Mental Health – Catherine’s Story

As we continue the Mental Health Series, Catherine Paour, shares her story with PCHA. Misdiagnosis and a CHD discovered later in life has forced her to face anxiety and urged her to spread awareness. 

 

 

Lots of prayer, purpose and passion has prepared me for a whole (hole) new way of life! I kept asking myself, “What is holding me back from living a life I would love?” I was always struggling and complaining that my lack of energy and stamina was really in the way of me pursuing my dreams after working full-time for the last 35 years and being a single mom to a child.
I’ve been a heart patient since the age of 12, when I was first told I had a heart murmur. I can visualize that appointment perfectly.  At 23, I failed my new hire physical at a local Medical Center, and required a Cardiologist to clear me for employment. In 1997, as a newlywed, I had a Trans Ischemic Attack (TIA), or a mini stroke, and thought I was going to leave my new husband a widower.  I had a baby in 1999, and I was surprised I survived.  In 2002, after a few years of unbelievable stress, when my baby suffered Shaken Baby Syndrome in the care of a licensed day care provider, I begged my Cardiologist of the last 20 years to “dig deeper.” I explained that I simply couldn’t breathe, couldn’t carry my baby, or get groceries from my car to the kitchen. I got resistance everywhere I turned, from being called a hyperventilator, to a hypochondriac, to just plan ole lazy, until finally I was diagnosed with Heart Failure at Urgent Care of all places. Yippee. At age 42? WHAT? How come my cardiologist hadn’t told me this?
 As time went on I was getting worse, shortness of breath, debilitating edema, my legs felt like concrete pillars, my digestion was horrible, my heart was pounding outside my chest, passing out, visual disturbances, wheezing like crazy and missing out of so much of life – it was never-ending. I ended up having not 1, not 2, but 3 Cardiac Ablations, with an additional 3 Cardioversions thrown in here and there. The ER knew me all to well. I was also on a home oxygen tank for a bit, and felt like a fool walking around work with a tank on wheels –  that was not fun. My lung function was always about at 52% with no known cause. My heart failure had no known cause. I thanked God everyday for my Handicapped Parking Placard, and my parents still ran circles around me. I finally bought a used electric scooter to take my son to places like amusement parks and zoo’s, and I excluded myself from anything physical for the last 15 years. This also meant loosing friends and countless opportunities.
And then I found my SPARK!!! I started working with a Certified Life Coach, and I came away with better than I could have ever expected. I made a commitment to my self-care, to “be bold,” and to seek out a new cardiologist after over 30 years.
Within a week, my new cardiologist sat with me in a hospital recovery room, after a TEE, and asked me if I knew or had ever been told I had a giant hole in my heart. He described it as an Atrial Septal Defect – something I have lived with my entire life, undiagnosed!!!!!  My mom and I were shocked, and I accidentally loudly answered “ uh… NO?”
I very quickly was scheduled for Open Heart Surgery at a top notch hospital to repair the hole. The surgeon said the hole was the biggest they had ever seen, and he specialized in this. He said I had a huge atrial aneurysm and the septal wall was basically non-existant. I didn’t have two chambers, just one big “swamp”. The hole measured 6 cm x 3 cm.
I was hoping to have a new found life, and love for it, but I find myself really struggling emotionally with the after effects and the bureaucracy of what I have been through in this experience. Three weeks after a succesful surgery, I went down hill fast and ended up in the ICU for a week with massive heart failure. My EF was at 10%. I had extremely low blood pressure, extremley high heart rate, every tachycardia there is, AfiB, blood clots, plueral effusion. It was terrifying, but I managed through it, finding hope in my future, the Lords breath breathed into my lungs, and my sense of humor.
From there on out, I wasn’t able to return to work. I finally made it to Cardiac Rehab four months later, but  broke my tailbone sitting on an exercise machine. So I’ve been compensating for months and months, and my body is in pain from head to toe. Yet I continue to find the joy in hopefully having a healthier future, and I can’t tell you how hard it is to have every Dr. I see still to this day say, “You’re lucky to be alive”.
I’m back into working with a life coach, writing two non-fiction books, doing some public speaking and podcast interviews.  However,  I still deal with the severe anxiety about my “ex” cardiologist and how my CHD could it have been missed all this time, my fear of what would have happened if I didn’t make the bold move to go to a new Cardiologist, and WHY is my “ex” cardiologist deemed as meeting the gold standard of care in my case.
I also struggle with anxiety regarding my workplace leave of absence, disability plans and managing a less stressful life moving forward. The systems and lack of education in CHD is so prevalent in my community, and I don’t know how anyone thinks they can can endure so much trauma and bounce right back into a very stressful workplace. I’m scared. My short term disability is over, my long term disability has been denied and currently being appealed, Social Security Disability has not made a determination and my place of employment can no longer hold my position, and I am facing termination. Another surgery  is on the horion as well, as soon as authorization is received.
But there is hope, I have faith. EARLY DETECTION is key. It is also important for adult patients to find medical professionals who know how to appropriately diagnose CHD and handle adult congenital patients, without making them feel like they’re just neurotic!

 

 

 

Catherine Paour was born and raised in Torrance California, and is the youngest of 3 children. She was born Frank Breech, which caused multiple orthopedic complications throughout her early years, that has limited her well into her adult years.  She suffered with heart issues all her life and had a cardiologist for over 30 years. Her ASD went undiagnosed until the age of 55. Today, Catherine is very open to sharing her story with a hint of humor to help others who may have to experience similar circumstances and to offer hope and healing about facing open heart surgery to repair a CHD. She is certified as a Vibrant Happy Women Meet-up Coach and continues to find ways to turn her pain into her purpose.

