REFLECTING ON 2018

The Downs and Ups (in order of appearance)

THE DOWNS

It was indeed a very cruel way to start 2018.

I guess the misery already started taking its course in late 2017 when a dear friend of our PH community lost her fight with PH and died.

Unfortunately this wasn't the only fellow PHighter.

Even if it sounds like lacking respect – which is definitely not intended – it felt a bit like this „political incorrect“ nusery rhyme called „Ten Little Injuns“.

What I mean to say is that 2018 started with bad news about the passing of PH patients almost every week. Every few days someone of the community posted something about someone losing her/his fight with PH.

At first it was just sad hearing about it. Then it turned out to affect people who were active members of the community, people who were taking part in the discussion and some of them were online only a few days before the sad news. This was really shocking. Shocking as it seemed to have happened without a warning. Devastating because this scenario got closer to yourself than you'd prefer.

Some fellow PH-friends had very unstable health issues or were already listed for a TX when their passing happened. This is of course neither a justification nor an explanation why it happened to THEM instead of someone else but still … I guess we all tend to forget at some point HOW seriously ill we really are since we have superb medication which do support us so much and help us lead such a better life. Also, most of us have dedicated and perfect PH specialists who monitor our figures and if there's a worsening in our status, they will try to do all that is humanly possible to improve or escalate the therapy. That's why most of us probably think, something like this could never ever happen to them, at least not at this point.

This has nothing to do with being naive or unrealistic or that we don't understand the gravity of the situation/condition we're in. It's simply some sort of self protection. A way to cope with the psychological stress we're often in. Life is cruel enough. But when suffering from such a complicated and rare disease like Pulmonary Hypertension, no day is like the other and in fact when you go to bed at night, you don't know what the next day holds in store for you – whether it will be a good or a bad PH-day.

Anyway … I'm running off the track, sorry for that.

What I meant to say is: many of us feel relatively good, well – at least given the overall health condition. We can be grateful to live in Germany with supportive health insurance, medication for everyone (even the expensive therapies such a Trepostinil) and to consult the physician of YOUR choice.

So when you suddenly hear of the passing of someone close, you realise how fragile you really are and you start to fear that next time it could happen to your best PH-friend or at worst – to yourself.

There are several ways to deal with these kind of news, everyone deals with it differently.

Some tend to hide and burry themselves, others act like nothing has happened and that they are indestructable or immortal. There is no right or wrong.

The only important thing is to realise about the severity of this disease early enough to do YOUR OWN part to have the best chances to live a long and at least a relativly uncomplicated life.

I would lie if I'd say that these shocking and sad news didn't leave me untouched and I can confess, that I was pretty scared and nervous myself. It also left me numb for a while and I felt a bit paranoid everytime I had side effects from my meds or other minor ailments. Yeah, I know …. crazy but then again, it's just human nature, I guess.

But luckily 2018 wasn't made of bad news only, in fact the positive news and events ended up to make the majority of the last year. Thanks for that.

The Ups

Maybe it was due to the shocking news of the passing of around 10 Phighters our PH community was facing at the beginning of 2018 which brought everyone closer together? It surely was also other circumstances which happened behind the scenes to motivate many people to take sides even without trying to persuade them to do so. I reckon many people just noticed the vast injustice.

Either way … all this accidently helped a lot to extend the community and the make the dealings with one another even more familiar.

Sometimes it really amazes me how complete strangers, people with the same disease you have never ever met in person kinda become your everyday friends, your partners in crime, your shoulder to cry on or the people you can share your achievements with. They will cheer for your blood test figures, they will throw their pompoms for you when you've reached a new personal best in the 6MWT and they will just be there for you to just keep their fingers crossed when going through a hard time.

But with that in mind, sometimes these „internet buddies“ become even more at some point.

