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Claire
Claire Elizabeth entered the world on January 18, 2007 at 5:28 in the morning. I heard her cry and I remember thinking, "She's fine!" But we knew she wasn't. Almost exactly four months earlier, Michael and I received the devastating news that our second baby had a "broken heart" - a congenital heart defect called Transposition of the Great Arteries with a Ventricular Septal Defect (VSD), a hole between her ventricles. As we had with our first daughter, shortly after our twenty week ultrasound, Michael and I went in for a fetal echo to look at the baby's developing heart. But unlike the first time, our appointment was far from routine. This time after the echo, we were sent across the hall to wait for the physician to come in and talk to us. I remember watching Dr. Allen, who was later to become our saving grace, walk in and inform us that our darling baby girl would require at least one surgery in order to survive. Because her anomaly prevented oxygenated blood from reaching her body, the surgery would be required shortly after birth.
We made the decision to deliver at the University of Virginia because there is no pediatric cardiovascular surgeon in Richmond and the team at UVA has lots of experience with babies. We had several more echos and everyone felt comfortable that Claire would be a candidate for an arterial switch operation that would completely mend her heart. Aside from the misplaced arteries, her heart appeared to be developing normally. The first few hours of Claire's life, all went as planned. I got to hold my new baby for a few seconds before the team whisked her off to the NICU. Once she was settled in her isolette, our families got to go in and meet her. Finally that afternoon, after assuring ourselves everything was under control and that in a few short days we'd bringing home a perfectly corrected little girl, Michael and I relaxed enough to fall asleep. We were awakened less than an hour later to the horrifying news that Claire's heart was actually "significantly more nuanced" than expected and that she was in critical condition, in need of immediate intervention. We were shocked by how blue and swollen she'd turned in the short time since we'd seen her. Thankfully, the emergency procedure, a balloon septostomy, created a portal for oxygenated blood to mix with the unoxygenated blood and make its way through her body. Claire responded beautifully and within twelve hours was off the respirator and began to thrive.
As expected, Claire did have Transposition of the Great Arteries. However, her VSD was larger than anticipated and in an abnormal location. Additionally, and most significantly, her pulmonary valve was malformed, making an arterial switch virtually impossible. The surgeons and cardiologists were unsure how best to proceed with this heart that was unlike anything they had seen before. Six days after she was born, we brought our baby home. The next eleven weeks were consumed with doctors' appointments, worry and frustration. We sent Claire's information to several pediatric heart centers in search of a surgeon who would tackle her "nuanced" physiology. We found him at Children's National Medical Center. On April 9, 2007, Dr. Richard Jonas mended Claire's heart with a modified Rastelli procedure. Dr. Jonas crafted a completely unique fix for Claire's heart, she is truly his work of art! Our little champion flew through open heart surgery like a prize fighter and we left the hospital five days later. Five days!
Claire is facing at least two more surgeries to replace the conduit from her right ventricle to her pulmonary artery. We continue to our pediatric cardiologist every few months and we will return to Children's National Medical Center in the spring for a catheterization. Because Claire's anatomy is so unusual, we can't get a good measure of the pressures in her heart without an invasive procedure. But aside from the badge of courage running down her chest, you would never know that Claire was anything but an active and happy one year old!
Nothing about dealing with a congenital heart defect is easy. Despite the breath-taking prevalence of these disorders, the epidemic remains virtually unknown and unpublicized and research is sorely under-funded. Receiving the diagnosis of congenital heart defect seemed so isolating and almost hopeless, but we quickly learned to rely on the strength and support of our family and friends. Mended Little Hearts has been such a source of comfort for us; finding other parents and families intimately familiar with the same experiences and concerns but also joys and accomplishments is an inexplicable relief! Each family has their own story and each is an inspiration and a wealth of knowledge. But most of all, watching these amazing hearts, from babies to teenagers, reminds us of what is most important in life!
Eli Smith
Eli is an energetic and strong willed soon to be four year old. His heart defect was detected during a routine prenatal ultrasound. Although the news was devastating it gave us time to learn about his condition, make choices regarding his surgery and plan for his birth.
