Thursday, 5 April 2018

Living with "Paul"


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.

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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