Gary and I went to our routine, 20 week ultrasound for the anatomy scan of our little nugget. We were excitedly watching our sonographer go through her hands, feet, arms, legs, kidneys, liver, then her heart. She finished and told us to wait in the room, while she went to talk to the doctor. We waited impatiently for her to come back. Gary and I were getting anxious, because we had to get to our restaurant, Kasey’s, to open for the day. When our sonographer finally came back, she brought the doctor. (Uh-oh!)
He said, “I hate having to tell parents this, there appears to be a defect in her heart.”
Our hearts dropped. Surely, there was a mistake. He referred us to see a Pediatric Cardiologist for a definitive diagnosis. Two days later we were at UNMH, with Dr. Goens, doing another ultrasound. Afterwards, she told us our baby girl had Hypoplastic Left Heart Syndrome (HLHS). After many discussions and many, many, questions to Dr. Goens and one of her nurses, Pamela, we were going to Children’s Hospital of Colorado to have our first baby. We were to live there through her first two planned surgeries, for about six months. We closed our restaurant on June 30, 2017. I was flown up to Denver on August 11, while Gary drove with my mom in our fully-packed car.
Katherine was born on Monday, September 11, 2017. I got to hold her for about one minute, before they took her away to do an echo and get the umbilical lines in. She slept for about three days, because of the medicine she was on, and had her first open-heart surgery when she was four days old. She did great. She had a rough first night post-surgery, but her recovery after that night went really well. She is a fighter!
We lived in the hospital with her for three weeks. We took her “home,” to the Ronald McDonald House, on October 6 and struggled with her eating until January. Before her planned second surgery, we were re-admitted to the hospital three times; once for an irregular EKG, once for blood in her stool, and once for her low/almost no weight gain. We fed Katherine every three hours, religiously, for four months. Katherine had her first Cardiac Cath just after Thanksgiving, where they determined she needed a stent in her BT Shunt (that was put in during the Norwood, first surgery).
Katherine then had her Glenn procedure on January 17, 2018 and woke up a different baby! Her first bottle after the surgery was almost four times what she was eating before the surgery. She was awake, alert ,and wanted to play and eat! However, she was not able to keep her oxygen level up in the good range, so she had another Cardiac Cath; they determined she needed a stent put into her Inferior Vena Cava (IVC). We got to take her home, to Albuquerque, on February 7, 2018. During the last Echo in Colorado, the doctor found that Katherine’s Tricuspid Valve was moderately leaking, but they would keep an eye on it and determine what needed to happen down the road. We made one more trip to Colorado for a Cardiac Cath in May of 2018, where they put a stent in her Left Pulmonary Artery. Since we’ve been home, Katherine has been having a great time. She loves to play and laugh, and she is pulling herself up on everything now! It won’t be long before she’s walking!
This week we are continuing with the theme of summer fun! Today we’ll hear from Margaret King, a Heart Mom, who spends her summer hanging out with her fun-loving son.
In the CHD life, sometimes it’s hard to get a break. While summer brings a brief reprieve from school expectations and IEP goals, we CHD families have our own challenges: from trying to cram appointments and therapies into the months when our child is off school to watching some of our children struggle with the loss of their usual routines and social outlets, navigating new extracurriculars staffed by people unfamiliar with our child’s diagnosis, and dealing with hot weather and physical activities that can be too strenuous for our kids.
Yet, summer is a wonderful time to experience new places and try new things that can open the windows of self-discovery, create family memories that will last a lifetime, and even increase skills for self-advocacy. Families living with CHD deserve, and need, opportunities for fun, exercise, and release from stress and worry. While as a heart mom, I’ve fine-tuned our family’s version of “fun” over the years, I hope to instill in my own child that “fun” is the adventure itself, not just the destination.
Learning Your Limits While Being Limitless
As someone with chronic illnesses myself, I want to teach my child to respect how he is feeling physically and mentally, and to know when he is reaching his ‘max” for the day. Some days he can go almost nonstop for 3-4 hours, and other days, he might struggle to walk even a quarter of a mile, depending on weather, whether he has a cold, or just has been on the go too much. Honoring this has been great for his self-awareness and self-advocacy in other areas of life.