We've had some meetings this year with our community of people. First a nice BBQ in June in the Ruhr Area in middle of Germany with around 25 people, then later in September a meeting with a Harbour Cruise in Hamburg with around 17 participants. More meetings planned for 2019 as we noticed, that others who couldn't make it to these Meet Ups in 2018 were almost begging for encores in 2019 so that they could take part as well.

A positive „side effect“ from meeting up with others was how the overall way of communication, the warmth of which people deal with one another took to a new level of encouragement, respect and understanding. This is really amazing.

These meetings also resulted in even more regular active users. More people who now have the courage to give advice to others, no matter if these people are „new“ or „old“. Also, more people who tell from their personal point of view, give their personal advice or explain what they've been through.

Maybe it sounds schmaltzy but it's like one big family, everyone is there to support one another.

Honestly, I never thought that this community would evolve so quickly and in this direction.

The group itself gathered momentum – no guidance needed, so it seems.

I'm not here to make the grass grow greener, we're not there to make the sun shine brighter or to take other parties jobs and do „politics“ or lobby work.

I am/we are solely interested in helping other people who just got their diagnosis and are now struggling with setting up their new life. The main aim is to make these people realise that they are not alone and that there is a way to live a life with Pulmonary Hypertension. Nothing more, nothing less.

So anyone reading this in search of help or advice from fellow patients maybe you'd like to visit our website at:

www.pulmonale-hypertonie-selbsthilfe.de

Of course there were also „personal highs“ resp. improvements in 2018 which I'm really grateful for.

Sometimes you really have to listen and take the advice from others. Luckily I did!

Extended therapies or additional devices are not always restricting your quality of life – sometimes it's right the opposite. You just have to change your point of view and accept changes to feel better in the long run.

These „changes“ were at first the cPAP therapy for me.

After having massive sleeping problems in the early half of 2018, I made an appointment in the sleep laboratory and I was diagnosed with severe sleep apnea with around 70 breathing interruptions within one hour of sleep. That's scary! After getting adjusted to the sleep apnea mask in August and getting a follow up check in October it turned out that I now have „only“ around 7 sleeping interruptions within one hour of sleep. Massive improvement!

I'm really curious to see what an effect this therapy will have on my heart when having my echocardiography in March this year.

Another extention to my therapy is oxygen at nighttime, as it was also discovered while being at the sleep laboratory, that I desaturate when being asleep. My oxygen level is still moderately okay at nighttime, nothing really to worry about but still … it won't do any harm, it doesn't bother you much at nighttime but will instead support the heart and body, when I get substitutional oxygen.

I'm on nighttime oxygen for 3 months now and like with the cPAP therapy, I'm eager to see if it affects my heart in a positive way.

The newest and also most important and effective addition to my therapy is physio therapy in form of „lifting weights“. Honestly speaking you can't really call it lifting weights, as the resistors are so minor, at least for someone being healthy. This special „lung sport programme“ was set up by the PH chief physician and the physio therapist at my local PH specialist center. Other PH patients take part at a course once a week, I have special training twice a week for an hour each, also to help losing some weight, as my BMI is far from the magical TX-marker. Not that I need to be scheduled for a TX anytime soon nor that I need to be listed. But it can't be bad to work at my patient's compliance just in case.

This training is amazing. I really, really like it and have less body ache from the side effects from the medication now. The main target is muscle building or at least to keep the current muscle at work and preserve them from degeneration.

Besides this sport programme, I also have nutrition counselling and I feel really proud to say that I actually lost some weight already. It's baby steps and really hard work, as there's not much room to juggle with food or sports to lose much weight but still … you have to take every chance to do a little bit. At least I'm currently still motivated to stick to it, let's see how long this will last …

As you can see, 2018 was positive in many ways.

Surely sometimes you feel gloomy or misunderstood or treated unfair. It's completely okay to surrender yourself to these feelings from time to time. But one should never forget that it's ONESELF who's in charge of your life and what you make of it.

