ANXIOUSNESS ABOUT FOLLOW UP MEDICALS

The longer we live with the diagnosis, the more anxious many of us get

I got my IPAH diagnosis in April of 2016 after having to some extent most serious symptoms of breathlessness, fluid rentention in my legs & ankles, weight loss due to the heavy working heart and sometimes even syncopes.

I was adjusted to several new meds in May 2016 and shortly afterwards felt a lot better. I also had a supporting rehab which was specially designed for PH patients. Around a year after being diagnosed I was introduced to triple therapy (incl. Macitentan, Tadalafil & Trepostinil via an implanted infusion pump) and within no time, my overall stats got a lot better with each follow up medical. The most amazing thing which happened after being introduced to Trepostinil was that my right ventricle shrunk by a third which was a MASSIVE achievement!

With my last check up medical in November 2018, I scored my current personal best in the 6MWT and managed to „run“ 535 meters but being completely shattered afterwards. And I mean WORN OUT!!! Run over by half a dozen trains.

I hardly ever felt the psychical stress before. I had no problems going to the follow ups, in fact I was even sometimes excited and nervous in a positive way because I was wondering whether I would be able to improve my results once again. I was hungry for new achievements, for better blood figures or even better results in the TTE (heart ultra sound). Until now, I was lucky and blessed that my figures got better or at least remained stable (with only one exception of a 6MWT when I walked 10 meters less than before).

I often talk to a friend and she tells me that she gets so very nervous even weeks before the follow up date. I couldn't understand all the hassle and fuss about it. I was always completely relaxed and trustful that the medication would do their best and keep me stable.

She often tells me „Wait for it … you'll get there as well!“

Well, I guess I am there now … and this feeling is awful. It's a mix of paranoia and anxiousness.

Basically I am feeling alright. I am even doing sports and I do have a feeling it is helping me a lot.

Despite all that, everytime there's something aching and you THINK your heartbeat is faster or stumbling you start to worry and paranoia is approaching. All this can put you under a lot of stress, which is surely not supportive for your blood figures or overall well-being.

But you just can't help it.

PH is an incurable disease. It's progressive. And with this awareness in mind, it's no surprise that you start to feel paranoia or anxiousness at some point.

The funny thing is, I always thought that I wouldn't be successful at the 6MWT but each time until now, I managed to improve my performance which is some kind of a curse also …

It's a curse and burden because you put yourself under so much pressure that you kind of need to prove to your physicians, that you can do better or at least, you can hold that score.

If you „fail“, they start asking questions like „Are you feeling worse?“, „How's you're breathing going?“, „Maybe your NYHA class needs to be adjusted“, „We need to check on your risk assessments“, not to mention possible questions about the enrollment onto the lung transplant list …. the list goes on and all these questions make your tummy turn upside down.

The end of the story: with the next follow up approaching, you start feeling nervous weeks or at least days in advance, you're lacking a good night's sleep and you just feel awful.

I'm sure, all this can actually affect your figures and results. But that's just my opinion or maybe just an excuse for possible poor performance.

I reckon that even with all the awareness of Pulmonary Hypertension and its severity, at some point we all start living in a bubble, in a parallel universe. We are provided with the best possible medication, with supportive and emphatic physicians and all this results in a more or less „careless“ living. We feel great, well mostly. One gets humble and grateful for being able to live, even if living is so much different to what it was like before diagnosis.

So it's completely understandable that one can get pretty nervous if there's something not running smoothly and we're brought back to the „real world“ and are reminded once again how serious, progressive and terminal life with Pulmonary Hypertension is.

My next follow up medical with TTE, 6MWT, blood tests, lungfunction test and BGA will be this coming Thursday (March 7^th) and yes, I AM nervous!

Fingers crossed for me, that I can at least hold all my stats. I will possibly update on the figures.

So how do you feel about it all? Do you feel similar? I'd like to hear your opinion on this topic so feel free to reply. Thanks! :)

WORKING OUT WHEN SUFFERING FROM PH?

Monitored excercise can support having an overall better status

My PH-specialist has been nagging me about losing some weight for the past months.

I know that she's right about it and it's essential especially when it comes to a possible lung transplant. Luckily I'm not neccessarily needing one right now. My overall status is pretty good and the figures currently show a low risk. Nevertheless, I'm far away from a TX BMI of 30.

That's why my physician kept encouraging me to seek assistance with the obesity outpatient's department. I wasn't impressed. I guess she kept mentioning it everytime I was a the hospital for getting a refill for my implanted infusion pump.

Then, one of the male doctors did the refill as my usual physian was on holiday. He did mention the obesity department as well and I felt really fed up and while watching him putting on the sterile disposable scrub, I noticed his chubby belly and thought „He's giving advice to others to lose some weight when he could lose some weight himself“. As if he read my thoughts, when he removed the disposable scrub after the refill was done, he caressed his tummy and said „Yeah, I know … I should better lose some weight myself!“

Oh my god … I felt so awful. I mean, I didn't say it out loud but still. Even thinking about it made me feel guilty.

