Progression

[Brittany Foster]

Hi Libby,
A lot of times the doctors are honestly unsure about how to answer or give timelines of things. People’s body’s adapt, change, and medicine and medical advances are made every single day. Unless someone is currently dying I feel like it it hard for doctors to tell a patient “you have x amount of time left” because most of the time , that number they give is just a number and people surpass expectations. I haven’t noticed much of a progression but I did at first. I was on oxygen all the time until they put me on o2 and a bipap at night. Now, it seems like my body has just adapted to the lower lung function and the lower oxygen levels and it has become more of my “normal” and doesn’t feel as overwhelming or as drastic as it first was when I noticed the symptoms. What symptoms have you been experiencing more or what signs of “progression ” have you noticed? Have you mentioned these to your doctors and are they attributing them to the PH?

[Libby]

Occasionally I feel like my breathing is shallow and that if I want to breath deep, it has to be conscious effort. Otherwise, no progression in symptoms. My feet are always cold and purple and I get chest pain during exercise but it has always been that way.

[Brittany Foster]

Hi Libby,
I have many similar symptoms to you. have your doctors given you any testing like the cardiopulmonary exercise test? They even do a right heart cath on people while they are exercising to get a better idea of what is happening to the body on exertion. This is actually what was able to diagnose me and pinpoint my underlying respiratory conditions.

Make sure to bring up this testing to the doctor the next time and even a pulmonary function test.

[Valerie]

Hello, Libby!
I’ve also always had congenital heart disease. I was diagnosed with PAH (congenital) only at the age of 18. I do not like sports and any kind of physical education, so for me the progression of the disease is not so much noticeable at the moment. But now when I watch old videos, I can see that when I was 4, 5, 6, 7 years old, I was an active child. I jumped, danced and frolicked like an ordinary noisy child. The only thing that bothered me was the pain in my left side during a long run. Now active physical movements cause me great difficulties. I think that this is due not only to the disease, but also to the lack of any physical exercise for all the years. My legs are not used to long walking, so I often stop. Perhaps if I did the simplest physical exercises in school, I would be hardier now.
I know I have to take care of myself and be careful. The doctor said to me, “If you’re late for the bus, you can’t run. Wait for the next one”. I try to follow this advice, although sometimes I can miss some things because of such slowness. It makes me regret missing out, but I remind myself how I would feel after a run.
You write about feeling of shallow breathing and conscious effort when you want to breath deep. I think that happens to me a lot. Sometimes I catch myself barely breathing, like hiding from someone with bated breath.
According to annual ultrasound studies, I am at about the same level as ten years ago. But for me personally, the progress of the disease is noticeable in decreasing endurance and a more depressive attitude to life. You’re right that all of this is different depending on age, lifestyle, cause of the disease, etc. I think, me personally, for example, in one and the same condition holds mainly my slowness in walking and avoiding heavy physical activities.

[Brittany Foster]

Hi Valerie,
Thank you for sharing all of this. I remember you telling me before that you were born with CHD too. I love what your doctor said to you “If you’re late for the bus, you can’t run. Wait for the next one”. It makes so much sense. It is always such a bad feeling when you notice your body start to slow down or are unable to keep up as you were able to before. Looking back at what I was able to do at one point always makes me feel so much worse. Thinking back though is just something that everyone does when they reminisce on younger days. It hits us a little harder though because then our thought processes can get depressing. I hope you are helping your mental health as long as your physical health. They are both important pieces to this disease.

[Libby]

Brittany – yes I have a pulmonary exercise stress test every year, along with EKG, MRI and pulmonary lunge function testing 🙂 I’ve never had a heart cath done WHILE doing the stress test (that sounds awful to me!) but I’ve had plenty of heart caths.

Valerie – we sound very alike! I do not like exercise but I am a horseback rider so I’ve got strong leg muscles, just not good cardio. I can totally relate to the bated breath thing. It’s weird because if I don’t take notice of it, I hardly notice that I’m barely breathing but then when I’m in bed or just not doing anything, I’ll notice it and it irritates me, haha!

