New Diagnosis – Mary Beth’s Story, Diagnosed in Adulthood

We continue the New Diagnosis series with Mary Beth Meyers’ story. Diagnosed at the age of 23, Mary Beth shares how she had to put her dreams on hold due to an undetected CHD. 

 

 

 

In May of 2012, I was a newly graduated young woman, with hopes and dreams to begin working with children, meet my husband, and have a family someday. All that came crashing down the day I was diagnosed with a heart defect. A few months after turning 23, having just earned a degree in Early Childhood Education, I learned that I was born with an undiagnosed Congenital Heart Defect(CHD). My specific CHD is an Atrioventricular Canal defect, which isn’t just one, but rather a group of small defects that include an ASD, a VSD and possible abnormalities of the AV valves(mitral and tricuspid).  Since this defect can only be repaired by surgery, I am now  a two time open heart surgery survivor. I had my 1st surgery in August 2012 and a full mitral valve replacement in May of 2015(pig valve). I will be facing my third open heart this coming Summer.

Every since that sucky diagnosis day, life has been an absolute roller coaster ride. One filled with many fears, unknowns, and blessings. The physical, mental, and financial aspect has been draining, not just on me, but family, especially my parents. I know my parents would blow through their savings and sell everything they owned to keep me alive, but as an adult patient, who is quite aware of how stressful finances can be, there is always that sense of guilt running through my mind.  I have to remind myself everyday to count my blessings and that somehow God provides, not just for me, but for my parents too.

Being a late diagnosis has it’s pros and cons. My childhood was thankfully a normal one, even though knowing about my CHD would have explained all those years of being “tired,” rather than writing them off as laziness. My childhood wasn’t filled with surgeries, multiple hospital stays, doctors appointments, and trauma of not being able to communicate or understand what was going on.

For me, however,  the cons outweigh the pros. Not knowing for so long your heart was in overload for the majority of your childhood and all of your teenage years can add up to some hefty damage. Sometimes waiting can do more harm than good. And in my case, I, along with my parents and care team, wonder if doctors had intervened early on, would I be facing my third open heart surgery in under 6 years. Unfortunately, that’s something we will never find out, but I firmly believe, the earlier intervention, the better the outcome. Of course, it’s not something to dwell on, but rather be grateful that my diagnosis was found in my early twenties, rather than my fifties, or worse, during an autopsy.

Trying to become a young adult after getting through my 1st surgery has been anything but easy. Open heart surgery changes you physically, mentally, and emotionally. You are not the same person. Trying to navigate through life with something you know will never go away and brings a ton of physical and emotional pain can be draining. Not to mention, it’s financially draining. It forces you and your family to make sacrifices in so many areas. That’s not necessarily a bad thing, but when it consumes your whole life, for the rest of your life, it can seem unfair and downright exhausting. It makes you feel like you don’t know where you belong in this world. All your hopes and dreams keep getting delayed, and you wonder, with another open heart around the corner, if they will actually ever come true.

My late heart diagnosis has left me confused, angry, and jealous. Jealous of family and friends building their careers, gaining independence, experiencing marriage and motherhood. The hardest part is realizing, because you survived, that you are here for a reason, yet  you feel like a failure. You feel like a failure at times because you are struggling to become the independent adult you thought you’d be since childhood, having no idea then that your defect existed. Even though a few years have passed since my diagnosis, there are still days where it is hard to swallow the news of this disease. But there is a lot  I cannot change in my life, I can only keep moving forward with a new perspective.

One of my favorite quotes that I’ve come to lean on is, ”Accept the things you cannot change, the courage to change the things you can, and the wisdom to know the difference.”  I had no idea this is what my life would become, but everyday is gift. If you find yourself in a similar situation, surround yourself with family, friends, and a community of patients like you. It has shaped me into a person I’d never imagined I would be, and, now, I couldn’t imagine my life any other way.

 

 

Mary Beth Meyer is a 29 year old, living with an AV Canal Defect. With a late diagnosis at the age of 23, she has since undergone two open heart surgeries. Her most recent was a full Mitral valve replacement. Mary Beth graduated college from Franciscan University in 2011, and is currently pursuing a masters degree in Elementary Education at Holy Family University. She loves being an aunt to two beautiful nieces and  a handsome nephew. She loves her faith, family,  and a good peppermint latte. As the author of Mimi’s Open Heart blog, Mary Beth shares the good, bad, and amazing things about having a congenital heart defect. You can find her on Facebook @Mimi’s Open Heart or Instagram @mimisopenheart.

New Diagnosis – One Day At A Time, A Heart Mom’s Story

As we approach Heart Month PCHA begins its series on New Diagnosis. This week, Heart Mom Alexandra Frost shares with us the story of her daughter Emersynn’s  diagnosis in utero and how she learned to take the news and life with CHD day by day.

 

 

It all started when we went for our 12 week NT scan, where they measure the fluid behind the neck. The doctor came into the room and we could feel the discomfort. He said that our baby’s fluid behind the neck was measuring double the norm. This was a red flag for either Down syndrome or a congenital heart defect (CHD). He sent me to get blood work the same day. Ten long days later, I got the results that our baby tested negative for Down syndrome and the other genetic stuff they tested for, and we found out she was a girl! We were so excited and thought we were in the clear since we have no history of CHDs on either side of our family.

Fast forward 8 weeks when we went for our 20 week anatomy scan. When the doctor came in, we got that same feeling. She said, “All organs look good, except her heart. Unfortunately, she has a heart defect which puts you in that 1%.” It was one of the worst days of our lives. My world shattered as I tried to process these foreign words – “congenital heart defect.” They weren’t quite sure how severe her defect was at that point, which is why we were referred to a high risk OB and then a pediatric cardiologist.

