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Broken Hearted in Nevada: Julie's Story

Part 1 of 2 Congenital heart defects are the most common birth defect in humans, affecting 1% of the world’s population. In the United States alone, 40,000 babies are born with CHD every year.1. Of those babies, about 25% will have a critical CHD, requiring surgery in the first year of life. There is no cure.

In the last few decades, advances in surgical techniques and research mean that today, about 95% of those born with non-critical CHD and 69% of those born with critical CHD will live to see adulthood. Those medical advances were largely in the pediatric space and focused heavily on survival.

In recent years, estimates show that over 1.4 million adults are living with CHD in our country. But for this population, the U.S. medical system is woefully underprepared to treat them. You may have seen stories from actors, politicians, or athletes living with CHD who seem to have no issues receiving appropriate care, but what about everyone else?

Meet Julie, a 53-year-old woman living with critical congenital heart defects. She is a daughter, twin sister, and loving mother. Julie has miraculously outlived every grim prediction doctors made in her early childhood, despite the odds being overwhelmingly stacked against her.

For babies like Julie, born with congenital heart defects between 1970-1974, the biggest hurdle was surviving the first year of life. If they did reach their first birthday, the chances of surviving to adulthood were just 77-83%.

Heart surgery on infants did not become commonplace in the United States until the early 1970s. After a year of infant CHD surgery at Boston Children’s Hospital in 1972, surgeons had some data to work with. They realized that babies with critical CHD who died generally did so in the first few weeks of life. The rest of that decade saw a shift from surgery in infancy to surgery in the newborn period for these babies, with the first successful procedure coming in 1983 on an 11-day-old.

Julie had her first open-heart surgery at the age of 5 1/2 in 1976. Her childhood was filled with summer vacations, school, birthdays, and lots of visits to the pediatric cardiologist. Because there were no specialists for adults with congenital heart defects until 2015, Julie continued to see her pediatric cardiologist as an adult. Her heart remained stable until her mid-20s.

In 1995, at age 25, Julie gave birth to a beautiful, healthy baby girl. Researchers had barely begun to study adults with CHD and no one told Julie she should have additional cardiac care and monitoring during pregnancy (probably because they didn’t know yet). Standards of care for adults with CHD were not issued until 2008 with the next update coming in 2018.

After the birth of her daughter, Julie’s heart really struggled. By age 26, in 1996, she was back in the hospital for her second open-heart surgery. There was no road map for Julie’s medical team to follow and they were dealing with a long list of heart-related issues in her body. Not to mention, Julie lived in Nevada, where the healthcare system could barely serve its healthy population.

The surgery did not go as planned and took far longer than anticipated. As the procedure entered its seventh hour, extensive bleeding and the length of time on the bypass machine forced the surgeons to place Julie into a coma, with an open chest. She wouldn’t wake up for almost two weeks.

What happened during her 13-day coma is largely unknown. Medical records were not easy to access, and communication from doctors to family members was limited. Julie woke from the coma completely paralyzed on her right side, unable to hold, feed, or care for her young daughter. She didn’t know it at the time, but this would be a turning point for her health.

Facing yet another uphill battle in her young life, Julie persevered and recovered function on her right side with months of intensive therapies. She would go on living life, raising her daughter, and continuing to follow up with her pediatric cardiologist.

As she aged, additional health conditions arose and everyday tasks became more difficult. Julie could feel that something was happening in her body, but unfortunately, Nevada still did not have any adult CHD specialists that could investigate. Despite the herculean efforts of her pediatric cardiologists, Julie declined into heart failure and would require a full evaluation by a specialized adult CHD center.

The team at UCLA’s Adult Congenital Heart Disease Center confirmed Julie’s heart failure diagnosis, but she was blindsided by the severity of her disease. Without knowing they even existed, Julie never had the chance to use the ACHD care standards for her anatomy, and was now looking at evaluation for a heart transplant.

If Julie’s life were a feature film, it

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I'm new here!

Hi, my name is gkpoole1227. I'm here because I had a congenital birth defect in my heart. I had a bicuspid instead of a tricuspid aortic valve which had caused an aneurysm in my aortic arch. In April of 2021, I had my aortic arch and my aortic valve replaced during the same operation. Directly after the surgery, for three days I suffered with delirium, where I did not know my husband or daughter. It was a very scary time. I would also say I experienced brain fog for months. I have had some vision problems as well- though that seems to be improving now that I am 2 years out. As some other women have stated, I also had some hair loss after the surgery.
Kimberly Poole


3 reactions 1 comment

I’m new here!

