Hi everyone! My name is Jennifer Hinkle and I’m a heart mom here in St. Louis. My husband and I started the Ollie Hinkle Heart Foundation five years ago in honor of our son, Ollie. The Ollie Hinkle Heart Foundation is dedicated to lessening the impact of CHD through love, awareness, and the advancement of congenital heart disease diagnosis and treatment. Recently we sponsored the A Dose of Magic event and are excited to help bring a continuing education series through Facebook live discussions. Today we’ll be discussing the topic of pulmonary hypertension with Dr. Mark Grady and Jessica York. Dr. Grady is a cardiologist and pulmonary hypertension specialist here at St. Louis Children’s Hospital. Jessica is a pulmonary hypertension mom and advocate. Thank you both for being here today. Thanks for having us. One last note before we begin, we’ll be giving prizes away to the first three people who submit questions during this discussion— all you have to do is be one of the first three viewers to submit a question and you can win some awesome prizes from The Heart Center and the Olli Hinkle Heart Foundation. To begin, Dr. Grady, can you help explain what pulmonary hypertension is? Sure it’s a disease of the lungs that makes it difficult for the blood to get through them. That’s a pretty simple explanation. There’s lots of different reasons why that may be the case but essentially it’s a problem with the lungs getting, I mean a problem with the blood getting from from the heart through the lungs to the other side of the heart. Okay. Jessica, I read about your daughter, Haley’s story on The Heart Center’s Facebook page. Can you tell us a little bit about her and her diagnosis? Sure, Haley is the youngest of our two daughters. She’s five and she has a sister Jordan who’s 15. For the first few years of life, Haley met all her milestones at a steady pace and as her sister had years before. She developed a magnetic personality always smiling, making jokes, and doing pranks to make people laugh. At our three-year-old checkup which was delayed, so we had it in October of 2016 her birthdays in July, was when her heart murmur was heard for the first time. Her doctor had decided that we would wait a month to see if the murmur was still present. If it was, then we would go ahead and schedule an echo. During that time my dad had become paralyzed. Six months prior, she’d been going to weekly appointments with him to a wound care specialist. That office actually noticed that she had purple lips, purple fingernails, and seemed really short of breath so they urged us to call her doctor, which we did. They immediately scheduled the echo which was when they discovered she has a complete AV canal heart defect also with pulmonary hypertension. Dr. Grady, as you know Jessica is a big PH advocate and she’s pulled together some questions from the PH community for today’s discussion. What signs are important to look for to rule PH out or get earlier diagnosis of pulmonary hypertension? That’s a good question! I think sometimes the diagnosis can be delayed as in Haley’s case for a number of years when it’s thought to be perhaps something else. It’s a rare diagnosis and so as a pediatrician you have to have that in the back of your mind as a possibility like lots of rare things that happen in kids, but in general if a child’s got significant pulmonary hypertension, at some point they become a little bit less active. They’re not as playful or as energetic as they used to be. Sometimes it can mask as asthma. I’ve seen situations where children are diagnosed with asthma for several years. It doesn’t seem to be getting better despite a lot of treatments and they come to discover that it’s actually pulmonary hypertension. Anytime a child faints for no good reason, that sometimes can be a sign of pulmonary hypertension. Now, lots of kids faint, so I don’t want everyone to panic, but if it’s something that happens repeatedly that would be another sign. How can we help educate pediatricians and nursing staff on local levels about early diagnosis and taking the observations of parents seriously? Well again, I think situations like what we’re doing right now just increases the awareness of pulmonary hypertension as a diagnosis. I’ve been involved with it for the last ten years and I can say that the number of kids that we’re diagnosing with it seems like it continues to go up each year. I think just having that in the back of your mind as a general pediatrician is hard because they have to have lots of things in the back of their mind, but again just letting people know that that’s always in the differential. What kind of challenges are presented when trying to treat a child with pulmonary hypertension with medications that are not yet approved by the FDA? Well that’s a problem that’s true in a lot of pediatrics. As a pediatric cardiologist, we do a lot of things