How Do We Meet the Needs of the ACHD Population?
September 24, 2013
Everyone would probably agree that the adult congenital heart disease (ACHD) patient population is one of the most challenging set of patients to provide comprehensive care for. Medically, their physiology and anatomy is complex, and from a sociologic and public health standpoint, they must navigate a difficult transition from pediatric to adult cardiac care. They also comprise one of the fastest growing patient populations in contemporary cardiology. As such, these patients are a natural and important group to take a closer look at in how to provide truly patient-centered cardiovascular care.
To help us understand how to best meet the needs of adults with ACHD, we interviewed Dr. Amber Khanna, who completed an Internal Medicine-Pediatrics residency at the University of North Carolina, Chapel Hill, and a fellowship in Adult and Congenital Cardiology at the Mayo Clinic, and currently practices at the University of Colorado Hospital (UCH) as a specialist in ACHD.
I imagine the transition from pediatric to adult cardiac care can be very difficult. What seems to be hardest part of making that switch?
I think the transition is very hard. First of all, children really bond with their pediatric cardiologist. A lot of them have known their doctor their whole life. They've seen them frequently, been through a lot together, inpatient and outpatient, post-op, made the decision to go to surgery together. There's a lot of bonding that goes on in that. So I think breaking that bond for no particularly good reason other than reaching a certain age, is very hard for patients to understand. And patients will say, "why don't you just call my doctor who knows me? Why do I have to tell you everything when there's this other person just down the road who knows all about that."
What kind of things can we do to make the process smooth?
I don't think that what we're doing here now is perfect, but one of the ways we're trying to smooth it out is by my going to Children's Hospital. Patients are followed by a pediatric Cardiologist at Children's, and then at a certain age, they are told, okay, the next visit will be with Dr. Kay (Program Director of ACHD at UCH) or myself, and we see them at Children's. So then it just sort of eases the transition. So they can see me, and meet me in a familiar environment and a familiar process. And then I can say, okay, next time, you're going to see me over at the other building. It kind of staggers the transition just enough so that it makes it a little bit easier to understand that we're not just going to a brand new place with strange doctors at a strange place all at once. And when that works, that seems to work pretty well.
We've also talked about not springing [the transition] on kids, and starting at a much younger age, at 12-14 years. We tell them, someday, you'll need to see an adult cardiologist, you can't see a pediatric cardiologist for the rest of your life. We also prepare them for what it's going to be like in adult cardiology, where there's an expectation that you know your disease. Adult cardiologists expect you to know your medications, to call us if you run out of medications. They expect the patient to call to set up another appointment.
We also educate these teenagers and young adults about when it's appropriate to call their doctor. A lot of people have no idea what kind of things to call your cardiologist for, what kind of things to call your generalist for. So just starting that whole education process of how to be an adult patient.
How do parents fit into this? I have adult patients who still come in with their moms, and they are around 60 years old, and their moms are in their 80's.
One of the things I try to do is make sure that the patient is sitting in the chair closest to me. For a long time, it's been the mom who sits in that chair and talks to the doctor. So just that simple thing, that shifting of it is important.
I always ask the first question to the patient. I don't ask mom what the diagnosis is, I tell the patient they can ask mom for help but I just keep bringing it back to the patient--what's your diagnosis, what medicines are you on, what symptoms are you having? Sometimes I'll ask, "are you having any shortness of breath?" and they'll look to their mom!
A lot of the parents are very nervous, but they know that this has to happen. So they are very supportive of me asking the kid the questions and let them take charge.
Because they recognize that they can't always be there.
Right, and a lot of them are envisioning their child go off to college, or going away somewhere. And I phrase it as, "I read through your chart, I know what you have. Now you're in the middle of nowhere, you're in the emergency room, and your heart's beating really fast. What do you tell the doctor? What's the name of your diagnosis?" And a lot of the 18 year olds don't know.
Do they have trouble understanding that they still have heart disease that still needs to be followed? Or trouble understanding that they have to stay in the medical system long term?
Not to bash the surgeons, but the surgeons like to tell people that they fixed them. And I think it's very natural--we'll take your child to surgery and we'll make him better. And the parents hear, you will fix him, and he will be normal. And the doctor says he can participate normally in everything.
Nobody likes to tell them, we're going to do this, but in 15 years, you're going to need another surgery. Or as you get older, you will probably have rhythm problems. You know, the parents don't hear it, but you know, I kind of think nobody really tells them. It's very focused on the here and now, and getting through what we need to get through, without the doom and gloom.
I was thinking about how hard it is to explain to someone what they have when they have simply bread and butter coronary artery disease, and yet with congenital heart disease, there is a whole other level of complexity that is even challenging for cardiologists. Are there constructs in place to help patient education, to get an understanding of their disease on their level?
There are two things that we do. I almost always bring in a picture of their heart, a diagram of some sort. I go through it with them, and say, okay, this is how your blood goes. This is how normal blood flows through the heart, and this is what's going on with your blood, this is the problem, and this is what they fixed, and this is what we're left with now. And I make sure that they know that, and then I hand [the diagram] to them at the end.