Hi, my name is MummyTut82. I've been diagnosed with so many illnesses diseases that at age 40 and Mum of 3 darling children, I have lost all quality of life and I'm scared of myself honestly.. For I can't keep just 'existing' like this and I know it is destroying my children's hearts watching me so sick ... it breaks me more every minute coz I honestly believe they shouldn't have to see Mummy this way anymore it isn't fair on them... #icantdothis😮‍💨 #heartbroken💔 #idontwanttoleavemybabies😪 #itisjustfartoomuchtohandle💔

#MightyTogether #PTSD #Fibromyalgia #Anxiety #Depression #ADHD #OCD #Grief #Leukemia #FunctionalMovementDisorder #DiabetesType2 #Cancer #RheumatoidArthritis #OpticNeuritis #NeurocardiogenicSyncope #DemyelinatingNeuropathies #CeliacDisease #InappropriateSinusTachycardia #congenitalheartdefect/Disease #BirthDefect #IronDeficiencyAnemia #Arthritis #ArteriovenousMalformation #SuicidalThoughts #chronicactiveepstein-BarrVirus #ChronicIllness #polycysticovarysyndrome(PCOS) #Hyperparathyroidism #ThyroidCancer #Cardiomyopathy #CardiovascularDisease #CongenitalVascularCavernousMalformations

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Congenital Birth Defect and Lonliness, Young Adult

Hi there,

My name is Theo and I'm currently a freshman in
college. I was born with an extremely rare birth defect called a type three laryngeal
cleft. I had my cleft repaired for the third time
about three months ago; I was in pretty
significant isolation during the summer as I
needed to limit my talking and couldn’t afford to get sick. I was extremely
excited for college, but unfortunately came down with a nasty case of COVID the first week. The repair failed due to coughing and I’ll have to undergo another surgery in early January. I'm
heartbroken and feel totally defeated. Being
isolated for 7 months sounds depressing
and demoralizing. None of my peers have ever
heard of a laryngeal cleft, much less know how to support me. No one I know can relate to having 12 surgeries and the toll that comes with them. So overall, I'm feeling a little lonley, fragile, and cheated. I would love to chat with/hear other peoples' stories! #MightyTogether #LaryngealCleft #RareDisease #Lonliness #BirthDefect


How to cope when you feel the burden of your disease is all on yourself?

A wonderful person on here gave great advice that knowledge is power. It feels very true for fibro at least for me. Along with a recent diagnosis of fibro, I also deal with OCD, Anxiety, & Depression. I also deal with recent diagnosis of PTSD & a birth defect in my kidney.

I recently had surgery on my kidney 4 days ago. I told the doctor that I had fibromyalgia before the surgery. Unfortunately, I just googled today surgery and fibromyalgia. I feel irritated with myself because now I’m in a lot of pain and I just found out there were things I could suggest to my doctor to decrease my pain. Like local anesthetic into injection sites and magnesium drip so I didn’t have to wake up from anesthesia with so much muscle cramp pain.

I feel irritated with my doctor that she didn’t know these things but as a healthcare professional I understand why.

I just feel like I have to know everything all the time and it’s exhausting, lonely, and disheartening. #Fibromyalgia #Surgery #DaVinciSurgery

3 reactions 4 comments

Difficult News

I had an x-ray done on my hips on the 20th and I got the results yesterday. I have a birth defect with both hips called hip impingement. It's causing damage to the ball joints I think. I haven't talked to the doctor yet about what's going on but that's tomorrow. I have to find an orthopedist so I can figure out what I'm supposed to do next. Treatment is typically physical therapy, injections, and sometimes surgery to do hip replacements. I wish I wasn't sick anymore but it's nice to know why I'm having such bad hip pain and why I've had hip pain for such a long time


Answer you do not want but get

I have a birth defect of the heart, that I found out at the age of 52. During the past 4 years I have had 19 cardiac surgeries, and 13 stents. I live with heart attack symptoms every day and have wanted my life to end. I just had my last cath on Monday, but before the surgery, I did not know what I was going to do if he could not fix anything- I thought the worse. Well I got the news that I didn’t want to hear - they cannot fix my heart. I was crying at first, then I realized that I no longer have to o have hope for a fix, and no one can give me any false hope. It was a relief if anything. Only 3 ways that my heart can be fixed - technology that hasn’t been developed yet, transplant, or death.