[Valerie]

Hello, Libby!
Do you ride a horse? It sounds incredible! I thought that for PH disease prohibited activities such as horse riding. It seems to me that even trying to just get on a horse takes a lot of effort. Recently I was offered to try it and I sarcastically refused. How long have you been doing this?

[Brittany Foster]

Libby,
I’m the same way with the breathing thing. If I stop and pay attention to it, I can tell that my breathing is a lot more shallow than everyone else and that my chest barely rises when I’m breathing. It irritates me that I physically can’t take a deeper breath even if I tell my mind to do it. I can do all the convincing I want and psych myself up for the tests to do well on things like the PFT but then the results are always the same! That in itself can be SUPER irritating too! I try to distract myself through the day though and like to stay busy as much as possible. Being a part of the forums definitely makes me feel less alone that’s for sure!

[Libby]

Valerie – I have been riding for 12 years (since I was 12, now I’m 24). When I am out riding alone on my horse I don’t have issues because I can go at my own pace but when I take riding lessons, the riding instructors can be pretty tough on you and I have to ask for walk breaks. At one point I got my dream job teaching horse back riding lessons and riding horses in training. I was riding 2-4 horses a day for almost an hour each and despite riding consistently for 12 years, that EXHAUSTED me. I ended up quitting after a year because I would come home and collapse with exhaustion. My PH is considered mild at this point. What happens is that, because I LOVE riding horses, I will frequently push myself and not realize until after I am done riding how much my chest hurts.

[Brittany Foster]

Libby,
I totally get the feeling of wanting to do something that you love and then unfortunately having to take a step back or do things at a different capacity than you once thought. It is hard to lose that active side of you and to feel how tired and exhausted you are. I remember working full time as a teacher and just coming home and collapsing with tiredness and whole body fatigue that is so hard to explain to others who don’t truly get it. Do you work right now part time or full time?

[Valerie]

Libby, I’m sorry it’s made it harder for you to do what you love, too. Horses certainly bring you a lot of positive, communication with them – it’s a kind of therapy. Sometimes I see a pony from the equestrian club on the streets of my city. Pony with braided pigtails is so cute, all kids want to ride it. I hope it won’t be so difficult for you to bear the symptoms of the disease and you can continue to communicate with the horses. Maybe if you now (knowing about the disease) will be more careful, to do something slower and more careful, take the proper meds, you’ll be hardier and will be able to continue riding? That’s my theory.

[Brittany Foster]

I hope that with a good treatment plan that you can do all of these things too! You are so right though, Valerie. There are actually programs that have been developed in my state that work with children with autism and disabilities and horses. They are supposed to have a lot of “healing powers” and are usually naturally calm animals and have a way of communicating with people that I find remarkable ! Are there any programs like that near you Libby? Maybe even looking into something like this and starting up a program with horses and those with disabilities would be such a great thing to do to share a little about yourself and your passion with others!

[Carol Volckmann]

Hi Libby, When I was first diagnosed with PAH, I was told I had 3- 5 years to live, and that exercise would not help. I searched for a new Pulmonologist and found the right team. Please do not fear a right heart cath under exercise – it does seem very strange but no problem except the shots to numb you up is not great fun. The new doc said he wanted to get very aggresive with this – so I was put on remodulin,letairis and adcirca – and was told excercise is very important (so keep on riding) treadmill is very good for me. All this was 7 years ago and I remain stable and expect to live a normal life span – I am 74 and have lots mote to do and enjoy. So give that horse a big HUG!
Carol

[Brittany Foster]

Such great and encouraging words for Libby ! Thank you Carol for your feedback. You’re so right that sometimes it really takes such a great team of doctors to give you that extra push or confidence that you might need. It can be very scary and overwhelming to get back into a routine, especially if you have ever had a bad experience while working out (especially prior to diagnosis). I have had to work through a lot of fear around exercise and when I am able to physically get back to it, I honestly can’t wait to start doing more because it was so beneficial for my physical and mental health.

