Beyond the Heart – Anna’s Story

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.

Letzer Family

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

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!

-Dawn White

Bryana Kennedy Walker

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

Recap – Arrhythmias and Cardiac Devices

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

 

Jarvik: For Smaller Hearts

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

 

Terri’s Story

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

 

The Elephant in the Room

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

 

Bradycardia Explained (Part One)

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

 

Tachycardia Explained (Part Two)

David

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!

-Sarah Shoemaker

Alee Faith

Alee Faith was born on June 16th, 2016 .After some poor feeding issues and a sweating episode we ended up at UK on June 27th and we were given her diagnosis: Truncus Arteriosis with vsd. We were transferred to Cincinnati Children’s Hospital and Alee had open heart surgery on July 5th, at 19 days old. The surgeons placed her conduit and corrected the VSD during that particular procedure.
Alee then had a heart cath where 5 stents were placed in December of 2016. She will continue to have surgeries/procedures throughout her life. Life changed drastically for our family when our precious Alee came along. We have follow up visits with our cardiologist at UK every couple of months and we never really know what we are going to be told. The conduit that they placed doesn’t grow with Alee as she grows and gets bigger and in her particular case she has had moderate leaking with that conduit/valve pretty much ever since it was placed. We don’t know if/when her cardiologist is going to come in and inform us it is time to have ohs again, another heart cath, or that we just need to schedule another follow up visit in 3-6 months and everything is still looking the same for now.
We have to hope and pray for the best but at the same expect the worst with her Diagnosis. Organizations like PCHA provided our family with so much hope & support. Although it very well seemed that our world stopped spinning the night of June 27th, 2016,  we didn’t feel so alone with this having the support that we had. PCHA is by our side as an advocate for our heart warrior and a voice alongside our own that she so desperately needs. This Heart Journey continues for our daughter and all other heart warriors. They can never be fixed, only mended time and time again.

Lucas Iguina

In January of 2007 Lucas was diagnosed via utero with Hyploplastic Left Heart Syndrome. We were given three options before his birth – one was a procedure of three palliative surgeries that he would need to survive. Lucas had open heart surgery at 3 days old, a second open heart surgery at 4 months old, and his final open heart surgery at two years old to repair the half a heart he does have, all performed by Dr. William DeCampeli at Arnold Palmer Children’s Hospital in Orlando. He has had many interventions since birth, his last intervention was a stent replacement in February of 2016. He also has a blood disorder that causes his red blood cells to not break down easily. CHD is lifelong, there is no fix or cure, and its care is complex but there is hope. Lucas is doing amazing today, he enjoys to live life to the fullest and does not let his CHD hold him back. Our family is active and we advocate for the CHD community to support families just like ours and raise awareness. Lucas has visited D.C a few times to advocate for CHD and plans to continue having his voice heard! Together we can conquer CHD!

Ezra Unzen

Ezra was born August 13, 2014 in Minneapolis, Minnesota with Hypoplastic Left Heart Syndrome, Double Outlet Right Ventricle, Coarctation of the Aorta, and Ventricle Septal Defect. He underwent a successful Norwood surgery at 5 days
old. He had a heart cath at 4 months old which
led to his 80 minute cardiac arrest and caused a severe anoxic brain injury. He wasn’t
expected to live 4 months following the arrest,
let alone to his 1st birthday. He lived a joyful life
with loving family and friends until November
4th, 2016 when he suddenly died of heart failure.
Ezra had a huge smile and touched the lives of
everyone who knew him.

Ezra’s parents are grateful for the lifesaving and compassionate care he received through his life. Together, with Project Heart To Heart, Ezra’s parents have started the Ezra John Unzen Scholarship. The scholarship will be awarded to high school seniors in the Hermantown, MN district who want to pursue a career in healthcare. With the first scholarship being awarded in May 2018, Ezra’s memory lives on not only through the life he lived, but through the future doctors, nurses, and healthcare professionals coming out of the Hermantown School District.

 

Emersynn Frost

Emersynn was born this past November and is our miracle baby! She is doing absolutely amazing!

At our 20 week anatomy scan, we found out that our little girl has a congenital heart defect (CHD). Shortly after this news, we were referred to a high risk OB and a pediatric cardiologist. When we saw the ped cardiologist, he broke the news that she does indeed have a complex heart defect which is VERY rare. Our baby has congenitally corrected transposition of the great arteries (ccTGA), ventricular septal defect (VSD), pulmonary stenosis (PS) and dextrocardia. 1% of pregnancies end up with a baby with a CHD. Out of those one percent, .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!

A little bit about her condition: In ccTGA both ventricles (pumping chambers) of the heart are reversed. Fortunately, the arteries are reversed too, so the heart actually “corrects” the abnormal development, thus the name “congenitally corrected transposition of the great arteries.” However, ccTGA is a complex malformation in which the heart is far from being normal.

In a normal heart, the left-side pumping chamber (left ventricle) sends blood to the entire body. The right-side ventricle pumps blood only a short distance, to the lungs. The left ventricle is built to last longer than the right ventricle: 80 to 100 years if no other health problems exist.

In ccTGA, the heart twists abnormally during fetal development, and the ventricles are reversed: The stronger left ventricle pumps blood to the lungs and the weaker right ventricle has the harder chore of pumping blood to the entire body. The right ventricle is not built to last as long as the left ventricle. Emersynn is currently almost 5 months 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 me more and more everyday! She is such an inspiration to everyone around her and is such a strong little warrior!