When a liquid medication becomes your magic potion
(ADVISORY: THIS BLOG OR ITS PHOTOS MAY DISTURB YOU!Maybe also because of my bulky & fat appearance)
I
was given the diagnosis of Pulmonary Hypertension in April of 2016, so around two years
ago.
My first meds were Macitentan (Opsumit) and Sildenafil (Revation), the ladder was exchanged to Tadalafil (Adcirca) in July of 2016. I felt pretty good with it and noticed tremendous improvements compared to the time before diagosis and medication.
Unfortunately,
around half a year later things slowly got worse and I knew that
there was something wrong with me.My first meds were Macitentan (Opsumit) and Sildenafil (Revation), the ladder was exchanged to Tadalafil (Adcirca) in July of 2016. I felt pretty good with it and noticed tremendous improvements compared to the time before diagosis and medication.
Being confronted with therapy escalation
So
I went into hospital in January of 2017 to have a RHC and boom! my
mPAP had risen by 13 points to now 66 mmHg. This came as a shock,
especially because I was now taking meds, so I really didn't expect
the pressure to still rise but to remain stable.As if things weren't bad enough there was this one doctor at the ward who must have missed the lecture when empathy was on study plan. The day after the RHC he came into my room during his medical round and the first and only thing he said was „Did you ever think about a lung transplant?“ I just call him „the butcher“ from now on.
Surely, I was aware of the fact, that there is the option and last chance of a TX but honestly and I really mean, HONESTLY, I thought I still had plenty of time.
Anyway, I was really scared about this twist. I heard about other patients enjoying a triple therapy with meds like Iloprost (Ventavis) or Selexipag (Uptravi) so when I spoke to the chief physician the next day, I touched upon trying a triple therapy first before plunging head over heals into the TX topic. I asked him directly whether I could try Selexipag as I thought taking another pill wouldn't harm anyone.
The "R"-word
Unfortunately
I didn't like his response. His exact words were „If you were
taking Selexipag or Iloprost it would have the same effect as if you
were taking M&Ms or TicTacs. You need something way more powerful
with you degree of PH. The only option for you is Remodulin!“
And
here it was: the nasty word I didn't want to hear either …
During
former hospital stays I got to know some other patients and some of
them had the sub q pump with Trepostinil (Remodulin) and all of them
had the most horrific side effect esp. suffering from site pain. One
thing I knew for sure: I never ever wanted to have this medication
and now the doctor only offered me this one option.
Luckily
there was still one straw I could grab.
In
Germany we are lucky to have the option to get a Trepostinil Infusion
Pump implanted to subserve the body with the Trepostinil through a
catheter which goes right into the superia vena cava. So to put it
short: no fuss with covering up the needle when showering, no site
pain and such. The only requirement for this implantation is that you
should have had sub q remodulin pump for at least 6 months. This has two
reason: 1. The doctors want to make sure that you respond positively
to Remodulin and 2. The health insurance companies only want to pay for this
surgery after you've been tested whether you do respond to the med or
not.
This is the LENUS pro Infusion Pump (size 40 ml) |
You
should have seen me … I literally begged my doctor to get this
implanted infusion pump right away. I guess I would have done
anything.
The
chief physician told me that he would check all possible options with
the other doctors and the surgeon and would tell me then, whether
they would try it or not.
What
I didn't know was, that I was patient number „Zero“ … the first
ever patient (at this specialized PH center) who would receive this
implanted infusion pump WITHOUT having any prior sub q Remodulin
before.
Of
course they've done surgeries like this before, they were trained in
implanting the pump apart from the fact that the patient haven't had
Remodulin before.
ICU, Remodulin Titration and Surgery
So
I returned to hospital in March of 2017 and went to the ICU for 5
days where I was titrated with Trepostinil. Every 8 hours the dose of
Remodulin (which I received intraveniously) was increased and I could
almost set the clock that half an hour after the the dose
increasement I needed to vomit and had diarrhea. These 5 days felt
like hell. I'm not exaggerating. So many doctors came to see me each
day simply because I was something special, something unique,
something they've never done before and therefore needed special
treatment, so to speak. I thought that even the Pope and the Queen
couldn't feel more special than me. Though I didn't feel special at
all, in fact, I felt like being run over by a train due to the
Remodulin side effects during titration.
Me at ICU starting the preparations for titration of Trepostinil/Remodulin |
On
March 29th, 2017 I had a 2 ½ hour lasting surgery and
became an „alien-mummy“. Paul (the alien) is now my lodger. The
infusion pump was implanted in my abdominal below the costal arch.
I
started calling him „Paul“ or „The Alien“ because my tummy
looked like I was pregnant with an alien shortly after the surgery.
Completely dented and bloated not to mention the rainbow colours of
the bruises I had both around my tummy as well as on my shoulder.
The
surgeon made two cuts, one to insert the pump in the abdominal and
the other cut in the shoulder area to thread in the catheter into the
vena cava superior.