Sometimes, it also means going somewhere fun when we’re feeling good, even if it isn’t “perfect” weather outside. This has resulted in many of our most magical days, because we’re often among the only people crazy enough, for example, to go the beach or Multnomah Falls in the pouring rain a couple weeks ago (and the only people crazy enough, apparently, to visit Oregon with only 1 umbrella for 3 people).
“Off” Days Are Our “On” Days
We try to avoid going places during “peak season” due to hotter temperatures, longer lines, and crazier parking. Often going to a fun destination (like a water park or amusement park) very early or very late in the season is much quieter, cheaper, and all-around a lot less wear-and-tear. Last summer, instead of going to a bigger our out-of-state destination, we took a week off and visited several fun places within a 2-hour radius of our home…on the weekdays, when they would be less crowded.
Reservations and Expectations Are Not Family Friends…But the Unplanned and Magical Are
Pre-paid tickets? Big expectations? These, at least for us, create a lot of pressure to get a certain level of experience out of what often turns out to be a major expense. Lowering the stakes allows us to be open to the unexpected, and results in a lot less guilt or disappointment if the weather is taking a lot out of my CHD child or one of us just isn’t feeling 100% that particular day.
This past winter, we drove from Milwaukee to Madison, WI to see the holiday lights at the zoo. We walked all over the zoo, saw all the animals, and got worn out before the lights even came on. Sure, we didn’t end up seeing the lights, but leaving through the back entrance, we saw the sun setting over a beautiful frozen lake, and joined the people playing on it. Honestly, playing on that frozen lake it something I’ll never forget–and was probably far more special to us than seeing the holiday lights, after all.
To quote the late, great Anthony Bourdain, “no reservations” has become my motto.
Fun, Fun for Everyone
Summer fun will look different for each family, and for many of those living with CHD and other special needs, that is particularly true. But over the years, here a few tried and true summer options we’ve found:
- Museum membership reciprocity: instead of buying several memberships to local attractions, we pick one different membership each year. Most museums, zoos, nature centers, and botanical gardens that sell tax-deductible memberships have reciprocity with other institutions, allowing us to visit several other educational sites per year with our membership–usually all for free. Some children’s museums also offer free tickets or memberships for families with special needs.
- Nature: We’ve discovered many county, state, and even federal natural areas and historical sites that have free parking and free entry. Many nature centers and parks have short (1-mile or less) nature/interpretive trails that are flat, easy terrain and often wheelchair and stroller accessible.
- Farms and farmer’s markets: Summer is the time to visit local farms that offer pick-your-own berries, peas, pears, and apples. We like these because you can go at your own pace, go early or late if it’s hot out, and of course, make delicious and healthy recipes when you get home. Local farmer’s markets are a great sensory experience for kids without being too overwhelming, and get us eating healthy in a season that’s ripe for indulgence!
- When in doubt…water: Swimming pools and wading in lakes are, of course, kid favorites, but going ponding at your local nature center, visiting splash pads, or just running through the sprinkler are great ways to cool off. When it is too cold for swimming, we enjoy simply beach hunting at local lakes for “meditation rocks,” “worry stones,” and other treasures.
- Or animals: The healing power of animals can’t be overstated. Being out and about in summer gives us more opportunities to view animals in their natural habitats, as well as safely encounter them in educational and recreational settings.
- Library programming: Summer reading programs promote reading for fun prizes, but many summer reading programs offer free events for children throughout the summer. From constructing marshmallow catapults to storytimes and magic shows, to kids’ concerts and reading to a service dog, my son has had some great experiences right at the local libraries.
- Flower hunts: When my son was recovering from his 3rd heart surgery one summer, our activities at home were limited for several weeks, especially because there was a major heat wave occuring at the same time. That was when we started our summer tradition of walking around the block, going on “flower hunts” to see what was in bloom every few days. Sometimes, simple is best…but there’s nothing wrong with becoming acquainted with your local wildflowers and garden blooms, with their accompanying butterflies, caterpillars, and birds!