other—I do—other than treat pulmonary hypertension and a lot of it involves medicines and that aren’t necessarily approved for children. We struggle with this all the time. Drug companies just aren’t as interested in developing that specifically for children, so we have to borrow from the adult world. A lot of times that works out okay. We just give the kids maybe a little bit less of a dose than we do the adults, but sometimes we’re not sure what the effects will be on a growing child. Sometimes the studies aren’t there and we just have to take our best guess with it. It can be problematic especially in the PH world. These medicines are rare or expensive. To be really effective there are some that you can take by mouth, but there are some that really require more invasive delivery like an IV that has a pump much like Haley has been wearing for the last several years that provides that medicine 24/7. Obviously, that’s a big challenge for an energetic three or four year old to keep that going. Right? Yeah. Does Haley have any limitations from her pulmonary hypertension? Yeah, she definitely does. I mean Haley pushes herself very well but she has limitations like climbing the stairs. If she climbs stairs, she gets out of breath after three stairs. We had to get a stair lift put in our home just for her to get upstairs to her room. She has to use a wheelchair when walking long distances always, but even sometimes short distances if she’s having a bad PH day. That can be a problem. She also has the Broviac in which she has to wear a backpack 24/7 to pump medication through so she can’t be submerged in the water. Bath time requires shower guards. If she swims, she has to wear a special dry suit for that. There are definitely limitations. Then, she’s getting ready to go into kindergarten so that’s a whole process because right now she goes to preschool just twice a week, three hour days and I mean that makes her exhausted. The fatigue is another big limitation for her as well. Mm-hmm. Dr. Grady, are there any new potential treatments on the horizon that you’re excited about? Well again, we have to rely on some of the adult studies for our medications. I don’t know that there’s anything pill wise that is really going to be effective in the pediatric world. There are some devices that might be of interest. I know Haley has to have a backpack where her pump is continually providing her with her medicine through a central line. In the adult world, now they’ve started to take those pumps and actually implant them under the skin, so now you don’t have a backpack anymore. Again that’s just getting under way in the adult world and whether something like that will be applicable to children, that would be exciting. Yeah, I was going to ask. Jessica, can you tell us a little bit about Haley’s medications and the process you and your husband carry out to prepare these medications? Sure two of our medications we have to order from a specialty pharmacy which that in itself takes about a 30 minute phone call every month to do. Then you have to sign for the package because they’re very expensive. Then her modulin—she used to be on Flolans—you had to mix that every single day. There are modulins that you have to mix. They actually have a module diluent now so we can premix it for like seven doses. You switch the cassette out though every other day so we have to do that, as well as her bandage changes we do once a week. Change those out. Then she’s on oral medications which she takes 2 at 6 a.m., one at 2 p.m., one at 6 p.m. and then one at 9:00 p.m. Those are her daily meds. Speaking of medications, Dr. Grady, to your knowledge, do any of the medications or pulmonary hypertension itself cause problems with schoolwork like attention, focus, and learning? It’s a good question. I would just preface it by saying miss York does a fabulous job in managing what really for a lot of people is almost inconceivable. It’s almost like having your own intensive care unit at home. She’s awesome at it. Thank you! I appreciate that. Haley has done as well as she has because of the dedication of mom and dad here. In terms of how it affects people at home, I mean at school, I’m not aware that they have direct, I have not seeing that as a common problem. I think they all have side effects. They can cause headaches, GI stomach upset, and things that may affect their performance in school or fatigue and things of that nature. We had a few questions about the surgical procedure Potts Shunt. Dr. Grady what is Potts Shunt and who is considered the ideal candidate for this procedure? Right so earlier you were asking me if there’s something new that’s coming out that may be of benefit to kids with hypertension. I would say this this surgical technique called the Potts Shunt, named after the surgeon who developed it back in the 1940s, was for treating a different kind of congenital