Port & Perm-Cath

Recently I had to be admitted again to have a port & perm-cath inserted.  This was my third anaesthetic for this year, & that thought alone terrified me as having MG is a huge risk when being put under.  We had to be at the hospital before 6am & I was second on the list to go through - so at least I didn't have to wait too long with a grumbling tummy!

I have never had a port inserted, although I know about them & have often had discussions with my doctors about having one inserted - especially recently when my veins have just been giving up on me!  The surgeon I went to has inserted all my other perm-caths & knows me well, so I knew that I could trust him completely.  He saw me before I went under, squeezed my hand & told me that everything was going to be ok.  I really appreciated it, as I was stressing whilst I was lying waiting to be wheeled into theatre (in my super sexy one-size-fits-all paper panties & hospital gown - please can someone design something that doesn't look so dreadful?).

The pics above show how the port is inserted, & what it looks like under the skin.  It takes away the horror of them having to dig for a vein as they simply insert the needle into the spongy centre part of the port.  Currently you can see the shape of the port in my chest, but as time goes on this should settle more & you won't even know that it is there!

I had my perm-cath inserted at the same time as I am still having to go in for plasmapheresis & they cannot continually insert temporary lines as my veins are filled with scar tissue & the pain is unreal when they try to break through this.  I didn't want to have to go under again, & also I don't think my medical aid would like me having to book in again!

About 3 hours after my operation I was finally wide awake enough to message friends & family to let them know that I was fine - and I took the beautiful photo above.  Can you tell I am still a bit high on the medication & very unimpressed with the pain?  Gee whiz - the pain.  It was unreal.  I felt like I had been hit by a train in my chest & was completely miserable.  I don't usually ask for pain killers, but this time I had to.  I just couldn't cope with it.  For such a small operation it sure did have a huge impact on my body!

A couple of hours after this my doctor came & said they were happy with how I had recovered so I could go home - that same day!!!  I have NEVER had such a quick hospital stay & I was so excited to go home to my own bed to recover.  I kept a cushion on my chest at all times to protect everything from my dogs as they love to give kisses & cuddles, & we couldn't risk anything being pulled out/scratched.

Since then things have got better day by day.   I am still quite tired & sore, but I am able to move around a lot more & at least I could take off some of my plasters - I react so badly to these & sometimes think that the itching may have been worse than the actual pain.

Three Days Post-Op

Tomorrow I am getting my stitches taken out & the perm-cath dressing changed with one of my favourite nurses - Jenny.  I haven't seen her since my last line at the beginning of the year & she is such a vital part of the team that takes care of me.  I can't wait!

We will be starting chemo (through my port - eeek!) in about 3 weeks time.  I am almost finished with my antibiotics for the PCP pneumonia, & once that is done I will have more blood tests & then the new treatment begins.  I am excited, but also very nervous.  Chemo is no fun, but I am hopeful this will make a difference in the quality of my life - and at least I have the pipes in for any plasma that I may need.

Onwards & upwards!

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.

An unexpected addition

When I finally moved to Jo'burg to be with husband, (the short story is that I was not coping health-wise anymore, & could not even make it through a full day of work without feeling like I was going to pass out driving home & so came to our new home earlier than expected.  I was worse than I have been in years & was terrified of hitting full-blown crisis & thus being separated from my husband for even longer.) I had an appointment with my new neurologist in order to discuss our plan going forward.

She examined me after discussing all my previous medical history & treatments; & said weakness of my facial muscles is pronounced, with my talking very nasal & my uvula not moving at all.  I could not get off the floor without assistance; could not hold my head up against any resistance; my eyes do not close; i couldn't hold up my arms for any amount of time & I was absolutely exhausted.  She was concerned at just how bad I was & sent me for a battery of tests to see if we can get any further answers.

Straight away I went to have blood tests to check my antibody levels; liver function & all sorts of other things - they drew 10 vials of blood in total (yes, I almost passed out & had to sit quietly once they had finished - I am still a huge wimp when it comes to needles).
I then had to go for a CT scan of my chest in order for them to check if my thymoma had come back (I had my thymus gland removed 2 months after I was diagnosed, but if they had left anything behind, this can grow back & cause further antibodies to be produced).  With a CT scan you have to lie very still - you have to hold your breath whilst they are taking the "pictures" so that nothing moves.  They run a drip of dye in that helps them to see everything more clearly - this drip makes you feel warm all over; gives you a metallic taste & makes you feel like you have wet your pants.  Not something they always warn you about so it can give you a bit of a fright!

In my sexy hospital gown waiting for my CT scan

The doctor & I discussed our plan going forward to try get me back to some form of normality & hopefully give me some enjoyment of life.  She has decided that I need to have a permcath inserted as I will need to go for multiple sessions of plasmapheresis & I cannot always have a temporary line inserted - this has become very painful & I have extreme scar tissue build up on the inside that makes it more dangerous & difficult for the doctors.  I will not be able to work for probably the next year due to all my treatment, & the fact that I will be in hospital for at least a week at a time for my plasma sessions.  This places huge financial strain on my husband who now has to carry both of us, & this stresses me out a bit.  However, my CT scan came back clear which is fantastic news!