School Intervention Series: Making A Difference

With the summer winding down, it’s just about time to head Back-to-School. Join us through a brief break from our Mental Health Series, as we re-introduce guest writer Kyle Herma, School Intervention Specialist at Children’s Hospital of Wisconsin. The blog will feature Kyle’s 3-part series for PCHA beginning with an introduction on what she does and why. Over the next couple weeks, we will share Kyle’s tips, tricks and recommendations for navigating a school’s system without a dedicated school liaison (and how to start advocating for one in your cardiac center) and provide a collection of resources on how to ensure your child is getting all of the services and supports required to have equal access to a quality education.

 

 

Twenty nine years ago my sister was born with Hypoplastic Left Heart Syndrome (HLHS), a congenital heart defect that left a chamber of her heart severely underdeveloped at birth. This was at a time when medical technology and surgical repairs options were very limited for a complex baby like my sister. While she ended up losing her battle with HLHS, her short time on Earth ultimately began a battle much greater – the fight to eradicate congenital heart defects completely and in the process, improve medical outcomes and quality of life for those currently affected. Today, the Herma Heart Center (HHC) at Children’s Hospital of Wisconsin is known for having the best published survival rates of HLHS in the world. However, a top recognition like this is not achieved without constant work towards excellence and innovation in all areas of care.

 

Herma Heart Center

How does this relate to PCHA’s “Back-to-School” theme this month? It does on so many levels! Two years ago I was a kindergarten teacher, loving every minute of every day guiding 4 and 5-year-old kids as they discovered their world. I worked in the inner city of Milwaukee at a low-income Charter School – all of my students considered “at-risk” due to a variety of different statistics. Every day I sought to plan lessons that not only were rich in academic content and student engagement, but also focused on building a classroom culture of strong future leaders and community advocates. While I had always been involved with the Herma Heart Center on various levels because of my family’s deep connection, I was a teacher. I loved being a teacher.

In 2015, I got a call. The Cardiac Neurodevelopmental Follow-Up Program, one of the HHC’s leading whole-child focused programs, was expanding and looking to hire a School Intervention Specialist after they noticed a very high need for multifaceted school intervention in students with complex health needs – specifically in the area of pediatric cardiac neurodevelopment. The job requirements outlined a liaison-type service, with the goal of working to ensure clear and consistent communication between the medical staff, the family, and the child’s school at all times. I began researching far and wide. I wanted to learn everything I could about how CHD affects a child’s neurodevelopmental functioning and what type of supports schools have in place to modify for or accommodate these children. All of my searches came up empty! There was nothing. While significant literary research supported that children with complex health needs and chronic illness are at a greater risk of reduced student engagement, higher disruptive behavior, lower academic achievement, an increased exposure to bullying (among many other well-documented negative educational outcomes), structured programs for school re-entry and intervention are rare. In this moment I knew that I needed to step in to fill a role much larger than a classroom teacher.

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The School Intervention Program officially “went live” in February 2015. I began with a very focused population of heart transplant patients that pinpointed those re-integrating into school post-transplant, but also offered intervention services to all of our heart transplant patients no matter how many years post-transplant they were. The response was huge. My patient population quickly grew to all heart transplant patients (including those wait-listed for transplant) and several patients with advanced heart failure who were anticipating a future transplant. My pilot study served 55 cardiac patients, ages 3 (preschool) to 24 (college). The schools’ concerns that were addressed included: attendance and absence support, special education support, attention and behavior plans, and documentation/medical record communication – just to name a few of the big categories. Of those 55 patients, 57% have exited the program with their school concern fully resolved, 36% still receive ongoing school intervention but are making great progress towards their school goals, and 7% transitioned to different medical centers where school intervention could no longer be followed. I’ve witnessed one of my high school student graduate with his class just 5 months post-transplant, I’ve heard from a school nurse that she could not believe a student is finally getting to live a “normal” life without any medical interventions needed during the school day, and I’ve helped a mom send her 7 year old to school for the first time because she finally felt confident the school could handle his needs.

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You may be thinking, “That’s great, but my child did not have a transplant. This doesn’t help us.” I am hear to tell you that it does! By doing a formal pilot study on a small population of patients and proving there is an urgent need for formalized school support, I am establishing both attractive outcomes data and the sustainability of this type of position. I frequently share these outcomes with colleagues, speak about my work at a director and leadership level, and even present at international conferences just so people can see how this seemingly non-medical work is directly related to improved medical outcomes. And guess what? People ARE listening.

 

Herma_Kyle

I am excited to be sharing with you over the next couple weeks to help bridge the very different worlds of medicine and education to ensure that children with complex health needs, specifically CHD, are not falling though the gaps. Here’s to a great school year!

 

Kyle Herma is the School Intervention Specialist serving the Herma Heart Center at Children’s Hospital of Wisconsin. Kyle has been at Children’s since February 2015 conducting a formal pilot study on school intervention and the impact it has on a child’s overall medical outcome and quality of life. Prior to this position, Kyle was a teacher at Milwaukee College Prep’s 38th street campus. In both roles, Kyle has shown her dedication to serving children who are placed at-risk for school failure and ultimate mission to achieve equal access to quality education for all.

Mental Health – Beyond Survival, The Struggle with Anxiety and Depression

PCHA continues its series on Mental Health with a piece by nurse and PCHA -VA Board Member, Sydney Taylor. Here, Sydney discusses contributing factors and prevalence of Anxiety and Depression in patients and parents affected by Congenital Heart Disease. 

 

 

 

For the first time ever, there are more adults living with a congenital heart defect than children. This is all thanks to improved medical technology, amazing advancements, and increased knowledge and awareness in treating CHD. While this is certainly incredible news, there are new – and unanticipated – aspects of treating survivors that we must now focus on.

