New Diagnosis – Pulse Oximetry

This week we will hear two perspectives on the benefits of pulse ox and the effort to make this non-invasive test a standard screening of newborns. Pulse Ox screening has made it possible to detect CHD in many newborns that would have otherwise gone home undiagnosed. This week’s contributions were provided by Dr. Gerard Martin, a pediatric cardiologist, and Ms. Lisa Wandler, a pediatric nurse, from the  Children’s National Heart Institute and  Dr. Matt Oster, MD, MPH , a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta.

 

 

Dr. Gerard Martin, a pediatric cardiologist, and Ms. Lisa Wandler, a pediatric nurse, from the  Children’s National Heart Institute discuss with us how pulse oximetry screening works , as well as its benefits and limitations. 

Screening infants for Critical Congenital Heart Disease (CCHD) using pulse oximetry is recommended in the United States, but this was not always the case!  As of this summer, all 50 states and the District of Columbia will be screening for CCHD.  In many countries, this important life-saving screen is not yet standard for every newborn.  

How CCHD pulse oximetry screening works:

CCHD screening is simple, painless and takes only a few minutes to perform.  It typically takes place around 24 hours after birth either in the newborn nursery or in the mother’s room.  A sticker with a special light probe is placed on the baby’s right hand and either foot.  The measurement that the light probe takes helps the medical team determine whether the baby may have CCHD and require further assessment and testing.

How it has changed standard screening for newborns:

Prior to the implementation of CCHD screening, as many as 50% of infants with CCHD were being discharged from the hospital without anyone knowing of their heart problem.  Without CCHD screening using pulse oximetry, routine newborn screening could help identify hearing and other rare but serious conditions in babies just after birth but not heart defects.  

Benefits/importance of screening:

An undetected heart problem in a baby can lead to severe health problems for the baby and even death.  This newborn screen has helped to decrease the number of babies lost to undiagnosed heart defects and saved many lives.  The CDC continues to investigate the impact CCHD screening has had in the U.S., but an early estimate is that the number of deaths due to CCHD has gone down by 33% in states requiring this important newborn screen.

Limitations of screening using pulse oximetry:

Screening improves the detection of CCHD, however, not all types of CCHD are able to be detected using this screening method.  It remains important to follow the instructions of a baby’s pediatrician and other doctors as newborn assessment and pre-natal ultrasound remain important other ways CCHD can be identified.

Early symptoms of CCHD can include rapid breathing, difficulty feeding and bluish skin.  If your baby has these symptoms, tell the baby’s doctor.

 

The Critical Congenital Heart Disease Screening Program at Children’s National Heart Institute is composed of Dr. Gerard Martin, a pediatric cardiologist and Ms. Lisa Wandler, a pediatric nurse.  The team at Children’s National has worked on implementation, education and CCHD screening advocacy for over ten years and has provided guidance at the local, state and international levels to those interested in CCHD screening using pulse oximetry.  The team can be contacted at pulseox@cnmc.org

Gerard Martin headshot, cardiologist, children’s national heart institute

 

 

 

Dr. Matt Oster, MD, MPH  discusses the progress brought by pulse ox and the distance yet to go. 

 

In my medical career, I’ve been able to see what was once just a promise or an idea be transformed into reality – the ability to screen well-appearing newborns for critical congenital heart disease. While a baby may appear completely well by anyone who sees her, lurking beneath the surface could be subtle hypoxemia – an abnormally low concentration of oxygen in the blood.- secondary to a congenital heart defect. The application of pulse oximetry to detect such hypoxemia has thus allowed clinicians to detect many previously undiagnosed cases sooner, a change
that has led to decreased infant mortality from critical congenital heart disease.

This dramatic change in the care of newborns did not come easily. It took scientists studying the issue to determine whether this could work. It took policy makers the vision to implement this change in their regions. And, most importantly, it took the tireless advocacy of parents and others to call for, and when needed essentially demand, such change.

The application of pulse oximetry to screen for critical congenital heart disease is a true public health success story. And, it has even seen spillover effects in that many children with hypoxemia due to causes other than heart disease are being detected and treated. However, there is still much work to be done. We need to figure out the best way to implement this screening in special settings such as the neonatal intensive care units, home births, or areas of high altitude. We need to improve the quality of the program so that it is implemented consistently and correctly for all newborn. We need to help public health agencies monitor and track the success of this program.

But the biggest change we need to make is figure out a way to increase the sensitivity of the screening. Yes, the program has detected thousands of babies that may have previously gone undetected. But there are still many newborns with critical congenital heart disease that are being missed, even newborns with hypoplastic left heart syndrome. This is typically due to the fact that hypoxemia may not yet be present. We need improved diagnosed methods beyond pulse oximetry to help detect these children. This is not an easy task, but scientists are indeed working on it. And when it’s ready, we’ll need the help of advocates and policymakers to make it a reality.