I was admitted last week Thursday & taken to theatre - right before I went under they were still discussing if they were sure they wanted to insert the perm cath as my MG was really misbehaving, & I am already a risk going under anaesthetic.  We didn't want to take any unnecessary chances!  However, the necessity of getting my strength up outweighed the risks & after a quick prayer I was told to count backwards from 10; & the next thing I knew I was in recovery coughing out the pipe that was down my throat.

The ladies taking care of me in recovery were lovely & took such good care of me.  They stick this amazing pipe under the blanket that blows hot air on you so you feel toasty warm!  They came to take x-rays of my pipe to make sure everything was in the right place, & then I was taken to High Care (where husband was patiently waiting!) where I would spend the next couple of days for my plasmapheresis sessions.

An illustration showing what was done to me & how the pipes work

I had no idea what I looked like, or how much was actually sticking out of me.  I had acted like an ostrich with my head in the sand beforehand & didn't research anything - in fact I thought I was having a little port fitted like what chemotherapy patients have done.  So I got quite a shock when I had a look down & saw all these plasters & 2 pipes coming out of my boob area!

During my very first plasma session after having the permcath inserted

I was very sore that first day & didn't want to move too much - luckily the anaesthetic kept me quite sleepy & the pain killers pretty much knocked me out after that had worn off!  I didn't try be a hero & cope without pain killers as I have learnt my lesson from this before.

The next day I could already smile!  One treatment down & I felt like a new woman!

The top plaster is where they went in - they feed the tubes from here into my heart, & then use a guide wire to feed the tubing under my skin & out a little lower down to attach the outer connections to.

Showing all placement - excuse the heart monitors but these are a necessary fashion accessory in High Care!  You can see some of the bruising already coming out.

After 2 sessions of plex (plasma exchange) I could already feel a huge difference.  For the first time this year pretty much I was able to swallow my supper without choking & wasn't having to toss my head with every mouthful.  The doctors were super chuffed with my improvement & decided I would have 6 sessions this time around & then I would come back in 3 weeks for another 6 sessions.  Having them closer together will help to remove all traces of my antibodies before my body stores up too many again & hopefully I won't head towards crisis again!

I was so lucky to meet a fellow Myasthenic who I have been chatting with online & is in a support group.  She has always been such a sweetheart, so positive & always encouraging.  In fact she was the one who recommended my new neurologist & made me feel safe about starting with someone new.  Dyanne  was admitted into the bed next door to me for her rounds of plasma & it was such a treat finally being able to meet face to face.  She had had a really long journey to receiving her diagnosis & had initially been misdiagnosed with all sorts of other diseases before her drooping eye gave the doctor her "aha" moment that this is MG.  It was so lovely meeting another snowflake & having someone I could talk to, knowing that I was completely understood & wasn't being judged.  We laughed together, bonded over our new book (both of us had bought "Harry Potter & The Cursed Child" to hospital with us to take our mind off things) & just chatted about life in general.  I am truly blessed to have made a new friend, & know that we would probably not have met were it not for our MG!

Dyanne & me showing off our ports!

The doctors & nurses couldn't believe the difference in my talking, swallowing & general strength after a few sessions of plasmapheresis.  I had some special friends come to visit me in hospital & they commented on how they couldn't believe it was the same person!  It always makes me feel so good hearing that other people are seeing a difference in me & when they can get excited with me.
The only thing that frustrates me is when certain people make out I am so much worse than I actually am in order to make use of the sympathy card for themselves; or when they tell my family outrageous stories that aren't true,but make it sound even more exciting - not cool guys.  I don't need any more drama/spice added to my health issues thank you very much :)

I was discharged & am now at home relaxing, taking things easy, but also loving the fact that I can crochet again & have a bit more energy to cope with life in general.  My bruising has come out a bit more since being at home & is now a beautiful dark purple (thanks cortisone for making this look even more dramatic!)

The bruise starting to come out a bit more

The pipe you can see under my skin.

These pipes can stay in for the next 2 years if I take care of them properly.  I am not allowed to get anything wet & have to go for a dressing change once a week where they will properly clean everything, flush out my pipes & replace the waterproof dressing (oh, did I mention that I have developed an allergy to this plaster, but just have to suck it up as there is no alternative!).  Showering is rather tricky & I am trying to figure out how I can wash my hair without getting any water down my chest, but I am sure that over time this will be something I can do without any stress.  I also can't wear a bra as my pipes are just in the wrong place & I can't risk moving them by having a bra rubbing against them throughout the day (#freethenipple was not something I ever thought I would be supporting!).  These are just a small price to pay to feel like I do & to have the quality of life I do right now compared to only a week ago.

Appreciate your health & never take for granted the miracle that your body is in its constant functioning.  It is only when things go wrong that you begin to realise how amazing the human body truly is!