heart disease. The utilization of this Potts Shunt in the last five to ten years has—I think—made a real difference in some children’s lives with pulmonary hypertension. Essentially it’s a connection between the right side of the heart and the left side of the heart. Instead of the the right side of the heart which has got to push all that blood through the lungs which it has trouble doing, you make this connection to give the heart a back door where some of the blood can escape. Perhaps that pressure drops a little bit inside the heart, so it’s not under so much tension. By doing that, you preserve the heart function longer, the kid feels a little bit better, they’re a little bit bluer because of it but their heart is a little happier. It’s a technique that was first developed and used a lot in France. We’ve now just done our sixteenth child with a Potts Shunt which is probably more than any other center in the United States anyway. I’ve seen where it makes a huge difference in a child’s life and how they feel. Are there any reasons that would exclude a child from being a good candidate? Well, yeah. It’s a careful selection process. It’s certainly not going to be for everyone. For Haley’s pulmonary hypertension, it would not be an effective treatment for her because she’s already got a hole in her heart that allows some of the blood to escape through it, but generally if a child has really very high pressure on the right side of their heart then they would potentially be a candidate for a Potts Shunt. Okay. Are there any benefits of the Potts Shunt being done before remodulin being started or before there is any heart RV damage? That’s a tricky question. Remodulin is one of several medicines that are called prostacyclins. They are considered by most people in the PH world to be the most effective medicine that we have to treat pulmonary hypertension. The downside is how they have to be delivered. Much like in Haley situation, it has to have to place for a central line or it’s given under the skin, but you have a pump. You’re given it 24/7 and as Ms. York can testify that that’s a huge change in your life and the quality of life of the child. Most people would feel like any child with significant pulmonary hypertension for the most part probably deserves, if it’s really severe, a trial of the prostacyclin. Where does the Potts Shunt fit into that? I think that’s a work in progress to be honest. We’ve had several children, one in particular that had severe autism, and there was no way that he was going to allow us to put in a line and he was not going to keep it in place; as a consequence, we elected to do a Potts Shunt without trying the prostacyclin and he’s done great, but in general we probably would give a go at the medicine before jumping to a Potts Shunt. Now how long you would try it, that’s a that’s an open question. Okay. Then, another question is can someone grow out of pulmonary hypertension or be cured of it? Well, there’s lots of different reasons why someone might have pulmonary hypertension. Some can be very straightforward like you have a thyroid disorder that can cause pulmonary hypertension so if you cure the thyroid problem the pulmonary hypertension goes away. Unfortunately for lots of people that develop pulmonary hypertension, we don’t really know the cause of it. Those are much more difficult to predict. In general, in children, I’m much more optimistic if a young child or an infant develops pulmonary hypertension than if it’s a it’s a teenager. An infant still has 5 or 6 years of growth ahead of it where the lung can develop new lung tissue. I always feel like if you can develop new lung tissue, there’s always a good chance that could be good lung tissue and that may be your pulmonary hypertension and will improve with time. Okay. Jessica, you’ve been such an advocate of the pulmonary hypertension community. Why is it important for you to be so active and spread the word about PH? I think it’s really important because PH is rare but it’s also considered an invisible disease. I think everyone knows what a lot of illnesses are like diabetes and things like that, but when you say PH people have no idea what that is. It’s hard for people to look at Haley and see this happy, smiling, active child and wrap around their head that she has a terminal illness, so I think it’s important to change the vision of what everyone thinks a terminal illness is supposed to look like and that it doesn’t necessarily have to look like that. It can be something like PH. Then, I would just really like to see PH be ruled out instead of ruled in so we can get earlier diagnosis. Maybe if we caught Haley’s earlier, the situation would be a lot different. I think it’s just important to get the word out so people know what it is and really make them aware. Mm-hmm. What kind of support have you and your family received from others