When the field of pediatric cardiology was born, the main goal was to keep patients alive. The beginning

of this delicate science was unfortunately wrought with struggles in patient survival rates. However, as time went on and improvements and advancements in the field were made, patients started living to reach adulthood. But still, the main focus was to simply get these patients to see age 18. On the whole, aspects of everyday life patients may struggle with were – and sometimes continue to be – unaddressed by providers simply due to a lack of research and knowledge.

One of the biggest (and most prevalent) concerns facing CHD survivors is anxiety and/ or depression. Frequent and lengthy hospitalizations, painful procedures, and traumatic surgeries in childhood often lead to profound psychosocial impacts on patients. These impacts can range from minor to life-altering. For example, I can always feel myself becoming anxious when I smell rubbing alcohol or “hospital smell.” I remember this anxiety from childhood, but it had typically been isolated to healthcare-related environments. However, it made the beginning of nursing school and working in the clinical setting very difficult. Other patients may avoid seeking medical attention due to this anxiety, turn to substance abuse to cope with depression, or experience any number of ineffective coping strategies due to a lack of recognition of their unique needs in the medical community.

In a particular study done in adults with CHD, researchers selected patients who appeared to be “well-adjusted”; that is, did not outwardly exhibit signs and symptoms of depression or anxiety. Despite their appearances, 36.4% were found to have a “diagnosable psychiatric disorder, with anxiety or depressive symptoms being prominent [1].” Another study revealed that 18.3% of adolescents (age 12-18) with a heart defect suffer from depressive symptoms, compared with 3.3% of the healthy control group. Additionally, 30% of the adolescent CHD patients displayed anxiety, compared to 10% of the healthy control group [2].

Patients are not the only ones to suffer psychological distress related to their heart defect and treatment experience. Parents of CHD patients are also at risk, and possibly experience greater distress than their children. In a recent study done by the American Heart Association, an estimated 25%-50% of parents experience symptoms of depression and/ or anxiety, “and 30% to 80% reported experiencing severe psychological distress [3].”

There are more factors at play in the development of depression and/ or anxiety in the CHD population than you might think. Patients with more complex defects seem to be at a higher risk of developing anxiety and depression, and interestingly, those who undergo more cardiac catheterizations than others [2]. It has also been theorized that separation from parents due to early life-saving interventions shortly after birth may contribute to psychosocial abnormalities. Other researchers have postulated that early exposure to traumatic events (such as open-heart surgery) contribute to the development of ineffective coping mechanisms later in life. Most intriguing is recent evidence suggesting higher rates of cerebral insult secondary to cardiac dysfunction in CHD patients: in one study, 24% of infants had abnormal brain scans prior to surgery, and a staggering 67% had abnormal brain scans after surgery [4]. Literature is even more scant regarding parental anxiety and depression, but older parents and unemployed parents seem to have a higher incidence of depression [4].

If you or a loved one are struggling with anxiety or depression, it is important to speak with a healthcare provider. Now that heart patients are surviving, we need help in thriving. Don’t be afraid to reach out to someone you trust. You are not alone, and you matter.

 

For an additional guide on symptoms, tips, and when to seek help, please visit PCHA’s Educational Resource on Mental Health. Although this guide addresses parents in particular, the guidance it provides can be applied to patients experiencing difficulty with andxiety, depression, and post traumatic stress, as well. 

 

 

References:

1. Bromberg, J.I., Beasley, P.J., D’Angelo, E.J., Landzberg, M., DeMaso DR. (2003). Depression and anxiety in adults with congenital heart disease: a pilot study. Heart Lung, 32(105–110).

2. Awaad, M. & Darahim, K.(2015). Depression and anxiety in adolescents with congenital heart disease. European Psychiatry, 30(1), 28-31. doi 10.1016/S0924-9338(15)31916-7.

3. Woolf-King, S.E., Anger, A., Arnold, E.A., Weiss, S. J., Teitel, D. (2017). Mental health among parents of children with critical congenital heart defects: A systematic review. Journal of the American Heart Association, 6(2). doi 10.1161/JAHA.116.004862.

4. Pauliks, L. B. (2013). Depression in adults with congenital heart disease-public health challenge in a rapidly expanding new patient population. World Journal of Cardiology, 5(6), 186-195. doi 10.4330/wjc.v5.i6.186.

 

 

 

Sydney Taylor is a congenital complete heart block survivor, registered nurse, and is the Adult CHD Board Director for PCHA-Virginia. She has required pacemaker therapy since she was 15 hours old. She enjoys coffee and a good book, hiking and kayaking in the beautiful Shenandoah Valley area, visiting national parks, and making friends with any and all dogs.

 

Mental Health – Courtney’s Story – A Mom’s Perspective

As PCHA continues  our current series, Courtney Kile  shares with us how her experiences as Heart Mom to son Sully impacted her mental health.

I still remember the first time it happened. The surgeon had come in to tell us that the open heart surgery performed on my 3-day old son, was an outstanding success.  I remember looking at the surgeon calmly and saying, “Thank you very much,” and I turned on my heel and nearly sprinted down the hallway.  My mom and step mom followed close behind, calling my name, but my brain wasn’t computing her words.  I grabbed the door to the lactation room at the end of the hall and swung it open to close myself inside.

Then I completely and totally lost it.

I sat on the sterile vinyl chair, hugging myself, sobbing, and I think I even drooled a little.  I’d been holding in all these emotions, trying to process everything that happened in the last 30 hours and once I knew he “safe”, I couldn’t hold back anymore.  Little did I know, this would be the start of a near constant internal battle.

My son Sully was born in November 2011, seemingly healthy.  Just 36 hours later, local doctors discovered a Congenital Heart Defect and he was airlifted to the larger, metro hospital.  The 3rd day of his life, a team of doctors and surgeons operated on his 6lb. 8 oz. body. After a spectacular recovery from his first surgery, we handed him off to the surgeons again just 6 months later.  That surgery was also a screaming success.

