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.

 

 

 

 

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.

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.

Wellness – The Most Wonderful Time Of Year

The Holiday Season is meant to be filled with family and joy. It can also be a time of great stress, especially for families with chronically ill loved ones. In today’s post, Becky Hunt shares her experience with losing a child to CHD as well as managing her own illness, and explains how to de-stress from it all around the holidays.

 

 

 

Ah, December! It’s the most wonderful time of the year! Time for enjoying the festivities of the season! Exciting, right?! Well, for many of us, the next few weeks bring along added stress that can dampen our spirits and make the season a little less bright. Making travel plans, buying gifts, driving the kids to this party and that program, it’s non stop! Most “wonderful” time? Talk about most STRESSFUL time of the year!

 

Christmas was always my most favorite time of year! That was until my world came crashing down on me many times over.

My story starts with a little girl named Gracie. My baby girl. Gracie was born August 2nd, 2012 with Hypoplastic Left Heart Syndrome, or “half a heart”. She lit up my life for 82 days. The girl who changed MY heart inspired me to start a non-profit organization that creates dream cakes for kids with Congenital Heart Defects to brighten their lives. 5 years later that non-profit, Cakes From Grace, is thriving and growing and reaching more and more heart families by the day.

3 years after we lost Gracie I heard the 3 words that no one ever wants to hear, “YOU HAVE CANCER”.

Cancer.

Me? Haven’t I been through enough? Why me? Why now? I have CAKES to bake!

But the truth is, Cancer seemed to be nothing compared to losing your child or even seeing THEM suffer the way she did in the hospital for 82 days.

But I am here 2 years later, gone through several surgeries, 2 years of treatment and no Cancer in sight.

After Gracie passed away I dove right back into work. Started 5 different businesses (like one wasn’t enough). And just buried myself in it. At first work was my place to hide, to escape, it was a distraction, a place I could numb the feelings and avoid the hurt.

My obsession with work grew and soon it turned into an obsession with stress. I couldn’t escape. I felt like if I stopped then the world would crumble beneath me. Like I was letting people down. Constantly giving of myself, my services, my time, never saying no.

Christmas was always my most favorite time of the year. That was until we lost Gracie. I found myself angry that there wasn’t a spot at the table for her. No gifts under the tree for her. She wasn’t there to decorate the tree with me or sing our favorite Christmas songs I always sang to her.

The Holidays for me started to turn into a chore. The lists, the gifts I needed to find. My loved ones started to become a checklist. Ok, that person is done, 3 more to go, and 1 week to get it done before Christmas!

The endless amount of things on the Calendar. Christmas concerts, parties we were invited to, family get-togethers, gift shopping, UGH and the standing in lines! When am I gonna have the time to put up a Christmas tree?! 23 days of December just are NOT enough to fit it all in!

Everything constant, work, grief, the to-do lists all piling up!

 

Then how in the world DO you fit it all in? How in the world can you DE-stress?

 

1. Master The Art of Saying “No”

You don’t HAVE to attend every party you are invited to. Make a list of all the parties you and your kids were invited to, have them pick 1 or 2 of them to attend, not all of them.

2. Skip The Lines

You don’t have to go out and stand in those lines waiting to purchase the ONE thing you came here for! Shop online if that stresses you out! Put up your feet and cuddle up at home! Let the USPS guy bring it to you!

3.Take Time for You

Instead of giving your spouse a list of things you would like for Christmas, as a gift, ask him to take the kids out for a few hours while you read a book or watch a Christmas Movie and drink a glass of wine in a QUIET house. Ah, doesn’t THAT sound nice?!​​​​​​​​​​​​​​​​​​​

4. Write a priority list, NOT a to-do list!

Instead of freaking out over the things you need to get done, try to organize your list in order from what the top priority/needs to be done right NOW to what can wait for a bit.

5. Put the work DOWN

For some of us we can make our own hours, like myself. For others, you don’t have that luxury. But if you DO have the option, carve out at least 1 hour during your day to do something for you. Something that makes you happy. You’ll go back to work feeling refreshed.

6. Acknowledge Your Feelings

The holidays can bring up a whole bunch of emotions from sadness & loss to anger & frustration. It is OKAY. Just because it’s the ‘happiest time of the year’, does not exclude you from feeling those emotions. Forcing that “happy” on yourself can weigh you down even more.

​What I’ve learned is you can’t take care of your house, your kids, your to-dos if you don’t take care of YOU first.

Life is so darn short, kids grow fast and days grow faster.

I challenge you this season. Take more time for you and your loved ones. Maybe this is the year you start new traditions of making home made gifts and cookies and treats instead of spending the time to shop, spending that time away from laughter and joy. Or in my case, just BAKE cake!

Laugh more, live more this season. I DARE you!

 

 

Hi Friends! I’m Becky, Mama of the funnest (yes that’s a word in my world) 4 year old and the bravest Heart warrior who lives in Heaven.

I consider myself a lifestyle blogger with a focus on all the things I love and am most passionate about!

I’ve gone, done and experienced a lot in my adult life and I have a passion for sharing and serving. From losing my first daughter, Gracia, to a Congenital Heart Defect at just 82 days old, going through Cancer treatments and surgeries over the past 3 years to running 5 separate businesses as well as a non-profit organization just within the last 5 years.

And now closing a toxic chapter in my life and choosing to start over. Starting fresh and following my BIG SCARY dreams of becoming an author, writing a blog, being a life coach, videographer, continuing to grow my non-profit and putting myself out there in hope that I may inspire and help someone going through their own struggles, triumphs and hurts.

I’m a work-aholic and a stress-aholic on the recovery train to freedom! My mission is to be the best me I can be and to help other women find out what that means for them too.

Learn more about Becky:   https://www.beckyhunt.me/

 

 

 

 

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.

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