Q&A: Transition to adult congenital heart care must be managed carefully

Wayne J. Franklin
Wayne J. Franklin

The proportion of children with congenital heart disease who survive to adulthood continues to rise. With this good news, however, comes a challenge: The cardiac care of these patients must be managed throughout their lives, and at some point they must transition from being overseen by a pediatric cardiologist to being overseen by an adult congenital heart disease specialist.

With this transition comes the need for patients to become more knowledgeable about their condition, to take a more active role in seeking care and to better understand which behaviors and medications are safe for them.

Wayne J. Franklin, MD, co-director of the Phoenix Children’s Heart Center, director of the adult congenital heart disease program and chair of adult medicine at Phoenix Children’s, spoke to Cardiology Today about how best to transition children and adolescents with congenital heart disease into adult care.

Question: When should the transition begin from pediatric cardiology care to adult care?

Answer: The book answer is age 12. Functionally, however, I’ve found it more realistic to start around age 14 or 16. It is flexible because some teenagers are slow to mature, but others are mature enough to talk about adult heart-care topics. We all agree that it should definitely start before age 18, and certainly before age 21.

Q: What sort of knowledge should a patient have as the transition is made?

A: There are different levels of understanding. I pick three to five things that I make sure every patient should have for when they transition. There is a 20-point list of things that they should be able to do, but if I narrow it down to three or five, that is more feasible for a teenager or college student.

I do have a top five list of points, in no particular order.

No. 1, they should know their diagnosis and be able to explain it medically. For example, “I have D-transposition of the great arteries, atrial septal defect and heart block” or, more simplistically, “My arteries were switched and I have a hole in my heart.” Either way, they understand what their diagnosis is and can name it. That is half the battle when you are a doctor seeing a patient who has a long medical history for the first time.

No. 2, it helps them to know what was done. Most of these patients have undergone some sort of repair, either surgical, catheter-based, medical or otherwise.

No. 3, it is also helpful for them to know what medications they are on and why, as well as the dosages. This is true for any patient.

Those are the three most important ones, the next two are a “bonus.” No. 4 is to know how to make an appointment. The patient’s parents have directed their health care for their whole life, but sooner or later, they have to be responsible for their own care. It takes some skill, courage and initiative to pick up the phone, send an email or go to a website to make an appointment.

No. 5 is where to go for emergencies. Should they go to the pediatric hospital or the adult hospital? Should they call 911 or not?

Q: What insurance considerations come into play when this transition is occurring?

A: In the United States, children may be on their parents’ health insurance plans until they turn 26. But I tell patients that they need to be very aware of getting good insurance, which means a plan that doctors and providers will accept and that covers most of their care. If a job offers you a lower salary but better benefits, consider taking the benefits, because you are going to meet your deductibles and spend all the health care dollars allocated to you each year. Try not to have any gaps in your insurance coverage because emergencies can happen at any time and can put one in a financial hole. Get COBRA when you need it. If necessary, look for other private and federal options, including Medicaid and Medicare. You need to plan for an emergency before it happens; it can take 6 months to 1 year to plan for the next move.

Q: How long should the transitional process take?

A: Ideally, this will happen over a 2- to 4-year process. The worst time to get a new patient is in the ED. If they have been cared for by a pediatric specialist their whole life and then suddenly show up in an adult ED, we have to reinvent the wheel.

Research has shown that if you can conduct this process slowly and methodically over several years, each time giving the patient a little homework, that is most effective.

Sometimes we do the “fast track” approach, which is to have a nurse assess whether the patient has picked up the knowledge they need or still needs help. This will catch issues such as, “This female patient is not on birth control and has questions” or “This patient is bad about taking their medicine, so you have to talk about compliance.”

Q: How do you educate these patients on appropriate exercises and activities?

A: We try to provide this education throughout the entire process. These patients are young, energetic and often athletic. Some of the activities they do are safe, and some are not. The parents are usually good about trying to nail down exactly what their child can and cannot do.

For the most part, they can do most things. There are some specific diagnoses where they are not supposed to do activities such as weightlifting or playing contact sports such as football. Who can participate in long-distance running is outlined in the guidelines. However, the guidelines do have nuances.

We try to guide the patient on this at least through high school and college, where they have the most opportunities for organized sports and could be pushed by a coach. If you are a 20-year-old trying out for Division I athletics, that is a challenge we have to address. It’s different if you are 30 years old and going out for a jog or a bike ride on the weekend.

Q: Now that more people are living into adulthood with congenital heart disease, the question of whether they should have their own children has become more common. What sort of counseling is needed there?

