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 ...