) about my eye sight. After an examination she confirmed that it was not MG that was causing my issues. I previously had lasik eye surgery to correct my poor vision that I had since I was a child and she recommended that I see my opthalmologist.

Off I went to see Dr. H and after his examintation he confirmed that as far as my lasik surgery goes my eyes were fine. (Although I believed my night blindness was caused by this and is a common side effect. Something he didn’t want to acknowledge.) He did state that he believed that I had secondary Sjögren’s Syndrome as my eyes were exceptionally dry and that is what contributed to my blurry vision. Dr. H presribed several eye drops and recommended that I see my rheumotologist.

So I went to see my rheumotologist Dr. S and explained the situation to him. He examined me and then ran blood tests to confirm the diagnosis. And true as Bob I had Sjögren’s Syndrome! But what did this mean?

,Sjögren’s (“SHOW-grins”) is a systemic autoimmune disease that affects the entire body. Along with symptoms of extensive dryness, other serious complications include profound fatigue, chronic pain, major organ involvement, neuropathies and lymphomas.

Although many patients experience dry eyes, dry mouth, fatigue and joint pain, Sjögren’s also cause dysfunction of organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Patients also have a higher risk of developing lymphoma.’ (From the Sjögren’s Syndrome Foundation http://www.sjogrens.org/home/about-sjogrens)

The interesting thing was that ‘Sjögren’s symptoms frequently overlap with or “mimic” those of other diseases including lupus, rheumatoid arthritis, fibromyalgia, chronic fatigue syndrome, and multiple sclerosis.’ Thus it was easy to miss as a diagnosis in my case.

In terms of diagnosis Dr. S ran the below blood tests and my ANA and SS-A as well as SS-B were positive – thus confirming my diagnosis:

  • ‘ANA (Anti-Nuclear Antibody)
    ANAs are a group of antibodies that react against normal components of a cell nucleus. About 70% of Sjögren’s patients have a positive ANA test result.
  • RF (Rheumatoid Factor)
    This antibody test is indicative of a rheumatic disease, including rheumatoid arthritis (RA), lupus and Sjögren’s. It doesn’t, however, specify which rheumatic disease a person has. In Sjögren’s patients, 60-70% have a positive RF.
  • SS-A (or Ro) and SS-B (or La)
    These are the marker antibodies for Sjögren’s. Seventy percent of Sjögren’s patients are positive for SS-A and 40% are positive for SS-B (these may also be found in lupus patients).
  • ESR (Erythrocyte Sedimentation Rate)
    This test measures inflammation. An elevated ESR indicates the presence of an inflammatory disorder, including Sjögren’s.
  • IGs (Immunoglobulins)
    These are normal blood proteins that participate.’

It is important to note in terms of a diagnosis that Sjögren’s Syndrome can occur as a primary disease or a secondory disease as a result of other autoimmune diseases. Sjögren’s is also an autoimmune disease and in my case I had secondary Sjögren’s.

Below is a great image from the Sjögren’s Syndrome Foundation depicting the symptoms of the disease.

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In terms of treatment it is treated similar to other autoimmune diseases with cortisone and immunosuppressants as there are no specific treatments for Sjögren’s Syndrome. I was already on cortisone and would later start other immunosuppressants and together with the eye drops it was manageable as I only had the dry eyes and skin to deal with. I later developed Raynaud’s phenomenon, but there is not much to do about that. Autoimmune diseases suck.

As always I was relieved to have answer, although knowing the answer didn’t really set me free. I now had three complex diseases to manage – Myasthenia Gravis being the most complex. Most importantly I had diagnosis’ and I wasn’t in the lost in the sea of no diagnosis anymore.

As I write this it is August 2017 and I can tell you that after being diagnosed a lot happened and battling these diseases, especially Myasthenia Gravis, has been complete and utter hell. Although I haven’t been diagnosed with more diseases I have had several health complications directly related to my diseases and just having autoimmune disease with a body going haywire in general. But that is another story for another day.