A: For women, we address that topic early. It certainly has to be addressed by high school, when people become sexually active. This is a very sensitive issue not only for the patient, but for their parents, who may not be engaged with what their child is really doing socially, whether sexually or with high-risk behaviors such as drinking and doing drugs.

We try to address pregnancy right away. We counsel them on which birth control medications are safe and which are not. Some patients are not truthful about their sexual activity, so we will try to interview them without their parents in the room.

I usually do not prescribe birth control medications myself, but will work with my OB/GYN colleagues to educate them about which medications are safe or recommended for this patient.

Once a patient wants to get pregnant, family planning comes in to play. We need to determine when it is safe, if it should be done before or after a planned surgical procedure. If necessary, we will advise whether a fertility treatment is safe.

For men, they need to know what sexual function treatments are safe.

For both sexes, they need to know what the risk is for passing a heart condition along to their offspring. For a mother with congenital heart disease, the odds are about 8% to 10% that a baby will also have it. For a father, the odds are about 3% to 4%.

Q: What other considerations must be made during the transition process?

A: We need a good receiving primary care doctor. For many of these patients, their pediatrician is not involved with their cardiology care because it may be beyond their capabilities or outside their comfort zone. That can also be true with adult primary care providers. But I have always found it beneficial for these patients to have me as an adult congenital cardiologist, but also to have someone else for primary care because there will be primary care issues that are not cardiac, whether they be reflux, prostate health, diabetes, etc. More patients than I would like to admit see their cardiologist as their primary care doctor. I don’t do things like mammograms, Pap smears and colonoscopies, which a good primary care doctor can and should do. I am there for all the cardiac issues.

We are fortunate that a lot of people are surviving to adulthood with congenital heart disease. A lot of them are coming out of the major pediatric centers. We need to challenge the pediatric heart programs to build the infrastructure to successfully hand these patients off to an adult provider. After 18 years, we can’t just kick these patients out. We need to continue to provide them care for the rest of their lives. – by Erik Swain

For more information:

Wayne J. Franklin, MD, can be reached at Phoenix Children’s Hospital, 1919 E. Thomas Road, Phoenix, AZ 85016; Twitter: @drwaynefranklin.

Disclosure: Franklin reports no relevant financial disclosures.

Wayne J. Franklin
Wayne J. Franklin

The proportion of children with congenital heart disease who survive to adulthood continues to rise. With this good news, however, comes a challenge: The cardiac care of these patients must be managed throughout their lives, and at some point they must transition from being overseen by a pediatric cardiologist to being overseen by an adult congenital heart disease specialist.

With this transition comes the need for patients to become more knowledgeable about their condition, to take a more active role in seeking care and to better understand which behaviors and medications are safe for them.

Wayne J. Franklin, MD, co-director of the Phoenix Children’s Heart Center, director of the adult congenital heart disease program and chair of adult medicine at Phoenix Children’s, spoke to Cardiology Today about how best to transition children and adolescents with congenital heart disease into adult care.

Question: When should the transition begin from pediatric cardiology care to adult care?

Answer: The book answer is age 12. Functionally, however, I’ve found it more realistic to start around age 14 or 16. It is flexible because some teenagers are slow to mature, but others are mature enough to talk about adult heart-care topics. We all agree that it should definitely start before age 18, and certainly before age 21.

Q: What sort of knowledge should a patient have as the transition is made?

A: There are different levels of understanding. I pick three to five things that I make sure every patient should have for when they transition. There is a 20-point list of things that they should be able to do, but if I narrow it down to three or five, that is more feasible for a teenager or college student.

I do have a top five list of points, in no particular order.

No. 1, they should know their diagnosis and be able to explain it medically. For example, “I have D-transposition of the great arteries, atrial septal defect and heart block” or, more simplistically, “My arteries were switched and I have a hole in my heart.” Either way, they understand what their diagnosis is and can name it. That is half the battle when you are a doctor seeing a patient who has a long medical history for the first time.

No. 2, it helps them to know what was done. Most of these patients have undergone some sort of repair, either surgical, catheter-based, medical or otherwise.

No. 3, it is also helpful for them to know what medications they are on and why, as well as the dosages. This is true for any patient.

Those are the three most important ones, the next two are a “bonus.” No. 4 is to know how to make an appointment. The patient’s parents have directed their health care for their whole life, but sooner or later, they have to be responsible for their own care. It takes some skill, courage and initiative to pick up the phone, send an email or go to a website to make an appointment.