 

Dr. Matt Oster, MD, MPH is a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta, and he holds Emory appointments of Associate Professor of Pediatrics in the School of Medicine and Associate Professor of Epidemiology in the School of Public Health. He earned his MD at the University of Pennsylvania School of Medicine and his MPH in epidemiology at Emory University Rollins School of Public Health. After completing residency training in pediatrics at the University of California-San Francisco, he did fellowship training in pediatric cardiology at Emory University. When not seeing patients, he serves as director of the Children’s Cardiac Outcomes Research Program at Sibley Heart Center. His research interests include newborn screening for congenital heart disease, the epidemiology of congenital heart disease, and long-term outcomes for patients with congenital heart disease.

 

 

 

New Diagnosis – Fetal Echo

More often now, than a generation ago, babies born with CHD are being diagnosed prenatally. This week, Dr. Sheetal Patel, from Lurie Children’s Hospital of Chicago and Assistant Professor of Pediatrics, Northwestern University Feinberg School of Medicine, explains the role prenatal echocardiography plays in diagnosing CHD and the benefit of that early diagnosis.   

 

 

Congenital Heart defects (CHDs) are among the most common birth defects, affecting 1 out of every 110 babies born. Each year nearly 40,000 babies are born in the United States with CHD, ranging from simple lesions that may not need any interventions to complex CHDs that can be fatal if appropriate treatment is not provided soon after birth. Research shows that prenatal diagnosis and early detection of these complex CHDs is associated with improved surgical outcomes. With improvement in diagnostic technology with Fetal echocardiogram today, about 70% of complex CHDs are detected prenatally. Goals for detection are targeted at 100%, and we are aiming to reach there with improved awareness and better screening techniques.

A fetal echocardiogram is an ultrasound test performed during pregnancy to evaluate the heart of the unborn child and can be performed as early as 18 weeks gestation. Diagnostic accuracy for detecting complex CHD with a fetal echocardiogram is as high as 95%. It is a non-invasive procedure performed with an ultrasound probe placed over mother’s belly. Generally, the pain or discomfort that results from the probe pressure on the mother is minimal. It involves detailed evaluation of baby’s cardiac structures including cardiac chambers, valves and major blood vessels. It also evaluates fetal heart rate and rhythm. This test can detect CHDs such as missing heart chambers (such as hypoplastic left heart syndrome, hypoplastic right heart syndrome, and many other variations), abnormal great arteries (such as transposition of the great arteries, truncus arteriosus, interrupted aortic arch, etc), abnormal cardiac valves (such as atrioventricular septal defect, pulmonary valve atresia), or large hole between cardiac chambers (such as a large ventricular septal defect). There are limitations of fetal echocardiography that it may not detect minor cardiac valve abnormalities, small holes between cardiac chambers or coarctation of aorta that develops after birth.

Early detection of CHD before baby’s birth has many advantages.

Prenatal diagnosis of CHD allows for necessary preparation to provide highly specialized care that the baby will require soon after the birth and prevents the hemodynamic compromise that can result if this CHD was undetected. This preparation involves coordinated care by multiple teams with expertise in pediatric cardiology, neonatology, pediatric cardiac intensive care and pediatric cardiovascular surgery. In addition, social worker, child life specialists, and palliative care teams may be available to help parents cope with the diagnosis and treatment. An important aspect of early detection is to provide expectant parents the opportunity to have detailed counselling.  This counselling helps parents to better understand their unborn child’s heart condition and interventions that might be needed.  This aids parents in their research to choose a center of excellence for their baby’s care. The goal of this prenatal counselling is for parents to be armed with knowledge, process the information over time, and maximize the family’s preparedness for the journey and transition to a birth of their new baby. Research shows that those mothers who knew about their baby’s heart condition prior to the birth were less anxious once the baby was born as compared to mothers who found out about the defect after baby was born.

There are some standard indications for fetal echocardiography during pregnancy.

Not every expectant mother needs to have a fetal echocardiogram. However, if the risk of having CHD in the unborn child is expected to be higher than general populations, a fetal echocardiogram is indicated. These risk factors include having a prior child with congenital heart defects, maternal diabetes, maternal infections during pregnancy known to affect baby’s heart, etc. Mother should discuss with her obstetrician if a fetal echocardiogram is indicated based on the family history and her own medical history. If indicated, a fetal echocardiogram should be arranged to be performed between 20 to 24 weeks gestation which is an ideal time for accurate diagnosis of CHD. Other indications for fetal echocardiogram include abnormal findings on obstetrical screening test such as increased nuchal thickness, abnormal cardiac images during the level II anatomy scan, chromosomal abnormalities (such as trisomy 21, trisomy 18, trisomy 13, Turner syndrome, etc) detected during the prenatal genetic testing, or other organ malformations noted during the anatomy scan. These abnormal screening tests indicate higher risk of CHD in the fetus and therefore, a fetal echocardiogram is indicated.