I considered myself a pretty together person.  I felt like I handled stress well and had healthy coping skills (scarfing a king size Reese’s is a healthy coping mechanism, right?).  As time went on, I noticed myself beginning to change.  When we were getting ready to leave the house for any reason, I would retch and gag, and sometimes even vomit.  I would get sweaty and nervous, and I had no idea what was going on.  If I wasn’t with Sully, I would think of all the horrible possibilities that could happen.  I would replay these scenarios in my head until I would end up in a crying ball on the couch.  What was wrong with me?  Everything was fine, but I just couldn’t figure out what was going on.  I was too scared to talk to anyone about it.  I have a job that keeps me in the public eye.  What if they thought I was crazy?  What would my family think?  I knew it wasn’t normal to need a gallon of Pepto just to go and get groceries.   I’d dealt with a mild form of anxiety all my life, but never like this.  This was different.

Right after Sully turned two, a friend a mine sat me down for a frank discussion.  Being a mental health practioner, she’d seen this before.  She told me that she wanted me to talk to my doctor.  I tried to blow her off, but she pushed further.  Then she said it, “I think you have PTSD.”

Umm… what?

I was stunned.  I wasn’t in the military and hadn’t been through a war.  How could I possibly have PTSD?  There had to be another explanation.

Even though I thought my friend was way off base, I decided to call my doctor.  After talking to my doctor and being referred to a therapist, it was confirmed; I had Post Traumatic Stress Disorder (PTSD). The diagnosis was hard to swallow.  I blamed myself for not being able to control my emotions and thoughts.  I felt crazy.

Then, I started talking to other parents who’d been through the same or similar situations.  It was during these conversations that I realized that I wasn’t alone.  All these other parents had feelings like I did.  Though none of us had been deployed to a warzone, we were on the frontlines of our own; battling for our children’s lives. We’d waged war against catastrophic medical conditions that threatened to kill our children; and sadly, some of those medical conditions took the lives of some sweet warriors.

With a newly restored hope, I decided to talk more about PTSD and mental health.  There is such a stigma surrounding mental health issues.  The world is judgmental, that’s a fact, but we cannot stand silent in battle alone.  We need to talk about it.  We need to let the masses know that this is a real struggle that stems from healing wounds.  Seeing your child hooked up to 20 IV racks, with chest tubes, and a ventilator, is not something you can easily get over.  When you face the mortality of your child, you change. It is something that rocks you to the very core of your being.

The biggest thing I’ve learned since my diagnosis is self-care.  I can’t be the mom Sully needs unless I take care of myself.  Admitting you need help can be tough for people, but it is a necessity.  You can’t do this alone. Do not be ashamed.  We are in this together.

 

I’m happy to report that Sully is almost 6 years old now.  He is starting kindergarten in the fall. He’s happy, healthy, and the joy of our lives.  As for me, I’ve learned tips and tricks to manage my anxiety and panic.  Don’t get me wrong, I’m not perfect.  There are still times that leave me in a nervous mess, reaching for my inhaler.  But those times are few and far between. Every day I choose to take care of myself and not let PTSD control me.

 

 

 

 

 

Courtney Kile hails from Duluth, MN.  She is the mom to an amazing CHD warrior named Sully and wife to Robert.  Courtney and her husband run Project Heart to Heart, a Minnesota based non-profit serving families who have children born with Congenital Heart Defects. She is a paralegal by trade and uses her skills to help CHD families.

Patient Engagement Tools: Comprehensive Single Ventricle Roadmap

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

 

Patient Engagement Tools: Comprehensive Single Ventricle Roadmap

Congenital Heart Disease (CHD) impacts about 40,000 newborns a year in the United States. Single ventricle defects are a complex subgroup of CHD, affecting approximately 5 out of every 100,000 newborns. In addition to normal pediatric and adult primary care needs, these patients are impacted by frequent follow up, complex testing, screening, re-interventions, surgeries, consulting providers, and care throughout a lifetime. This care not only impacts the patients, but the entire family system, including siblings, parents, grandparents, and extended family. The Comprehensive Single Ventricle Roadmap is a novel idea stemming from the persistent questions families have brought forward trying to understand the process of single ventricle disease over time and its neurodevelopmental effects.

Speaking from my personal experience as a younger sibling of a single ventricle patient, this kind of guide has been desperately needed for many decades. The first “blue babies” were given options for a better quality of life, and as research and outcomes have improved, these patients are now living into adulthood. Since arriving at Lurie Children’s Hospital in 2016, I am pleased to see the transformation of the idea evolve so promptly into a formal patient engagement strategy (available in English and Spanish) under the guidance of our entire team. I am specifically incredibly grateful to Dr. Kiona Allen and Amelia Aiello who agreed with this vision, making it a reality for patients and families.

Fontan Roadmap

https://www.luriechildrens.org/en-us/care-services/specialties-services/heart-center/programs/single-ventricle-center-excellence/Pages/single-ventricle-roadmap.aspx

Guiding Families Through the Journey

Now that you have been introduced to the roadmap, imagine yourself learning about the diagnosis of single ventricle CHD prenatally. The typical excitement and thrill of learning you are on a road to being a parent of a healthy new baby is not the same joy for parents and families faced with an incurable single ventricle disease. The stops along that road and the topics you’ll discuss are critical, important, costly, and personal. The unknowns, outcomes, and trajectory of this road trip are overwhelming to comprehend. Emotions and fears are often high, breaking down the normal anticipatory excitement and joy.