Along these lines: Make 2019 YOUR year! Make 2019 the best you can.

SLEEP APNEA AND cPAP-THERAPY

There is a cure for sleepless nights and so called „PHainsomnia“

I can't even remember when it all started?!

Guess it must have been March or April. It started slowly and I didn't really think of it at the beginning and to be some sort of a problem. I just accepted it as a fact, something I needed to adjust with.

I usually go to bed pretty early, between 10/11 pm.
What happened was, that I started waking up again from my „first sleeping period“ after around only 1 ½ or 2 hours, which of course isn't very long. I didn't bother at first but got up instead, had something to drink, went for a pee and returned to bed. Of course it always takes a while before you fall asleep again. Guess most of us PHighters can relate to that.
I also put the blame on the fact that I'm not fully strechted, that I had too much time to relax and therefore was rested already when going to bed.
So at some point I fell asleep again and what happend? Two hours later I was awake again. This went on until the early morning hours. THEN I got really tired and could sleep until noon.

I started finding the weirdest explanations for this condition.

It was due to the late coffee I drank the evening before, I slept too long the morning before, the weather at nighttime was too hot to fall asleep, the list went on.
Then at the end of May I asked my PH outpatient's department whether I could have a check on sleep apnea.
I was given this little portable device which consisted of a pulse oximeter, nasal prongs and this little box which looked a bit like a pedometer and was strapped to your chest, recording the O2 saturation and the apneas. I could use this at home, which was good, as I thought it was easier to sleep in your own bed.

When I went to my PH physician the following day, the collected data was checked and surprise, surprise … I was told I was suffering from sleep apnea with around some 70 or so apneas that night. WOW! I didn't expect that.

So my next step was getting an appointment in a proper sleep laboratory. I sent all the documents along with my diagnosis of PH to the pulmologist and received a letter telling me that they reserved a spot for me at the beginning of April 2019 (!!!). I thought this was a joke or typo and called the doctors office, stressing out how urgent my case was because due to the underlying diagnosis of PH every disturbance (such as bad sleep or apneas) could fuel and speed up the original disease.

They put me on an emergency waiting list and luckily shortly afterwards I received a phonecall whether I was free that one night to check in the sleep laboratory. Of course I agreed.
What a strange night that was … I couldn't sleep at all. I was so worried moving from one side to the other in case I might rip off any of the cables and electrodes.
This first night was only some sort of evaluation of the current status as the portable device is not at all as highly diagnostic as the results in a sleep laboratory are.
So when they told me a few days after, I was IN FACT suffering from sleep apnea, I was kind of relieved as I knew there were ways to treat this and make it better.
I needed to have a second night in the laboratory, testing with a cPAP mask and adjusting pressure aso.
Again I needed to wait for an appointment but luckily this only took two weeks until I returned to the laboratory.

Second night at the sleep laboratory testing the cPAP mask

Surprisingly getting the mask applied to your face and sleeping with it was less stressful and uncomfortable than I thought it would be.

And what a relief … the collected data even showed that it helped a lot reducing the apneas. I was told I „only“ had 7 during that night, so coming from 70+ this was a huge improvement.

The doctor then prescribed me a cPAP-mask and a „Dreamstation“ by Phillips which controls the pressure.

I went to this shop who helps with breathing devices and the guy gave me a proper introduction for this device and I could even take it with me that same day.

So since August 7^th, 2018 I'm a proud owner of a cPAP-mask for sleep apnea therapy and somehow I do sound like Darth Vader and look like Maverick from Top Gun ….

♫ "Highway to the danger zone" ♪♫
That's me, Maverick Vader - a total success until now

I know some people who are giving this device a hard time and kind of refuse to accept that it might actually help them. Also, some may say that it makes them look „unsexy“.

Guys … when you sleep at night, your partner's eyes are closed as well when he sleeps.

I'm only two weeks into therapy with this device and I could sing praisals …. REALLY!