Summer with CHD has its own considerations, but it’s also the perfect time to find out what you love to do as a family, try new things, and gain important insights and life skills. “Fun” doesn’t have to be a big production or involve “big ticket” attractions–though there’s nothing wrong with doing those once in awhile, too! We’ve found that simple spots are some of the most relaxing and beautiful–and easiest for us to adapt to, depending on our own needs.
Margaret King is a Wisconsin writer who enjoys penning poetry, short stories, and young adult novels. In her spare time, she likes to haunt the shores of Lake Michigan, similar to many of her fictional characters. Her recent work has appeared in Unlost Journal, Verdancies, the Ginger Collect, Foxglove Journal, Moonchild Magazine, at art shows and in various other spots on the web. She was recently featured as Poetry Superhighway’s Poet of the Week, and is the author of the recently-published novella, Fire Under Water.
The effects of Congenital Heart Disease aren’t limited to the heart. Patients will often experience complications with other body systems. This week Anna Archer shares her how her late CHD diagnosis caused a brain abscess.
Growing up and Diagnosis
I was your average, ordinary, little girl for the first decade of my life and lived an idyllic life with my (incredible) parents and eight sisters and brothers (yes, you read that right), in the Pacific Northwest. When I was ten, I started showing symptoms of my undiagnosed CHD. I started becoming slightly short of breath and cyanotic with exertion, I was easily fatigued, and I probably slept more than most kids my age. After running through a whole gamut of testing, we finally received my diagnosis of Tetralogy of Fallot with a VSD, Eisenmenger’s Syndrome, and Pulmonary Arterial Hypertension.
Surgery to repair my heart was not an option for me, so life meandered on after my diagnosis, but I maintained my health for most of my second decade, it declining only in the last year or two. A few months after I turned 20, I was listed for a double-lung and heart transplant. Many years later, I learned that my parents thought they might lose me that winter, but I survived, and, a few years later, I was thriving again. I became healthy enough to go off the list after six years on it. In September of 2012, I got married and moved to Southern Nevada, where I currently live.
To better understand the plot twist, you need to have some understanding of my heart. I have what is called a bidirectional shunt with my VSD. It acts as a “pop-off” valve if you will, allowing my blood to change directions as needed, to relieve the pressures between my heart and lungs. Bacteria that should get caught in my lungs and give me a cold, is able to bypass them, through the shunting in my VSD, and maneuver its way up to my brain. There it made itself comfy cozy and became a brain abscess.
Plot twist! AKA: The Brain Abscess
On Monday, February 25th, 2013, I had a headache. Now, this isn’t abnormal in and of itself, except it didn’t go away. I still had a headache on Tuesday, and Wednesday. It was a pulsing kind of headache. Sometimes it would be a dull ache, then I would get these waves of intense pain. By Thursday, I was becoming concerned that I had injured myself while working out with my husband the weekend before. Friday came and went with no improvement, despite caffeine, ibuprofen, oxygen, and resting all day. On Saturday, I had decided if it wasn’t better by Monday, I would call my cardiologist’s office and see what they recommended. I had only seen my new cardiologist twice at this point, so I still wasn’t very familiar with how everything worked at their office.
Around 3am on Sunday, March 3rd, I started vomiting. This is always taken very seriously, as I dehydrate quickly, and what little energy I normally have is drained almost immediately. After a second round of vomiting, I woke my husband up and asked him to take me to the ER; something was definitely wrong.
The ER was fairly quiet that night, and I was taken back almost immediately. They worked through everything respiratory/heart/lung related first, chest x-ray and bloodwork, the most obvious concerns with a CHD/PAH patient. After everything came back clear, they sent me for a head x-ray. I remember someone coming into my room and asking”have you ever had anything show up on your scans”, I said “not that I knew of,” and thought it kind of a silly thing to ask (little did I know). Then I was sent for a head MRI… I wasn’t as concerned as I, perhaps, should have been. I supposed that since my head hurt, it made sense they were checking it out. I never thought something would actually show up on those scans.