No. 5 is where to go for emergencies. Should they go to the pediatric hospital or the adult hospital? Should they call 911 or not?

PAGE BREAK

Q: What insurance considerations come into play when this transition is occurring?

A: In the United States, children may be on their parents’ health insurance plans until they turn 26. But I tell patients that they need to be very aware of getting good insurance, which means a plan that doctors and providers will accept and that covers most of their care. If a job offers you a lower salary but better benefits, consider taking the benefits, because you are going to meet your deductibles and spend all the health care dollars allocated to you each year. Try not to have any gaps in your insurance coverage because emergencies can happen at any time and can put one in a financial hole. Get COBRA when you need it. If necessary, look for other private and federal options, including Medicaid and Medicare. You need to plan for an emergency before it happens; it can take 6 months to 1 year to plan for the next move.

Q: How long should the transitional process take?

A: Ideally, this will happen over a 2- to 4-year process. The worst time to get a new patient is in the ED. If they have been cared for by a pediatric specialist their whole life and then suddenly show up in an adult ED, we have to reinvent the wheel.

Research has shown that if you can conduct this process slowly and methodically over several years, each time giving the patient a little homework, that is most effective.

Sometimes we do the “fast track” approach, which is to have a nurse assess whether the patient has picked up the knowledge they need or still needs help. This will catch issues such as, “This female patient is not on birth control and has questions” or “This patient is bad about taking their medicine, so you have to talk about compliance.”

Q: How do you educate these patients on appropriate exercises and activities?

A: We try to provide this education throughout the entire process. These patients are young, energetic and often athletic. Some of the activities they do are safe, and some are not. The parents are usually good about trying to nail down exactly what their child can and cannot do.

For the most part, they can do most things. There are some specific diagnoses where they are not supposed to do activities such as weightlifting or playing contact sports such as football. Who can participate in long-distance running is outlined in the guidelines. However, the guidelines do have nuances.

We try to guide the patient on this at least through high school and college, where they have the most opportunities for organized sports and could be pushed by a coach. If you are a 20-year-old trying out for Division I athletics, that is a challenge we have to address. It’s different if you are 30 years old and going out for a jog or a bike ride on the weekend.

PAGE BREAK

Q: Now that more people are living into adulthood with congenital heart disease, the question of whether they should have their own children has become more common. What sort of counseling is needed there?

A: For women, we address that topic early. It certainly has to be addressed by high school, when people become sexually active. This is a very sensitive issue not only for the patient, but for their parents, who may not be engaged with what their child is really doing socially, whether sexually or with high-risk behaviors such as drinking and doing drugs.

We try to address pregnancy right away. We counsel them on which birth control medications are safe and which are not. Some patients are not truthful about their sexual activity, so we will try to interview them without their parents in the room.

I usually do not prescribe birth control medications myself, but will work with my OB/GYN colleagues to educate them about which medications are safe or recommended for this patient.

Once a patient wants to get pregnant, family planning comes in to play. We need to determine when it is safe, if it should be done before or after a planned surgical procedure. If necessary, we will advise whether a fertility treatment is safe.

For men, they need to know what sexual function treatments are safe.

For both sexes, they need to know what the risk is for passing a heart condition along to their offspring. For a mother with congenital heart disease, the odds are about 8% to 10% that a baby will also have it. For a father, the odds are about 3% to 4%.

PAGE BREAK

Q: What other considerations must be made during the transition process?

A: We need a good receiving primary care doctor. For many of these patients, their pediatrician is not involved with their cardiology care because it may be beyond their capabilities or outside their comfort zone. That can also be true with adult primary care providers. But I have always found it beneficial for these patients to have me as an adult congenital cardiologist, but also to have someone else for primary care because there will be primary care issues that are not cardiac, whether they be reflux, prostate health, diabetes, etc. More patients than I would like to admit see their cardiologist as their primary care doctor. I don’t do things like mammograms, Pap smears and colonoscopies, which a good primary care doctor can and should do. I am there for all the cardiac issues.

We are fortunate that a lot of people are surviving to adulthood with congenital heart disease. A lot of them are coming out of the major pediatric centers. We need to challenge the pediatric heart programs to build the infrastructure to successfully hand these patients off to an adult provider. After 18 years, we can’t just kick these patients out. We need to continue to provide them care for the rest of their lives. – by Erik Swain

For more information:

Wayne J. Franklin, MD, can be reached at Phoenix Children’s Hospital, 1919 E. Thomas Road, Phoenix, AZ 85016; Twitter: @drwaynefranklin.

Disclosure: Franklin reports no relevant financial disclosures.