Instead of waking up every 1 ½ – 2 hours for around 3 – 4 times a night, now I only wake up once and have extended my sleeping periods to up to 3 – 4 hours.
I know that it will take up to three months until you have fully adjusted to the mask & therapy and will completely benefit from it but I just can't wait for it, as already now I see how much of a difference it can make.

SO … in case you have sleeping problems, often wake up at night, feeling worn out in the morning and still feel tired after „sleeping“ for 8 – 10 hours … it's not always just the side effects from PH medication but could mean you're suffering from sleep apnea. Make sure to have it tested to get back to a better sleep which also relieves the heart.

Just be open for the therapy!

I will keep you posted on further progress at this topic.

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!

Living on borrowed time

What does it mean to suffer from a fatal disease?

To be terminally ill?

I guess many people don't believe me or can hardly understand how severe my condition really is.

It took me some time myself, to fully accept the impact on my life. Wait … did I really accept and understand it myself already? Sometimes I keep wondering …?!

The problem: Every day you keep learning new things and aspects which shed some more light on the complex topic.

I am suffering from Pulmonary Hypertension (PH), a disease which affects both your lung AND heart. This is a chronic, progressive and incurable condition.
So … to put it straight. There is NO cure, NO medication which makes it disappear, NO magic fairy dust you spread all over me and then it's miracly gone. There is NOTHING which can be done to make it go away. This fact is scary. And I mean SCARY!!!
Knowing that you will be dying. And not dying like everyone else but knowing that the clock has already started ticking. And it's ticking loud and clear!
I need to add, that there is IN FACT a chance to „heal“ or at least rescue you from this devils circle and this option is a lung transplant.
I don't need to explain that this is linked to even more complications and failures. So even a transplant is no guarantee to lead a healthy and happy life …

I received my diagnosis around two years ago (April 2016) and am now living for two years with this disease and during these two years there have been 3 risky incidents where I almost died. Luckily I only heard and realized this some time after. But when you actually have had these moments, you really are aware of the fact how precious and unpredictable your life is.

Just to explain the disease in short and for „dummies“, without too much medical knowledge: With Pulmonary Hypertension, your blood vessels in your lungs are narrowing.

Nobody knows why this is happening, the disease is simply still unknown in many parts. The right heart needs to pump much harder to overcome this resistance due to the narrowing of the blood vessels to make sure pumping enough blood into the lungs where the blood will be enriched with oxygen and returns to the left heart chamber, where it will be redirected into the systemic circulation.

As the right heart needs to pump harder, the consequence is that the right ventricle gets bigger and bigger. It also crushes the left ventricle due to its increasing size.

To make you understand what an enlarged right ventricle means or what can happen, just imagine that you are blowing up a baloon. You keep blowing & blowing, the baloon is getting bigger and bigger and at one point the baloon will be bursting. This is when right heart failure will occur … flatline … game over!

And this is what will happen with ANY PH patient at some point. No chance to escape!

Another thing to illustrate the impact of PH is the following. Someone not affected by PH has got a middle pressure in one's blood vessels of the lung of up to 25 mmHg. Many of us PH patients have got a pressure of double or even triple this figure. To make you understand what this means to us, is showing you the seriousness of the disease and its level by a simple analogy.

A normal person's blood pressure is 120/80. Now imagine you would double or even triple this pressure. That would be 240/160 or even 360/240. Imagine you would have to LIVE with such a blood pressure in everyday life. What do you think how long could your organism cope with such levels? Before collapsing? Scary, isn't it?

Okay, so this explains the disease in short.

Now onto the struggles in everyday life.

When I tell people that I am given medication and that I have stable results in tests, I suppose many of them are often mislead to think that I'm okay, that the disease is cured or at least at bay and I don't need to worry about my health condition anymore.

I simply don't look sick, you couldn't tell from my appearance that I am battling a death struggle. This has pros but a lot of cons too.