[Chris LaRose Mev]

Libby –

You don’t mention what PH drugs that you’re on.
According to PH stats, without treatment, the ave life expectancy is 2.8 yrs.
So the key is getting effective treatment; have your pulmonary pressures gone down?
What were they @ diagnosis? Now? Also, do you feel better or worse since dx?

chris

[Anthony Collins]

Hi Everyone

I do not know whether what I have to contribute will give others hope or make them depressed. Obviously, I would very much like it to be the former for all forum members with CHD-PAH themselves, or with relatives who have this combination of conditions, but I realise that this is probably unrealistic. According to Paul Bresser et al, PAH associated with CHD has the best outlook of all types and 80% of patients in this category are still alive five years after diagnosis. (“Predictors of long-term survival in pulmonary hypertension,” Journal of Heart-Lung Transplantation, 2011). One caution is that this pronouncement is based on research conducted before the turn of the century. The huge value of it is that these findings were made in a period when our current drug treatments for PAH were all just dreams. My CHD-PAH was first diagnosed in 1963 and primitive cardiac surgery the following year failed to resolve it. My parents were given a prediction that I would not reach adulthood but I was shielded from knowledge of this until my 21st birthday, the additional reason for celebration then being disclosed. I had no more treatment of any kind for CHD until I was 43, when I commenced taking medications for arrhythmia, and no treatment of any kind for PAH until a few months ago, when I began nocturnal oxygen therapy at the age of 67. The member of my family who lived longest with CHD-PAH was the one who had no cardiac surgery and no PAH treatment of any description. Brittany is so right about the adaptive capacity of people’s bodies. Another thing to consider is genetic variables. McLaughlin and her co-writers (“ACCF/AHA Expert Consensus Document on Pulmonary Hypertension; A Report of the American College of Cardiology Foundation Task Force and the American Heart Association,” 2009) suggest that these could be the explanation of why some children with a given type of CHD “develop irreversible PVD [pulmonary vascular disease, of which PAH is a variant] in the first year of life”, while others with that identical shunting defect “may maintain acceptable levels of pulmonary vascular resistance for many years.”

If all of this provides any of you with reason to be positive and hopeful then I am pleased.

[Brittany Foster]

Hi Anthony,
I really appreciate you sharing this information with all of us. Especially those on here with CHD that may have contributed to their PH. I agree that genetics can play a huge role in all of this and how our bodies adapt to certain situations and anomolies that we have been born with. Have you looked into any type of genetic testing for yourself? How many other members of you family have variations of what you have ? Genetics plays a huge role in a lot of my conditions and chronic illnesses and after going to q genetic counselor it provided myseld qnd my family with a lot of clarity.

[Anthony Collins]

No problem, Brittany. As I say, if the sharing helps others I am happy to contribute. I don’t know that genetic testing would add anything much more than a label (mutation of GATA4, or of NKX2.5 perhaps) to what is already entirely obvious. My paternal grandfather had cardiac disease. The degree of penetrance in his sons was 100% (4/4), all born with the same defect; the daughters were spared (0/2). In my generation congenital anomalies were inherited by 33% of the males (3/9) born to those sons and, once again, by none of the females (0/4). My own sons, who currently show no signs of having inherited the condition, have been completely uninterested in the screening offered to them. In the early 1960s cardiology research quantified the familial penetrance as the second highest identified to that point in my small country.

Keep up your good work.

