I'm Dr. Angela Bates. I work at the Stollery Children's Hospital in Edmonton, Alberta, Canada. I take care of the pediatric pulmonary hypertension program there. I'm the medical lead, as well as I do pediatric cardiac ICU. So I see both inpatient and outpatient kiddos, all across Western Canada.
I think pulmonary hypertension in kids - the thing that sets it apart from other lung diseases is we talk about being subclinical, meaning a lot of these kids can live with this disease without having any symptoms. By the time they actually show up, they can be very sick. They can be as sick as being in the ICU. They can show even as sick as dying from this disease. We have this window of opportunity to make a huge difference with all available drugs that we have now-a-days, including oral medications we didn't have 10 or 15 years ago.
The other thing that's really remarkable is that this is an amazing group of children that really can do a lot despite being quite sick, if you look at their echocardiograms and look at some of their other different investigations. That's kind of remarkable with these kids. Again, [it can] also kind of trick us practitioners in terms of how aggressive we are with treatment. I think sometimes we aren't thinking about screening them, we're not thinking [about] doing an ECG or ECHO. They give us very few clues early on.
Also a lot of our gold standard of tests are invasive. The gold standard would be doing a cardiac catheterization, which requires sedation [and] a general anesthetic. It requires actually putting catheters into the groin. Those are all things that sound scary to parents, scary to kids, and we respect the fact that some of these kiddos; they have a high risk of something going bad in these procedures. So I think it makes it a bit difficult. That being said, we have really come a long way in terms of noninvasive investigations. Our ECHO technology is better. We now have cardiac MRI and everyday people in the radiology world are looking at trying to find more reliable noninvasive tools.
For us the challenge is a few things. For one, our group of pediatric pulmonary hypertension is very diverse. So two big groups are very different. You have these arterial pulmonary hypertension kiddos. They can either be hereditary or idiopathic related heart disease or other things like connective tissue. Then you have another group of developmental lung diseases. Even [with] treatments. Are there certain treatments that are useful in one group versus another? Some of these kiddos are very little, looking at non-invasive techniques of diagnosing and following these kids is very challenging. That's really where a lot of our research is headed now-a-days.
Then just even clinical end points. Because it's hard to really identify what matters the most for these kids. Obviously, yes, survival matters, but is there something that we can follow as a marker that's a little bit more sensitive? That we can follow that will change the earlier on disease progression that will help us to modify the therapy. Then again another thing that's just a little bit challenging is even different drugs that are available. So, oral medication we do have a lot more. A lot of them aren't approved in pediatrics, so we certainly use lots of medications off label but even what's available for them to get at home. Whether it's subcutaneous delivered or IV and so forth. That's some of the challenges, but also exciting areas for us to move forward in PH.
If you look back at the older literature, diagnosing kids late in the game - their prognosis was quite terrible. Median survival without treatment was around 18 months. Now that we know that introducing different therapies earlier on, these kiddos can live 10-15 years, which gives them that window that; one to obviously be kids and do well but, two to get to a point where maybe lung transplant is a better option than when they were 5 years of age. It also gives us more time to find better treatments. Certainly implementing therapies early on, not only improves their survival but just their quality of life which really is what's most important to parents, to kids, and as well as to us as health care professionals.
In terms of advice for a general practitioner or a pediatrician in the community following these kiddos is, even though it's a scary disease educating yourself is very important. Empowering the families, listening to the families. And then, always not being afraid to ask advice. We're never too busy to talk on the phone. We are never too busy to help follow these kids in the community with you, to see them in our clinic, to provide more education.
The biggest thing for these kiddos is quality of life. Although sometimes we may have to sort of negotiate what sort of therapies are best in these kids. Really in the end, these kiddos should be at home with their families, out doing activities that they enjoy. We work within those constraints but to truly focus on how can kids best live out their childhood.
If you have a kiddo that just doesn't fit the normal mold of what you're used to seeing for common childhood illnesses like asthma or that sort of thing, think about pulmonary hypertension. And if your tests don’t make sense, certainly think of pulmonary hypertension. And I think the other thing is you can do a simple cardiac exam. If you can feel a second heart sound that's abnormal, if the kiddo looks like there's a right heart failure, there's a reason for that. A simple thing like an ECG is a great place to start. There is a lot of things you can do in the office to screen these kiddos and then pick up the phone.
The field of pulmonary hypertension both in the pediatric and the adult world is always moving forward and we really try to empower our families to really help facilitate that, because that is what's the face of pulmonary hypertension is and that's what it's all about.
Moving forward, some of the focus we really want to do is looking at identifying kiddos and family members earlier on and using things that we have; these biorepositories and trying to identify what makes certain patients different than others and then really try to tailor therapy to get the best outcome for these kids. There is going to be a lot of excitement moving forward and the biggest thing will be that this should improve care for our kids.
My name Angela Bates and I'm aware that I'm rare.
Angela Bates, MD, is pediatric PH specialist from Stollery Children’s Hospital. She is committed to conducting research in pediatric patients in the areas of critical care, specifically those with congenital heart disease and those affected by infectious diseases in the critical care arena, as well as patients presenting with pulmonary hypertension of all etiologies. Her research is focused on improving diagnosis, treatment, outcomes and resources available to these patients.
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