When we saw the pediatric cardiologist, he broke the news that she does indeed have a complex heart defect which is VERY rare. Once again, my heart sank to the floor after hearing this news. Emersynn has congenitally corrected transposition of the great arteries (ccTGA or l-tga), a large ventricular septal defect (VSD), pulmonary stenosis (PS) and dextrocardia. Out of those one percent that end up with a heart defect, .5 – 1% end up with ccTGA. That is how rare the defect is! Only 5,000-10,000 people in the US have this condition! After leaving the cardiologist that day, my husband and I looked at each other in the car and looked back down at the paper that had a drawing of our baby’s heart. We were so confused and wondered if we would ever be able to understand the anatomy of our little one’s heart. Overwhelmed was an understatement.

 

 

 

 

 

 

 

 

 

We live in Fort Myers, and, unfortunately, the hospitals around here are not equipped to deliver babies with severe heart defects. We were given the choice to deliver in Miami or Tampa. Although Emersynn’s ped cardiologist (who is exceptional) is in Tampa, we decided to deliver in the Miami area at Memorial Regional Hospital (connected to Joe DiMaggio Children’s Hospital). This decision was made through countless hours of research after finding out (this is how I essentially coped with the news). During our research we came across a Facebook page for a boy that has the same defect. I reached out to his mother who led me to a private Facebook group for Emersynn’s particular defect. Only immediate family or the person affected is allowed to join this group. It turned out to be an extraordinary group that helped us tremendously! We found out, through this group, that a top surgeon for Emersynn’s defect was mentoring at Joe DiMaggio Children’s Hospital, and was there about once per month. This made us feel comfortable, as her defect is so rare and there are only a handful of surgeons that are successful in doing the major surgery that she will eventually need in the future.

 

We were told throughout the pregnancy that Emersynn would need a shunt within a week of birth, as they thought she wouldn’t get enough blood to her lungs. She would then need a major surgery called the double switch down the road. I ended up delivering on November 22, 2016 and she was a big baby, which defies odds already! They watched her closely in CICU for three days as her PDA closed. Once it was closed they monitored her saturation. She proved to be miraculous and was sent home on day 4, with no surgery and thriving!

Emersynn just turned one year old and has not had any surgery thus far! She is defying all odds and doing SO amazing! She is growing well and hitting all of her milestones on or before (!) she is supposed to! This little girl amazes us more and more everyday! She is such an inspiration to everyone around her and is such a strong little warrior! Emersynn will need a major open heart surgery in the next few years. The doctors say her body will tell us when it’s time. This is a very risky surgery and hard to put her through when she is currently doing so well. We know she will eventually need this surgery to continue to thrive and give her the best life. Some days, I hardly think about her heart defect, and other days it totally consumes me. It’s something I wouldn’t wish on anyone. However, we wouldn’t change Emersynn for the world. We know that we were chosen to be this sweet girl’s parents, because we could handle the challenge. Through this experience, we have learned to take it one day at a time!

 

 Alexandra Frost currently lives in Fort Myers, FL. She is originally from Long Island, NY and graduated from Villanova University in 2012. Alexandra has been married to her high school sweetheart for almost 2 years. She juggles real estate and being a mommy to a very adventurous one year old heart warrior. Alexandra and her husband are excited for their second baby on the way and know Emersynn is going to make a great big sister!

Wellness – Balance

In the latest post in the Wellness Series, Megan Horsley, heart mom and Pediatric Dietitian at Cincinnati Children’s Hospital Medical Center, discusses how to find balance with your nutrition.

 

 

Have you ever felt off balance? I am not specifically talking about when you were walking on a balance bean during gym class or gymnastics; or stretching your quadriceps after a run; or learning how to ride a bike for the first time. I am talking about BALANCE as you journey through life. I can assure you, from heart mom to heart mom and heart dad to heart dad, having your child affected by heart disease creates imbalance in your life at times. I think this unevenness, even though challenging in the moments, help us to grow into better individuals in the long run.

What about when you ignore your alarm and wake up late, panicking because you have to get to work, an appointment, or make sure the kids make the bus, or let the dog out before he or she pees on the floor. Then like dominos, the rest of your day may seem off. You either eat late, skip eating or choose something half satisfactory and unhealthy and then before you know it, it is dinner time when you stop and think about your eating and drinking choices throughout the day!

Well, I often think of one’s health and nutrition in the same manner. Sometimes, our eating and healthy behavior is “off balance” and we need to get it back into check. At times we are not mindful of what we are entering into our mouths or how it will affect our body’s long term. We put the nutrition of the ones we love and care for every day before our own nutrition. This is being a parent, right!? Maybe, some of us are better at it than others but no matter what we can all use reminders of how to be more mindful to eat healthier.

I believe mindful eating is vital to maintaining a healthy weight and eating behaviors. Listen to your body. Slow down. The more mindful we are, the more balance we have around our nutrition and diet. It is especially important to practice around the holidays, celebrations or social events. For example, have you ever actually counted out the number of chips/crackers the label says is a serving? or measured out the appropriate amount of salad dressing or cheese for your salad?  This practice encourages more self-education but you also get to visually see what a portion size looks like for that item. Try it for the next food you eat! Learn what normal portions look like for foods. Read those food labels! They have been updated this year and are more reader friendly. This includes even those calorie-dense fluids like juice, Kool aide or, yes, alcohol. I think you will be surprised. Conscious awareness will go a long way in healthy decision making. You may find yourself eating from smaller plates or drinking from thinner glasses. Use mantras to keep you aligned with your goals. One of the mantras I use to remind myself of mindful eating is: Eat, Drink, Be Mindful.

