Janette Reyes, NP - phaware® interview 504

Nurse Practitioner Janette Reyes from the Pulmonary Hypertension Program at the Hospital for Sick Children shares her extensive experience caring for pediatric patients with pulmonary hypertension. Reyes discusses the unique challenges and developmental considerations involved in treating children from infancy through their teenage years, as they transition to adulthood. Reyes also touches on the emotional and social struggles these young patients face, such as bullying and anxiety, and highlights the role of the multidisciplinary team in supporting their mental well-being.

Hi, I am Janette Reyes. I'm a Nurse Practitioner in the Pulmonary Hypertension Program at the Hospital for Sick Children in the cardiology department. My connection to PH is not just seeing patients in the clinics, but also when they're an outpatient and inpatient, thus promoting continuity of care for the patients and families.  Whenever the parents and patients have questions, I'm there for them.

Today, I want to talk about the range of ages of our patients and how pulmonary hypertension can happen as early as birth. We follow these patients until they're 18 years of age. I've been in this role for quite a while, so I have had the experience of and privilege of caring for these patients as they grow older, from infancy sometimes to their teens and transitioning to adulthood. So, I've had to also consider their developmental stages that they're at when providing care.

It's also been a pleasure and exciting to see them grow and change and have different types of concerns and issues, particularly as they get older and they become more trusting of the medical team and you start to observe the changes in their moods during the appointments. So, you have to be cognizant of what they're going through. Perhaps there have been changes in their school or interaction with their peers or how they're coping with their PH as they change schools as well, making new friends and the challenges that they go through. They're a bit more different in a sense that they can't do all the things that other children and teens can do. They're a little bit more limited, let's put it that way, at times. But, we discuss that there are other positive activities or issues that can come out of their current situation, as well.

One concern is sometimes they somehow with our interaction with each other, something at school may bother them and so we would focus on that. When I ask them how they're doing, it does come up that perhaps something happened at school and they're feeling sad about it. Sometimes the parents inform me that there's teasing or bullying. One patient was experiencing some bullying at school, because he couldn't keep up with his friends. There's also psychological issues that could make them feel sad as they report. We're fortunate to have a multidisciplinary team of social worker,  psychologist-- and psychiatrist -- that can help us with such issues, including anxiety and how they're dealing with their illness, family and friends.

For some patients, I start to see them at a young age. As they go through their stages of growth and development they go from being very chatty at their clinic appointments. They're not aware of their diagnosis, so they come to a clinic visit happy because most of them will get a treat after their appointment and they're not really understanding what's going on. They're eventually get used to taking their medication on time. They hear from us that “You have to take this in the morning and then at night and they understand that it is good for their heart. It interesting to witness the change in their disposition as they grow older, where the chattiness starts to become less. This mood start to occur  around 11 or 12 years of age, the tween years, when they are quiet during the appointments. They sit in the room and not being very verbal. You have to really try to get information out of them, so the parents are very much helpful and involved in letting me know what they’ve observed and how their child's feeling.

Then, as they patient becomes older, and mature, they start to understand their health and illness. At around age 14 years old, I will start more in depth teaching by discussing and illustrating, “This is what's wrong, or this is what's happening to your heart. This is pulmonary hypertension.” Then, once they start to understand, then they start to engage more in conversation, become more responsible and involved in their care over the years. There are still some patients that require more teaching and there are others that understand and being to ask more in depth questions and discuss what they're going through.

With respect to the transition process, as the patients get older, they become more responsible in taking their own medications, reporting more of their symptoms to me. This is the one of the goals. I also encourage them to communicate directly to me rather than having their parents inform me and the doctors. The parents start to release some of their, interaction with me. They start to understand that they know what they're feeling, they know their symptoms, they know what their concerns are more than anyone. A typical interaction would be when the parents will wait and remain quiet, allow the child talk and answer the questions and sometimes when the child doesn't... Or the teen forgets something, the parent(s) will say, "Oh, remember you did have chest pain last week." Just sort of as a reminder. The parents and the PH Team help prepare the teen towards transitioning to adult care as they leave Sick Kids.

The way I deal parents who remain very involved with the care and responses to my questions, where when I ask the question to the patient and then the parents will right away answer, I turn back to the patient and say, "What is your description of how you feel? What is your pain like? How often?" I turn the conversation back to the patient. However, if the teen is not able to further provide a detailed response, then I'll turn it back to the parents.

We discuss goals. I would typically say, "When the next time you come, I want you to tell me how much have you done." For the older patients, I would say, "I want you to call your prescription to the pharmacy. Your parents can be there, but I want you to make a phone call. Then, when you come back, you tell me if you did that." I discuss little goals to help them with that independence. I let the parents know it is also for the best, because you're not going to be there with their child at all times when they're in university or college. We do need to prepare them to be a bit more independent."

I've been in my role over 20 years. In the 20 years, we went from just Flolan, intravenous Flolan with the emergence of sildenafil in the 2000s. Then, came the bosentan. Then, what was wonderful was the treprostinil, which then was an option, because Flolan as many of you know, you have that six minute half-life. It's a very demanding, stressful therapy. So with the four-hour half-life of Remodulin, that was something that was positive that came out of these medications. Then, more oral medications started coming to a point where there was even an oral prostacyclin, where we could at least prolong the subcutaneous and the IV medication therapy. Sotatercept, with that coming up, that's another exciting new pathway of medication. So everyone's certainly looking forward to that.

We've come a long way, and my conversations with families, it's still the same in the beginning where it is overwhelming. I tell them not to Google, although they will still Google, which is understandable. But now we have so many resources in the PHA Canada website and PHA Association in the US and other countries that we could ensure them and  say, "there is no need to Google. Go to these websites, and that's where you'll find your information." That's the change in helping these parents in the beginning who are so overwhelmed with the diagnosis, particularly with idiopathic pulmonary hypertension. Then, over the years, it has been easier because there are medications. We'd no longer say, "Your child has three to five years to live." Now we don't know. These children are becoming adults, growing up to be adults, perhaps only on one medication, maybe on just two oral medications.

The types of conversations have certainly changed more in a positive way. It's an exciting future up ahead for even new patients who are being diagnosed. So, hopefully there's going to be more medications and maybe a cure. A cure is what we're looking for. We're fortunate we have different options and now a different pathway of medication, but let's hope a cure is coming soon.

I think one thing that I'd like to add is that parents should be open to allowing their child to grow up as normal as possible and allow them to experience life. These children can do many activities of daily living and be happy. They know their limits. If they want to go out with their friends, just set out some guidelines in case there's issues or emergencies. That way they can hang out with their friends. It's okay. It's okay if they want to do a little bit more, physical activity such as sports and employment. Talk to the PH team. Together, we can help the child grow up as normally as possible and experience life to the best that they can live.

One last thing that I'd like to add is that I'm very fortunate and privileged to work with children. Each child is different and unique in their own way. So, the way we treat them and my interaction with each and every one of them is unique. The care comes from the heart.

My name is Janette Reyes, and I'm aware that my patients are rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Engage for a cure: www.phaware.global/donate #phaware Share your story: [email protected] Like, Subscribe and Follow us: www.phawarepodcast.com. @phacanada