Sep 20, 2022
Board certified pediatrician and mother to a young child with idiopathic PAH, Chandani DeZure, MD, shares advice & tips for parents navigating this rare diagnosis and offers a unique perspective from both the patient/caregiver and physician side.
My name is Chandani DeZure. I'm a board certified pediatrician.
I’m also a caregiver to a child with pulmonary hypertension who
just turned four years old. I wanted to share some tips and advice
that I've picked up along our journey as both a parent and a
pediatrician, that would be helpful for other parents of children
with rare diseases to hear.
The healthcare system has a lot of challenges and so as a physician that's been able to be on both sides of the lane, there are a few things that I can offer advice or tips based on my experience. Feel free to kind of adapt it if you're an adult with PH, as well.
The first arena that I would tackle is when you're actually meeting with the doctor. Now, usually this is a short visit or there's limited time, or there's too much to cover for the amount of time it is. We've actually been pretty lucky that our PH team spends as much time as we need with them, but we know that may or may not be the case for everybody. In terms of when you're at your appointment, use your time wisely. Prep for your appointments. Prioritize which questions you need answered. Know the role of everybody on your team and direct the most appropriate questions to that person. Write it down. Walk into the appointment with these questions, because you'll forget. When you talk about one thing, you'll just get off topic.
If you're on multiple meds, verify all your meds. Get those refills at each appointment. Sometimes you have to do prior authorizations. Get all that taken care of so you're not spending time on that outside your appointment. PH is a rare disease. There are usually some specialized centers. Ask what kind of support they have locally, regionally, nationally. There are conferences that also exist for patients. See if they have any that are happening locally anytime soon.
You can ask for a second opinion and ask your team to facilitate that. PH is a small world and they refer to each other all the time and nobody is offended if you ask for a second opinion. I think it just puts your mind at ease to get that second or third opinion. So ask for that. Ask them while you're there how to contact or communicate with them once you do leave the clinic or the hospital. For the “in case of emergency info,” who do you call? How do you call them?
Some of these medications are really expensive and aren't always covered by insurance. Find out if they know any co-pay programs that they may have used in the past with other patients. For example, my son is on Letairis and the manufacturer also does offer a copay program for kids, which many manufacturers don't. You have to be 18 for most other manufacturers.
Some other questions to help you prepare for what's coming is to figure out what the plan is and then what would be your next steps. If it works, what's our next step? If the plan doesn't work, what's our next step? Just so you can kind of frame where you are in this marathon that is PH. Other things to ask at the appointment, are there any clinical trials that you or your child is eligible for that you could enroll in?
I think the biggest discord I find between patients or parents and the physician is the expectations. I think it's important to set realistic expectations of what is going to happen at that appointment. The amount of information you're going to get, the fact that you may walk out of there with not clear answers. I mean this in the nicest way, but you need to set the bar low. If you set the bar low, I don't mean about clinical care, but about the rest of it, right?
When we're hospitalized, I don't expect, even though it should -- trash to be taken out. I don't care if we're sharing a bathroom. Even though I don't need all of that, there's a piece of that working that feels like you're in control of something, or it feels more convenient. If you acknowledge that some of that isn't going to happen or it's out of your control, I think those visits go better. The clinical care, you should absolutely advocate for to the best of your ability.
Then the last piece that I find the most challenging in medicine, in the healthcare system, is communication. So I always like to do, and I've gotten better as a physician doing this, closed loop communication. Once the doctor tells you what they said or what the plan is, you repeat back to them your understanding of it, and then just make sure that you're both on the same page. Have them write it down or you write it down just so nobody forgets or there isn't some miscommunication.
I bring this up because I'm also in some parent groups on social media and I read the stories that some parents who are not medically savvy have about their kids with PH. My interpretation is that's not quite what's going on. There's something lost in translation for these parents. I think that's why it's so important that you understand what the doctor is saying. Sometimes it's they're not saying it well. Sometimes we are not hearing it well as parents. That tends to be the biggest source of discomfort or annoyance or frustration or anger with the system, in my opinion.
That's the things I would say at the visit. When outside the visit, stay on top of your stuff. Physicians, nurses, everybody else involved in your care are typically great and they mean well, but the system itself is broken beyond repair. Things don't always connect well. The concrete things, I would say is have copies of all your reports and imaging readily available. If your health system offers a portal, connect with it. Verify your appointments. Follow up if you don't hear back in a timely manner. Establish contingency planning if things get canceled or rescheduled. Your team has very little control over these things, but it will drive you insane, probably.
I like to keep a binder of all the labs, tests, imaging, all that for my son. So that if you can't remember when something happened, I have something easily that I can pull up. Also, because I have a portal and it's easy to do, I request all his medical records every six months, so that it's updated.
Other things for me, find a therapist and a support group. Find healthy outlets for self-care and do them, which I know is easier said than done. If you're really in a hard place mentally, 988 is an emergency number that has just launched in the U.S. for mental health.
Little things, find out what formulations you can get a med in a liquid, a crush pill, capsules, and then what it tastes well in or hides well in. Make sure you're updating your regular pediatrician of all the updates. Even if they're not PH specialized, they can advocate for you or write letters for school and even do onetime medicine refills, if it's not a specialty pharmacy. They're there for you so make sure they're in the loop.
Know your insurance benefits in and out. Learn what a deductible or a co-insurance is. What's in network. What's out of network. Then for PH, know what transplant options are and what coverage you have for transplant.
Just in general, since I have a child, I make sure his school has a health plan, that all his teachers know the meds, the side effects, what to do in case of emergency. He does have an IEP. Make sure you're hooked into your school if you need that or a 504 plan. Put all the numbers that are relevant into your cell phone: pharmacies, clinics, social workers, teachers, school nurses, all of that straight into your cell phone so that when you need something, it's at your fingertips.
One little other thing, at least for clinics I like to do is put a note in the chart of the best time to call, because I feel I'm very busy with lots of things. Even though they may not call at that time, maybe that's the best chance that they're able to get ahold of you. These are a few of the tips or advice that I would offer to parents from a pediatrician perspective of things that might make their life easier if they're a parent of a child with a rare disease.
My name is Chandani DeZure and I'm aware that my son, Ryan, is rare.
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