Just breathe...

At the end of last year, a friend reminded me how important it is to go for all my check-ups - I had been so focused on my MG throughout the year that everything else had fallen by the wayside.  I hadn't even gone to the gynae since my miscarriage in January 2016!  After her constant nagging reminding; I made an appointment with the most amazing doctor & went in for all the normal checks & tests.

On my husband's birthday, we got a phone call to say they had found abnormal cells & I needed to go into his rooms asap for a biopsy of my uterus - aka a "womb punch".  I have had abnormal cells before, but they lazered them away without too much concern.  However, my new gynae wanted to make sure he knew what it was before we did anything.
I went into his rooms, absolutely terrified of this unknown!  Let me tell you something now - they say it doesn't hurt, but that's a great big lie...  Take a pain killer before. You are awake & quite able to feel everything that happens.
He also told me I am now insulin resistant, so need to add Glucophage to my daily intake of meds, as well as cut out all additional sugar from my diet.  No more chocolate or sweets, but rather try get my body as healthy as possible.  We think this is all as a result of the prednisone I have been on for many years, & the horrid side effects this has.  What may help me now has so many long-term effects, & I am only seeing a lot of these now...

I am very glad I went for the biopsy though, as I needed to know the results.  As painful & terrifying as it was; when they called to tell me I had tested positive for early stages of cervical cancer; I was so grateful to my friend for pushing me to make an appointment & that we could sort it out now.

My surgery was booked for 10/01 to remove endometriosis; ovarian cysts; CA cells; do a full D&C; & remove my remaining tube as this seemed to keep blocking up & causing infection.  I wasn't too nervous as I knew I was in the best hands possible & it would be a quick recovery - I have had them often enough to know I can handle this!

More scars added - luckily just little ones though!

The morphine drip that was my very good friend

My surgeon was happy with the results & I was discharged the following day with strong painkillers & instructions to just rest & take things easy - follow up in a months time.

Ok, I've got this!  Not the best start to my 2018 (I was meant to go back to work on the 10/01 & instead was being wheeled into theatre), but now all the baddies have gone; he managed to get all the cancer cells & now we can focus on moving forward...

The recovery from this op seemed to be more difficult than before, & I was absolutely exhausted the whole time.  I thought perhaps the pain killers were too strong for me, as I hardly ever take them so I decided to stop these.  Still I felt completely lethargic & constantly out of breath; plus my heart would go crazy then seem to slow right down.  I researched the Glucophage to see if it could be that as my dose had been increased & I found the following:

• tiredness
• weakness
• unusual muscle pain
• trouble breathing
• unusual sleepiness
• stomach pains, nausea, or vomiting
• dizziness or lightheadedness
• slow or irregular heart rate

Well, this is pretty much everything I was experiencing, so it must just be the meds right?  My body just needed to adjust & I just needed to push through.  I spoke to some other friends who had been on it as well, & they said they also battled initially but once their bodies got used to it then everything was much better.  Right, so I'm just a wimp!  Time to put on my big girl panties & deal.

However, the following weekend my husband & I quickly went to the shops to get a birthday present for a friend's daughter & I almost fainted while we were out.  I could hardly keep up with him in the shops, & I was sweating by the time we made it back to the car.  I honestly thought my lack of fitness was catching up to me, & I was more embarrassed than anything else.  I took things easy for the remainder of the day, & missed our friends' braai so I could have an early night cuddling my dogs.

On Monday morning I woke up & still felt a bit short of breath, but knew I needed to pop into the shops to get my sister's 40th birthday present.  I parked at the closest parking possible, & made my way inside - taking my walking stick just in case.  I had to pause at every seat along the way to the shop; take a breather; & almost passed out a couple of times.  I realised something was not right.  This is more than just my meds!  So I phoned my doctor who had administered my Rituximab & made an emergency appointment to go in & see her - she had said to me that if I feel any signs of a cold or flu coming I needed to go to her so we could sort it out quickly.