What happens after a fetal echocardiogram detects CHD in fetus?

A pediatric cardiologist performing the fetal echocardiogram will discuss the findings of CHD in details with the expectant parents. Tailoring the counselling to the parent’s needs over time is critical as parents can be very overwhelmed during the initial hearing of a diagnosis of CHD. Counselling should include discussion about implications of this CHD on baby during the pregnancy, what support and care this baby would need soon after the birth, what interventions, procedures and surgeries would be necessary during neonatal period, and what other procedures or surgeries would be needed later in life and what is expected overall prognosis with this CHD. The goal of this counselling process is to provide information to parents that would help them with their decision making to choose their options. The options are described in details that include preparing them for this journey to have child with CHD, palliative care or other family planning options.

Each Fetal Cardiac Program have unique set up to provide this detailed counselling. At Lurie Children’s Hospital; these services are provided through our fetal cardiac program at The Chicago Institute for Fetal Health. Following the initial consultation, parents have a “Comprehensive Fetal Cardiac Consultation” which includes a follow up fetal echocardiogram to assess the evolution of the CHD (if any), consultation with a fetal cardiology team composed of a pediatric cardiologist, neonatologist, cardiac intensivist, cardiovascular surgeon, social work, and other specific team pertinent to the diagnosis. For example, a consultation for prenatal diagnosis of HLHS would include a specialist for “Single ventricle Program”. Parents may also choose to meet with “cardiac neurodevelopment team” and “Child life Specialist” if they are interested in learning more about these important aspects of their child’s quality of life in future.  The number of partners at the table during this meeting can seem overwhelming to some parents and modifications are made to this process to meet each family’s needs.  

Our goal as a comprehensive fetal cardiology team is to arm families with information, answer questions, form a united care team to provide cohesive CHD care pre and post-natally, and optimize the chances for the most successful outcome and quality of life throughout a lifetime.  

 

References:

Fetal Echocardiogram: https://www.luriechildrens.org/en-us/care-services/specialties-services/medical-imaging-radiology/diagnosis-services/heart-evaluation-testing/Pages/fetal-echocardiograms.aspx)

CDC: https://www.cdc.gov/ncbddd/heartdefects/data.html

 

Dr. Sheetal Patel is Associate Director of Fetal Cardiac Program at Ann & Robert H Lurie Children’s Hospital of Chicago and Assistant Professor of Pediatrics at Northwestern University Feinberg School of
Medicine. Her clinical interest lies in fetal, neonatal and pediatric cardiology. She is passionate about prenatal diagnosis of congenital heart defects. Her research interests are in evaluating outcomes in
congenital heart defects, with special focus on single ventricle heart defect and Fontan palliation.

New Diagnosis – Prenatal Conquering CHD Kits

This week, our State Chapter Coordinator, Melanie Toth, shares her experience with us as  a new mom finding out her son would be born with a congenital heart defect and how PCHA helps to make sure other families don’t have to go through what Melanie’s did alone.

 

Like many heart families, when diagnosed with their child’s heart defect, your world is flipped upside down. It was no different for our family. I remember us walking out of the room not really understanding or knowing what just happened. We had only walked into the doctors office for our 20 week ultrasound, and left finding out our child would be born with a heart defect, that I couldn’t even pronounce. While I have always had a love and hate relationship with congenital heart disease, over the past decade I have learned a lot. I can’t change the diagnosis we were given that day, but I did wish I could change the information that our family was given when we left the doctors office.

We weren’t aware that CHD was the #1 birth defect or that too many families walked in similar shoes as us. We left feeling hopeless and alone, with a paper that said, “Tetralogy of Fallot”. My husband and I went online to get more information on our son’s diagnosis and, to say the least, were completely overwhelmed. There was different information on various websites, and we just felt more hopeless.

If 10 years ago our doctors would have handed us a PCHA Prenatal Conquering CHD Kit, life would have been just a little easier. From resource cards to navigate important CHD information, to guided questions on what to ask your medical team, and most importantly, how to connect with other heart families, the prenatal kit is like a life raft for new families. Through PCHA State Chapters and working with hospitals, prenatal kits have offered the much needed information and personal connection that every heart family deserves. If 10 years ago our family was given a prenatal kit, instead of walking out with our sons defect written down, our family would not have felt so alone in our heart journey, during a difficult first year with our son’s surgeries.