The ER doctor came in, and told me I had a mass in my brain, and I needed to have a brain biopsy done. I was shocked, and in some sort of denial, because I told him flat out that surgery wasn’t happening. They couldn’t do surgery on me, it was too risky, it was reserved for life and death situations, and this was obviously not a life or death situation. He insisted that I needed to wrap my mind around it, because it would have to be done. Then he told me to look up glioblastoma, then left the room after reiterating that I would need to have surgery. I knew what a glioblastoma was, one of our family friends had died from it. I looked it up anyways; one to two year survival rate with successful treatment. I called my Dad and cried as I explained what little I knew.
I have been blessed with amazing parents, my Dad is a respiratory therapist and my Mom went to nursing school. It took my Dad almost an hour to calm me down when I called him about my brain scans, and finally, he said he would call my Mom and tell her what was going on. I didn’t think I could do it and be any amount of comprehensible. A little while later my Mom called, and I cried with her. Somebody, I’m not sure if it was myself, or one of my parents, let the rest of my family know what was going on. I have also been blessed with amazing siblings. Each and every one of them has supported me through thick and thin. They worked things around in their lives so that Mom could fly out to Las Vegas to be with me for the impending surgery and recovery.
Somewhere in the mess of things, I was coherent enough to call my cardiologists office and let them know what was going on, as well as telling the ER people that they needed to connect with my cardiologists office. The neurosurgeon who was going to be performing my surgery came in sometime that Sunday. He looked over my scans and to talk to me and my husband. He was much more calm, and kind about things. He told us that “the mass in your brain could be any number of 100 or more different things, and I simply wouldn’t know more until I get in there to check it out during the biopsy”. I felt much better about things after talking to him; I’d had enough of this glioblastoma talk.
Surgery was scheduled for the next day, Monday, March 4th, but it ended up being postponed, because they couldn’t get my blood thick enough (I’d been on blood thinners since I was diagnosed). After a few platelet transfusions, and a couple of vitamin K shots, it was finally thick enough to schedule the biopsy. I count this as a blessing on multiple levels, as it allowed some extra time for my Mom and one of my brothers to fly into town that day.
Tuesday, March 5th, 2013, my six-month wedding anniversary, I had brain surgery. My family was told the biopsy would take around one and a half to three hours, so you can imagine the almost heart-attack they all had when the surgeon came out after 45 minutes! He said everything went great, the abscess was right next to my skull, and not buried into my brain, so he was able to remove the entire thing and flush it out with antibiotics and antibacterials, before closing me up again. The abscess ended up being about the size of a golf-ball or meyer lemon.
For the next day or two my timeline is a little wonky. It was my first experience being intubated, and my first experience with any kind of narcotic (I’m apparently fluent in ASL when I’m on narcotics, I don’t remember this). What I do remember, is that my husband, mom, brother, mother-in-law were all there. I was surrounded by a million beeping machines, and my family. My best friend flew into town a few days later, followed by my dad the next weekend. I was surrounded by those who loved me. I remember distinctly getting my hair dry-washed by a nurse, which was the most incredible feeling in the world at the time. The night nurse, who made my life sane again. My Dad talking medicalese to the doctors, to explain that resting oxygen levels in the mid 70’s were perfectly normal for me. Slowly entering the social media field again, and the outpouring of prayers and support from family and friends across the country. Continual jokes about having a “few loose screws” now.
The “short-term” recovery ended up taking just over two months. I was released from my Infectious Disease doctor on May 8th and Neurosurgeon on May 16th. I say “short-term” recovery, because I was still recovering from some aspects six months later, a year later, two years later, even now.