I walked into her rooms & collapsed on one of the chairs in the chemo treatment room, where I promptly burst into tears.  I was absolutely exhausted & so confused about what was happening.  Why was my body doing this?  What was going on?  Am I taking too much medicine now?
My pulse was 172, & BP was 145/100 - just from walking the 10m from my car to the rooms.  They decided to do blood tests & a blood gas (from arterial blood - not fun & so painful...) & they made me a cup of tea while we waited for the results.

Surprise!  Hospital admission - urgent.

A porter was called from the hospital & I was taken straight through to the ward where I was put onto oxygen & sent for X-Rays & a CT scan of my lungs.  They knew that something was wrong, but we weren't sure exactly what.  
Their fears were a clot in my lungs & we needed to see this asap!
I was not prepared for a hospital visit - all my meds were at home, my car was still in the car park at Dr Gunther's rooms & my cell phone was about to die & I had no charger.

I rushed off to get the Xray & CT scan then returned to my room to wait for the results...  That evening my doctor came to say the results weren't conclusive so we were going to have to either do a needle biopsy of my lung or a scope to try find out more.  
"But don't worry, you will be asleep for these!"
Well thanks, I am so glad.

Just after she left, the originals were all delivered to my rooms & I got to see what my lungs were doing...

You can see my perm cath & stitches from my Thymectomy here too

Not very pretty lungs

Those white bits in my lungs?  Those shouldn't be there...

I had no cooking clue what this all meant, & sent the images to my boss & family to let them know.  They were clever - perhaps they could tell more than me.  But nobody was saying anything.

The next day my doctor came back to see me & said she had found a cardio thoracic surgeon to perform the biopsy but she was currently in surgery so would come see me when possible; but they were starting me on an antiobiotic straight away just in case.
I asked her for the truth of what my scans were saying, as they all seemed a bit nervous.  She told me it was one of 3 things - a really bad infection (e.g. TB); another autoimmune disease; or lung cancer.  Given the fact that I had CA cells removed a week earlier, this didn't sit well with all of us.

My friend came & sat with me for the whole day so I wouldn't be alone with my thoughts & concerns & she was also there to help me sort through my emotions.  We discussed the reality that this could be it for me.  I may not make it through this op, as my lungs are already compromised from whatever this is; plus my muscles are so weak from my Myasthenia Gravis; plus I had an anaesthetic just over a week ago.  It really hit home for me.
I did realise the following:
I do not want extreme measures to be taken to keep me alive.  I don't want to be a vegetable or a burden on everyone else forever.
I am signing DNR forms.
When I die, I want a celebration of my life!  No sadness & tears, but a party with music & laughter & balloons - think of Kate Hudson's character in "A Little Bit Of Heaven".  That's what I want!

My friend helped me ask the right questions to the doctors & could take in more of what they were saying at that stage than I could.  She also organised for my non-invasive ventilation machine to be delivered to me so I could hopefully start sleeping better at nighttime, & rallied around to get me food, visitors & all sorts.  

So little & neat!

My sexy mask - the machine forces air into my lungs which helps a lot when I can't get a deep breath in

The surgeon arrived that afternoon, introduced herself & picked up my scans.
When she just went quiet I knew something was wrong.  
"Megan", she said, "I am not happy to take you to theatre now.  I need to talk to the anaesthetist & prepare for this operation.  We won't be doing it tomorrow, but rather on Thursday morning first thing.  Recovery is going to be hard.  With these lungs I need to do an open lung biopsy.  This means I have to cut between your ribs under your right arm & take samples of your lungs.  We may have to deflate your lung to do this.  It is going to be difficult, & you are going to be very sore.  But we need to do this."