I guess part of it is my own fault also.
I have always been a chirpy person. Always funny & friendly and curious towards new people. I am often sarcastic & humoristic when it comes to my disease. I guess part of it is also self protection. If I would always and openly admit that I'm afraid of the future, afraid of the following day, scared about going to the follow-up appointments not knowing whether your figures may got worse and reading new proven outlooks and statics on the internet, I guess I would go insane and fall into a deep depression. And besides: it wouldn't change a thing about my status. So I try to make the best of it, well at least sometimes.
I am glad that I found a support group of people who suffer from the same disease as I am. It encourages you loads when you read that some people are living with PH for 7 years+.
Cause nobody actually knows when your time will be up. There is no such thing like life expectancy with PH. There's no such thing of being „PH-free“ like it is with cancer patients, when you've passed your 5-year-post-surgery/chemo you're officially cancer free or HEALED. With PH there simply isn't anything like that. You keep battling and hoping to survive the next day and dreaming that one day a cure will be found and that hopefully you will still be alive to experience that day.
This kind of life is tiring, I tell you.
I get tired of explaining my health status to others. When I need to cancel appointments with friends short notice, when I have to decline invitations months in advance simply because I don't know how I will be feeling on that certain day and you don't want to disappoint someone who solely relies on you.

I often hear from other people „But you don't look sick at all. In fact, you're looking really great. You've lost weight and overall look rested“. Oh … what a curse this can be …

How can you make others realize how severe and dangerous your health condition is, when you are looking like pure spring awakening?
I have started to answer with the following saying: „Outside hooey, inside phooey!“
Cause that's what I still feel like.
Taking medicine, being on TRIPLE therapy, the maximum for PH patients at the moment, doesn't mean you're anything better than before. The meds are simply meant to „improve quality of life“. Are you aware of what this means? Again, there is NO cure. Even physicians are talking about of simply and ONLY improving quality of life on your way to death. Well, I guess most of the doctors wouldn't tell their patients this right to their face.

But in all guidelines and essays it's always only talk about improvement in quality of life instead of healing or extending life itself. Simply because there are no statistics about how a certain medication could actually extend life expectancy, well .. there are no statistics just yet.

So, in general it means: you still gonna die but you will be feeling a little better on your way towards the bright light.

Scary, isn't it? Just try to imagine it was you …
Yeah, I know … many people keep arguing, saying „we all have to die at one point“.
Agreed! This is surely right! But most people don't know when and how this will happen.
Now try to imagine knowing that you could be dying EVERY DAY within the next 5 years and due to right heart failure. Or simply because you've caught the flu and your weak immune system is not able to fight against it …
Just food for thought and to raise awareness WHY we PH patients are sometimes so sobby and weak and simply angry when others don't understand or try to have a little sympathy.

Which brings me to the next topic.