I feel honored and blessed to help our PCHA State Chapters as National State Chapter Coordinator. Personally helping heart families and helping set up chapters to empower families, is the best pay it forward our family can offer.

 

Melanie’s heart journey began in June 2008, during a routine 20 week ultrasound. She and husband were devastated by the news that their unborn son Luke would be born with a congenital heart defect (Tetralogy of Fallot) and required heart surgery at a week old and again at 9 months old. Feeling very scared and alone during the roller coaster ride of a CHD journey, Melanie decided that no other heart family should feel alone. In 2010, she has started a support group for heart families in Chicago. Working nationally and locally with various CHD organizations. In 2016, she began volunteering with PCHA’s new state chapters, to offer families more than just support. She is currently the State Chapter Coordinator for PCHA National helping to develop state chapters.

 

Recap – Arrhythmias and Cardiac Devices

Complications from surgery, scar tissue left behind, and an underlying disease can bring about arrhythmias in CHD patients and lead to need for a cardiac device. Gathered here are the contributions and resources from PCHA’s Arrhythmia Blog Series. 

 

Jarvik: For Smaller Hearts

PCHA’s next series introduces the various Cardiac Devices involved in the treatment of Congenital Heart Disease and the associated conditions. In the first post, Dr. Adachi tells us about the Jarvik for small hearts, a ventricular assist device used to help pump blood through the body.

 

Terri’s Story

Often times, CHD patients face issues with the rhythm of their hearts. In some patients, this can mean additional treatment is necessary, with either medication or a cardiac device. In this week’s post, Terri Elliott, an adult CHD patient, shares her experience with an arrhythmia that led to her receiving an implantable cardioverter defibrillator. or ICD.

 

The Elephant in the Room

With each CHD, there is no certain path, no one course looks like the next, and complications vary widely. Though a patient may have a specific structural diagnosis, different arrhythmias may develop over time, however patients and families are not always made aware of this possibility immediately. That’s why the diagnosis of an arrhythmia may surprise parents and send them for a loop, just when they think they’ve seen it all.  This also makes it difficult for parents to know just how much to share with teacher and other adults in their children’s lives, just as Alison Connors shares with us this week.

 

Bradycardia Explained (Part One)

This week former Cardiac Nurse, Carol Raimondi, provides us with the first of a two-part resource on arrhythmia and the various diagnosis and treatment. Up first: Bradycardia.

 

Tachycardia Explained (Part Two)

Wellness – CHD and Exercise

With the brand new year, many of us are making resolutions to better ourselves and our lives. Exercising more is often at the top of the list! This week, Kathleen Baschen, an exercise physiologist at Ann & Robert  H. Lurie Children’s Hospital of Chicago, shares tips on how patients with CHD can get FITT. 

 

 

Your cardiologist suggests that you start exercising regularly, but where do you start? What type of exercise can an adult with congenital heart disease even do? You can do much more than you think! The key is to start small and build it up gradually as your body adapts.

 

We all have heard the benefits from regular exercise; how it improves lean mass in the body, lowers body fat and reduces stress.  But how can it help adults with CHD specifically? It can help decrease shortness of breath and fatigue in day to day tasks, it lowers blood pressure and heart rate and has been shown to reduce hospitalization stays (and duration of stays).  The benefits of exercise span from your head to your toes, with having positive effects for most systems in the body.  It sounds like a no-brainer, but developing and executing an exercise plan can be hard.  Here are some areas that will help you become more FITT, focusing specially on how to incorporate more aerobic, resistance and flexibility exercises into your weekly regiment.

 

Frequency: How often should I exercise? For a beginner, it is recommended to participate in aerobic exercise 3 days out of the week, resistance exercise 1-2 days out of the week and flexibility at least 2 days out of the week.  These can be spread out over the week or can be combined into 4-5 days to allow yourself breaks through the week.  For example, you could complete aerobic exercise and flexibility on the same day, or resistance and flexibility on the same day.  For those who have busy schedules, it can be more time efficient to complete two types of exercise in one day.

 

Intensity: How hard should I be exercising? For patients with CHD, using heart rate during exercise can be inaccurate due to medications or pacemakers.  For these patients, it is recommended to use the Borg Scale (see chart below) for aerobic activity.  During your warm up and cool down stages, you should be working from 8-10 on the scale (mild intensity). During the bulk of your aerobic exercise, the intensity should increase to 11-14 (moderate intensity). For resistance training, beginners can start with body weight or light hand weights. Increase your resistance every 2-3 weeks to progress strength. When stretching or performing other flexibility exercises, be safe and stretch to a point of feeling a tightness.

https://www.cdc.gov/physicalactivity/basics/measuring/exertion.htm

 

Time: How long should I exercise for? The ultimate goal is to exercise continuously for 30 minutes. When starting this program, start with any aerobic activity for 10-15 minutes and gradually increase your time over the course of 4-6 weeks.  Take breaks when necessary or complete aerobic exercises in an interval format to allow for periods of rest.  For resistance training, complete exercises in 10-15 repetitions and 2-3 sets.  If you exceed 15 repetitions, it’s time to increase your intensity! When performing flexibility exercises, hold stretches for 10-30 seconds and repeat stretches 2-4 times.