My heart sank.  It was my sister's 40th over the weekend & we had planned a trip to KZN to see her as they had just returned from the UK.  I had been planning for this for months.  Could the op not wait until next week?  I promise we can do it on Monday!!
"No Megan, your lungs won't take the air pressure of a flight.  We are afraid that if you fly you may die in the air.  They cannot cope with that."
Right.  Ok.  Gee.  This is actually pretty serious. 
Phone my sister.
Cry.
Hand phone to friend to pass on the news.
Throw temper tantrum.
Cry some more.
Swear.

Wednesday was spent with friends from work constantly popping in with treats & spoils, visiting the cardiologist ("You have a good strong heart Megan!  Nothing to worry about here!" - good news at last!); & going for more tests.  Operation is set for Thursday 8am.  Shew.

Thursday...

I'm ready!

They bring me the super sexy hospital gown & one-size-fits-all broeks - which I promptly place on my head & wait for the nurses to say something.  I needed laughter & happiness to fill the gaps that fear & concern were making in my soul.  They told me I'm not normal & gave me a big cuddle to help ease my heart.  My friend arrived by my bedside, squeezed my hands & told me everything would be ok.  Pre-op meds were given & off we went...

This was it.  I didn't even get a chance to message everyone.  
Was I going to make it through this?  
What was the outcome going to be?  
Can I go home now?  
My mind was full of so many questions, but at the same time I was already getting sleepy from the meds & just wanted to relax into the bed - now I understand why they give you these!  We were told the op would take maximum an hour & I would then be taken to ICU so they could keep an eye on me.

127 minutes later I was wheeled out of surgery into ICU.  I had made it, & didn't even need to be on a ventilator!  They had needed to reinflate my lung, but otherwise all went well.  They also removed my perm cath so for the first time in a year & a half I don't have a pipe hanging out of my chest!!  They were happy.  I had morphine, a thoracic epidural for my lung area & I was floating - as long as I didn't try to move.  I had a drainage pipe to get rid of any muck/blood that pooled around the biopsy area & I was left to sleep for the remainder of the day - except for when the physio came to get me to sit up.
I almost vomited from the pain & they quickly realised it wasn't going to work today!

Friday...
Sleep, & I managed to sit up in a chair thanks to the physio.  They said I needed to fight through this as we needed to start expanding my lungs & get them working again.  I was determined to be able to breathe better asap so I didn't give my body a chance to back out.  We still didn't know what this was, but my samples were being tested & I knew we would have answers soon.
I even managed to walk to the entrance of ICU with a walker & the physio holding on to me very tightly!  However, going off the oxygen is not an option as my stats dropped every time we tried.

Saturday...
Drainage pipe out!  Catheter out! Arterial line out!  Woop woop!  I manage to walk to the bathroom on my own with the walker & don't pass out.  I still can't move too much without pain, but every day is progress.
The doctors come that evening to say that the blood tests etc have shown that I don't have an infection as my white cells are completely normal - so no TB.  This is good news, but at the same time it says that this is most probably lung cancer.  I don't know how to take this.  My mind is going crazy.  How is this happening to me??

Sunday...
Morphine drip removed - I was managing without it & didn't want to become addicted.
I sent the following message to my family:
"Hi guys.  Managed to sneak my phone in.  Still in ICU - had a bit of a scare this morning when they thought I had an embolism in my lungs but luckily it seems to be ok.  Tests for TB & other infections have come back clear, but we are still waiting for the others.  I was on antiobiotics just as a precaution after the op too!  I'm very sore but managing to walk short distances on my own.  I'm going to be on oxygen for a while now until we can sort my lungs out but I'm feeling positive :)"
My heart kept going into V-tac & they sent me for more xrays, & the cardiologist came back to see me.  Everything came back clear & we just put it down to my crazy body.
Still no clear diagnosis as to what was wrong with me though.