going through a PH diagnosis? I think our support stems from what Haley has been able to give to the PH community. When I looked at Haley when she was 2 weeks old, I just thought there’s something special about her. She’s going to have a big purpose in life and I think now her purpose is clear. We’ve had people reach out to us telling us that seeing Haley smile gives them inspiration to keep going and helps them get through their hard days which makes us feel better. I really feel like she’s fulfilling her purpose here which is nice to do. Yeah. What kind of support have you been able to offer other families? Just really going to the events, reaching out to people on Facebook, answering questions, just trying to really do anything we can. We do an Easter exchange and Christmas exchanges with the PH community. Facebook makes it really easy to connect with everyone now. It’s been really helpful. That’s awesome! Well keep doing what you’re doing for sure. Do we have any questions from our audience? Okay. Here’s a question coming in from Nicole Phillips, “There are PH support groups around the nation, but many are focused on adult patients where can pediatric patients turn for support?” That’s a good question. There are family support groups on Facebook, but I think that’s why we’re doing things like this to try to connect more PH families. I think when it comes to your child some people deal with PH differently than others but that’s what we’re hoping to do by doing things like this is to gain more support. I would add that there are some organizations that actually do cater to families with children with Pulmonary hypertension, in particular I think you’re involved with the Pulmonary Hypertension Association. I think their website is pha.org. If you go there they have I think a lot of information for families with kids that have pulmonary hypertension Team Phenomenal Hope also has a support group,a call-in support group, and things that we’ve been doing as well. That’s great information. Tanya Stark has a question. “Do most kids with PH also have CHD?” That’s a good question from Ms. Stark, who I know well. It’s actually becoming a bit more common to see Pulmonary hypertension associated with congenital heart disease especially as we’ve gotten more sophisticated in helping young children and babies with congenital heart disease. They’re living longer and so now we’re starting to see some of the secondary consequences of of that disease as they as they grow. By and large, it’s probably a smaller subset of kids that have pulmonary hypertension. Haley is a little bit unusual in her presentation. Miranda Hughes asks, “My daughter was diagnosed with PH at three months old. She has a repaired repaired AV canal defect. I have been told her lungs will get stronger as she ages. She’s now 18 months old with a trach and weaning from her vent. I have noticed how much more active and stronger she has become as we are weaning. How can we tell if she is really actually doing better from her PH?” Well first of all, the fact that she seems like she’s doing better is a good sign. I think if her pulmonary hypertension was truly worsening she wouldn’t be as active as she’s describing there. I think that certainly the child’s physician should be able to assess the pulmonary hypertension relatively easily through things such as echocardiograms. Occasionally if you need, to you do things like cardiac catheterizations. It should be fairly straightforward to get a good handle on how her pulmonary hypertension’s evolving. It’s a good sign that she’s becoming more and more active. Again, the nice thing about little children is that they grow and they can actually get better. Okay, we have another question from Christine Car, “When is the Fun Walk and how do we sign up?” It’s this year on May 5th which happens to be World PH Day. There are links on the PH Fun Walk for Hay page on Facebook as well as Pray for Hay on Facebook. It’s going to be a great event. Hopefully, it’s going to be annual. It’s going to have a one-mile fun walk. We’re going to have Louie from the St. Louis Blues there, Fredbird from the Cardinals, face painting, balloon animals, and airbrush tattoos. I just hope to get as many people out there as we can along with the PH patients, and raise some money. All the money is going to go to the PH Robin Bars Pediatric PH fund to help with research for a cure. It sounds like a great event. Yeah. Do we have any more questions from our audience? Okay. If we don’t have any other questions, I’d like to thank Jessica and Dr. Grady for joining us today. Thank you all for tuning in and remember this discussion will be available in our video section shortly after we finish streaming. Always feel free to leave us a comment if you have a question or concern and someone from The Heart Center will get back with you. Thanks again! We will see you next time.