Even if it comes across like fishing for sympathy or trying to make others feel sorry for me, I am not.
I am simply trying to make others understand, why I often feel weary and spiritless. With PH, not one day is as good as the other. You can have two good days and then 4 bad days. Or also the overall condition is going up and down and up and down again.
And you often can't say in advance, whether you will be feeling good enough on a certain day next week, to agree on an appointment. I guess this uncertainty has cost me some friends. I reckon' some of my friends or people around me are simply unable to cope with this condition or how they should react towards me without hurting me.
Or they are tired about the fact, that I only keep talking about my health condition and nothing else anymore. Sorry for that!
Well, I once said somewhere that I accept and respect this nasty disease but that I don't want it to dictate my life.
I was so foolish. PH IS DICTATING my life. And you can't escape from that or do anything about it.
Also, many people suggest to try to stay positive and don't concentrate on the disease so much. I simply can't! Call me weak, call me negative but try, only just TRY walking in my shoes for a month or so.
I'm on medication which's primary cause is to make my everyday life more bearable. The PH symptoms are still there, sometimes more, sometimes less. I still get breathless, I am still weary and tired, I still have water retention in my legs but luckily not as severe as before. I take around 20 pills a day to keep these symptoms and their side effects at bay.
These meds and their effect on the PH can easily be explained with a link to having toothache. When you're suffering from toothache it is because you're suffering from tooth decay. You're taking a painkiller and the pain is gone. The tooth is still rotting away unless you're having a filling or have it removed. Same with the PH meds. The meds are simply calming the symptoms and the „pain“ … but still, the narrowing of the lung vessels keep going on and can't be stopped. If you are lucky and the meds really do work good for you, the speed of the narrowing can be slowed down … but that's the only positive thing about it. The disease itself will progress.
Some side effects of some of the meds are really inconvenient and I MEAN inconvenient.
How would you feel, when due to one of these meds you can't control your digestion anymore and every now and then (mostly when it's the worst moment ever, for example when you're out and about) you have no control about it and simply shit your pants.
Sorry for being so plastic and open … but this feels just destressing.
This happened to me in public for three times already. Maybe that's another reason why I tend to stay at home more and more?
That's the reason, why PH is so present in my everyday life and on social network platforms.

When I was still working and was on a sick leave because of having the diagnosis and being treated and adjusted with the first PH meds, I updated my teamleader and job colleagues about my status and I would often get text messages saying „Get well soon“ and „Speedy recovery“.

At first, I made the effort of explaining them back & forth, what this disease really meant, how severe it was and how tiny my chances were. They kept wishing me a speedy recovery aso.
Honestly … I was really angry! Angry about the fact that they seem not to care and listen to what I was trying to explain to them. About my crappy health condition, the poor outlook on life and such … There was no RECOVERY for me! Didn't they understand what I was trying to tell them?
It took me quite a while until I realized that they were probably all overwhelmed by this news as well.
I had to ponder about it for quite some time, thinking about what I WOULD SAY to someone being terminally ill with no outlook of any cure. Urgh … awkward feeling to say the least. You simply wouldn't go gatecrashing saying „ah well … I hope you don't need to suffer for a long time“ or „try to see it positive, you don't need to worry about job changes and all the shit which is going on in the company right now as you're not returning anyway“. You just wouldn't say something like that, would you? So to overact your own insecurity and anxiety towards this severly and fatally ill person you would end up saying „Get well soon“ as well …
That's what I needed to realize and learn as well …

Honestly speaking, I really don't know what to expect from family, friends and people around me. They are all different. They all deal with this information differently. Some may backtrack and let the friendship cool down – maybe to protect themselves from negativity and anxiety about this horror disease? Or maybe because they don't know what to say?

There's this saying: „if you can't say anything nice, say nothing“
Maybe they are acting this slogan?

Well, I want to reach out to all of you there …

My results and figures may be stable for now … but my spiritual condition often is not.
I have my ups and downs … sometimes I'm positive and confident that this third and very potent medication will actually help me to live longer. What else can I hope for? Sometimes it's good to just dream and think everything will get better ...
But then you hear from the PH community that another fellow phighter has died and you keep worrying who will be next. Will it be a friend closer to you or even worse: are you the next? Nobody can convince me that he/she hasn't got these thoughts on his/her mind.

So when people keep asking me about how I feel, I usually say „Good“. That's such a cliche as well … hardly anyone would be prepared for the real answer in full effect. When you keep answering what you've been going through for the past days or weeks. And honestly speaking, the question is, do you really want to bother them with all the details and sad facts? If you really spread all the details, you may get branded as a whiner. Though there are truly differences in being a whiner and stating the depressing facts.

Still … I am convinced that most people wouldn't be prepared for the truth so I keep answering „good“.
And sometimes I catch myself believing that I AM ACTUALLY „good“. One gets humble when being burdened with such a disease and you start valuing the little things.

With PH, no day is like the other. The nightmare starts again every single day ...