 

Type: What kinds of exercise should I be doing? Whatever you like to do! Aerobic exercises include running, walking, swimming, elliptical, biking, stair master, aerobic classes, Zumba and many more! Resistance training should consist of major muscle groups (upper body, lower body and core). There are countless exercises that you can do right at home with little or no equipment. Flexibility exercise can be anything from traditional static stretching to yoga.

 

Use this FITT principle when you are ready to start your exercise program and make time in your week to complete it.  Set a goal and stick to it. Find what you enjoy and do it! Remember to be safe when exercising. Check with your physician prior to starting an exercise plan, and if you ever experience symptoms while exercising, stop immediately or contact your physician if they persist.

 

Kathleen Baschen received her MS from Benedictine University. She is currently an Exercise Physiologist at Ann & Robert H. Lurie Children’s Hospital of Chicago, and her  focus is in cardiopulmonary diagnostic testing, pulmonary rehabilitation and cardiac rehabilitation.

The Promise of Research for CHD, and Our Responsibility to Advocate

Advancement in standards of care and best practices can only happen with research.  This week, Margaret King discusses the implications increased CHD research has on not only the community but society as a whole, as well as how important it is for each one of us to contact our representatives to increase research funding. 

 

 

The Promise of Research for CHD, and Our Responsibility to Advocate

 

This past month, I and a group of several other local heart families were treated to an astonishing behind-the-scenes tour of the Mitchells’ research lab at Children’s Hospital of Wisconsin, where we learned about the vital work they are doing to identify the genetic processes, risks, and factors in CHD. Just a few weeks prior, I also had the opportunity to attend Mayo Clinic’s Feel the Beat event, where the HLHS Program shares research updates with heart families. Both of these research programs are committed to improving treatment of CHDs across the lifespan, with the further goal of exploring targeted treatments based on individual risk factors.

 

Many leading pediatric cardiology centers are working tirelessly behind the scenes to make game-changing breakthroughs for current and future CHD patients. From stem cells to genetics, new medical devices and drug therapies, and of course, developing best practices for everyday care and management, research underlies almost all aspects of CHD care. It has enormous implications for the quality of life and outcomes CHD patients will experience.

 

As members of the CHD community, we can advocate for lifesaving research funding to our representatives, as well as urge our friends, families, neighbors, and colleagues to do the same.

 

Research Breakthroughs: A ripple effect

 

With 1 in 100 babies being born with a heart defect, there is an urgent need for research breakthroughs in preventing and optimally treating CHD of all kinds. However, studying individual types of CHDs can have tremendous implications that extend far beyond CHDs themselves. For example, understanding the possible cascade of genetic events that causes hypoplastic left heart syndrome (HLHS) sheds light on the broader process of cascades that cause a number of complex diseases, many of which have stumped researchers for decades.

 

If we can offer anything from targeted stem cell therapy to genetically-tailored drugs for one disease, it is just a matter of time until these technologies can be used to treat a wider and wider range of illnesses.

 

These cutting-edge research discoveries have great potential to alleviate suffering, not just in the CHD community, but across entire our society. They offer hope that we truly can “conquer CHD” and many other conditions that have proved to be extremely challenging to treat and manage using the life-saving advancements of the twentieth century. While the breakthroughs of the past were revolutionary, we now know that we can do so much more if we put resources into the proper channels.

 

Advocating for Research: Our responsibility

 

Researchers and doctors cannot shoulder the burden of advocating for research alone. The more we help advocate, the more time they can spend on research and collaboration. As it is, researchers often spend a lot of time identifying avenues of funding and writing grant applications for scarce funds, which takes valuable time from their work in the lab.

 

Many of us understand the importance of advocating to our political representatives, but do we talk to our friends, families, neighbors, and colleagues about how important it is to fund public research through institutions like the National Institute of Health (NIH)?

 

The NIH is the largest source of biomedical research funding in the entire world, but the process is fiercely competitive, with less than 20% of applications being approved at any level of funding. The number of projects the NIH can fund, as well as what level of funding projects receive, fluctuates with the national budget.

 

Advocating not only for increased funding, but stability in the NIH budget from year to year, is of utmost importance to make sure the lights stay on in some of our most promising, dedicated labs. After all, when the lights stay on at the lab, researchers can shed light on life-and-death health problems that affect many of us personally, and all of us as a society.