In concluding my diagnosis story I want to come back to the purpose of sharing it – to help others find their diagnosis and to not loose hope in the sea of no diagnosis. In hindsight it is ‘almost’ easy to see why I was sick and it feels like I could have been diagnosed earlier. I don’t know if I am right or wrong in saying that. But I do hope that someone reads this and perhaps see symptoms matching theirs that they can discuss with ther Dr. Although that being said I don’t hope for anyone to have these diseases.

Thank you for reading and sharing my story. There will be more to follow. I wish you good health and happiness.

XO

The previous blog post about my diagnosis journey ended where I was finally diagnosed with Myasthenia Gravis. I was elated to finally know what has been wrong all along and to treat it, even though Myasthenia Gravis is a horrible and dibilitating disease to have.

It is bittersweet to get an accurate diagnosis. You are relieved to have an answer and to know that you haven’t been imagining symptoms. However finding out that you suffer from an incurable rare disease is not such a big relief.

MG (Myasthenia Gravis) has a very specific treatment process, whereby you start with baseline of medication and if these don’t work you progress to ‘stronger’ medication/s. The primary medication that is always used for MG and never changed is Mestinon, which  works against the acetylcholine antibodies that are classically involved in MG.

Remember that MG is a disease that affects the normal functioning of your neuromuscular junction. More specifically it affects the acetylcholine neurotransmitters in the junction, whereby antibodies attack these neurotransmitters and prevent normal functioning. Acetylcholine is not the only neurotransmitter or protein affected; there is also MuSK, LRP4 and Agrin. Researchers are still discovering more. So it is important then to note that Mestinon only works to defend the acetylcholine neurotransmitters.

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In addition to Mestinon the next thing you get when you have an autoimmune disease is cortisone or steroids, which has an anti-inflammatory effect. So my current cortisone dose was increased and it was the start of an annoying weight gain and moon face journey. ‘Sigh.’

The next medication to be added is an immunosuppressant. I was already on Imuran, because of my Lupus diagnosis and we continued on this. Unfortunately we had to stop it soon after my diagnosis as I couldn’t tolerate the side effects.

Fortunately my weakness improved, but I still had a lot of other symptoms, especially a lot of pain. Dr. P – The Wonderful – referred me to a terrific rheumatologist Dr. S. Dr. S made a surprise finding – I didn’t have Lupus (YAY!), but Fibromyalgia! I was shocked and relieved, as having both Lupus and MG are very trying. This also explained why some of my symptoms didn’t improve with a treatment regimen that should have worked for both MG and Lupus. I also found it very interesting that Lupus and Fibromyalgia or often cross-diagnosed as they have similar symptoms. This is a link to a piece explaining how these two diseases are often cross diagnosed –  http://www.fibromyalgia-symptoms.org/fibromyalgia_lupus.html

Fibromyalgia has a shockingly long list of symptoms and complications, which explains why you generally feel absolutely shitty if you have it. Some of the symptoms are below, but this link takes you to a piece on ALL of the symptoms – https://www.verywell.com/fibromyalgia-symptoms-716139#unusual-pain-types

Thus if you have Fibromyalgia you might experience some of the following symptoms:

  • Chronic muscle pain, muscle spasms, or tightness.
  • Moderate or severe fatigue and decreased energy.
  • Insomnia or waking up feeling just as tired as when you went to sleep.
  • Stiffness upon waking or after staying in one position for too long.
  • IBS (Irritable Bowl Syndrome.)
  • Chest Pain.

Fibromyalgia is a controversial disease that many – including doctors – believed was a physical manifestation of psychological distress. It was only two years ago that researchers established that it was a central nervous system disease. (https://www.sciencedaily.com/releases/2015/05/150517071813.htm) Because of this I wasn’t thrilled about the diagnosis as I was faced with the stigma of having a disease that people think you make up in your mind.

Unfortunately there are not really any effective (or curing) treatments available for Fibromyalgia. I was put on Lyrica, a nerve pain medication, and Cymbalta an antidepressant approved by the FDA for Fibromyalgia as it also helps for pain. I also use Stilpaine for when the pain gets worse. Some patients are prescribed opioids as well to deal with the pain, but I cannot have these as they are contra-indicated for MG. But let me tell you a little secret; none of these really help, because you are still in a lot of pain. Always.