Another contributor to a healthy diet is pre-planning. We are busy people and on the go. If meals and snacks are not planned, it is easy to give in to fast food or limit food groups when grabbing something quick. This can lead to overeating or uncontrolled snacking. Take a few minutes to pack ahead some healthy snacks so you are ready to fuel yourself as well as your family when you hear, “I’m hungry!” from your child in the back seat. Get a dry erase white board and plan your meals out for the week. Incorporate the kids in the process. Taco night or breakfast for dinner can still be offered as a balanced meal. If headed to a party, plan to make a dish that you know has good nutritional value and that your family will eat at the party. I like to think as planning ahead as “mapping out your nutrition” for the day/week. Now, yes, this does take effort but so does getting dressed or maintaining hygiene. If you make it part of your routine, you won’t be bothered by the time it takes.

Keep your plate colorful! Try to eat from a variety of colors during your day. Each color represents different phytochemicals, antioxidants and nutrients for your body. They are all important. If you only eat one color all the time, you may be missing out on other vitamins and minerals important to your heart health, the skin or immune system. Be creative with your colors. Try new colors of foods. We have over 10,000 tastes buds and they change often and over time. Repetitive tasting of foods will help one acquire a taste. Don’t get frustrated but find different ways to incorporate a new food if your child/ren won’t accept it.

Pair protein with each meal and snack! Protein is the building blocks of our bodies. By pairing protein with a carbohydrate or fat, your digestion will be slowed and you will have less spikes in your blood sugar. Try the peanut butter with the apple or banana; add a cheese stick to your crackers; dip sliced peppers or carrots in your hummus or veggie dip; add avocado to your toast; try almonds with your dried fruit. You will have more lasting energy and feel stronger.

Choose fruit for dessert if looking for something sweet! This is a nice way to add more nutrients while feeling like you are fixing your craving for sweet. Yogurt and berries, banana bread, mango sorbet can be a lower calorie, higher protein, nutrient-enriched option.

I would like to end this blog with Role modeling good healthy behavior. Eat to live, not live to eat! As a heart mom, and a cardiac dietitian, I am endlessly reminded of how precious life is. Be kind to your heart and live as you want your children to live! Yeah, it sounds cheesy but it works. If they see you exercising routinely, packing your lunch for work, making meals as a family, downing a kale smoothie, or trying something new they will follow this learned behavior. Find balance again when you may have fallen off. Be mindful of your body and health and don’t waste time with weight loss pills or quick fixes. Use hard work, education and dedication to help you be successful in maintaining a healthy weight.

 

You can find more healthy nutrition information at various nutrition blogs. See nutrition blogs for all categories at: http://www.nutritionblognetwork.com/

 

Megan Horsley, heart mom, heart dietitian
Pediatric Dietitian @ Cincinnati Children’s Hospital Medical Center
Megan.horsley@cchmc.org

 

My Heart Hero: Maggie

 

 

 

 

Megan Horsley is a Clinical Pediatric Registered Dietitian at Cincinnati Children’s Hospital Medical Center. She currently practices in the Critical Cardiac Intensive Care Unit as well as outpatient cardiology areas. She is a Certified Nutrition Support Clinician in addition holds a Certification as a Specialist in Pediatric Nutrition. She received her undergraduate degree from the University of Cincinnati in 2006 and completed her dietetic internship with the University of Northern Colorado in 2007. She has been a lead dietitian for the Children’s Heart Institute and participates in many projects and community services such as, The National Pediatric Cardiology Quality Improvement Collaborative, family education day and heart camp to name a few. She recently joined the Cincinnati Children’s Heart Association Board in October of this year. She has ten years of professional practice in pediatric nutrition and thirteen years of personal experience as a heart mom herself. She plans on continuing to concentrate her efforts on improving the nutritional care, experience and outcomes for all children affected by heart disease.

 

 

 

 

Arrhythmias – Tachycardia Explained (Part Two)

Last week we discussed the issues that can cause the heart to beat too slow. This week, we look at what happens when the heart beats too fast. Carol Raimondi, completes part two of her resource on arrhythmias, this time covering tachycardia.

 

 