The Comprehensive Single Ventricle Roadmap is not a pathway any parent eagerly seeks out; yet, it is essential to living life with single ventricle disease. It requires thoughtful planning in an already busy family life schedule to organize the daily care that must be performed seamlessly within the diagnosis and treatment of single ventricle disease. This population is only several decades old; thus, the unknowns within single ventricle care are many. Investigating the latest research outcomes is an essential part of the journey — this includes understanding and coping with the lack of care options. Medical science and care have often not evolved fast enough to benefit children with single ventricle disease. Discussions with families about the surgical and other milestones on the journey are not easy conversations. The unique framework of the roadmap provides a visual guide, allowing families a way to understand the disease process. It also allows for valuable transparent discussions about opportunities for positive coping, hope, and fostering resilience along individual family’s pathway.

The Roadmap is not a “cookie cutter” framework meant to fit every family’s story completely; no two patients (and families) will have the same journey. However, it helps families visualize and more fully understand what care throughout a lifetime looks like, and allows families to anticipate major milestones in a specific time span of a child’s life, such as the newborn surgery. Identifying this point in time allows for transitional discussions regarding navigation in and out of acute and chronic care. These conversations often raise questions about the acute issues currently present for the patient and allow opportunities to explain our other patient engagement tools. For more helpful tools follow: https://www.luriechildrens.org/en-us/care-services/specialties-services/heart-center/programs/single-ventricle-center-excellence/Pages/home-monitoring-program.aspx

Typical questions from families during the newborn surgery period include:

  1. How will I be able to care for my newborn after surgery?
  2. What is home surveillance monitoring and will I be able to breastfeed?
  3. What if I need to go to the ER or another health care provider?
  4. What does follow up look like in the HeArT clinic (High Acuity Transition Clinic) and the pre-Glenn visit?

Not all stop points are anticipated. A couple examples of unplanned cardiac triggers across a lifespan include a 12-month-old s/p Glenn with moderate to severe AV valve regurgitation failing to thrive on medical therapy. Because of the cardiac issues, this patient moves into the blue circle entitled additional procedures. This may include potential re-operation for valve concerns before the anticipated next surgery in the journey, the Fontan operation. A second example is a 15-year-old s/p Fontan with arrhythmias requiring placement of a pacemaker/AICD that moves into the additional procedures post-Fontan for arrhythmias not responsive to medical management. Lastly, a 40-year-old s/p Fontan with failing function requiring listing for transplant that moves into the additional procedures post-Fontan and in essence trades one disease state for another (single ventricle physiology for transplant).

Striving for Anticipatory Guidance and a Successful Transition to Adult Care

One goal of the roadmap is to provide cardiac anticipatory guidance for families on the normal developmental milestones in life (marked by schoolhouses and graduation caps) and indicates the need for continued cardiac neurodevelopmental screening. The roadmap creates a framework to discuss difficult topics, potential complications, disease trajectory, issues that develop because of single ventricle physiology, and new cardiac concerns. When new issues develop that require attention, we have open conversations with the family that outline goals, medical options, surgical palliation and outcome statistics. Included in the conversation is a diagnosis review utilizing images that are tailored to the child’s individual anatomy to explain the current anatomy and potential next phase of the child’s journey.

The second goal of the Comprehensive Single Ventricle Roadmap is to foster developmentally appropriate health-promoting behaviors as our patients transition to adulthood to enhance the longer term quality of life. In the early-late teen and adult years, decision-making shifts from primarily parent-driven to patient-driven. This can be challenging for all involved. The milestones on the roadmap visually guide patients and families along the valuable process of each child’s maturation, identifies opportunities for transition of care from parents to patients, and highlights ongoing surveillance monitoring of the many consequences of Fontan physiology to achieve the ideal outcome with the best quality of life. This process is individual for each patient and evolves over time. Success is achieved when coordinated, developmentally appropriate, and psychological supportive care creates patients that advocate for themselves in adulthood and maintain the most positive health promoting behaviors in lifeTo see more on developmental Milestones follow: https://www.luriechildrens.org/en-us/care-services/specialties-services/heart-center/programs/single-ventricle-center-excellence/Documents/developmental-milestones.pdf

Lurie Children’s Hospital has a creative way of facilitating this transition within the Single Ventricle Program. The pediatric single ventricle clinic overlaps monthly with the single ventricle adults being seen in the Adult Congenital Heart Disease (ACHD) program. This allows for collaboration, a slower transition, and a formal hand off of care over time instead of a more rigid fixed timeline. To learn more on our website, follow: https://www.luriechildrens.org/en-us/care-services/specialties-services/heart-center/programs/single-ventricle-center-excellence/Pages/index.aspx

 

 

Michelle Steltzer has 20 years of nursing experience in fields from oncology to pediatric cardiology. She received both her bachelor and master’s degrees in nursing from the Univeristy of Wisconsin-Madison.

Michelle had a critical role in the development of the first home surveillance monitoring program for pediatric cardiology patients way back in 1999. She then worked collaboratively with the Joint Council on Congenital Heart Disease Quality Initiative while employed in Boston. Michelle expanded feeding protocols within congenital heart disease to include breastfeeding.

In addition to having worked at Boston Children’s Hospital and Children’s Hospital of Wisconsin, Michelle now works as a pediatric nurse practitioner at Ann & Robert H. Lurie Children’s Hospital of Chicago. Growing up with a sibling with a CHD, Michelle learned by experience and by watching her mother just what services were lacking for CHD families.

 

American Stroke Awareness Month – Terese’s Story

For the month of May, PCHA has been focusing on American Stroke Awareness Month. In the 4th week of our series, Terese Quarino shares her experience with stroke.

