THE ODDS OF DAILY ROUTINES AND RANDOM ACTIVITIES

When even the littlest efforts backfire on you

Two days ago I had my 2-year-“anniversary“ of being diagnosed with Pulmonary Hypertension. During these two years I had in total an amount of 3 incidents who almost sealed the end of everything for me. Luckily I only realized about this months later.

But when you do „stupid“ things and don't listen to your body or ignore warning signs it usually backfires on you, even more with PH.

So during these two years I managed to learn a lot about activities and when it's too much or what too much actually is. I developed strategies how to schedule the duties and things I had to do.

That means, usually only two appointments a week (doctor or others) and most of the time I make sure that there's at least one day inbetween to „recover“.

Same with duties around the house. I do very little each day as even the tiniest things really tire me out and I'm exhausted afterwards.

For someone healthy, doing the dishes is nothing to worry about. For me, it is.

I don't have a dishwasher, as my kitchen is pretty tiny. Maybe I should really consider of getting one installed somewhere.

Anyway, when I've done the dishes, I'm also „done myself“ and need to rest for at least half a day.

Even showering, washing and blow-drying the hair, lotioning my body and getting dressed is at some days a milestone achievement. Usually, I sit down afterwards and stick a pulse oximeter at one of my fingers, watching my pulse decreasing and my oxygen level increasing. There I sit, breathing deeply in and out and try to catch my breath again.

I can continue this list forever …

And then there are the days when you actually KNOW that certain things shouldn't be done by yourself as they are way too exhausting for you and you KNOW doing these things will strain your heart for a long time if not forever and they will generally backfire on you the following day.

So I ways enjoying the warmth and sunshine of spring on my balcony, sitting there in my deck chair and starring at the balcony panelling which was GREEN of dirt.

A friend of mine tried to see it from the funny angle, calling it „Wildlife Balcony Patina“ but honestly, it HAD TO GO!

Wildlife Balcony Patina

So what happened was, that I started cleaning part of it on Tuesday this week. The dirt was so sticky to the panelling, it took so much of my energy trying to remove it.

No surprise I was completely shattered after doing around ¼ of the whole panelling.
It looked good, no doubt about that but still it was very hard work for me.

So I rested on Wednesday, just went shopping with my mum and then later sat down on the balcony again.

So yesterday (Thursday) I decided to clean the rest. I have this mop with a telescopic handle and even though it helped me loads, it was still VERY exhausting removing all this sticky dirt. I was sitting on a stool, while doing it but I needed a lot of energy to handle the mop.

Patina Free Balcony

When the mission was accomplished, I sat on the balcony enjoying the clean panelling and the sunshine.

I FELT okay after a while and was certain that I dosed things correctly by splitting it in two parts.

Enjoying a little treat: Coffee in my favourite Coldplay mug

A Magnolia Tree I can see from my balcony

Then came last night. I woke up because my shoulders, neck and arms were hurting.

DAMN!!! I really should have known better and not being so careless.

Luckily I got back to sleep and even though waking up for my first meds at 7 am, I fell asleep again and overslept my second meds at 8 am by only waking up at 9 am. I honestly felt like being run over by a train. Luckily this didn't last too long and right now I'm okay again.

But what I wanted to say is, even sometimes if we feel okay and think we can handle things we are always proven wrong and getting the bill afterwards.

Although I am grateful for all the different medication I'm on which help making me feel better, they are a "curse“ also …

A "curse" because we sometimes tend to forget how poorly our health is, as the meds are covering the sympthoms and most of us feel so much better.

Even the best educated and long-term PH patients sometimes make the most stupid mistakes …

I just hope I won't make too many of them. This „cleaning-session“ taught me a lesson!

At least for now.

Time Frames

What considers a disease to be fatal or terminally?

A friend of mine, who is also suffering from Pulmonary Hypertension, and I were discussing a controversial topic the other day.

What considers a disease to be fatal and terminally?

Just to explain what I am up to I need to go far afield.

My friend was diagnosed in spring of 2013 and her doctors predicted that she probably wouldn't witness Christmas that same year because of the severeness of her disease.

She has an amazing doctor. Well, most of the PH doctors are truly, really special. They have this empathy many other physicians have lost.

Back to my friend … she took part in a study and received a certain medication in a very short titration phase and this medication literally saved her ass.

She was lucky to experience Christmas of 2013. And she even celebrated Christmas 2014, 2015, 2016 and 2017 also.

She is living with PH for 5 years now, although she was given the worst prospects and was doomed.

So this brings up a scientific assessment and a pragmatic question:

1. SCIENTIFIC: Even doctors can't predict or rate the severeness of the disease. Every patient is different, there are no such things as rules for everyone, medication for everyone or a general outlook. Every person is different and needs her/his personal mix of medication & dosis to be treated.
   Which makes things a lot more complicated as doctors need to juggle with meds and doses and this takes up a lot of time until every patient's individual therapy is found. And some patients are lacking this extra time. In reverse others, who were given only a short time to live are still around due to competent doctors, the perfect therapy and just pure luck.
2. PRAGMATIC: You tell others who are not familiar with PH that you are suffering from a fatal disease and that you are terminally ill. Do they expect you to die within a certain timeframe? Like with cancer patients, where the doctors often can considerably estimate way better how long a patient still has to live.
   How long is this timespan for someone suffering from PH? From which time on do others don't believe you anymore, cause „you are still around and alive“?
   Do they possibly think that you are actually exaggerating your condition to make them feel sorry for you?
     

To get back to the beginning of my blog and to the conversation I had with my friend, she told me, that at the beginning, shortly after she had been diagnosed with PH, she told her friends and relatives about her condition and that she was terminally ill.

Now she is joking, that sometimes she feels the need to say sorry to her friends that she is still around. She worries that some of them may think she is a liar and is only exaggerating.

So, do you understand what I mean? Being terminally ill and suffering from a fatal disease doesn't mean you only have to live days, weeks or months. Sometimes, when medication works good for you and you are lucky that maybe the size of the right ventricle is decreasing, you have a few more years on your bill. You never really know WHEN you have to die. It could be tomorrow but sometimes it can also be years away.

This disease is considered to be fatal and terminal because there is still no cure to heal it. All the medication we receive are only meant to make life more bearable and to increase the quality of life. Also, the amount of stays in hospital should be reduced due to the medication. The overall outlook is still the same.

Unless we don't get a lung (or sometimes even heart-lung) transplant we are cursed to die way earlier than a healthy person due to the fact that the heart will at some point fail.

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 29^th, 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 3^rd, 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!