[Brittany Foster]

Wow Anthony,
I find genetics so fascinating and am really interested in all that you are saying here. It seems you are a wealth of knowledge about this. My family has genetic mutations that are autosomal dominant meaning whoever carries the gene has a 50/50 chance of passing it on to their child (male of female). There is such a variety of phenotypes that can be presented in the gene. For our family it is sacral deformities, a large sacral tumor (that needed removal from my mom and my 2 sisters and me), partial breakdown of the lower spine, neurological defects (central nervous system because of the spinal cord) and also phenotypes like congenital anomolies of the heart and vascular system and skeletal abnormalities like hip dysplasia. We have a mutation in the PCSK5 gene and the 7q chromosomes. It’s very interesting to look up if you are interested in genetics. I personally think that it is good for all family members to get as educated as possible (even if there are family members who don’t want to believe it or are scared for their own reasons of getting the testing done) . I think knowledge is power and the more info we have and the more info science has, the better awareness we can bring!

[Anthony Collins]

Hi Brittany
I was interested to learn of hereditary skeletal defects in your family. I cannot be sure that this is the case in mine. My father, his father, his brothers, did not have spinal conditions. I do not know about earlier generations. I have read that incomplete septal formation and imperfect evolution of the spine can occur together during embryonic development. Should this have happened to me – and the medical opinion is that it likely did – then my hyper-kyphosis is at least congenital in origin. However, if this is correct it did take around a decade to manifest itself, unlike the winged scapulae that had long been evident. When it comes to something as complex as CHD-PAH I think that it always pays to bear in mind the possibility of multi-factorial causation. It is not inconceivable, in my view, that the development of normal spinal curvature in those with paediatric PAH could sometimes be compromised by chronic hypoxia and consequent weakening of the supportive musculature. Finally, there is the controversial issue of what permanent damage such surgical approaches as thoracotomy and combined sternotomy-thoracotomy might do to the prepubescent spine. I was not the only one in our male paediatric ward to be left bent over by cardiac surgery. My new and highly exaggerated angle of deformity at least reduced. But getting to the point where the reduction was entirely apparent to those looking on did take months.

[Jimi Mcintosh]

Progression is measured by the 6 minute walk test, heart cath, sometimes a double cath,blood chemistrys . The walk test does not measure the effort and the shortness of breath, when going up stairs or inclines, I can walk a mile on a level surface LOL. Blood gases look good, but I am winded.

I know that everyone is different, based on age, other medical issues, region you reside in, and above all else your determination to not let this disease, control your morality. It feels like you have been given a death sentence, I look at it as being a “life sentence”, what am I gonna do with the time I have left? I use to worry about, what I was going to miss, the people, I was leaving behind, and, and what did I do wrong to deserve this.

I remember being told that I had 6
months or less, I was too sick to be on the transplant list. Here try this drug, it might work. Be careful flying, do not climb Mt Everest. I aged 10 years worrying, about what I could not control. Live your life to the best of your ability, take some chances.
DO NOY SIT AT HOME AND WASTE AWAY.

There is a progression, determined by the classification of your PH (PAH) and other medical issues and risk factors. I have taken medications that I felt allowed me to walk further, breathe better, then been given some that minimizes the progress made. Talk to your doctors, ask them to put on their
Big person pants and to tell to give you their best “guess-timate”. It takes a team to win this game. Try to not worry about what you cannot control and focus on what you can impact.

[Brittany Foster]

Jimi,
Your words are inspiring and motivating and I hope that everyone gets a chance to read what you wrote. It’s so true that people can use this diagnosis and have it destroy them (which initially it’s okay in my opinion to grieve and have time to allow yourself to be upset and sad) but then people have to start thinking about all the ways they CAN actually control their life. Once the control is taken back over these things then life starts to feel more like your own. Making the “best” out of life circumstances has been something that I have always done since I was younger because I didn’t know any other way. It helps to have people like you around who are supportive and encouraging for this community ! Thank you 🙂

[Robin Webster]