 

I remember the day, like it was yesterday…. The Wednesday before Labor day weekend, I called my cardiologist to say I felt some funny beats in my heart, but no other symptoms.  He said he would send me a holtor monitor overnight to wear for 24 hours. I did exactly what he told me to do, and when I received a call from him late on a Saturday night, a holiday weekend, I knew something was up!  He told me to pick up two medications at the pharmacy that night, start taking it, and that if I have any fast heart rates, go straight to the ER.  I agreed.  If I didn’t see him in the ER, I would see him first thing Tuesday morning in his office.  He explained everything  to me on the phone, but it came out of the phone like Charlie Brown talking  (Mwa-Mwa-Mwa sound).  I hung up the phone and didn’t tell my parents anything, except that I needed to pick up the medications and be at my doctors office Tuesday morning.The rest of the weekend went fine and we went to my cardiologist office Tuesday morning.

As soon as I arrived, I had an EKG, an echo, and blood work done.  It was determined that I was in heart failure. My heart rate was at about 300 beats per minute.  I met with an electrophysiologist and my cardiologist to determine what would be the best course of treatment.  We decide cardiversion would be the best route. Since I had already eaten breakfast that morning, we would have to wait until the next day.

Wednesday morning, my doctors started with a TEE (Trans-Esophageal Echocardiogram) to see if there are any blood clots that they could see before they performed the cardioversion.  When they didn’t find anything, they moved forward with the cardioversion. After the procedure I was feeling great.  The doctors decided to keep me overnight, until my Coumadin was at a therapeutic level. One of my parents always stays with me after any procedure or surgery.  I told my parents I was fine, and they could go home. Twelve hours after my procedure I had  stroke.

My nurse was in the room, checking on me, and I could not speak. Then my arm started to bend, and it went behind and arched my back. Next, I could not feel anything in my hands or feet.  The nurse left the room, and I remember thinking to myself, “why is she leaving me?” But looking back, I realized she pushed a button before she left. Seconds later, a rush of doctors and nurses came to my rescue.   If it was not for their quick response, the lingering symptoms from my stroke may have been worse.

After my stroke, I had cognitive, physical,and speech therapy, which I’m truly thankful for. I will be honest, I struggled with it! But, step by step, I got through it! I still struggle with how my stroke impacted my everyday life. My handwriting is horrible, it’s significantly worse than before my stroke.  I also have a hard time with recall. At work, when I need to speak at a meeting, I would jot down notes to prepare. Now, if I just write it down, I may not remember what I need to say, so instead I type ord for word in the note section of my phone, so I can read it.  All these issues add up to big issues.

Having a stroke has changed my outlook on life. It has taught me to live everyday to the fullest! My experience with stroke was quite a scary time for me, and I will never forget  how it felt!

 

 

 

Terese was born with Tetralogy of Fallot in 1973, which was repaired in 1974 at Children’s Memorial Hospital in Chicago, IL. In 1992, she had her second open heart surgery, and mitral valve replacement. Terese’s other medical conditions include a lumpectomy in 1990, and multiple treated aneurysms. She also started dealing with rhythm issues, and still is to this day, which required a cardioversion in 2007. Twelve hours after her cardioversion, Terese suffered a stroke. She has also had two ablations, and had a pacemaker implanted in September 2014.  Since then, Terese’s medical history has been further complicated, in recent years, by being diagnosed with Lupus (SLE) in 2013 and Shrinking Lung Syndrome in 2015. In her free time, Terese loves spending time with her family, friends, and her puppy, Payton. She also enjoys swimming and listening to great music.  She lives each day to the fullest!

 

 

 

American Stroke Awareness Month – Molly’s Story

 For the month of May, PCHA will be focusing on American Stroke Awareness Month. In the 3rd week of our series, Alison Ogden shares the story of her daughter Molly’s stroke at age sixteen.

 

Molly awoke for school on November 5, 2012 as if it were any other Monday in her Sophomore year of High School.  But life suddenly changed that morning, as her mother, Ailson, found her collapsed and unable to speak.  Even though Alison knew the signs of stroke, she didn’t recognize them in her physically fit, distance running 16 year old. Fortunately, the first responders did. The local hospital determined Molly had suffered a massive stroke following a dissection of her carotid artery.  After a scan was performed it was confirmed that Molly had a clot in the brain.  She was transported to a larger hospital where she would spend the next month and undergo a series of procedures designed to save her life. 

She was then transferred to an inpatient rehabilitation hospital in a neighboring state where she would spend the next 2 1/2 months.  She returned home on February 12, confined to a wheelchair and still unable to say more than a few words.  She started out-patient therapy right away.  Her determination and drive, along with her positive attitude and faith, kept her focused on recovery.  She returned to school on a part-time basis in April while still spending many hours in therapy each week. 

By taking a full load of classes, working closely with the help of a para-educator and devoted school staff, she graduated with honors, on time, with her class in May of 2015!

Molly has attempted college classes, but, because of her aphasia, she found them to be extremely difficult.  She still struggles to regain communication, her right hand and foot don’t work the way she would like, but she is determined to continue therapy and still sees improvement.  Molly brings joy to every situation she is in, including the volunteer work that she loves.  She is open to talking about her situation and feels that spreading awareness of the signs and symptoms of stroke is important. While caring for Molly, Alison has been inspired by her attitude. She has learned from her daughter to look for the best in all people and to live life to the fullest each and every day.

 

 

Alison grew up in Kansas City. She and her family, which includes her husband, Brad, daughter, Molly and son, Graham, has recently moved to central Oklahoma, where they live on a cattle ranch. Alison is a recruiter for Jos A Bank and feels lucky to work from home! Her husband owns a home restore/remodel business, with which Alison also assists.

American Stroke Awareness Month – The Asher James Foundation

For the month of May, PCHA will be focusing on American Stroke Awareness Month. In the 2nd post of our series, learn about the Asher James Foundation, founded by Jen DeBouver after the loss of her son Asher.

 

In October 2012 our whole World changed. The outcome of our son’s life was about to change. On October 5th our son Asher looked like a beautiful, somewhat healthy baby boy who was recovering from his heart surgery. He had been doing well when it came to his heart related procedures. He had gone through a fetal intervention, months before being born, a cath procedure at hours old, and then his heart surgery. He had a few minor hiccups along the way, but for the most part was doing well with recovery, so we thought.