Some things I wish they had done, was suggest I see a psychologist, and do neuropsych testing. My abscess could’ve been much worse, bigger, deeper into my brain, in a different spot, any number of things could’ve made recovery that much harder and longer. There wasn’t anything obviously different, or wrong, after the surgery, but lots of teeny tiny things that slowly built up, and built up, and built up, to become large problems later on. My abscess was in the frontal left lobe of my brain, and the frontal lobe functions include things like mood, personality, memory, behavior, decision making, judgement, initiative, planning, reasoning, intelligence, movement, and inhibition. I lost all semblance of self-confidence. I forgot how to cope. I became incredibly jealous. I wasn’t the same, and I didn’t know it for awhile. When I figured it out, I didn’t know how to fix it. Rewiring the brain is hard work. It’s an exhausting, frustrating business.
After I had a bad reaction to some allergy medications in the summer of 2016, along with some of life’s tougher moments, I became severely depressed, and developed anxiety. I had called my cardiologists office to see if there were any medications I could take, and as I’m bawling my eyes out with my nurse, they told me they had a psychologist I could see. We made an appointment for the next day. It’s been an incredible, life-saving, life-changing experience. For the past year and a half, I’ve seen my psychologist every week to two weeks, and I can’t recommend it enough. We’ve worked a lot on rewiring my brain, learning how to cope in various situations, and to remember that I am enough.
It’s a continual process, learning who you are, and what you are capable of, if only you give yourself half a chance.
Anna (Becky) Archer is 31 and lives in Las Vegas, NV with her husband and kitten. When Becky isn’t busy at her part-time job as a filing clerk, you can find her playing video games, knitting,
crocheting, swimming in her pool, shooting photos, hiking, going to the movies, and hanging out with family and friends. She is a Heart Warrior, PHighter, and Survivor. She has a fierce
perseverance for living.
Our story began at Freddie’s 19 week ultrasound. The sonogram technician kept circling around his heart and said “there’s a problem with his heart anatomy”. We were first told he had Truncus Arteriosus; subsequently we learned his true diagnosis: Tetralogy of Fallot with Pulmonary Atresia with Multiple Aorto-pulmonary Collateral Arteries (MAPCAS). A ruined pregnancy turned into a silent blessing as we had time to research the best medical facilities in the country to cure this defect. After much prayer and research, Children’s Hospital of Philadelphia became the obvious place for our family in order to save Freddie’s life. It was made clear to us that not only would he need his full repair., but additional surgeries would be required during his life to replace his conduit, and address any other repaired that might be required.
Freddie was born August 5, 2005, but after 11 days in CHOP’s ICU he was released only to be admitted to Johns Hopkins Hospital 2 weeks later for failure to thrive. A feeding tube was placed in order for him to gain enough weight for his full repair, which took place on December 22 2005, under the expert care of Dr Tom Spray. After 5 ½ hou
rs in the OR, including 19 minutes under circulatory arrest, where he was cryogenically frozen, Dr. Spray emerged to utter the best words Melanie and I had ever heard: “he’s fine, everything went well”! It went so well in fact, that the Assisting Surgeon later confided to Melanie and I that for a surgery of this magnitude, it was outcome they had all year.
When Freddie was just over three at a routine follow up a pseudoaneurysm was diagnosed via echocardiogram. A cardiac catheterization ensued and confirmed a pseudoaneurysm.
Freddie underwent a homograph conduit replacement on October 20, 2008, again under the expert care of Dr Spray at CHOP. Due to the amazing network of physicians and all staff aat CHOP, we have been blessed that Freddie has had no restrictions of any kind, and in addition to a great scholastic experience to date, he has been able to play recreational soccer, flag football, baseball and basketball.
Just recently, Freddie had his 3rd open heart surgery to replace his homograph conduit on February 6, 2018. He did develop a pneumothorax postoperatively and needed a chest tube placed at bed side. Amazingly, he was discharged 74 hours after surgery.
On 10/26/2015 our 3rd child, Nicholas was born also with Tetralogy of Fallot that was diagnosed at a 22 week fetal echocardiogram. Nicholas was born in the SDU at CHOP and became symptomatic of heart failure at 2 weeks of life. Nicholas was also admitted to Johns Hopkins for failure to thrive. We gave Nicholas fortified breast milk thru the bottle. He had his full repair at 6 weeks old at CHOP by Dr Spray. He is currently undergoing speech therapy for speech delay.