Monday...
My sister & Dave arrived from KZN to come see me.  They spent nearly the whole day at my bedside talking to me & keeping me calm.  Just after they left for the evening, both my doctors arrived at my bed & I went absolutely cold.  This was it.  I could feel it...
"Can I phone my husband?"  I asked them before they could say anything.
"No Megan, it's ok!  You don't have cancer.  You have a very rare lung disease called PCP Pneumonia.  It is common in AIDS patients.  Because you have no immune system from all your treatment for Myasthenia Gravis, you white blood cells couldn't fight it & thats why it didn't show up in your blood results."
They went on to tell me that they have never seen this so advanced in anyone that was so alive, & by my results I should be either frothing at the mouth or 6 feet under.  I should not still be sitting up & talking to them, let alone walking around.  The technician who did the biopsy asked if it was part of an autopsy!
I was immediately started on very strong antibiotics, as well as nebulizers & I finally slept through the night.

People asked why I was in ICU for so long if it was "just Pneumonia".  For one, I needed to be constantly monitored as my oxygen levels were dropping; secondly my lungs are so damaged that I cannot breathe properly; & thirdly this isn't your average case of pneumonia!  I wish it was that simple...

I was finally discharged on Wednesday at about midday, after receiving an oxygen tank for the trip home & my own machine to provide me with oxygen for whilst I am away from the hospital.  My sats kept dropping when I went off the oxygen & we didn't want to take any chances!

So now, this is where I am.  I am taking 4 antiobiotic tablets 4 times a day; along with 12 prednisone every morning.  Add to that my pain killers, anti-nausea, tablets to prevent stomach ulcers & sleeping tablets means that I seem to be swallowing more tablets than food.  I don't have much of an appetite, but am now managing to be without oxygen for much longer periods of time & can even walk up stairs without needing a break!

The doctors told me that they were convinced it was cancer & they were terrified to tell me that - they also didn't think I would make it through the op so I am a walking miracle.  So now, now I am going to live my life.  I am going to take a deep breath in, breathe out & march forward through everything that life has to throw at me!

Reflections

2017 was a difficult  horrid trying year for me, & for many others that I have spoken to!  I don't know what it was about it, but it just was not pleasant & I am so glad to have it behind me...

But, this post is not going to be about all the bad times, or the fact that I was hospitalised more in 2017 than I probably have been in my entire life put together (& that's a lot for me!); instead I am going to write about the lessons I have learned.

Happy New Year from Warren & myself!

1.  Family are there for you no matter what - even if they aren't able to be there physically.  
I think I have given my poor family a few grey hairs over the past year, but they still love me.  They message me; visit when they can; FaceTime me at least once a day (ahem - this would be my sister who was living in England & wanted to check up on how I REALLY was!)  They know that we are not exaggerating the situation, in fact we are most probably underplaying just how scary & difficult things are.  Although we are living further away from most of our family than we ever have before, our relationships are stronger than ever & I really feel blessed to have them all in my life - even my bossy aunties!!!!
They love me for me, warts & all.

Mummy & me

With the in-laws at Christmas having some fun

Me & my special mother-in-law

Owen, my precious nephew, & me

Millie, my cousin's daughter & my goddaughter had such special bonding time this year when I went to Cape Town for a doctor's appointment.  It is scary how much she reminds me of a younger me!

My special family - only missing a few here!

My sister; Dawie-darling & me on our trip around London!

2.  Friendships can be found in the most unexpected of places!
I have made such amazing friends here - the type of friendships that you read about in books or see in movies.  Friends that I could phone at 2am & they would rush over to be with me immediately.  Friends that brought me home cooked meals when I was going through chemo so I wouldn't have to cook.  Friends who would send me an encouraging message when I was having a tough day - without even knowing how much I needed it.  Friends who drove out of their way on Christmas Day just so they could come give me a squeeze & we could have a quick catch up.  Friends who get my crazy & love me for it.  Friends who drop everything to take me to/collect me from yet another hospital stay.  Friends who didn't mind me going to lie down on their bed to have a snooze while we were there for a braai.  Friends who added me into their inner circle as if there was always that spot just waiting for me.  Friends who understand that even if I don't always reply to messages, they are always in my heart.
I have made friends in hospital; at an amazing international conference that I was lucky enough to attend; through my support groups; through my job but mostly just from people who were there for me no matter what!  This is a shout out to those friends who make my world a better place - I have never known such support & love; & it certainly makes everything a whole lot easier.