On the bright side I had two correct diagnosis’ – Myasthenia Gravis and Fibromyalgia. And I could start to manage these diseases as much as is possible, which I did.

Next time I will tell you about my third  (and hopefully final) correct diagnosis.

XO

This is an interesting article via Lunatic Laboratories about the use of our own stem cells to treat in this case Muscular Dystrophy a degenerative muscle disease.

Similar research is being done for Myasthenia Gravis with great success.

Are stem cell transplants our future?

XO❄️💙

Satellite cells are stem cells found in skeletal muscles. While transplantation of such muscle stem cells can be a potent therapy for degenerative muscle diseases such as Duchenne muscular dystrophy, these cells tend to lose their transplantation efficiency when cultured in vitro.

via New method to grow and transplant muscle stem cells holds promise for treatment of MD — Lunatic Laboratories

Every year in June there is a targeted effort by the myasthenia gravis community to spread awareness about this rare disease. There are however many people and communities that strive to do this every day.

In order to do my part and to spread more awareness I have created a Facebook page ‘Myasthenia Gravis – Red and the Big Bad Wolf.’ It would mean the world to me if you would like my page and help to spread awareness. You never know who it might reach and more importantly help. Especially those that have not been diagnosed, have been wrongly diagnosed, or struggling with mysterious and debilitating symptoms.

You can find my Facebook page at https://www.facebook.com/redandthebigbadwolf/ You can also follow me on Twitter on @ItsRedRidingH.

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You can search for the following hashtag #IhaveheardofMG on Twitter in order to read and share tweets about myasthenia gravis. Do follow the wonderful creator of the #IhaveheardofMG campaign on Twitter on @ihaveheardofmg.

I also suggest that you follow the inspiration behind ‘Myasthenia Gravis Unmasked’ on Twitter on @RebekahDorr or on Facebook on https://www.facebook.com/mgunmasked/

Then there is my biggest inspiration and snowflake shoulder to lean on. Someone that I am honored to call my friend that you must follow on Facebook  – Retha. You can find her on https://www.facebook.com/chronicallytwentysomething/

I will leave you with a brief explanation of myasthenia gravis (MG) and its primary symptoms. ‘Myasthenia gravis (pronounced My-as-theen-ee-a Grav-us) comes from the Greek and Latin words meaning “grave muscular weakness.” The most common form of MG is a chronic autoimmune neuromuscular disorder that is characterized by fluctuating weakness of the voluntary muscle groups.’ (Courtesy of the Myasthenia Gravis Foundation of America http://www.myasthenia.org/) The most common symptoms of myasthenia gravis are:

  • A drooping eyelid
  • Blurred or double vision
  • Slurred speech
  • Difficulty chewing and swallowing
  • Weakness in the arms and legs
  • Chronic muscle fatigue
  • Difficulty breathing

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Wishing you good health and happiness. XO

I ended off my last blog post in April / May of 2014, where I finally had a diagnosis! Lupus! I was so happy to finally have an answer to ‘What is wrong with me?” after 2 and half years of illness! (This would turn out not to be the Eureka diagnosis I expected. :() Now lupus isn’t a happy diagnosis – no autoimmune disease is – but it was something. We could name it and we could treat it and I continued on the immunosuppressants (Imuran) and cortisone (Prednisone) per instruction from my specialist physician Dr. K.

I even started my blog 🙂 Red and the Big Bad Wolf – with the name inspired by the origin and name of the disease Lupus. I joined Lupus support groups and I finally felt a sense of belonging. I no longer had a mystery disease. People could no longer doubt my illness and whether it was all psychosomatic.

I felt slightly better on the new medication, but far from good. I also struggled with constant nausea as a result of the immunosuppressants. We increased the dosage to see if my condition would improve, but after a two months it became unbearable and I couldn’t even eat – so we reduced the dosage again. I would come to realize over the years that my body is very sensitive in terms of the impact of certain medications; and I would often struggle with severe side-effects.

