Seeing more than face value

This year has been a complete mishmash of emotions & events -
Hospital visits at least once a month; new drugs; new treatment; new doctors... 
Relapses; tears; laughter; hope...
Insecurities; positivity; negativity; love...
Learning my limits; pushing too hard; walking sticks; medical mask...
Friendships; braais; naps; working...
Care; support; loss; family...

I wrote a story on my private Facebook page about how I had been judged for wearing my mask out in public - one that I was told I needed to wear as the treatments I am on has destroyed my immune system & there is a high chance of infection.
I had an amazing response to this (with a few negative comments, but I have learnt that you will always get these trolls!)
My main point of ranting & writing what I did?  To create awareness for Invisible Illnesses - as we are all fighting a battle that many may not know about.  And we need to learn to be kind to everyone that we come into contact with.  You never know whose day you may be brightening!

My story was then shared by "I Have A Name"

This was amazingly picked up by "Good Things Guy" (if you haven't subscribed to this blog then you need to do it right now!  We all need to remember the good in life!)

This has lead to so many patients coming forward, registering with Rare Diseases SA & we have been able to offer support to all of them & help them realise that they are not alone.

The Times also shared my article, & this completely blew my mind.  I never expected my little rant to reach so far, or for the support & love to be poured out from all over SA.  Invisible Illnesses are now being recognised; kids are being taught to ask questions & not just point & stare; parents are reading up more; patients are not simply being judged; & slowly slowly the world is becoming a better place - step by step.

Working for RDSA, I have realised the need to create awareness & I will never give up on this!  I do not want it to be a "look at me, look at me situation"; but rather for people to try to understand so many of our patients who are in constant battle with their bodies & never give up.  They are superheroes in my eyes; & I am inspired on a daily basis by what I hear & learn from those around me.

So please, learn about the different Rare Conditions, ask questions, offer support & know that you are never alone!

To finish off, I really want to share this amazing interview that I had earlier on in the year with ANN7.  I was so grateful that I had just come out of hospital as I was feeling strong, & was able to talk without slurring.  This was definitely a good day for me & a highlight of my life!

I am still waiting to hear back from my medical aid with regard to the latest treatment plan, so once I know more I will update all of you.  We were really hoping the last lot would put me into remission, but unfortunately this wasn't the case.  My lung capacity has also dropped significantly, meaning that the muscles in my lungs & diaphragm are very weak & not working to their full potential.  This terrifies me, but I am hopeful that this next round will make the world of difference!

As Ellen says, Be Kind To One Another.

Chat soon xxx

Taking The Next Step

After my last blog, we received such an amazing outpouring of love & support.  It honestly blew me away & made my heart feel so full & happy.  It reminded me of the wonderful people that we have in our lives, & how many people TRULY care.  So thank you!

Following my last blog, we have more of a plan going forward...

My boss flew me to Cape Town to see the top neurologist in Myasthenia Gravis in South Africa (Prof Heckmann at Groote Schuur Hospital).  We were at the hospital for close on 3 hours having muscles tests & talking through my previous treatments, as well as my symptoms.  I was having to use my cane again, even though I had only been out of hospital for just under 2 weeks.
Prof Heckmann has advised that I am classed 3B at best, & I was 4B at my appointment.  The table below shows the severity of this, & how close I am to needing intubation.

I have never been informed of my classification, so when I saw this I was quite shocked!  It is never a great feeling to be shown in black & white just how terrible your health truly is.

Anyway, Prof said that I am bad, but I am certainly not the worst Myasthenic that she has seen - every little bit of positivity counts right??  She has given us a way forward with regard to treatment & trying out something new in order to try improve my quality of life.

We have increased my Ciclosporin dose to 175mg twice a day - and these tablets are not for sissies!  The 100mg look like they could be suppositories, & I have nicknamed the little ones "ticks" as that is what they remind me of.  The number of times I have choked on the big ones & had them dissolve halfway down my throat - bleaugh the very thought makes me miserable!

Another important point that she brought up is that I am depressed.  People so often hide their mental health issues, & when she told me this I actually burst out into tears (proving her point I guess...).  I have never wanted to admit just how tough this journey has been on me, & always try to show up with a smile on my face & showing a positive outlook on life.  It has got me through a lot, but everything has come to a head this year.  I have been put onto a very low dose of antidepressant in order to just try take the edge off things & help me cope a little bit better - it has been almost a month & I can already feel a difference in my stress levels & I am not bursting into tears every 5 minutes.