The next thing I always do is I send my patients a copy of their clinic note, so that they have the medical jargon. It's not addressed to them, it's addressed to their primary care doctor, but a copy is cc'd to them for their records. A lot of times they'll read through it and a lot of times they'll call us and correct us, "No no, I have two sisters, not three sisters." But at least then they have it.
The anecdote you related at your recent Denver Echo Society talk, about a patient telling you they had "something about flow" as a child that needed to be repaired, when it was Tetralogy of Fallow, was very funny. It sounds very similar, and it also kind of makes sense. So I'm sure that having the medical jargon in print would be very helpful.
How about access? A lot of patients don't live geographically conveniently located to any cardiologist who is familiar and experienced with adult congenital heart disease.
So Joe Kay and I, we two are really the only adult congenital providers in Colorado. There's nobody in Wyoming, there's nobody in half of Nebraska, there's somebody in Salt Lake City, but we're really it for a very geographically large area. We do some outreach, we go down to Colorado Springs, Joe tries to go out to Cheyenne, but with just the two of us and the patients so spread out. it's not really worth it to go out once a year or twice a year. It's just not how you deliver good care anyways, so patients end up driving and coming out here. And we try to coordinate it, and either have a really good solid primary care doctor or a local cardiologist. Local cardiologists don't always like sharing.
A little bit of a tangent, but do pediatric cardiologists sometimes opt to continue following their patients into adulthood, especially if there is limited access to a cardiologist comfortable with ACHD?
Yes, but one of the problems with that is the doctor will keep telling the patient to come back every year (appropriately), but at some point the patient decides, clearly, I don't need to come back, and there aren't any other adult patients in the waiting room. And so they just drop off. And the patient stops seeing anybody.
And then another problem is that the patient then ends up getting transitioned at a time of crisis. So everything's fine, going along, and then they become pregnant. And, all of a sudden it's time to become an adult, even though they're like 28 years old. But it would have been better if they had come earlier, that we had talked about things. We are very aggressive about recommending certain forms of birth control, we have this talk all the time, and I don't think pediatric cardiologists necessarily do. We always do a lot of high risk obstetrics.
It's much better to transition before a time of crisis. Another time of crisis is when patients end up in the hospital. Something happens, they call, and the ambulance isn't going to take them to Children's, they are taken to the University, and now they're there, and they don't know us, we don't know them, and it's just traumatizing and not the best time to transition.
I wanted to ask you about technology. I came across Follow My Heart, but I don't know how much it is used in real life. It seemed like a great thing. All of the patients that I've had with ACHD, didn't know what was wrong with their hearts at all. Has technology been helpful for those patient whose records have really started at birth?
Yes, it has. I've heard of Follow My Heart, but I've never had a patient who's used it. We have little ACHA, patient-based advocacy group, health care passports, little booklets with contact numbers, a diagram of the heart, write down medicines and allergies, staple an EKG.
Nothing on the web or cloud yet?
I think that's definitely a direction we need to go. Wouldn't it be great if it was on the phone?
Do you still have trouble getting records? Do they not have separate standards for saving records for children with serious medical problems issues, do they get destroyed just like everyone else's gets destroyed?
The other issue with access is insurance. And we deal with this on a daily basis. So I've got a patient who had surgery in 1955, and needs another heart valve. She's virtually disabled, but she's able to work at the Double Tree part time, so because of that, she's never applied for disability. But she has a peak VO2 of 14, she's pretty darn limited, but she doesn't have disability and she doesn't have insurance. And now we're trying to get her surgery done. And we have some ability at the University to do patients without insurance, but it's tricky, it takes a lot of paperwork and justification, and convincing the right people that it really is necessary, and no, we can't wait another 5 years until she's 65, we need to do this now. So the insurance issues are a big problem.
What do you think about the medical home concept for ACHD patients?
I think it's very hard. I actually don't think that having a specialist as your home is a good idea. I think that Mayo kind of works like that because it's such a referral clinic. It's fine if someone flies in once a year specifically for a particular evaluation, but in terms of primary care, I think the primary care doctors should be very much against this idea. I don't think that I'm up to date with primary care. I don't do pap smears, and I'm not going to start. There's so much to keep up with in my field, I'm not going to keep up with what's going on now with prostate cancer screening--it's changing, it's complex. I think that's what primary care physicians (PCPs) do. I also think there should be a bigger role for PCPs managing a lot of other issues with my patients, who can check electrolytes and a weight.
I find it interesting that the ACHD is very much a growing population. I don't know if you want to comment on that?
It's also an evolving onewhich makes it hard to learn about. Fifty years ago, they all died. Somewhere about 45 years ago, they figured out how to do the Mustard and Senning procedures, so now we have a bunch of 25-45 year olds who have had a Mustard or Senning. Then about 25 years ago, we figured out how to do coronary buttons, and how to do arterial switches. So we know something about how to deal with Mustards, but it's going to be an obsolete problem. We're just starting to learn about the problems with arterial switches. So it's fascinating because it's always changing.
Any other comments on staying patient-centered?
I think it's important to think about patient-centered outcomes. The outcomes that we have are real and easy to document, but don't make that much difference to the patient. The patient doesn't care if they're intubated 3 or 4 days, but that's a real outcome, and we judge surgeons on that. Most patients want to know, when can I return to full work, or when can I drive? We need to be more patient-centered in judging outcomes.