As we were making our final plans for Eli's arrival and care for our two older children he decided it was time for him to make his way into the world, a month early. He was born in Virginia via c-section and when he was two days old he was flown to Pennsylvania where he would have his surgery. He was only six days old when he underwent open heart surgery for transposition of the great arteries with ventricular septal defect, arch hypoplasia, coarctation of the aorta and hypoplasia of the aortic valve annulus. The most amazing part of it all is that he had his surgery on a Monday, was helicoptered back to a VA hospital on Friday and was at his home on Wednesday. It was very scary bringing home a baby that had just gone through all of this and weighed barely five pounds. Each day Eli seemed to get stronger and stronger and we really felt our prayers had been answered.
Eli has been doing great and sees his cardiologist every year for a heart ultrasound. He is not on any medications and leads a wonderful little life. He may need another surgery when he is about twelve but we can't worry about that now because Eli has taught us how to live. His zest for life is astounding. To see him run and play reminds us how precious life really is.
Jennifer and Greg
Madeline Sadie
On the morning of September 4, Madeline "Sadie" Thies made her entrance into this world. Sadie had a very rapid birth, arriving only seventeen minutes after her parents entered the labor and delivery room. Upon birth, she cried, and her mother was relieved - her first daughter had been born with the umbilical cord wrapped around her neck so tightly that she had to be revived for several minutes. Little did her parents know that, though her mother had several "abnormal" ultrasounds, her OB-GYN had failed to detect that Sadie had a life-threatening heart defect.
Sadie's parents watched with growing concern as the nurses tried to "pink up" their tiny daughter. After a few minutes, a nurse brought the baby over for her parents to see briefly before whisking her away to the nursery for intervention.
Minutes turned into hours before, finally, the doctors came in to tell Sadie's parents that she had a heart defect called Transposition of the Greater Arteries, or TGA. TGA results in the aorta and pulmonary vein being transposed, causing the blood to flow in a parallel circuit, preventing oxygenation. Open heart surgery was required to save little Sadie's life.
Sadie was rushed to the nearest location to undergo treatment and surgery. On the way out of the hospital, the transport team let Sadie's parents see her and hold her hand through the incubator, tubes and machinery that were keeping her tiny body alive. Already she had began to turn blue and swell and was barely recognizable from the baby they had seen for only moments earlier that evening after delivery.
After her arrival at the UVA PICU, it took 11 days to stabilize Sadie enough so that she could undergo an arterial switch surgery. During that time her family was constantly prepared for the worst case scenario. Many different doctors throughout those trying days tried to prepare her family when it appeared that her kidneys were likely failing, that her pulmonary hypertension would be permanent, that she may be retarded as a result of lack of oxygen to the brain, and even that she may lose toes or an entire foot due to the poor circulation. On more than one occasion nurses told her mother that they had never seen a baby with TGA that was "so sick."
Finally, 11 days after her birth, Sadie underwent surgery. The day before the surgery Sadie had opened her eyes for the first time that her mother had seen. For her mom, it was the reassurance she needed that the surgery would be successful, and that Sadie was alive and intelligent behind the veil of her drug-induced coma. Four hours later, Sadie was in recovery - the operation was successful.
After Sadie's surgery, she made a remarkable recovery - stunning the doctors, nurses and caregivers alike. On one of the last days of her stay in the PICU, Sadie's mother finally summoned the courage to ask one of the doctors what the long-term prognosis and implications of the defects would be for Sadie. He said that after Sadie healed, her family could expect to have "a normal baby."
A little more than two weeks after surgery, Sadie was released to go home. Though she faced several months of severe withdrawal symptoms from narcotics (similar to the reactions a heroin addict has when trying to break their addiction), she gradually became the normal baby that the PICU doctor predicted she would be. No ill effects have been found to date in regard to heart, coronary, kidney, brain, lung or muscle damage from those early days of heart failure.
Today, Sadie is a healthy and active one-year old. She is extremely bright, inquisitive, feisty and headstrong, and development is considered normal for a one-year old. She loves music and playing with her big sister, Katie.
In the future, Sadie is expected to lead a normal life. As she grows, her heart will be closely monitored through annual checkups with her cardiologist to ensure that it is growing properly, and that the transversed vessels grow with it. Sadie is expected to live a normal life, with only minor modifications to accommodate her heart condition (such as avoiding contact sports and scuba diving). Only time can tell if additional surgeries will be needed, though her outlook is bright.