 

Research takes an enormous amount of time, especially when dealing with pediatric populations and small pools of patients. Simply gaining approval for a clinical study is a complicated process, because researchers have to demonstrate their studies will not cause foreseeable harm to their subjects. With today’s advanced technologies, the studies we need in key areas like genetics and stem cell research are expensive, and can even face ethical and political hurdles. Many of them have several phases, each taking years to complete.

 

When scientific and medical studies of repute are finally completed, they must undergo peer review to withstand scrutiny from professional colleagues in their field. After that, usually further studies are needed, and even the most promising results need to be duplicated elsewhere before becoming mainstream practice. Each promising finding is simply a building block for further findings, hopefully leading to an eventual “big picture.”

 

Sharing Research with CHD Families: An institutional necessity

 

Touring the lab at Children’s Hospital of Wisconsin, and seeing firsthand how dedicated the researchers there are to helping CHD patients gave me new hope as a heart parent, as well as an inspiration to keep advocating for all the lives that will be touched by CHD. It gave me hope that there is either a cure or a radical shift in how we understand and treat CHD on the horizon.

 

Many heart parents rely on social media posts from other parents in order to learn about important research findings and the results of the latest studies. Many of us only hear about this vital work after the fact, and have little means of learning about the latest, cutting-edge discussions and studies that are happening at our own centers.

 

I commend Mayo Clinic’s Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) for their dedication to sharing their current research with families. Their blog and Facebook posts, as well, as their annual Feel the Beat gathering, which includes a science fair and demonstrations of their current projects for all ages, is refreshingly accessible. The biennial Heart Parent Education Day at the Medical College of Wisconsin/Herma Heart Institute also strives to inform heart parents about standards of care in pediatric cardiology, as well as their latest programs for patients.

 

I urge all pediatric cardiology institutions to get the word out about the great work they are doing–whether through a newsletter, blog, social media page, or in-person events–to the CHD community. Making in-person events family-friendly helps heart parents attend these events without the stress and expense of finding childcare. When heart parents are empowered not only with knowledge, but also the hope of such inspiring research, they are even more motivated to spread the word to their social and advocacy circles–which is a win/win for everyone.

 

*** Update***

Upon reading this article, one of our legislative champions reached out to us to share that this piece  “really helps underscore the Bilirakis-Schiff CHD approps letter for FY18 (attached)!  Give yourselves (and our CHD friends in Congress) some credit. These lawmakers are after all, #CHDWise 😉

Bilirakis_Schiff_FY18CHD

This reminds us of the work our legislators are doing because of our advocacy efforts. Advocacy works!!!!

We’d like to thank the lawmakers who are already supporting increased CHD research funding, as highlighted in the letter, but we can’t stop!  We need to develop more legislative champions!! You can help by contacting your reps and letting them know you want them to support increased research funding. 

To learn more about CHD related legislation and how to contact Members of Congress from your state, check out PCHA’s Post on the Congenital Heart Futures Act

 

 

 

Margaret King is the mom to the 9- year old mighty K-man, a spirited boy with half a heart who is determined to live fully, and is married to the awesome heart dad, Shawn. A content marketer and writer in Wisconsin, her other interests include hiking and being outdoors, reading, and avoiding going down the thrill water slides her son is passionate about. She hopes to have a small goat farm someday

Recap – Patient Engagement Tools

Take a look back at our Patient Engagement Tool posts.

 

The Care Partnership Pyramid

It is often difficult for parents and loved ones to know what to do in caring for their child during hospital stays, and it can often be a difficult topic to discuss. Christine Martinenza, RN, has implemented this month’s Patient Engagement Tool, The Care Partnership Pyramid, at Nemours/Alfred I. duPont Hospital for Children, which aims to help foster conversation and understanding in how to best allow for parents and medical staff to work as a team.

 

Transition Top 10 to Remember

As the seasons change, new milestones come and go.  This can be an especially trying time in the life of an adolescent with congenital heart disease, especially as they are undergoing the major transition of leaving the nest and going off to college, joining the workforce, or just moving far from home.  Dr. Aaron Kay, Director of the Adult Congenital Heart Disease Program at Indiana University Health, has the following Top Ten list to help ease the transition.

 

 

Comprehensive Single Ventricle Road Map

When a family learns their child will be born with a Single Ventricle CHD, they are thrust into a world of uncertainty. It is sure to be a daunting and overwhelming experience. The plan for care of these patients has not typically been clear. As outcomes have improved, providers have been able to imrove their plans of action. In PCHA’s first Patient Engagement Tools Series post, Michelle Steltzer, Nurse Practitioner from Lurie Children’s Chicago, shares their Single Ventricle Roadmap.