On October 6th, Asher was red and puffy. My husband questioned the doctors as to why he was so red and puffy, but they didn’t really have an answer. It was the weekend, not much was really done to get one. By Monday, we really had no answers until finally they told us he had blood clots. The clots were in both jugular veins, and both arms.

It’s funny how dates stick with you. October 8, 2012. The day my son got his death sentence. When they told me he had blood clots, that is exactly what I thought because I didn’t really know much about them. What I thought I had heard was that they were deadly. And. They. Are.  I had no idea babies could get blood clots.

We met with the Hemotologist and she started him on different blood thinners, but she explained to us that there were no medications for children, especially infants when it came to blood clots. She reached out to colleagues across the country to find a treatment plan for Asher. Adult medications was the only option. We began treatment and it seemed to be working.

And then it wasn’t. The clots would redevelop.

Sadly, on October 23rd, Asher would lose his battle with these blood clots. Asher was a fighter and SO strong. He battled these clots for over 2 weeks. He inspired me with how much he fought, so before we even left the hospital, I had decided, in my shocked, grief filled state that I wanted to start a foundation in his memory. I wanted his fight against blood clots to continue.

So a few months later, Asher James Congenital Heart Disease & Thrombosis Foundation was created. I knew I didn’t want another parent to be shocked that their child could get blood clots. I wanted them to receive information while they were in the hospital of signs and symptoms of a blood clot. We started working with groups that deliver care bags to CHD patients and supplying them with our signs and symptoms cards.

Asher James Foundation has worked with Ann and Robert H. Lurie Children’s Hospital on spreading awareness in children’s blood clots by sponsoring Children’s Blood Clot Awareness Day. We have also been to Washington D.C. to advocate on children’s blood clots.

 

Each year for Asher’s birthday we hold a softball event where we play Chicago style softball against our local PCHA IL group. It’s a fun day remembering Asher, spreading awareness, and collecting things to donate to the CHD and blood clot patients in the hospital. We usually sell stuffed giraffes and donate one with each purchase. This year we are doing a book drive to collect giraffe themed books to donate to the patients. Asher would have been going to Kindergarten this year, and I’m sure he would have loved reading books.

 

A lot of research has been done in the past 5 years and treatment in children’s blood clots has come a long way. There are better treatments and survival ratings than when Asher was in the hospital. We had the privilege to meet with researchers at the University of Iowa to learn about some of the advancements.

If you provide care bags to hospitals where children could be at risks for blood clots and would like to add our signs and symptoms cards, please reach out to us and we would love to send you some. www.asherjamesfoundation.org.

 

 

Jen is a bereaved mom of 2, Olivia and Asher and a rainbow mom to Nina. Jen and her husband JD started Asher James Congenital Heart Disease & Thrombosis Foundation after losing Asher to provide support and awareness in children’s blood clots. In the past, Jen was the bereavement coordinator for Mended Little Hearts National and Mended Little Hearts of Chicago She decided to take a break to focus on her rainbow after all. She continues to do projects such as Grief: A Mother’s Project and currently the Rainbow of Hope Project. Each year she and her husband hold a softball event for Asher’s birthday, which includes collecting something (stuffed puppy or dog or books) to donate to CHD patients at Lurie Children’s Hospital. She also works with her sister-in-law to collect blankets for Olivia’s Blankets in memory of Olivia.

Jen is very open about her losses, and feels that by being open she can help others through speaking about their own loss or help others to learn how to be sensitive to those that have gone through a loss.

Donatel Life Month – A Tribute to Triston

For the month of April, PCHA has been focusing on the theme of National Donate Life Month. In the 6th post of our series, Charity Robert shares a tribute to the boy whose heart saved her nephew Jacob’s life.

 

October 21, 2015 is a date to remember for 2 very special families.  This date marks an event that would forever change the lives of many, yet also forever combine two families that live very far apart.  It’s a date that was not planned, not foreseen, nor marked on anyone’s calendar.  This particular date was meant to be, yet unknown to anyone.  You see, when someone is waiting for their future to open up, sometimes another person has closed it forever.  We don’t know when or how or who’s future will end, but we do know that when someone is on the organ transplant wait list, this is the inevitable outcome we have to hope for.  October 21, 2015 Jacob Kilby got the call.  Jacob received the news he and his family had been waiting for, there was a perfect match…..a match that Jacob desperately needed in order to glimpse a future for himself.  A donor heart was going to be transplanted this night so that Jacob could walk into his future.

 

Triston “Shorty” Taylor Green was born on November 16, 1995 in the state of Alaska.  Triston is known for having a “wild” side growing up, yet had a loving and caring side that won over hundreds of friends.  Triston decided to attend college at the University of Nevada where he lived his life to the fullest each and every day.  Many friends would call him by “T.Dog” or “Shorty”, as he was musically inclined, while others knew him by “907SquadGod” on social media for being a unique, individual music writer, rapper, and beat mixer.  Some of the memories I’ve heard contain the “fearless” ways he enjoyed his life, such as motorcycle riding while being chased by police, a move that won the heart of his beautiful girlfriend.  Triston had the handsome looks, the genuine love of people, and that bit of bad boy personality that many people find attractive!  I’ve heard that he listened to friends and encouraged everyone to be the best version of themselves, often quoting “Nobody can tell you how to be yourself, you are the best you.  Appreciate being an individual and do you!”  Triston called himself “The Man” often and on October 21, 2015 he became just that for Jacob.  Just 3 days prior, Triston Taylor Green passed away from an accidental gunshot wound.  Triston had previously made the decision to become an organ donor,  a choice that was made of his own doing, and one that has proven heroic.  Yes, Triston Green, you are definitely “The Man” in the eyes of Jacob Kilby and his family.