With our JHB "family"

A friendship that has stood the test of time.

With my boss/mentor/friend

FaceTime with Farlz - one of my absolute FAVOURITE people from school days

My beautiful friend Kim (from Canada) who I met in Spain.

Me & Helen on our bonding trip to Spain.  I couldn't imagine my life without her & her cartwheels!

Navasha & I met on a FB group that we were both in & we just clicked.  This girl has been there for me through everything & always finds the time to pop in with a smoothie & a smile.  I am so grateful for her!

Candice is a fellow snowflake & someone very special to me

3.  Opportunities fall into your lap when you least expect it.
I think so often we take life for granted.  Being able to rush around with work & play; run a 5km fun run; attend events; being able to actually swallow & enjoy a meal etc etc.
This past year has taught me my limits, but it has also taught me that I need to appreciate every single moment of every day. 
Read that book; tell people you love them; work harder & with more heart; be kind; & live every day as if it is your last.
This year I got to go on my first ever big overseas trip; I had some amazing interviews to create awareness about Myasthenia Gravis & Rare Diseases as a whole (some when my talking wasn't great; but this helps to show a side of the disease that I often don't want to); & I got to meet & spend time with our beautiful Mrs South Africa - who I can now consider to be a friend.
None of this would have happened if it wasn't for me having Myasthenia Gravis.

Nicole Capper featured me on her "I See You" FB page; we had a whole episode of Bophelong devoted to Myasthenia Gravis & Rare Diseases; I was interviewed by my favourite radio presenter, Jane Linley-Thomas from East Coast Radio; one of my favourite blogs that reminds us of the good in life, Good Things Guy, did a feature on me; my special friend from "I Have A Name" did a feature on creating awareness for invisible illnesses; ANN& Shape Your Life did a segment on living with a Rare Disease; I was featured on The Mighty - with 2 different posts! - here & here; I was one of the patients featured on Rare Diseases SA platform for MG Awareness Month; my mum-duck wrote an amazing article from her heart about being the mother of a chronically ill child; & News24 helped create awareness through this article.
Shew - writing those all down really made me realise how much awareness was created this year!  It blows me away.  Next stop Ellen - hey, a girl can dream right??

With Zahidah (another MG warrior), Nicole Capper (Mrs South Africa) & Jonathan (Top Billing presenter) from our Top Billing experience.

"Wonder" movie premier

Tourist selfie in Spain

With Nicole Capper for her "I See You" campaign on Facebook.

Me, Nico & Kelly at our first ever Patient Huddle

Some special friends I made earlier in the year, & we were all interviewed by Anele!

4. Mental Health is just as important as physical health
For years I was too ashamed to admit that I wasn't coping with my diagnosis & the limits it was placing on my life.  This year I finally owned up to it & was put onto antidepressants.  I was so ashamed; until I realised just how much they helped!  I could cope with life.  I wasn't crying every day.  I wasn't lashing out at people.  I was finally getting back to my old self, my happy self.
Take care of all the different parts of you - emotional, physical, mental & spiritual.  It really does make a difference.

5.  Doctors & nurses are human too
I have been so lucky to have found the doctors that I have here in JHB & Cape Town (my MG specialist).  They truly care about me; give me hugs at the end of my appointments & only want the absolute best for me.  They have cried with me when I have relapsed & done a happy dance with me when things improve.  The nurses have become my friends & I often pop in just to go say hello to them - I am always welcomed with hugs & "when are you coming back?".  They have held my hands; brought me tissues; welcomed me "home" & truly cared.
We often forget that they are people too that have their own lives; their own trials; their own battles...  Yet they put this all aside to care for others & provide support.  They see the real side of patients, yet they still love us & do all they can to make our lives that much easier.
My doctors have never given up on me.  They have fought for me & kept trying new treatments to improve my life.  I wouldn't be here today if it wasn't for them.