 

Patient Engagement Tool – The Care Partnership Pyramid

It is often difficult for parents and loved ones to know what to do in caring for their child during hospital stays, and it can often be a difficult topic to discuss. Christine Martinenza, RN, and Janie Klug, MSN, have implemented this month’s Patient Engagement Tool, The Care Partnership Pyramid, at Nemours/Alfred I. duPont Hospital for Children, which aims to help foster conversation and understanding in how to best allow for parents and medical staff to work as a team.

 

Many infants born with congenital heart disease (CHD) are transferred to the Cardiac Intensive Care Unit (CICU) immediately after birth or diagnosis for stabilization. As the mothers are still recovering from the delivery, many babies will undergo their first of many surgeries. Following surgery, these newborns are at risk for life threatening complications, require minimal stimulation, and around the clock medical care. It is very common for the medical team to ask families to remain hands off during this critical stage. The period of time spent in this phase of care is variable and for some can be several days to weeks. As the baby heals, the medical team is able to lessen sedation, remove lines and tubes, and gradually increase the amount of stimulation. This process of de-intensifying support can be lengthy and sometimes unpredictable leading many families to describe it as a roller coaster of emotions. Continue reading

Teen Topics – The Top 10 Things to Remember

As the seasons change, new milestones come and go.  This can be an especially trying time in the life of an adolescent with congenital heart disease, especially as they are undergoing the major transition of leaving the nest and going off to college, joining the workforce, or just moving far from home.  Dr. Aaron Kay, Director of the Adult Congenital Heart Disease Program at Indiana University Health, has the following Top Ten list to help ease the transition and cap off, for now, PCHA’s Teen Topic Series.

TOP 10 THINGS TO REMEMBER

  1. Congenital heart disease requires lifelong follow-up
  2. Where operations were completed
  3. How to find a copy of all operative reports as available
  4. The current list of medications, especially blood thinners and rhythm drugs
  5. If they have a pacemaker or not, and a wallet card
  6. Whether they need antibiotics before dentist, tattoos, piercings and similar procedures
  7. What restrictions their heart disease places on activities they can do, and how that can affect jobs, etc
  8. Need to maintain health insurance whenever possible
  9. How to find a new doctor that specializes in their disease if they have to move
  10. Who to call if they have any questions or concerns

 

 

Congenital heart disease requires lifelong follow-up

Many patients with CHD feel well at this point in their life, and in many cases, without significant symptoms, they may not see the need to see a doctor.  There is a common misconception that CHD can be “cured” (which is possible in a very small minority of lesions).  Some conditions require follow-up more frequently than others.  The “transition” period is an especially risky time for teens to become “lost to follow-up” as they may not necessarily know how to make their own appointments, and may not know how to find a new doctor.

Please discuss with your CHD provider who they should be seeing as they become adults, and if your child has not seen a CHD provider in the last 12 months, now would be a great time for a quick visit to make sure your teenager has a better understanding of their specific problem and how frequently they need follow-up.

 

Where operations were completed / How to find a copy of all operative reports as available

Ideally, your family would already have a copy of all the operative notes from all surgeries, catheter procedures, pacemaker implantations, and electrical studies of the heart.  A great deal of time and unnecessary testing can be saved if you have a copy of this information. Knowing the exact history of what was done, who it was done by, where, and when can really help a new CHD physician rapidly identify the best strategies for follow-up for your child.

 

For example, it is very helpful to know what kind of artificial valve, as well as what size of valve, was implanted. It can help a CHD physician understand how long to expect the valve to last without replacement, as well as the likelihood of being able to replace it in the cath lab, versus being able to follow by just echocardiography or cardiac MRI imaging.

 

 


The current list of medications, especially blood thinners and rhythm drugs

If your child is on Coumadin to prevent a blood clot, this is incredibly important information.Likewise, rhythm control drugs are important to know about. Many of these drugs will have interactions with other new drugs that are commonly prescribed, for example, antibiotics.  In some cases it may be important to consider getting a medical bracelet for your loved one in case of emergency.

 

 

If they have a pacemaker or not, and a wallet card

A wallet card identifying the manufacturer of a pacemaker or defibrillator is very important, especially in case of emergency.  You don’t want to think about bad things happening to your loved one, but if they suffer an ICD shock and are temporarily unconscious and unable to speak for themselves, emergency personnel with access to this information will be much better able to care for your child.

Additionally, as patients move, they often will need to have one cardiologist for the “plumbing” and another for the “electrical work.”  It can save a lot of frustration to know ahead of time that a patient requires appointments with both types of cardiologists, and at most centers we can make arrangements for your loved one to see both types of cardiologists at a routine office appointment on the same day

 

 

Whether they need antibiotics before dentist, tattoos, piercings and similar procedures

Although endocarditis is rare, the consequences can be very severe. Guidelines changed about 10 years ago, but overall whether or not a patient needs endocarditis prophylaxis is a matter of clinical judgment. I advise you to ask your physician or other provider about this.