So this was the incident which made me realise I should go for it. This doctor had so much empathy, it wasn't just giving advice but he admitted that it wasn't easy and I believed him that he meant it in a good way.

I was really impressed with him and therefore thought I would prove I could do it.

That was back in October 2018. I started nutrition advice and also received a prescription for physio therapy in form of minorly lifting weights.

Losing weight is working out okay. I haven't lost too much, it's just that you change your eating habits and keep monitoring WHAT you eat. Keeping up an eating diary helps loads as you stop eating all the junk inbetween or at least keep minimising them.

I've lost around 4,5 kgs since late October and my PH-physician was thrilled to bits about this achievement when she did the refill for my infusion pump around a week ago. I use to joke that my goal is, to lose 1 kg every 4 weeks, so I can kind of brag to her when I go for the refill next time.

Anyway … but this blog shouldn't be about losing weight in the first place but about working out when suffering from Pulmonary Hypertension.

As I mentioned, I have special physio therapy twice a week also at the Universitätsklinikum Hamburg-Eppendorf UKE where I'm under medical treatment at.

This special lung sport programme was set up by the chief physician of the PH department and with one of the physio therapists.

It works wonders for me and I really enjoy it! Over all, I'm sure it supports me massively in losing some weight and a nice side effect is also, that I have less body ache since starting to work out. It's probably just the sheer fact, that I use more muscles and keep them from degenerating.

Many people will probably argue that it can be very dangerous doing sports when suffering from such a severe disease like PH is. It sure is. But you also need to do some exercise to keep fit.

So instead of overdoing it, this programme is really light activity & weights. The bottom line is to watch your saturation and pulse throughout exercising. As we all know, saturation shouldn't go anywhere below 90 %. The important thing about the pulse/heartrate is, that it shouldn't rise higher than 120 bmp. Furthermore it is essential that you keep doing PLB / pursed lip breathing while exercising, trying to breathe normal and regularly. If you start rapid heavy breathing while pushing weights, the resistances are too high.

With this blog I will try to explain what I'm doing when working out and what the differences to a normal gym are.

Healthy people often lift higher weights or work out with higher resistance, while doing few repeats.

The difference with my programme is, that I lift minor weights & resistance while doing more repeats. That means two sets with 25 repeats each at every gym machine with a minute of relaxation inbetween before you start with the second set.

Before I start it's essential to warm up by cycling on the ergometer for around 10 minutes at 25 watts. Then I'm doing my round at the physio gym by using the following machines:

• Leg Extension Machine
• Crunch
• Leg Press
• Adductor & Abductor
• Pec Deck (butterfly)
• Chest Rowing Machine
• Pulldown Dip (I usually skip that, due to the catheter of my implanted infusion pump)

There is always a physio therapist there to assist me or checking on me and my health status or asking how I'm feeling. I am monitored!

When I started this programme, I started with a resistance of around 25 – 50 newton depending on the machine I was working out.
10 newton are around 0,981 grams which means, 25 newton are approx. 2,5 kgs. I will round it up.

Many people probably start laughing when they read that I „lift weights“ of only 2,5 kgs. It's the repeats and the breathing which are important.

I have also realized, that exercises at thorax level, for example the pec deck are more exhausting than doing the leg extension machine. Working out with the legs is overall less tiring.

At the moment, I'm exercising with around 125 newtons (which are rounded up 12,5 kgs) at most machines except for the pec deck, where I only do 75 newtons. The leg press is also an exception, I manage to push 300 newton here, as you usually have loads more power in your legs than in the rest of your body without feeling worn out.

I will add some video for you to see, maybe it inspires you to start your very own training as well.

The most important thing is that you discuss all this with you PH specialist BEFORE you start doing anything. Ask her or him, whether you can do sports and which sort of sports and maybe which maximum restistance or other things you need to pay attention to.
I guess the reason that I am allowed to do it or even able to do it, that my current overall status is pretty good.

Leg Extension Machine:

 

And here is how the monitor of this machine looks like. Other machine's monitors look similar.

It shows the resistance in newton you are working out with as well as the current repeat you are at from a total of 25.
The blue bar is showing you the speed in which you should exercise. The white bar shows your speed and it would be best, if they move in syn. They ARE usually in syn, but when filming this, I was distracted and didn't watch the monitor for a while.

Crunch:

Leg Press:

Adductor & Abductor:

Pec Deck (Butterfly):

Chest Rowing Machine:

Wishing everyone of you loads of energy and motivation to start doing it and sticking to it.
Maybe it helps you feeling better as well.

It helped me loads. My next check up with heart ultra sound is at the beginning of march, maybe it will then also show a positive result of my training?!

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.