June 10, 2019 at 8:00 am #17725Colleen SteeleKeymaster
Are you an adult who was diagnosed with PAH as a child? Can you share comparisons in what it was like battling this disease as a child compared to now as an adult? Have you noticed a difference in the treatments and symptoms of PH as you got older? What was the most difficult part of having PH as a child compared to your greatest challenges now?
June 12, 2019 at 10:39 am #17809Anthony CollinsParticipant
With CHD/PAH, distinguishing those symptoms caused by the heart issues from those caused by the pulmonary hypertension is probably impossible.
In my earliest years, so I am told, my facial skin was highly coloured and there was one instance of cyanotic collapse, followed by unconsciousness and a dash for medical assistance. Those symptoms did not endure. Anaerobic exercise was always easier for me than aerobic. There was a single occasion of exercise-induced pulmonary haemorrhage. PAH was diagnosed by cardiac catheterisation in middle childhood.There were no specific treatments for pulmonary arterial hypertension in those days.
PAH, called “the lung issue” by my parents, at least in front of me, was not reversed by cardiac surgery. Just the same, only in recent years would I say that my quality of life has declined: gentle cycling was abandoned because dyspnoea made even small slopes unmanageable; on foot, slight upward gradients and stairs are increasing challenges; bending has induced arrhythmias; on occasion, strong pulsations have been weirdly visible just below the left shoulder. I cannot seem to control tachy-brady syndrome by reaching for the supplemental oxygen but it has reduced quite markedly the duration of flutter-fib incidents. They could run for days before the oxygen was prescribed; now, even on a low flow rate, it’s usually only an hour or two. Saturations dropping into the low eighties seem to be the most common trigger of flutter-fib.
My self-prescribed treatment for limiting physical and psychological impacts is exercise, currently walking a minimum of seven kilometres per day, except in the worst weather, and on the flat obviously.
June 12, 2019 at 11:12 am #17820Colleen SteeleKeymaster
You have had quite the PH journey Anthony, thank you for sharing! Diagnosing PH in children is a challenge because it’s so uncommon. My son went misdiagnosed for 2 years. Luckily there were treatments available for him by then but the medications had primarily been used on adults. 10+ years later and I have seen developments in pediatric PAH and I think some improvements in diagnosing it early. I think personal stories like yours would be helpful to the medical field to evaluate the progression and challenges of PH from childhood into adulthood.
I am so sorry to hear that you are experiencing increasing symptoms of progression. May I ask what treatments you are currently taking? Has transplant been discussed? I’m impressed by your drive to fit in at least some type of exercise each day. It sounds like you have always been pro-active in maintaining exercise despite your health challenges.
I appreciate that you included physical and psychological needs together because we discuss the two a lot on these forums. Both really need equal attention in order to stay well.
June 12, 2019 at 11:32 am #17825
I couldn’t agree more about the psychological as well as physical impact of chronic illness on the body. It certainly takes it’s toll in more ways than one and the mind and body both need to be talked about. I’m glad our forum members are so open to having these conversations.
June 12, 2019 at 11:30 am #17824
As someone with CHD an PH, I can relate to what you’re going through. I know sometimes (more often than not) it’s so hard to determine what the cause of our symptoms are whether they are cardiac related or lung related. I was born with pulmonary hypertension as a child because of a very large VSD that they found. My pulmonary artery was also compressed for a really long time before surgeons just recently divided my vascular ring that was causing compression.
I know how scary it can be to feel like you’re so out of breath and need to use that oxygen more often than not. It caused a lot of arrythmias for me too. I’m curious, do you have a pacemaker ? I see that you are mentioning tachy/brady. I used to alternate between the two of these before I got my pacemaker in. The pacemaker and the medications that I have been on for the past 7 years really have helped me a lot. Are you currently seeing an adult CHD doctor or are you still being seen by your cardiologist you had when you were younger? Feel free to reach out any time. Like I said, as someone with both of these two I get the difficulty of juggling both conditions and know how hard it is.
June 16, 2019 at 10:37 pm #17888Vanessa VaileParticipant
Going off topic, Anthony, but I’m on a walking kick. 7K a day, Wow. I was thrilled to crack the three mile wall recently. Now you’ve got me thinking about the next walking distance wall.
June 17, 2019 at 7:44 am #17891
I think it’s great that you do 7k a day! Whenever I have some good days of exercise tolerance I don’t take it for granted because as you know so many of this can be so unpredictable ! I usually keep a log of my exercises so I can sometimes see patterns if I’m having a bad week. My phone has an app that allows me to track the steps and miles that I take.
June 12, 2019 at 11:17 pm #17842Anthony CollinsParticipant
Hi Colleen and Brittany
Taking your questions in turn:
Drug treatment for pulmonary hypertension in my country has been so different from standard practice in the USA that it could seem to American members of these forums I lived on a different planet. Access tightly controlled by a state-appointed committee has only very recently been relaxed, at a time when I am no longer convinced that I should even try a first-line PAH treatment lest there be cardiac side-effects.
The difference between systems was amply illustrated a half dozen years ago when, following right-heart catheterisation, I bundled up all my records and sent them to America for a second opinion. I was advised to undergo re-catheterisation, the clear implication being that it would establish my need of PAH drug treatment. Because my lifetime exposure to medical IR was already so high, I did not want a follow-up RH Catheter study and I knew anyway that the level of PAH had not changed sufficiently in a very short space of time to meet the local threshold for drug therapy. So, no PAH drugs.
Very few heart transplants are performed in my country and, at my age, I would not even make it onto the waiting list – nor do I think that I should.
A pacemaker has been discussed in recent times. However, my ventricular function will need to be more compromised before the discussion becomes really serious and heavy scarring of the chambers could, I’m informed, represent a considerable challenge to appropriate anchoring of the leads. I have been told that it will happen, but only once I start passing out and so become a danger to myself and others.
Of the team of cardiologists I had in childhood, I know of two still living, neither a practitioner any longer. Adult Cardiology is not so much a specialty here. I see an EP.
Lastly, I am happy to rely on my own intuition much more than ongoing echo studies which provide PAH readings that fluctuate wildly – mild, moderate, severe – and come in no logical order. IF it is PAH more than a failing heart that explains the progression of symptoms, my physical activity level suggests to me that the degree of pulmonary hypertension falls between mild and moderate or is, at most, “mildly moderate”. That’s not too bad for someone whose parents were told that he would not reach adulthood!
June 13, 2019 at 8:39 am #17846
I am glad that you are beating the odds and outliving what the doctors thought at first. I think you must be really good at listening to your body and what it’s telling you. I also see an EP. Do they have pulmonary hypertension or congenital heart disease specialists?
When you write that your PH levels are probably mild/moderate does this mean that your exertional capacity seems okay for you? I’m sorry to hear that they think you won’t be a good candidate for a pacemaker because of the scarring. I have had other friends with this problem with the scarring too and they had to approach the pacemaker from another way but they had adult congenital heart specialists who weighed in on this too. I wish you were able to get good specialty care for your heart and your pulmonary condition. It makes a difference. But I’m glad you are aware of your body and what it needs and you speak up for yourself!
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