 

 

 

 

What restrictions, if any, their heart disease places on what type of activities they can do, and how that can affect jobs, etc

 

If your child is not allowed to lift heavy weights due to their condition, they should be steered toward a job that does not require heavy physical activity.  For the most part, pediatric cardiologists already do an excellent job with this type of counseling.

 

 

 

Need to maintain health insurance whenever possible

Fortunately, at least for the time being, CHD patients can remain on their parents’ medical insurance until they are 26 years of age.  This policy gives ample time for most patients to have a plan to have insurance for themselves, although there are exceptions.

Without insurance, many preventable things can’t be appropriately prevented.  Virtually no one can afford open-heart surgery, catheter procedures, or other major heart procedures out-of-pocket.  If your family does not have private insurance, please ask your care team for resources to learn how to find alternative insurance through Medicaid or other similar State programs.

 

How to find a new doctor that specializes in their disease if they have to move

A great resource on the web is the Adult Congenital Heart Association clinic directory, which can be found here.  This directory can be searched by each state and virtually every large city currently has a program listed on the website.  Contact information to help you make an appointment is available in this directory.

You should also discuss with your CHD care team which doctor they would recommend.  For some very complicated types of congenital heart disease, it may be important to determine where to move based on the availability of appropriate ACHD resources.  Of course, in many cases, patients may still continue to “go home” during summer and college breaks and continue to see their original cardiologists.

I would recommend that if a young adult with CHD lives more than a few hours away from their hometown that they should have at least a one-time “checking in” appointment at a local hospital or clinic, so that they can have their chart on file in the electronic medical record of the nearby hospital, mainly in case of emergency. Also have a plan in place for where to be transferred in case of a serious medical emergency.

 

Who to call if they have any questions or concerns

This may be the most important issue.  The default option in case of emergency is the emergency department; however in many cases, a timely call to the right person can save an unnecessary trip as well as a lot of frustration. You should discuss with your current CHD care team who to call in the future, and when.  Many routine and unexpected questions are going to occur throughout your child’s life, so knowing who to consider a “medical home” is quite valuable.

 

 

 

 

Dr. Kay is the Director of the Adult Congenital Heart Disease Program at Indiana University/Indiana University Health.  He is trained in both Adult Cardiovascular Medicine and Pediatric Cardiology.  He is originally from Huntington, Indiana and has degrees in Spanish and Engineering from Purdue University.  He completed training in Internal Medicine and Pediatrics at The University of Cincinnati/Cincinnati Children’s Hospital, and completed a dual fellowship in Adult Cardiology and Pediatric Cardiology at Nationwide Children’s Hospital and The Ohio State University.

 

Teen Topics – Q&A: Your Questions Answered

Back in September, patients and families submitted the questions they most wanted answered. We caught up with members of PCHA’s Medical Advisory Board, at last month’s Transparency Summit, to ask those questions. Check out the videos below, featuring Dr. Marino, Dr. Madsen, Dr. Gurvitz, and Dr. Sood’s answers on teen and young adult topics.

 

 

Dr. Brad Marino – Transition, Staying in Care, Protecting Your Health

Dr. Marino is an attending cardiac intensivist at Lurie Children’s Hospital. He is a Professor of Pediatrics and Medical Social Sciences at the Feinberg School of Medicine at Northwestern University, also serving as the Director of the Center for Cardiovascular Innovation in the Stanley Manne Children’s Research Institute.

 

 

 

 

 

Dr. Nicolas Madsen – Activity, Drugs & Alcohol, Related Conditions, Staying in Care

Nicolas L. Madsen, MD, MPH joined the Heart Institute at Cincinnati Children’s Hospital Medical Center in July 2012. He is the Vice Chair of PCHA’s Medical Advisory Board.

 

 

 

 

 

 

Dr. Michelle Gurvitz –  College, Tattoos, Contraception, and Transition.

Dr. Gurvitz is an assistant professor of pediatrics at Harvard Medical School and a staff cardiologist with the Boston Adult Congenital Heart program at Children’s Hospital Boston and Brigham and Women’s Hospital.

 

 

 

 

 

 

 

Dr. Erica Sood – Behavioral Health Topics/School Resources

 

Dr. Sood is a pediatric psychologist in the Nemours Cardiac Center and Assistant Professor of Pediatrics at Sidney Kimmel Medical College at Thomas Jefferson University.

 

 

 

 

 

Thank you, Dr. Marino, Dr. Madsen, Dr. Gurvitz, and Dr. Sood, for your willingness to share your knowledge and experience!