I still struggled with severe fatigue, general weakness in my body and limbs and pain. It was odd as I should have been improving on the treatment. And this did have Dr. K. and my neurologist Dr. P. (the Wonderful) scratching their heads.

I saw Dr. P. (the Wonderful) in July of 2014 for a follow-up. While we were talking she kept staring at me and then suddenly said –  ‘I have seen you looking tired and with bags under your eyes, but I have never seen your like eyes this. They look like they are drooping.’ She came over for a closer look and asked me if I had noticed this before. I said that I had actually noticed it on photos and even showed it to the Viking, but  he said I was imagining things and I thought so too.

Dr. P. (the Wonderful) looked at me with a big smile on her face and said ‘I know what is wrong with you! You have myasthenia gravis!’ This was the EUREKA moment of my diagnosis journey ! Of course I had no idea what myasthenia gravis was, couldn’t pronounce it, never mind spell it! 🙂 Dr. P. (the Wonderful) briefly explained what myasthenia gravis (or MG for short) was and wrote the name down so that I can also read up on it. She also said that we had to do a few tests to confirm the diagnosis – blood tests and an EMG test. Although the EMG test confirmed it the blood test didn’t, but she was still positive that I had myasthenia gravis.(This is a topic that I will cover in a separate blog post as testing for antibodies to confirm a MG diagnosis is not straight forward.)

Myasthenia gravis as a diagnosis made perfect sense and also explained why I wasn’t improving based on the treatment I was receiving for lupus. (Although it was a bit of a downer that I had both diseases – it is rare and troublesome.)

Like lupus, myasthenia gravis (MG) is an autoimmune disease, but it is very different. ‘Myasthenia gravis (pronounced My-as-theen-ee-a Grav-us) comes from the Greek and Latin words meaning “grave muscular weakness.” The most common form of MG is a chronic autoimmune neuromuscular disorder that is characterized by fluctuating weakness of the voluntary muscle groups.’ (Courtesy of the Myasthenia Gravis Foundation of America http://www.myasthenia.org/) The most common symptoms of myasthenia gravis are:

 

  • A drooping eyelid
  • Blurred or double vision
  • Slurred speech
  • Difficulty chewing and swallowing
  • Weakness in the arms and legs
  • Chronic muscle fatigue
  • Difficulty breathing

 

As you can see from above the symptoms in bold already ticked quite a few boxes for me. Over time all of the symptoms would be come applicable to me. 😦

The following paragraph provides a detailed description of what myasthenia gravis is and how it works. You can skip this if you are not into long medical descriptions! 😉 (Ps. I still cannot properly pronouce ‘acetylcholin’ :))

‘The voluntary muscles of the entire body are controlled by nerve impulses that arise in the brain. These nerve impulses travel down the nerves to the place where the nerves meet the muscle fibers. Nerve fibers do not actually connect with muscle fibers. There is a space between the nerve ending and muscle fiber; this space is called the neuromuscular junction. When the nerve impulse originating in the brain arrives at the nerve ending, it releases a chemical called acetylcholine. Acetylcholine travels across the space to the muscle fiber side of the neuromuscular junction where it attaches to many receptor sites. The muscle contracts when enough of the receptor sites have been activated by the acetylcholine. In MG, there can be as much as an 80% reduction in the number of these receptor sites. The reduction in the number of receptor sites is caused by an antibody that destroys or blocks the receptor site. Antibodies are proteins that play an important role in the immune system. They are normally directed at foreign proteins called antigens that attack the body. Such foreign proteins include bacteria and viruses. Antibodies help the body to protect itself from these foreign proteins. For reasons not well understood, the immune system of the person with MG makes antibodies against the receptor sites of the neuromuscular junction. Abnormal antibodies can be measured in the blood of many people with MG. The antibodies destroy the receptor sites more rapidly than the body can replace them.'(Courtesy of the Myasthenia Gravis Foundation of America http://www.myasthenia.org/)

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I will continue in my next blog post with my treatment plan for myasthenia gravis and also more surprise diagnosis’. ‘Sigh.’ It never ends.

XO