Another point of her plan is the necessity of supplements - due to all the side effects of my medication that I need in order to live; I need to take some other supplements to help me through this & protect my body.  I am now taking Calcium, Slow-K (Potassium), Iron, Vitamin D & a Multivitamin.  Although this seems to increase my pill intake to a ridiculous amount, if it will help prevent further damage I will take them without a single complaint!

And now for the exciting part...
Prof Heckmann wants me to start on Mabthera/Rituximab.
Rituximab is a monoclonal antibody, which is a type of biological therapy.  It is a treatment for chronic lymphocytic leukaemia (CLL) and some types of non Hodgkin lymphoma. It is also used for some non cancer related illnesses. 
Rituximab targets a protein called CD20 on the surface of the leukaemia and lymphoma cells. The antibody sticks to all the CD20 proteins it finds. Then the cells of the immune system pick out the marked cells and kill them. In other words, it has been specially formulated to target the B-Cells in the body & attack these (this is where my antibodies are).

It was quite a mission to get authorisation for this, as it is not currently a proven treatment for Myasthenia Gravis & from what I understand, it is still in the trial phase (as such).  However, my medical aid has been amazing & authorised 4 rounds of the treatment so far.  We were hoping for at least 6 to be authorised, but we are currently discussing this with them & Prof has sent them papers proving the importance of this therapy.  Holding thumbs these will be authorised soon, as the drips are over R60,000 each!!

I had my first treatment last week Tuesday.

I arrived at the Day Clinic at 9am, & husband collected me at 4pm - so it was a very long day.  We started out trying to find a vein for over an hour, before they eventually put a hot wheatie bag on my arm & leaving this for sometime to try bring my veins to the surface.  These poor little veins of mine hear the word needle & burrow as deep into my body as possible, trying to stay safe.  Even when I spoke nicely to them & tried to encourage them to show face, it was of no use.

We managed to get a vein & have a line put up before beginning the rigmarol of all the premeds & actual treatment.  It went something like this:

Saline Solution

2 x Panado tablets

Cortisone Drip

Anti-Nausea Drip

Antihistamine Drip

Rituximab

Saline Solution

I had to be under the eyes of the nurses so my comfy chair was right under their noses - there is a chance of reacting to the treatment & we weren't taking any chances.  Luckily I didn't react while I was there.  All the meds just made me very very sleepy so I caught up on my lack of sleep in one foul swoop.

Wednesday (the day following my treatment) was a tough one.  Because my immune system is being attacked, I am more prone to infections etc, so when I went out to collect my medication from the pharmacy, I had to wear a medical mask.  I was so terribly nervous about how people would react, but the amount of kindness I was shown was amazing.  Smiles from people, people offering to help me & no funny looks - I honestly think sometimes I expect the worst which is not at all fair to others.

Snapchat mask - still looking for one that is this pretty!

I went out to get my medicine, & when I got back home I felt extremely short of breath & so nauseous.  I do not usually like reading up what the side effects are as I think your mind is a very powerful tool & can make you experience these symptoms...

I messaged my friend/boss, who sent me the following info:

Get emergency medical help if you have any of these signs of an allergic reaction: hives; chest tightness, trouble breathing; swelling of your face, lips, tongue, or throat.

Some people receiving a rituximab injection have had a reaction to the infusion (within 24 hours after the medicine is injected into the vein). Tell your caregiver right away if you feel dizzy, weak, light-headed, short of breath, or if you have chest pain, wheezing, sudden cough, or pounding heartbeats or fluttering in your chest.

Rituximab increases the risk of a serious viral infection of the brain that can lead to disability or death. Call your doctor right away if you have symptoms such as confusion, trouble concentrating, problems with speech or walking, vision problems, or weakness on one side of your body.

Call your doctor at once if you have any of these other serious side effects, even if they occur several months after you receive rituximab, or after your treatment ends.
fever, chills, body aches, flu symptoms, feeling weak or tired;
ongoing cold symptoms such as stuffy nose, sneezing, sore throat;
headache, earache, painful mouth ulcers, skin sores, warmth or swelling with skin redness;
pain or burning when you urinate, urinating less than usual;
severe skin rash with blistering, itching, peeling, or pus;
weak pulse, fainting, overactive reflexes;
muscle weakness, tightness, or contraction; or
lower back pain, blood in your urine, numbness or tingly feeling around your mouth.

