Aug 25, 2017
Dr. Juan Alejos currently serves as the Medical Director of the Pediatric Heart Transplant/Cardiomyopathy Program at Mattel Children’s Hospital at UCLA. He discusses the differences between treating pulmonary hypertension in children and adults and ways to increase a child’s quality of life. Dr. Alejos also serves as Medical Director of the Pediatric Cardiothoracic Intensive Care Unit, Director of the Pediatric Cardiac Catheterization Lab and is the Director of the Pediatric Pulmonary Vascular Diseases program.
My name is Dr. Juan Alejos. I'm a pediatric cardiologist at Mattel Children's Hospital UCLA. I'm also the director of Pediatric Pulmonary Hypertension Program, and, as such, I have the privilege of seeing many patients who come in who are thought to have pulmonary hypertension.
In children, that is a whole different story than in adults. Pulmonary hypertension in children can be either a result of congenital heart disease or the result of having true congenital or inherited pulmonary hypertension, and our ability to treat them totally differs from one patient to another.
As we see these kids come in, I think our first obligation is to try to determine what the cause is. We can do many tests including CT scans, blood work, cardiac catheterizations. We may never find the cause in the kids who have a normal heart but have pulmonary hypertension. Many infants are born with pulmonary hypertension, present from the very moment that they deliver. Other children have congenital heart disease and have what we call pulmonary hypertension, but truly is really just an elevated vascular resistance, which means that their pressures are not really what meet the criteria for pulmonary hypertension but, rather, what effect it has on the way that their heart is working is essentially the same.
The toughest part about taking care of these children is that many of the medications that we use are not trialed in children yet, so really we have to kind of think outside the box and work outside of what is FDA-approved dosing-wise and delivery-wise. Most of these medications are designed for adults, and as a result, they're tablets, so giving it to an infant obviously is very difficult, requires a little bit of innovation and imagination and work with the pharmacy.
We use the same type of drugs, same type of medications, ranging from oral medications to inhaled medications to IV medications. As we use these medications, we have to be aware of what doses we're using, how we adjust the dose as the patient grows, and almost as importantly is their potential for side effects.
I do give children credit. I think that children tend to be a little bit more stoic than us adults. They're tougher. They want to have a normal quality of life. They don’t want to complain about side effects quite the way adults might, but I think that for us, it puts a little bit more of an onus on us to really be vigilant in watching them and being in contact with them as we start these medications to make sure they're not having any untoward effects.
It's a challenge for us to take care of children because, obviously, children ranging from zero to 21 in our practice obviously are not the same size, and a lot of our dosing is done based on weight. We have to continuously monitor our patients to make sure that we're giving them the proper dose, advancing it as they get bigger, also as their symptoms warrant it.
There’s a lot of collaboration between pulmonary hypertension or pediatric pulmonary hypertension centers that helps us work together in terms of determining what experience we have on using certain types of medications and what experience we have on the types of doses that are provided or that are started on these medications that can be used.
When we talk about pulmonary hypertension in kids, we're talking about very small numbers that make up these studies, and there are some companies that are very progressive in trying to get their product to be more easily or more user-friendly for pediatric patients, such as chewables or liquids. I think that that is a very positive thing for the pediatric population, because these kids - not only is their heart at risk, but their quality of life is at risk, as well. They're not able to play the way other kids do. Their life is very different, and for us, that's very important.
We recognize that, unfortunately, the fact is that these medications, as great as they are, and there are new medications that are superb that keep coming out, but as superb as they are, they're not cures, but they do help prevent disease from progressing. Years ago, the average life expectancy for kids with pulmonary hypertension was between three and five years. Now, we have kids who are on medications for 10 to 15 years who have not progressed or deteriorated any further and continue to have fairly normal life, able to attend school and to do some of the things that their colleagues - their friends are doing so they don’t feel like they're totally outsiders in this.
There's a lot of progress being made. Some of the more newer medications coming out are basically prostaglandin or prostanoid analogues that are oral that basically work in the same fashion as some of the IV medications work. For a child who has an indwelling IV line, to be able to get off their IV medication and get the IV line removed, that’s a huge thing for them to be able to attend school without having to worry about their line getting yanked out.
I've had patients with lines who lost them at Legoland®, who lost them at a petting zoo. Those things are very traumatic experiences for children. That’s just a child trying to have somewhat of a normal life. What we are seeing is that some of these kids now… Seven-year-olds who had never been able to go into a swimming pool or have a pool party can now go into the swimming pool and have a pool party for her birthday.
It's not just pulmonary hypertension medications, but it's really all medications are not really FDA-approved in pediatrics for the majority of cardiology or cardiac medications. The way we approach it is always with a lot of caution in terms of being careful, how to dose it, how to use it. I'm not the person who jumps into using it on a small child. We do it with care, with very close monitoring. We have the patient come into the hospital several times. We have home nurses come to visit them, to measure their blood pressure, take their oxygen levels.
I think that’s the most important part, building up a very close rapport with the parents and with the patient so that, in a way, we're kind of in their living room as they're growing up on the medication and they feel like they can reach out to us if there's any concerns, because that’s very important.
I think that it's important for them to realize it's a very serious condition, but it's one that we've come a long way in being able to treat. I want them to understand that it is a disease that’s most likely going to require lifelong treatment, and it's not something that can be taken lightly, but it also will require a lifestyle change, but that there is a lot more hope and a lot more optimism that as things improve, that the children will be able to have kind of a quality of life and be able to do most of the things that other children can do their age without much increased risk.
I think it's important for families to realize that the medical team in a condition like this is there, is there for them on a day-to-day basis, the nurses are there for them, and that it's important for them to feel comfortable in reaching out as a team - meaning the patient and the medical team, that we can do a lot to make this in control and in check.
My name is Juan Alejos, and I'm aware that I'm rare.
Learn more about pulmonary hypertension at phaware365.global. Never miss an episode with the phaware® podcast app. Follow us on facebook, twitter, instagram, youtube & linkedin @phaware #phaware #phawareMD