The event that took place on October 21, 2015 may not resonate with the everyday individual.  Organ Donation and all that it entails may not necessarily seem important or relatable at this time in everyone’s life.  It’s very difficult to talk about losing a loved one or to even think about the unimaginable trauma one might endure in a traumatic loss such as the life of Triston.  Believe me, the need for a life saving organ transplant is bittersweet when you have to hope for someone’s loss so that you may gain another year with your nephew, as I have had to do for Jacob.  Please know that if Triston had not thought about life after death, my nephew may not be here today.  I can only think that without the loving family and friends that created this unique young man, organ donation may not of been agreed upon by Triston.  On October 21, just 2 years ago 2 young men, smart beyond their years were joined together with one healthy heart.  My nephew has a future today and Triston will be loved, remembered, and treasured to infinity and beyond!

 

 

 

Charity Roberts is the youngest sister of Jacob Kilby’s mother, Mellissa Johnson. Charity became an active advocate and supporter of CHD and organ donation awareness when Jacob was awaiting his second heart transplant in 2015.

Donate Life Month – Choosing to give the Ultimate Gift

 

For the month of April, PCHA has been focusing on the theme of National Donate Life Month. In the fourth post of our series, Emily Inman shares the story of the day her family decided to give the gift of life, after her mother’s unexpected passing.

 

 

My mom had just gone back to work after taking off another long stint under the Family Medical Leave Act. She worked the 3pm – 11pm shift as a secretary at a trucking company. This meant I was back spending my evenings and parts of my nights at my grandparents. I didn’t mind. Grandma spoiled me. But I was used to having my mom around since she took off so much time under the FMLA. I was waiting for a bone marrow transplant. I didn’t have a match because I’m an only child, and, as luck would have it, no one in family was closer than a half match. I had just undergone several rounds of chemotherapy, steroid treatments, and an experimental stem cell transplant. I was still in reverse isolation and unable to attend school and activities.

I admit, it was a little scary not having her there even though I was at Grandma’s. She would call me during her lunch break to check on me and what not. On this particular day, the phone didn’t ring. Fifteen minutes into her lunch break it still didn’t ring. I knew something was wrong. I asked my Grandma if I could use the phone to call her. She said no. I defiantly used Grandma’s bedroom phone to call her office. One of the office ladies made up some story about how she couldn’t find her. Now I definitely knew something was wrong. About 5 minutes later the phone rang. Grandma picked it up. I deviously picked up the phone in Grandma’s bedroom to listen in on the conversation. I couldn’t believe my ears: “Mrs. Vasquez, we found Patty passed out at her desk. She was rushed to the hospital. You need to get there as soon as possible.”

The next couple hours were a complete blur. I don’t know if they were a blur from all the commotion, from my young brain trying to block it out, or from all the cancer drugs I was on. The next thing I remember is walking off of the elevator and down the hall to the ICU at the hospital. My dad, who was an over-the-road truck driver at that same company, was sitting there still wearing his Carhartt and covered in dirt and oil, with his hands over his face. I had never seen him cry before. And there he was. Beet red and crying like a baby. The nurse came in and explained to me that she had a brain aneurysm. She was basically brain-dead by the time she got to the hospital. We walked over to her room. I peered in the glass and she was laying there lifeless. Tubes, wires, and equipment were everywhere. If I close my eyes, I can still see her laying there with the breathing tube in her mouth. I said goodbye to her. And I thanked her for being the best mom I could ever ask for.

Grandma took me home, gave me all of those cancer drugs no child should ever have to take, and put me to bed. Behind the scenes, the pediatric cancer doctors we were working with at the University of Wisconsin-Milwaukee flew down by helicopter and harvested her stem cells in case I never found a bone marrow match or the experimental stem cell transplants didn’t work. The doctors then pulled the plug. Patricia A. Inman passed away March 1, 2001.

My mom helped to organize multiple blood and bone drives not only in hopes of finding a bone marrow match for me, but for finding matches for all others out their searching for their continued gift of life. We learned that what made it so hard to find me a bone marrow match was that I am of fifty percent Hispanic/Latino origin. My mom made it her mission to spread awareness about minority blood, bone marrow, and tissue donation, and to register as many people as she could to donate.

She then became an organ donor and her heart, both eyes, both kidneys, both lungs, liver, and pancreas were all donated. They went to recipients ranging in ages from 7 to 73. The mom of the 7-year-old boy who received one of her kidneys sent us an update on his condition right after his transplant. I was elated to read it. He was doing well and his prognosis looked positive. He liked to play baseball and couldn’t wait to be back out on the field. She asked if I would mind sharing something about my mom. The 73-year-old woman who received her heart also reached out and asked if I would mind sharing something about my mom. I shared that she is dancing in heaven knowing her organs went to so many people. She devoted her life to giving back, and now her afterlife is devoted to giving back as well.

 

 

 Emily Inman is originally from the Chicagoland area, and is an only child from a large Latino family. She was diagnosed with a very rare form of bone marrow cancer, called aplastic anemia, when she was 10-years-old. Emily needed a bone marrow transplant, but never found a match. She received several experimental stem cell transplants that ultimately saved her life. Emily’s mom, Patricia, passed away from a brain aneurysm while Emily was still undergoing treatment. She grew older, entered remission, and eventually went back to living a “normal life.” Emily received a B.A. in Journalism & Mass Communication and Global Health Studies from the University of Iowa. She stayed at U of IA and got her Masters of Public Health in Community & Behavioral Health, as well as Health Communication with a concentration in Cultural Compentency. Emily is now serving her second term with the Illinois Department of Public Health AmeriCorps. She serves as the food access for an organization that conducts homelessness prevention and intervention in the South Suburbs of Chicago.