Tachycardias

There are many types of tachycardias, but here we will focus on the most common tachycardias seen in congenital heart defects. When diagnosing these arrhythmias, the same tests as mentioned in part one are used to determine the arrhythmia. The only additional diagnostic test that is common with tachycardias, is an electrophysiology study (EP study). This test helps to confirm diagnosis and locate where an arrhythmia originates. It is done by a cardiologist who specializes in heart rhythm disorders in the EP lab. This is an invasive test where thin catheters are placed through the arm, leg and/or neck, then threaded through the blood vessels to the heart. The doctor can then use these to map the electrical conduction of the heart and reproduce arrhythmias in a controlled setting. This is very useful in planning for treatment.
Sinus Tachycardia
This refers to a heart rate that is more than 100 beats per minute.  Common reasons this occurs is due to a physiologic response to exercise, fever, dehydration,stress, pain, or stimulants, such as caffeine. When the factor that caused it is resolved, then generally, the heart rate returns to normal.
Atrial Fibrillation
A-Fib, as its often called, is the most common type of tachycardia. It occurs when, instead of the sinus node initiating the electrical conduction, there are many different impulses firing throughout the atrium. This results in a fast and very chaotic rhythm. Since it is so fast and irregular, the atria quiver or “fibrillate” and are unable to effectively squeeze the blood into the ventricles. A-Fib can come and go, or it can be continuous. Symptoms may include palpitations, lightheadedness, shortness of breath, fatigue or fainting, though some patients have no symptoms at all. The main concerns with atrial fibrillation is the increased risk of stroke and heart failure. As the atria are not emptying fully, blood can pool within the chamber and form blood clots.The clot can then travel to the brain and cause a stroke. Also, the irregularity of the rhythm makes the pumping action of the heart less efficient. If this continues for a long period of time, it can weaken the heart muscle, causing heart failure.
Atrial Flutter
Similar to A-Fib, atrial flutter occurs when the electrical conduction originates from an abnormal circuit in the atrium. With atrial flutter, there is still a very fast heart rate, but the rhythm is regular. It is not uncommon have atrial fibrillation in addition to atrial flutter. Like atrial fibrillation, the symptoms of atrial flutter can include palpitations, dizziness, fatigue, shortness of breath or fainting.
Supraventricular Tachycardia(SVT)
Supraventricular, or “above the ventricle”, tachycardia, is also referred to as atrial tachycardia, It occurs when there is an extra conduction pathway in the atria, usually present since birth, that creates a loop of overlapping signals. This arrhythmia is rarely life threatening. It is not uncommon for the atrial heart rate to get up to 300 beats per minute during an episode of SVT.  An episode may last for a few seconds up to a couple of hours. Symptoms of this include palpitations, feeling as though your heart is “racing”, lightheadedness, or shortness of breath. If the arrhythmia lasts for more then 20 minutes, it is recommended to go to the emergency room to receive medication to treat the irregular rhythm.
Ventricular Tachycardia
This refers to a very fast heart rate the comes from abnormal electrical signals in the ventricles, or lower chambers, of the heart. Since the heart rate is so fast, it does not allow adequate time for blood to fill into the ventricle, thus a much lower amount of blood is delivered to the body. These arrhythmias may last only a few seconds and not cause any issues. If it continues longer than that, it can be very serious and requires emergency treatment.  Symptoms are similar to the other tachycardias, but fainting can occur if it is sustained for more than several seconds.
Ventricular Fibrillation
This occurs when very fast, chaotic impulses in the ventricle cause it to quiver, or fibrillate. When this happens, blood can not  be pumped efficiently to the body. This is considered a life-threatening emergency if not treated within minutes and requires a defibrillator to “shock” the heart back into rhythm.

Treatments

Treatment of tachycardia can be divided into two separate categories, prevention of arrhythmias and treatment during an arrhythmia.
Prevention
Catheter Ablation- This is an invasive procedure that, like an EP study, involves insertion of small catheters through the arm, leg, and or neck, that are threaded through blood vessels into the heart. Once the abnormal circuit is found, the catheter uses either heat or extreme cold to damage the extra pathway, thus preventing it from allowing the arrhythmia to occur.
Medication
There is a class of medication know as anti-arrhythmics that doctors can prescribe to help prevent fast heart rates. These may be used in conjunction with other cardiac meds, such as beta- blockers or calcium channel blockers, to provide optimal coverage in keeping the heart rate and rhythm regular. Another class of medication that is used frequently for atrial fibrillation is anti-coagulants, or blood thinners. Warfarin, or Coumadin, is still the most commonly used type of blood thinner, but there are newer medicines that can be used as well. While blood thinners do not prevent atrial fibrillation, they help prevent blood clots from forming, thus reducing the risk of stroke associated with this arrhythmia.
Pacemaker
When a fast heart rhythm, such as A-Fib, is not successfully prevented by medicine or catheter ablation, a pacemaker can be used to help control the arrhythmia. As discussed in part one, a pacemaker will watch the heart for abnormal rhythms, and then initiate the correct conduction when needed.
Implanted Cardioverter Defibrillator(ICD)
Similar to a pacemaker, this small device is surgically implanted under the skin. It has leads that are threaded through a vein into the heart. The ICD then “watches” the heart, and, if a dangerous arrhythmia such as ventricular tachycardia is detected, it will deliver electrical shocks to the heart to restore a normal rhythm. The device is programmed with specific parameters so that the device does not shock inadvertently. It sends data wirelessly to the physician via an in home monitor, and also requires semi-annual visits to the pacemaker/ICD clinic for a full interrogation.
Surgery
Though rarely done for arrhythmia prevention alone, a surgical procedure can be done to block the abnormal pathways. Known commonly as a MAZE procedure, a number of small incisions are made in the heart tissue in a specific pattern. The scar tissue that forms from these incisions does not conduct electricity, so the abnormal pathway that was causing the arrhythmia is blocked. This procedure is usually done if the patient is having open heart surgery to treat another heart disorder.
Treatment During an Arrhythmia
Medication
There are a number of medications that can be administered intravenously to stop an arrhythmia while it is is happening. The type of medication will vary depending on the patient and the arrhythmia. There are also oral medications that can be taken at home to help control fast or irregular heart rhythms.
Vagal Manuevers
The vagus nerve is responsible for heart rate. There are certain maneuvers that affect this nerve, and can be used, with physicians instruction, to help lower a fast heart rate. Some of these include coughing, bearing down (as if having a bowel movement), and applying ice to the face and neck area.
Cardioversion/Defibrillation
Cardioversion can be done to help “reset” the conduction system of the heart. It may be performed as a scheduled procedure for someone who has had ongoing atrial fibrillation.This is done while the patient is sedated in a controlled setting with a lower amount of electricity. It also is used in emergency situations when ventricular tachycardia or ventricular fibrillation is present. This is done by placing paddles or patches on the chest. Then, a charged current is delivered that affects the electrical system of the heart and intends to restore a regular rhythm.
Carol Raimondi is an adult CHD patient and nurse, living with Congenitally Corrected Transposition of the Great Arteries.  She has had 4 open heart surgeries in her 40 years, as well as a pacemaker since the age of 6. After spending a large part of her childhood in and out of hospitals, she developed a passion for nursing. She went to school to become a cardiac nurse. Carol’s many hospital experiences helped her as a nurse to better understand what her patients were going through and  to care for them with that much more compassion and empathy.
Due to worsening medical issues, Carol had to give up the profession she loved. That did not stop her from being a patient advocate, however. She joined her local hospital’s’ Patient Family Advisory Council, which she now co-chairs, and shortly thereafter she joined Mended Little Hearts Chicago(MLHC) as an adult CHD liaison. She then expanded her work in the CHD community by starting an adult and teen CHD group within MLHC and became an  Ambassador for the Adult Congenital Heart Association. Currently she sits as the Pediatric Congenital Heart Association of IL(PCHA-IL)  President. Her proudest moments are when she is advocating and raising awareness for the CHD community, both on Capitol Hill in D.C. and locally.