How to cope with ptsd while experiencing acute illness and pain?

I was recently diagnosed with chronic ptsd after the memories of my childhood abuse resurfaced. Around the same time, I was diagnosed with a birth defect in my kidney (supposedly it’s rare to diagnose this defect in adults). The birth defect has contributed to swelling in my kidney causing a lot of back pain. Last week, they placed a stent in my kidney. one of the side effects of the stent is bladder spasms and cramping. I take pain medicine but when I get a random really painful flare up it triggers the memories of my childhood sexual abuse. It has been hard to ground myself lately because when I bring myself to the present, I come back to the physical pain. Strong pain medicine helps in those times but the emotions from the flashbacks are hard to calm down. Does anyone have any suggestions or resources? Thank you ❤️


"Can you walk down eight steps?#Disabled #SpinaBifida #Ableism #Halsey #HandicappedParking

This past weekend - Friday June 24th - I got to see Halsey at the Shoreline with my two best friends. To be clear, Halsey was AMAZING! But the venue was shitty...

I was prepared. I brought my handicapped placard and researched the venue before going. I purchased tickets online that said handicapped accessible tickets with a companion so I got the seat with a handicap and companion seat and also the seat next to the companion - three tickets in total. A week before the concert I checked again to be sure there was handicap parking and indeed it said handicapped parking available.

The day of the concert we got to the venue, and parking was a mess. We asked eight parking attendents where handicapped parking was, and they just pointed - and didn’t say anything. We finally parked but it wasn’t handicapped parking. The parking lot was uneven and gravely and not paved, which is kinda difficult when you're in a wheelchair. We got out of the car to assemble my wheelchair.

We had to walk a little ways to get to the venue. On our way over, we saw the parking lot where all the handicapped parking was. Austin asked one of the staff members if we could park there and she said yes just ask one of the shuttles to shuttle you back to your car and put his hazard lights on. But, once Austin got his car but yet another staff member said he couldn’t park there, and made him go back to his original parking spot. It was confusing and disorganized, as if people at Shoreline aren’t used to disabled people coming to concerts with their friends at all.

If people get treated like I did, that’s kind of not surprising.

We got to the venue and they wanted to do a bag check - including looking at the bag where I carried my catheters. It seemed like too much of an invasion of privacy, so I left the bag in the car… It turned out that the bathrooms (porta-potties) weren’t wheelchair accessible anyway… even though they were supposed to be.

We got to the gate to scan our tickets. I asked where the accessible section was. They just pointed and said on the other side of the theater. It turned out that the seats we had that were supposedly handicap accessible …weren’t. My friends Austin and Brooke talked to one of the staff and told them the situation. The staff member looked obviously irritated, pulled all three of us aside and radioed her supervisor. Austin was still talking to the staff member still telling her the situation and then she made a finger gesture telling him to be quiet.

Her supervisor came and after hearing the details, she looked at me and asked, “Can you walk down eight steps?” I said no. Then she asked “What if one of your friends carried you down?”

I …couldn’t even believe they asked me that. I was born with a birth defect. I have spina bifida. My spine was formed outside the skin, and my nerves weren’t formed in my legs. Right after I was born, doctors had to put my spine back into my body. I can’t feel below my knees, so I'm paraplegic. I don’t expect strangers to know my whole history but… I was in a wheelchair and I bought handicap accessible tickets. Why was she asking me that?

I turned and looked at Austin and Brooke and I just shook my head.

The staff lady rolled her eyes and sighed. Then she said, “Let me see what I can do.

Eventually we got to our seats, and they were accessible, but they were segregated off in the corner.

And I don’t know how I feel - maybe disappointed? It's a RARE moment when I’m feeling really good about navigating as a disabled person in public spaces. It is also RARE to get to hang out with my best friends. I go to dialysis three times a week, and right now I don’t work, so the tickets were an expensive treat. Most of the time I’m feeling tired and sh*tty, but on Friday, I felt good - it was going to be a good night.

And Shoreline ruined it.

…But Halsey was AMAZING!

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