While I think it’s important to accept the reality of what having PH (or any incurable disease) means and to get an emotional grip on that, I also think it’s paramount to keep in the forefront of our minds and our daily lives that while we may not be able to be “cured,” we can and often do “get better.” When I was diagnosed a little over five years ago, I literally felt like I was dying, and I figured it would be soon. When I found out I also needed a pacemaker and then found out I had breast cancer and then found out I had liver tumors and then developed a mysterious G.I. bleed … well, you can see how this tale sounds as if it goes … but it doesn’t! I may have a bunch of things wrong with me “on paper” and my medical file may be like 6 inches thick, but I’m in better shape as far as how I live my daily life than I have been in 30 years. I’ve lost almost 130 pounds, and my last 6-minute-walk test was my pesonal best score ever. Yes, I have good days and bad days. And if I make a choice to do something awesome, I know it may take me several days to recuperate from it, but I usually decide it will be worth it. I’ve been on fabulous family vacations and hosted huge parties (for over 500!) and I rode the world’s longest zipline over water in Haiti, sending postcards to all of my doctors with a photo of me zipping along at 500 feet in the air and thanking them for their efforts that got me there. Don’t fall into the trap of thinking that a PH diagnosis is “the beginning of the end.” Allow yourself to have the hope that it could just be the very beginning of an exciting life full of as much hope and as many joys as it has frustrations. Having a serious disease can give us the clarity of being able to see what really matters in life. So, I don’t regret any of it. I wish all of you the blessing of a meaningful, joy-filled life, doing whatever it is you love.

[Brittany Foster]

Thank you so much for all of this encouragement, Robin. All of your words you just said are so true. It’s all about how we view our illnesses and yes, there are some out there who honestly struggle so much with pain and discomfort, but it helps us to appreciate the good days when they happen and to not take them for granted. Before getting as sick as I am now, I took a lot for granted and I have always promised myself that when I start feeling better I will not take any of those things for granted. It makes me appreciate any bit of love, kindness, and joy even more than I did before. It’s just like your quote above that stated, “Having a serious disease can give us the clarity of being able to see what really matters in life.” Thank you for writing such great and powerful words for other members to see on here too.

[Libby]

Hey all – sorry for such a late response! I’ve been away from the forums recently. Brittany, to answer your question about if I work, I do work part time as a waitress and I am a foster parent.

Chris – I am not on any meds except for baby aspirin. My doctor says that all the PAH meds on the market will not help me (I am not entirely sure why, I forgot his reasoning but I will be asking him soon!). Part of my body’s issue is that my PAH has gotten worse due to a newly replaced pulmonary valve. I had a very leaky pulmonary valve (leaking 30%) which was replaced 1.5 years ago. The valve itself works awesome but because it’s the valve that runs to my lungs, it’s new efficiency is actually making the pressures in my lungs go up! Ironic isn’t it?

[Brittany Foster]

Libby,
I have heard of this happening because your body wasn’t used to the actual pressures and blood flow before. Does your doctor think you will adjust to this as time goes on or are they doing anything to help your heart work more effectively with the better bloodflow ?

[Libby]

But wait, there’s more! 😉 my doctor does not think it’ll adjust. The only real treatment I have at the moment is the ballooning angioplasty. I have had 4 and about to have a 5th. The other complication is that my two heart muscles have been switched from birth. The smaller chamber that is supposed to pump blood to my lungs is actually pumping to my whole body so it is working very hard! Then the large chamber is pumping to my lungs which also adds to high PH pressure. It’s a total mess! My heart is working very hard. I was on digoxin for first 9 years of life but then my doctor at the time said I didn’t need it.

[Brittany Foster]

WOW !! Sounds complex for sure. Have you ever gone to an ACHA (adult congenital heart association) conference? I went to one and learned SOOOO much. The next one is October 2020 in Minnesota and I’m pretty psyched about it. You should definitely go.They have conferences about CHD and PH too and defect specific talks too. I would definitely ask your doctor if he would recommend a medication like that again to help with the workload of the heart ! Also, are you seeing an ACHD doctor or are you seeing a pediatric cardiologist? If you are seeing just a regular adult heart disease doctor I would really recommend looking at the ACHA.org website and finding a doctor that specializes in adult congenital heart disease.