Other common side effects may include:
mild stomach pain, nausea, or diarrhea;
muscle or joint pain;
back pain; or
night sweats.

OH JOY!

The nausea has been something out of this world, but luckily my breathlessness seems to have come right.  But I am on "house-arrest" now in order to try protect my body & get the most out of this treatment.  I don't think I actually realised how much this would take out of me, or how crap I would feel.  I have slept for at least 2 hours every afternoon & struggle to find something that appeals to my stomach at the moment.  I am hoping that my body will adjust to this new treatment, & hopefully things will get better as time goes on.  Positive thoughts people!

The ladies that I work with have taken up a roster to bring us food, meals & happiness so I don't have to risk an infection going out shopping & I can focus on getting better during this time.  I honestly don't know what I would do without them & I really feel so blessed by their friendship & compassion.  It has made my life so much easier & seeing friendly faces when I have been cooped up in our little house makes the world of difference.

So, this is where we are right now.  We don't know when/if we will see results.  They have advised that it will be at least 3 weeks before we see any improvement at all, & I am going back to Cape Town after 3 months to reassess & see what our options are.  This is an exciting time, & although it may be difficult - it is only for 3 months & may last for a lifetime :)

50% of patients with Refractory Myasthenia Gravis go into remission on this treatment & thus we are praying that I am one of those!

In other news, I was recently interviewed about living with MG & you can view this at https://www.youtube.com/watch?v=zbEUqF9q_K4&sns=fb  Any awareness created for this horrid disease is something I really appreciate & I was so grateful for this opportunity.  Plus my furkids made their first TV appearance & stole the show!!

Acceptance

My last blog was a very long time ago...  There has been so much happening & we have been trying to process it all before we let everyone in on it.  My emotions have been all over the place, & I think (thanks to a friend for pointing this out!) that I hadn't fully accepted what has happened.

It has been an extremely busy year so let me quickly try summarize what has been going on - otherwise you could be here all day reading my story & I think that will drive you mad!

I am still going into hospital to plasmapheresis - we have realised that this is what is keeping me going & unfortunately my health has declined to the point where I spend only 2 weeks at home before needing to be admitted for 4-5 days of plex.  The effects of the plasmapheresis only last for 4 days before I start declining again, & then I battle for as long as possible before needing to be admitted.  I am now making use of a walking stick to help me keep my balance & take some of the unnecessary stress off my body when I go out in public.  I am exhausted constantly - from fighting my body, from putting on a happy face & from the emotional turmoil that I have been through (more on that later).  I have been on 4 different immune suppressant drugs over the past year & a bit to try control my MG, but unfortunately these are not working.  Methotrexate, CellCept, Azathioprine & Cyclosporin are just not making a difference.  I am on 4 different other medicines on top of these as well - each with their own array of side effects.
Before I used to come in for plasma once a year (as a maintenance treatment - think of it as changing my batteries to keep me running for another year), & now I am coming in after a fortnight (my cup has a hole in it, causing my health to leak out - the plasma is replenishing the liquid in the cup, but we are in a constant battle to try fill it up & cannot find the "leak" or how to clog it up).

Feeling grim & unable to smile.

 My darling precious granny passed away earlier this year,  It was the absolute worst thing I experienced.  Gogo was so much more than just my granny, & being away from my family at this time was extremely difficult.  She passed away in her sleep, but was battling for some time with her body slowing down & her memory falling away.  I miss her every single day, & often find myself picking up my phone to give her a ring & let her know what has been happening.  She truly "got" me & could always make me smile when I was having a down day.  I am so lucky to have had her as such a big part of my life for so long, but it doesn't make things any easier. The stress of losing her definitely affected my health, & I was admitted for emergency treatment just after her passing to try lift me out again.

With Gogo at our wedding

Together with the cousins at Gogo's memorial - bright colours for our precious granny.

Husband & I have been trying to still live our lives & suck the marrow out of it whenever possible.  This isn't always easy - especially with my health as it is.  We cannot make plans for more than a week away, as we don't know what will happen with my health or when I will relapse again.  We cannot travel too far from the hospital & need to be constantly aware of my abilities.  One braai out with friends means almost 16 hours of sleep to try recover - and paying for it for the next week.  We have amazing friends that truly understand this & take such good care of us - having husband over when I am in hospital & constantly checking up on me to make sure I am behaving!!