The Biggest Gift

After  receiving a heart transplant, Megan Horton,  a Texas Children’s staffer shares how she celebrates the biggest gift she’s ever received . Happy Holidays, Everyone! 

 

 

Twelve years ago, a family lost their daughter. Twelve years ago, a 17-year-old lost her best friend. Twelve years ago, friends, family and loved ones had to say goodbye to a girl who passed away too soon.

Twelve years ago, I received the gift of life at Texas Children’s Hospital with a heart transplant. It’s always hard for me to celebrate my “heart birthday” each summer, when I know a family is grieving. The girl who donated her heart would have been 27 this year. I hope her family would find joy in all the things I’ve been able to accomplish by receiving the gift of their daughter’s heart.

In the past 12 years, I’ve accomplished so much. I graduated from high school and college, moved to a new city, landed my dream job, celebrated so many birthdays and anniversaries, and traveled to paradise.

I was only 14 years old when I received the greatest gift of my heart transplant, and while I’ve faced many challenges, I’ve always tried to have a positive outlook on life and remember that my life is a gift.

Each and every Christmas, no matter what presents are under the tree, my greatest gift is always the fact that I am there to celebrate with my family with a strong and joyful heart.

I’m very blessed that I received my transplant when I did. Every day, 22 people will pass away because they didn’t receive an organ in time. Please sign-up to be an organ donor and make your wishes known to your family. If you’d like to learn more about organ donation, please visit DonateLife.net.

 

 

Megan Horton is a heart recipient and the blog content manager for Texas Children’s Hospital.

Recap – Teen Topics

The 2017 Teen Topic Series posts are all here in one place. Take a look back on the important issues discussed!

 

Medical I.D.’s and Taking Ownership of Your Care

PCHA began its Teen Topics Series with a post about employing medical IDs as a useful tool. American Medical I.D.s, introduces IDs as one step toward  families helping their teens take ownership of their own care. Read further on the importance of involving teens in self -care and for a special offer .

 

Going Off to College

Going off to college is a major milestone in a young adult’s life. For many, it is the first time living away from home, from their parents, and from everything familiar to them. A chronic illness can complicate the transition. In this week’s blog, Abby Hack shares what it was like for her to gain her independence while managing atrioventricular block.

 

Preparing Your Child for Independence

We heard from Abby Hack on heading off to college. Now, we will hear from her mom, Janice. Watching your child leave home for the first time can be worrisome, especially with cardiac issues to consider. Janice shares with us how she has helped prepare Abby to take greater ownership of her own care.

 

Q&A: Your Questions Answered

in September 2017, patients and families submitted the questions they most wanted answered. We caught up with members of PCHA’s Medical Advisory Board, at the Transparency Summit, to ask those questions. Check out the videos, featuring Dr. Marino, Dr. Madsen, Dr. Gurvitz, and Dr. Sood’s answers on teen and young adult topics.

 

The Top 10 Things to Remember

As the seasons change, new milestones come and go. These can be especially trying times 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.

 

Arrhythmias – 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 this week: Bradycardia.

 

 

 

 