Afterwards
I looked like as if I have been a victim of domestic violence, maybe
the surgeon used me as his punchbag (well, only kidding of course –
he was very nice!) ...
Luckily
I hardly had any post surgery pain, at least not as severe as I
expected. In retrospective, the titration phase in the ICU was far
worse than the surgery itself.
This is the abdominal surgery site, pre-haematoma - The sitches looked tiny & nice |
Unfortunately
I needed a second surgery a week later as there was a haematoma which
spread around the pump in the abdominal. This second surgery was
performed with local anaesthesia only and the surgeon was truly
worried I would go through the roof because of the pain and
discomfort but the only thing which bothered me was that my butt
started feeling numb after around 30 minutes. So everytime the
surgeon stopped removing the haematoma I asked whether I could
relocate my bottom. As there was a surgery drape in front of my face,
I could only hear but not see the surgeon and the nurses.
I
was later told by one of the nurses from the Pump Support Company
that all the doctors as well as nurses were almost biting their hands
because of laughter because the only thing I worried about was my
numb butt and not the pain I was going through.
Stitches at the abdominal post-haematoma |
Stitches at the shoulder, massively bruised as well |
Anyway,
after this trouble everything when uphill again despite some trouble
during the first „external“ filling of the pump.
Monthly feeding of "Paul"
The
infusion pump needs to be refilled every 28 days. This is taking
place at the PH center which is luckily here in the same city where I
live.
So
when the pump was refilled for the very first time on May 3rd,
2017 due to the massive swelling I had because of the two surgeries incl.
haematoma, the doctor had some trouble „finding“ or feeling the
filling membrane. There were in total 3 doctors as well as a nurse
from the Pump Support Company present and they all tried to find the membrane
by feeling it. In the end I needed to have x-ray to locate it
properly. Said and done but then when puncturing the position where
the membrane was, suddently seroma was regurgitating. In total it was
3 canulas of seroma which the doctor removed from the area above the
pump.
Then
he punctured the membrane again and succeeded and had a regurgitation
of a mix of Remodulin and NaCl. It is important that there is a
regurgitation of „old stuff“ when getting a new filling. To run
empty of your pump filling can result in dangerous withdrawl
symptoms, esp. when you're on a single or Trepostinil-Only-Therapy. A
low regurgitation could mean that the pump isn't working properly or
that you've maybe had an infection or flu, a rise of the body
temperatures results in an flow increasement of the pump. Same
happens when you are maybe sunbathing. You need to be careful what
you do, even with a fully implanted infusion pump.
Anyway,
afterwards the pump was refilled with an injection of NaCl, then an
injection of Remodulin and another injection of NaCl. This order is
immensively important!
The
doctors would never ever directly or firstly inject Remodulin, as it
is so concentrated it can literally KILL YOU!! So you always start
with an injection of NaCl, this doesn't do any harm! The second
injection is then Remodulin and it mixes with the NaCl inside the
pump. The doctors always finish the filling process with an injection
of NaCl again, simply to save the Remodulin which is still in the
tubes. Remodulin is damn expensive, 18 ml cost about € 20.000,-.
And
I'm currently at my „end dose“ of 18 ml Remodulin which I need
EVERY 28 DAYS!
Finding the filling membrane by my doctor by "feeling" it |
NaCl being drawn up in a syringe |
All utensils needed for refilling (2 syringes with NaCl, 1 syringe with Remoduling & needle on the left for regurgitation) |
Being sprinkled with disinfection spray (several times!!!) |
Creating a sterile environment for regurgitation |
Filling of the infusion pump with NaCl & Remodulin Hard work, as the doctor needs to press against the resistance in the pump triggered by the gas filling |
Mission accomplished, "Paul" fed, Patient happy! |
Massive improvement thanks to Remodulin Therapy
Only
around 3 months after surgery and implantation of „Paul“ I could
notice the severe improvement of my overall status.
I
added 50 m to my 6MWT and am now at 525 m. My pO2 is currently at 69
% and …. hold on tight now …. when I had a heart ultrasound in
December of 2017, my right atrium reduced 1/3 in size. From looking like as
flat as a pancake with a size of 42 cm² now down to a size of „only“
25 cm². Still a nightmare for any cardiologist but for a PH patient
a good reason to go down to your knees and praise the lord (not that
I'm religious …)!
So
there's this slogan some Remodulin patients and I came up with: REMO
CAN DO!
It's
our magic potion to feel so much better. I would go through all the
hassle again and again and I can strongly urge everyone to take
advantage of this medication when you are offered it.
And
just to put this straight: I seem to be the only one with this much
trouble during surgery and shortly afterwards. And it's not down to
the clinic or the doctors. Trust me, they are just superb! I can
truly say that they have saved my life already 3 times within the
past two years!
To say that I'm grateful is a complete understatement!
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