I was blessed to go to Barcelona, Spain for the EURORDIS Summer School on Patient Advocacy & Clinical Trials with my job for Rare Diseases SA.  What an amazing opportunity!  I met the most amazing people & came back with so much knowledge & excitement for the future.  We also managed to squeeze in some sightseeing as this was my first trip to Europe, & I really felt truly blessed.  The doctors did 4 rounds of plasma before I left so I was strong for my trip & could properly enjoy it without missing out on too much.  I did go for naps every lunchtime & pressed snooze on my alarm more often than I should have, but otherwise it was even more I could dream of.  I did choke on my food occasionally; missed out on meals as I couldn't swallow; & kept quiet more than I wanted to as talking was too much of an effort - but my heart was happy & I was determined to not let my health make me miss out on this experience of a lifetime.

With Helen from Genetic Alliance

Being a proper tourist

After our time in Spain, I stopped off to see my sister in England.  Katie is my best friend & biggest supporter - being away from her is so difficult & we talk almost every day on FaceTime.  Because I was so close to her, I couldn't miss out on an opportunity to spend time with her & get to see their everyday life.  I was spoilt rotten & it was such special bonding time.  We got to chat, cuddle, catch up properly & see so much!  I noticed myself declining during this time, & was napping for about 3 hours every day.  It was horrid missing out on time for her because my body was letting me down.

My life is enriched by this beautiful sister

Proper High Tea

And now, on to the real point of this blog...

This is so difficult for me to write, & this is why I have avoided writing anything for so long.

We have been through the stages of Grief:

1.  Denial  (This can't be real!)

2. Anger  (Why me?  This just isn't fair)

3. Bargaining  (If I get better, I won't take my life for granted; & all the "what-ifs")

4. Depression  (I didn't want to leave the house, & cried for anything)

5. Acceptance  (I am still trying to get here - but its getting better)

We have spoken to many doctors & they have met with each other to discuss my case.  This is the cold, hard truth.  We have not really sat & thought about what this means for us, until this most recent relapse.

The doctors have now said that they do not have a plan for me, & there is nothing else they can do for me.  

We were really praying that the newest medication would make a difference in my life, but I am slipping further back every time.  My relapses are coming quicker & closer together, with it now affecting my breathing.  My MG has officially progressed to the brittle, refractory form & what used to work for me no longer does.  I am in a constant fight against myself & it is absolutely exhausting.

Trying to stay positive!

We are not giving up hope, nor are we just being negative,  We are being realistic, & trying to find ways to make my lives easier to try take some of the stress off my body - hence the walking stick, having a snooze every day & listening to when my body says - woah, ok that's enough!

For so long I have tried to push it to the back of my mind & not allowed myself to "think myself sick".  But we have been told we need to face up to reality.  The longer I have my Hickman Line in, the fewer options I have.  They are concerned that soon I could become immune to this treatment, & it will not make such a big difference to my health.  Plex/plasmapheresis is what is keeping me alive.

Before coming in for this treatment, these were my symptoms:

I was unable to get off the floor; battling to swallow right from breakfast; slurring my words; unable to pick anything up from the cupboards; unable to reach for anything above my head; unable to hold my head up when bending forward; drooling constantly; unable to give husband/our animals a little kiss; I battled to breathe when lying flat on my back; I had to rest after having a shower & just felt completely drained.  I just cried constantly.

Just starting my third Plasmapheresis in this round of treatment

I am going to see a specialist in Cape Town early next month to see if she has any advice or ideas of what we could do.  My doctors here have been communicating with her, but we have decided that it would be best to actually meet with her in person so she can test my muscle strength herself.

We do not know if we will get any more answers, or if she will have a plan.  This could be the time to try make my life as comfortable as possible; or it could be something completely off the grid that she thinks of that could work.

We are not giving up hope of a miracle, but we are also acutely aware that there may not be a way forward for my Myasthenia Gravis treatment.

My heart is heavy, & writing this blog is extremely difficult.  I don't like to face up to this reality, & never, ever want to be seen as being negative.  Being positive & seeing the good in life has got me through so much.

Please stand with us in prayer.  

We need a miracle.