Congenital heart defects are frequently accompanied by issues with the electrical conduction of the heart. These issues can cause a slow heart rate( bradycardia), a fast heart rate (tachycardia), or an irregular heart rhythm, also referred to as an arrhythmia. There may be one of these conduction defects present, or several at the same time.This can be due to the structural defects that were present at birth, or related to scar tissue that develops with each surgical intervention.  Fortunately, there are options to treat these irregularities.
Here we will be discussing the most common types of issues related to the electrical system of the heart. In order to discuss these abnormalities, first you should understand how things are supposed to work.
In normal conduction of the heart, the impulse is initiated in a group of specialized cells, called the sinus or sinoatrial (SA) node. This is located in the right atrium, or upper chamber, of the heart. Once initiated, is causes the both the right and left atria to contract. The signal then continues to the Atria-Ventricular (AV) Node. This is found between the upper and lower chambers. The signal slows slightly through here, allowing the blood to empty from the atria and enter the ventricles, or lower chambers. From there, the signal continues down through the lower chambers via the left and right Bundle of His, causing the ventricles to contract. Each time this cycle occurs, it results in one complete heart beat. When working properly, it should repeat this cycle without interruption 60-100 times per minute.
Bradycardias
Sinus Bradycardia:
This describes a regular rhythm with a rate of less then 60 beats per minute. Sinus bradycardia is usually a benign finding. It is frequently seen in athletes or during sleep. There is generally no treatment for this unless the patient has symptoms, such as lightheadedness or fatigue. In such cases, further evaluation is done to determine an underlying cause, such as medication side effects or metabolic disorders.
First degree AV block:
This refers to a prolonged delay in conduction between the atria and ventricles. Generally, there are no symptoms associated with this, as the ventricle still does contract with every beat. No treatment is required for this type of heart block.
Second degree AV block:
Type 1- This is the less critical of the two types of second degree blocks and rarely requires treatment. In type 1, there is a progressive lengthening of the time it takes for the signal to travel from the sinus node to the AV node. With each contraction, it takes longer and longer until the ventricle “skips” a beat and resets the cycle.
Type 2-  In type 2 AV block, there is no specific pattern to when the signal is blocked from the atria to the ventricles. When this signal is blocked, the ventricles do not get the message to contract, and thus the blood is not pumped to the body. This often leads to third degree heart block. A pacemaker is implanted to treat type 2 AV block.
Third degree AV block:
Third degree, or complete heart block, refers to when the signal between the top and bottom chambers of the heart is absent. The ventricles use a “back up” electrical system, which produces a much slower heart rate then normal. The atria continue to contract as well, but there is complete dissociation between the top and bottom chamber, so the amount of blood being sent to the body is lessthen usual. Symptoms associated with complete heart block include lightheadedness and fainting. If left untreated, this block can cause death. This type of heart block  is critical and requires emergent treatment with a pacemaker. Often a temporary, external pacemaker is placed until the patient receives the permanent pacemaker.
Bundle Branch Blocks (BBB):
The Bundle branches are the fibers that carry the electrical signal from the AV node down through each ventricle. They are described as either the right or left bundle branch. When there is an interruption in this pathway, it is referred to as a bundle branch block(BBB). Generally, bundle branch blocks themselves do not cause any symptoms or require treatment. Further cardiac assessment should be done, however, to determine if there is an underlying cardiac condition. Right BBB is often seen in Congenital Heart Defects, especially in atrial or ventricular septal defects. Left BBB can frequently be seen in patients with coronary heart disease, or with weakening of the heart muscle.
Diagnosis
The cardiac conduction irregularities listed above can be diagnosed in several ways. The most common is by performing a 12 lead electrocardiogram (ECG) This is a non-invasive test where patches, connected to leads, are placed on the skin. The technician ensures that the patient lays still for 15-30 seconds while a reading of the electrical signals through the heart is done. A printout of these readings can immeadiately show the heart rate, rhythm, and any damage to the muscle of the heart.
A holter monitor, similar to an ECG, involves applying electrodes to the skin in specific locations. Generally, there are 5 leads or less. These leads are connected to a small device that has recording capabilities. The patient can wear this device for 24-48 hours, depending on the physicians preference.  The device continuously records during the time that it is worn. Once returned, the ECG recordings are downloaded to a computer for a physician to review. The advantage to this test is that  with a prolonged recording period, there may be abnormalities detected that an ECG, which gives a “snapshot” can not detect.
An event monitor is similar to a holter monitor, in that it it is a small portable device that allows for prolonged recording of heart rate and rhythm. This device is usually worn for up to 30 days, and usually the patient can push a button on the device when he/she is experiencing any symptoms. Then they can call in to the device monitoring company and transmit the reading, which is reviewed and forwarded to the patients physician.
Treatment for Bradycardia and Heart Block
Of the above conduction issues, treatment is generally only required for second degree type 2 and third degree heart block. A small, electronic  device, known as a pacemaker, is used to restore normal conduction. This device is surgically implanted in the upper chest or abdomen, just under the skin, in a “pocket” the physician creates. Leads connected to the pacemaker are then threaded through a vein into the heart, where it is attached. The pacemaker “watches” the heart rhythm, and when it detects abnormal conduction, it sends an electrical signal to the heart. The pacemaker is programmed to not let the heart drop beneath a certain rate. Newer pacemakers transmit data wirelessly to their doctor or pacemaker clinic via a small device that can be kept near the patients bedside at home, This allows the physician office to monitor the functioning of the device without requiring the patient to call in, as was done in the past . Usually, every 6-12 months, an in-office pacemaker check is required to do full testing on the device, but this is quick and non-invasive. Pacemakers can last 7-10 years on average, depending on what percentage of time the pacemaker is in use.
Please join us next week for Part Two – Tachycardia.

Carol Raimondi is an adult CHD patient and nurse, living with Congenitally Corrected Transposition of the Great Arteries.  She has had 4 open heart surgeries in her 40 years, as well as a pacemaker since the age of 6. After spending a large part of her childhood in and out of hospitals, she developed a passion for nursing. She went to school to become a cardiac nurse. Carol’s many hospital experiences helped her as a nurse to better understand what her patients were going through and  to care for them with that much more compassion and empathy.

Due to worsening medical issues, Carol had to give up the profession she loved. That did not stop her from being a patient advocate, however. She joined her local hospital’s’ Patient Family Advisory Council, which she now co-chairs, and shortly thereafter she joined Mended Little Hearts Chicago(MLHC) as an adult CHD liaison. She then expanded her work in the CHD community by starting an adult and teen CHD group within MLHC and became an  Ambassador for the Adult Congenital Heart Association. Currently she sits as the Pediatric Congenital Heart Association of IL(PCHA-IL)  President. Her proudest moments are when she is advocating and raising awareness for the CHD community, both on Capitol Hill in D.C. and locally.