6.  Find a job that you love
I was terrified leaving my stable job with a stable income & doing something that I knew & understaood when we moved from KZN.  I wasn't able to find a job here in JHB due to my constant hospitalizations; & this is where Kelly stepped in.  She offered me a part-time job that I could do when I felt up to it so I could still earn a form of income & not put undue pressure on my husband.
Over time, I have realised that this is my passion!  I love what I do.  I love interacting with patients & helping them as best I can.  I love the ladies that I work with & the fun that we have when we are together.  I have never been happier doing any type of job & I look forward to turning on my laptop every day.  No 2 days are the same & I am constantly learning.  I have become more compassionate; more understanding & also finally found my voice in society.

Kelly, Shevaun & myself - some of the team

So, 2017, you have taught me a lot of lessons.  But, you have shown me that there is always sunshine after a storm.  I have learnt to see the positive in every day.  I have learnt to #ChooseKind. 
But most of all, I have learnt to never give up.  Even when it feels like it is the end of the road; we are still on a journey.  Enjoy the views.  Sit back, relax & look forward to what is still to come!

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.

Under the effects

Yesterday I was re-admitted into hospital to have a new permcath fitted (it was taken out last month after it was constantly blocking during treatment & giving me all sorts of hassles - let me tell you, being free of it for a whole month was better than I could have ever expected!) & a fistula for future treatments.

We were at hospital bright & early, & did my admission so they could start prepping me for surgery.  The idea of going under anaesthetic is never something I look forward to.  With Myasthenia Gravis your muscles are already so weak, that going under & getting certain muscles relaxants causes huge stress to the body & it can be fatal. So even though it is just a small operation, I always start to panic when I know what is coming.  I signed all the necessary forms then waited - and waited.  I was starting to get hangry (angry from the hunger, as the last meal I ate was 7pm the evening before!) & the longer I lay there the more my thoughts started going crazy.

Eventually they wheeled me into theatre at about 1pm & started scanning my veins to see where they could insert the fistula.  It was not good news.  My veins are so tiny & run away the moment I come into hospital so the vascular surgeon said it is impossible to do a fistula as my veins wouldn't hold.  The vascular surgeon scanned both arms & even in my neck but none wanted to participate.  The anaethetist even said I have baby veins & took about 15 minutes just to find a vein that she could put the drip up into.  But, she got one in eventually!

I woke up after the op feeling good & my permcath in with no complications.

They have inserted it more along the side of my chest, rather than down the centre. So movement is a lot easier & the only real pain I am battling with is in my neck.  I think the surgeons are a lot more rough on us when we are under anaesthetic than we realise.  Plus this is the original incision site & where they insert the pipes so its expected to be sore!

My doctor who oversees my plasmapheresis came to see me & she said she hasn't seen me this good in ages, so it actually looks like everything is working now!  She was ok with the fact that they couldn't do the fistula as we are now hoping I won't need this on such a long-term basis.  Wouldn't that be great??  So this permcath will stay in for another 3-4 treatments & we will reassess to see if we think I should get a fistula fitted, or just come in every couple of months for a 'top-up'.

For now though, I would like you to meet my new friend...

Every day I now have to squeeze this stress ball - not only to help get rid of any stress I may be fighting, but also to try strengthen my veins & build them up in case I need a fistula to be fitted.  So it really kills two birds with one stone!  Hopefully this will help make it easier to take blood as well, as this is never a pleasant experience.  I'm telling you, my veins know when it is hospital time & go running to their secret fort, only to return once I am home safely...

Be safe, be kind, be strong xxx