Ethan was born July 11, 1998 with a congenital heart defect called VSD. He received his first pacemaker at the age of 4, another 2 at the age of 9 and will have his 4th pacemaker replacement surgery in April. He is nothing short of amazing. We are so thankful for the strength and courage he has shown everyday of his life. This kid has been through it all. He lives life to the fullest. He plays baseball, rides motorcycles and loves cliff jumping. He is in his sophomore year of college and wants to be a nurse to give back. I am so happy to say I am a proud heart warrior Mom!
At 20 weeks in utero, my baby was diagnosed with an Interrupted Aortic Arch, VSD and Aortic Stenosis. Bryana Kennedy Walker was born September 25, 2016 at Advocate Christ Hospital under the care of Dr. Michel Ilbalwi, Bryana underwent her first open heart surgery at 10 days old on October 5, 2016 to repair her Interrupted Aortic Arch and Aortic Stenosis. Bryana came home on continuous oxygen, an NG tube for nutrition and over 12 meds to be given daily with daily weights. In Nov 2016, Bryana suffered a stroke, rendering Right Sided Weakness to upper and lower extremities. At almost 6 months of age after a cardiac catheterization, Bryana needed her second open heart surgery on March 9, 2017 to repair her VSD and insert larger shunt. Post-op, Bryana left lung collapsed, requiring Echmo for 13 days. A chest ultrasound revealed a Chylothorax, requiring a chest tube to her left chest wall to relieve fluids. Continue reading
Complications from surgery, scar tissue left behind, and an underlying disease can bring about arrhythmias in CHD patients and lead to need for a cardiac device. Gathered here are the contributions and resources from PCHA’s Arrhythmia Blog Series.
PCHA’s next series introduces the various Cardiac Devices involved in the treatment of Congenital Heart Disease and the associated conditions. In the first post, Dr. Adachi tells us about the Jarvik for small hearts, a ventricular assist device used to help pump blood through the body.
Often times, CHD patients face issues with the rhythm of their hearts. In some patients, this can mean additional treatment is necessary, with either medication or a cardiac device. In this week’s post, Terri Elliott, an adult CHD patient, shares her experience with an arrhythmia that led to her receiving an implantable cardioverter defibrillator. or ICD.
With each CHD, there is no certain path, no one course looks like the next, and complications vary widely. Though a patient may have a specific structural diagnosis, different arrhythmias may develop over time, however patients and families are not always made aware of this possibility immediately. That’s why the diagnosis of an arrhythmia may surprise parents and send them for a loop, just when they think they’ve seen it all. This also makes it difficult for parents to know just how much to share with teacher and other adults in their children’s lives, just as Alison Connors shares with us this week.
This week former Cardiac Nurse, Carol Raimondi, provides us with the first of a two-part resource on arrhythmia and the various diagnosis and treatment. Up first: Bradycardia.
David was born on August 2, 2006. He is a twin. At my 18 week ultrasound the tech couldn’t find his aorta and the four chambers of his heart were not the correct size. We were referred to Dr. Goble. As she was doing an ultrasound she kept saying, “I can’t believe they found this”. Normal ultrasound techs are not trained to see that much detail in the heart. We were referred to UofM Mott. David’s twin Matthew is completely healthy.
David was diagnosed as HLHS until he was born. We lived in Lansing and due to David’s heart and twins we were scheduled for a C-section. Once David was born his diagnosis changed to Shone’s Syndrome. He had ADS, VSD, Coarctation of the aorta. He also has issues with his aortic and mitral valve. His first surgery was when he was five days old. He’s had a total of four open heart surgeries and two caths. He has been surgery free since May 2012 and isn’t even on any medication. Other then his learning disabilities and that he’s super skinny you wouldn’t even know he had anything wrong with him!