Cardiac Devices – 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.

I’m often wondering what to tell people when they ask me about our children. It’s something most parents think about I’m sure. What details will help this teacher, this coach, this babysitter take care of my child? What can I share with them that will give them a view of who they are while they are in their care? For me there is always this looming question of what to say when discussing my children. On one hand I want them to treat my children as the unique individuals they are, discover new things about them, but on the other hand we do have this elephant in the room which is CHD.

I am a mother of three with two children who have faced Congenital Heart Defects. Like most Heart Parents it’s not something you plan, but it’s our life and we do a pretty good job at navigating C.H.D. It is always on my mind what and how much to share especially since two of my children have irregularities in their heart rhythms or arrhythmia.

Arrhythmia isn’t something I thought about when my children had their open heart surgeries. Actually, it was never mentioned when they told me about their hearts and never mentioned until few years ago. I think in the medical profession they are more of the approach of “we will get to that hurdle when and if we get to it.” Even the vague definition for Congenital Heart Defects is ‘problems with the heart’s structure present at birth’ that doesn’t really lead you to believe you may also have “electrical” issues as well or it didn’t in my non-doctor mind. CHD has so many forms and complexities that there isn’t a one size fits all explanation or treatment, so I see their hesitation to lay it all out there. Unfortunately that makes CHD affecting your family a situation of having to roll with the punches of what is going to happen, or at least that is how I have felt.

About two years ago, we were told our daughter had 1st degree heart block. After 4 years of seemingly good appointments, it flung me back into that intense worrying state of mind that we were in for her first year of her life.1st degree heart block, once again something was affecting my child that I had never even heard of before. I remember thinking, “ok, block doesn’t sound good, especially when describing my daughter’s unique heart.” “Why wasn’t this mentioned as a possibility? Or was it, is that the same thing as a murmur? Maybe they mentioned it? No, I would have remembered that.” We were told not to worry, but of course one of my favorite quotes is ‘telling a mother not to worry is like telling water not to get wet.’ I remember crying in the bathroom after we got home from the appointment. Although I knew CHD was lifelong I felt like we were ahead of it or at least had it under control. Arrhythmia seemed like the scariest effect of her surgery. I may or may not have googled how much an AED costs.

With this new information we did what we could and I quickly learned that we did not in fact need our own AED. We did our research and we asked questions. It turns out that arrhythmia is pretty common among some patients who are affected by CHD. That was reassuring for some reason, at least she wasn’t the only one and her pediatric cardiologist was very familiar with this. We followed up about Holter monitor results and asked what to symptoms to look for in the future. We were told that pacemakers would most likely be in her future, but time would tell on when. We were met with some ifs and buts since, unfortunately, our cardiologist is not a fortune teller. Wouldn’t that be nice though? Note to self: google physic pediatric cardiologist. Just kidding. Fast forward just 6 months later and our youngest and second heart warrior was too experiencing irregular heart rhythms. This time, I was more prepared and less shocked. Of course his arrhythmia is not the same as our daughters, but arrhythmia was now on our radar so to speak. Isn’t it weird how things like this become your new normal? Now we had a new discussion point when it came to our kids and how to approach this new element of their health concerns added a new layer to who our kids are.

 

Thinking about my children at school and how to explain CHD and arrhythmia to their teachers worried us. I would go back and forth on how much to disclose at their schools. I wanted to keep them safe of course, but I didn’t want them to be known for just one thing, that being just their special hearts. We decided to air on the side of caution and give specific details about our children’s hearts to their teachers and school nurse. I am so glad we did because our daughter’s school nurse kept referring to it as congestive heart failure. Yikes, right? While we were worried what to share, our kids were busy telling everyone about their hearts and showing off their Holter monitors. They decided to take the lead and give full disclosure to anyone they met. We were surprised, proud and kind of felt silly. We worried what to say about the “elephant in the room” and how to say it, but when it came down to it, it didn’t matter. To their teachers, they were still just two happy kids in their school and their friends think it’s cool they had heart surgery. At this moment, we have things figured out, but of course life has a way of happening while we’re busy pretending we know what we’re doing and planning.

 

Two of our children are affected by CHD and have irregular heart rhythms. So where does this lead us? Like most Heart Parents we just don’t know what our children will experience next. This CHD journey isn’t written in stone and we will always have looming questions on what will happen and how to handle social situations well into adulthood. Will I one day have a heart warrior grandchild? Whoa, ok, slow down, but it’s already on my radar. We just have to stay tuned because with CHD we just don’t know exactly what path we will take. At least we no longer see CHD as an elephant in the room.

 

 

 

 

Alison Connors is a mother of three children: McKenzie, Jackson and Archer. Her oldest McKenzie and youngest Archer both had open heart surgery for congenital heart defects. McKenzie and Archer have been in the care of the PSHU team at Advocate Children’s Hospital in Oak Lawn, Illinois since birth. Alison has been married since 2009 to her best friend Christopher, and she recently went back to work as a 1:1 teacher’s aide for children with special needs. She and her family have a busy life, but she has a passion for volunteering and believes that there is healing power in taking part in something that is bigger than yourself. Having two children with CHD threw Alison onto a path she never expected to be on, but a path her family is very grateful to be on. It’s an honor for Alison and her family to share resources, give hope, and support to others who face the same situations that they have.

 

 

Cardiac Devices – 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. 

 

My name is Terri.  I am 61 years old, a wife, mother (of 1 daughter), grandma of 5, and a Licensed Veterinary Technician.  I was also the 1992 Hunter Jumper Champion in my state!! (…my cardiologist was not happy about my jumping fences on a horse, of course, until I WON!! LOL!)  I live an active/fulfilled life, despite living with CHD, arrhythmias, and a lovely ICD which I have named affectionately “my friend”.  My original defibrillator was named “Sparky”, it was replaced with a larger one and we are trying to bond!! I was diagnosed as a child with a VSD, Pulmonary Stenosis, LTGA, and a “blue baby” which I had surgery in 1960 (yes, with ether!)!  I have been told that I am one of the oldest complex congenital heart patients in Michigan). My childhood was difficult with much bullying to endure.  Funny (but sad story), the kids in my school teased me so unmercifully, that I told them that I would “give them my bad heart if they didn’t stop”!  Well, you can see how that would turn out (and did), so I did not have any friends, as they were then afraid of me.  As you can see, having CHD was not an easy feat, as a child.  More of this at another time.

Living with arrhythmias can be challenging.  Mine have caused me several trips to the hospital.  They feel as if my heart is racing like the “Top Thrill Dragster” at Cedar Point, OR  it skips beats like a “cell phone with bad reception”.  Either way, intense! Medication helps to control them, and if I don’t “listen” to them, (which I can usually hear or feel 80 percent of), I barely know they are there. That seems to be a learned trait.  There are times, however, that arrhythmias can make me feel like I will “pass out”.  Those are the difficult ones to deal with.

As for my defibrillator, well that is a totally different situation.  My defibrillator was placed after I had sudden cardiac arrest (due to v-fib) in 2009.  I was on life support, and when I woke wondering what happened, I was told that I needed one for “insurance”.  At that time, I had heart surgery to put in a shunt and implant the device.   Having a defibrillator is challenging, but also frightening at times. With my first defibrillator (“Sparky”), it was placed in my abdomen.  I did a lot of exercising and fractured wiring a lot!!  Funny story – My first fracture – I thought was my brand new smart phone!!  I remember wondering why I got the stupid smartphone in the first place, since it chimed relentlessly, and I could not figure out what was wrong!  The problem was that it was not my smartphone at all!!  It was ME!!  I had fractured wiring.  Surgery is always needed when something like that goes wrong.  But in “my friends” defense, it is always there to protect me.  It can deliver many joules of electricity to my heart if needed to save my life.  I have had 3 defibrillators, so far, and my current one is an advanced version of a sub-cutaneous unit.  I am one of the lucky ones in that none of them needed to “fire”.  Patients like me live in fear, have challenges, pain, can be limited, and sometimes breathless.  But, I for one, am positive, strong, determined, full of love, am caring, and even defiant!!

Don’t be afraid of your defibrillators!  They are there to help and possibly, keep you alive!!  That’s a lot of power in one little box!!  I have a lot to say after this many years living a life of CHD!  That, my friends, would need a bit more time and space to cover!  Another time and place!  Happy if this helps even one person!

 

Terri Elliott is a Licensed Veterinary Technician of 28 years who lives in Oxford, MI with her husband, Mike and their 2 dogs, “Madison” and “Charlie”. She holds an Associates degree in Veterinary Technology and is currently earning her B.S. in Business Health at U of M. She enjoys travelling and summer activities, especially golfing, horseback riding and swimming. Mostly, she enjoys spending time with her 5 grandchildren, daughter and son-in- law. She is a volunteer chairwoman at the Michigan Congenital Heart Walk and enjoys speaking at hospital heart events. She has just been accepted and trained as an ACHA Heart to Heart Ambassador.

Cardiac Devices – 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. 

 

At our institution, we are working to create a smaller, implantable ventricular assist device
(VAD), specifically designed for small children. This device is called the Infant Jarvik VAD, named
for Dr. Robert Jarvik, the inventor of the first successful total artificial heart.

Why do we need this device?

In the world of pediatrics, most VADs are pulsatile, which means they pump like the heart.
However, these sometimes fail. This is why most adult VADs are now continuous flow pumps,
meaning they help push blood through the body using an impeller. Currently, there are no
pediatric continuous flow devices available for our smallest patients. We are hoping to change
that with a small, AA-battery- sized device.

It is more challenging to make a smaller pump with a favorable performance. After failing to get
FDA approval two years ago, Jarvik made significant design changes, and I came on board to test
the redesigned device in animals as a preclinical trial. The data we collected was much more
favorable than the previous testing, resulting in approval for a clinical trial by the FDA. The
Pumps for Kids, Infants and Neonates (PumpKIN) trial has just begun with seven major pediatric
heart centers in North America, including Texas Children’s Hospital, ranked #1 nationally in
pediatric cardiology and heart surgery by U.S. News & World Report.

Currently, hospitals across the country have limited options on the types of patients they can
support. For example, at Texas Children’s, we implant about 20 to 30 VADs each year. In many
of these cases, we have to use an adult device in a child, which inevitable causes patient-device
size mismatch. If the Jarvik infant pump becomes available, that may change device support
paradigms, leading to more potential candidates who can benefit from this implant at hospitals
nationwide.

 

 

Dr. Adachi has written extensively regarding surgical, morphological, and physiological aspects of congenitally malformed hearts. His clinical and research interests also include pediatric heart failure and mechanical circulatory support including ventricular assist device and total artificial heart. He is also studying cardiac cellular and sub cellular differentiation prior to and following application of mechanical assistance. He also has an interest in pediatric lung transplantation. For better preservation of donor lungs, he seeks to develop a pediatric-specific lung preservation device for deployment in the lung transplant program at Texas Children’s, which is the largest in the world.