Sadie's family's motivation to join Mended Little Hearts stems from their experiences in the hospital and in the first year of Sadie's life. "Those days in the hospital, watching your child hover at the brink of death are the longest and most terrifying thing that any parent can face. I really wished I could have someone to talk to that could relate to our situation. Family and friends were wonderful support, but no one can relate to an experience like this until you have been in those shoes," says Sadie's mother, Kim. "Through Mended Little Hearts we hope that one day we can fulfill that need for another family.
Our Stories
Show all storiesTransposition of Great Arteries
Claire
Claire Elizabeth entered the world on January 18, 2007 at 5:28 in the morning. I heard her cry and I remember thinking, "She's fine!" But we knew she wasn't. Almost exactly four months earlier, Michael and I received the devastating news that our second baby had a "broken heart" - a congenital heart defect called Transposition of the Great Arteries with a Ventricular Septal Defect (VSD), a hole between her ventricles. As we had with our first daughter, shortly after our twenty week ultrasound, Michael and I went in for a fetal echo to look at the baby's developing heart. But unlike the first time, our appointment was far from routine. This time after the echo, we were sent across the hall to wait for the physician to come in and talk to us. I remember watching Dr. Allen, who was later to become our saving grace, walk in and inform us that our darling baby girl would require at least one surgery in order to survive. Because her anomaly prevented oxygenated blood from reaching her body, the surgery would be required shortly after birth.
We made the decision to deliver at the University of Virginia because there is no pediatric cardiovascular surgeon in Richmond and the team at UVA has lots of experience with babies. We had several more echos and everyone felt comfortable that Claire would be a candidate for an arterial switch operation that would completely mend her heart. Aside from the misplaced arteries, her heart appeared to be developing normally. The first few hours of Claire's life, all went as planned. I got to hold my new baby for a few seconds before the team whisked her off to the NICU. Once she was settled in her isolette, our families got to go in and meet her. Finally that afternoon, after assuring ourselves everything was under control and that in a few short days we'd bringing home a perfectly corrected little girl, Michael and I relaxed enough to fall asleep. We were awakened less than an hour later to the horrifying news that Claire's heart was actually "significantly more nuanced" than expected and that she was in critical condition, in need of immediate intervention. We were shocked by how blue and swollen she'd turned in the short time since we'd seen her. Thankfully, the emergency procedure, a balloon septostomy, created a portal for oxygenated blood to mix with the unoxygenated blood and make its way through her body. Claire responded beautifully and within twelve hours was off the respirator and began to thrive.
As expected, Claire did have Transposition of the Great Arteries. However, her VSD was larger than anticipated and in an abnormal location. Additionally, and most significantly, her pulmonary valve was malformed, making an arterial switch virtually impossible. The surgeons and cardiologists were unsure how best to proceed with this heart that was unlike anything they had seen before. Six days after she was born, we brought our baby home. The next eleven weeks were consumed with doctors' appointments, worry and frustration. We sent Claire's information to several pediatric heart centers in search of a surgeon who would tackle her "nuanced" physiology. We found him at Children's National Medical Center. On April 9, 2007, Dr. Richard Jonas mended Claire's heart with a modified Rastelli procedure. Dr. Jonas crafted a completely unique fix for Claire's heart, she is truly his work of art! Our little champion flew through open heart surgery like a prize fighter and we left the hospital five days later. Five days!
Claire is facing at least two more surgeries to replace the conduit from her right ventricle to her pulmonary artery. We continue to our pediatric cardiologist every few months and we will return to Children's National Medical Center in the spring for a catheterization. Because Claire's anatomy is so unusual, we can't get a good measure of the pressures in her heart without an invasive procedure. But aside from the badge of courage running down her chest, you would never know that Claire was anything but an active and happy one year old!
Nothing about dealing with a congenital heart defect is easy. Despite the breath-taking prevalence of these disorders, the epidemic remains virtually unknown and unpublicized and research is sorely under-funded. Receiving the diagnosis of congenital heart defect seemed so isolating and almost hopeless, but we quickly learned to rely on the strength and support of our family and friends. Mended Little Hearts has been such a source of comfort for us; finding other parents and families intimately familiar with the same experiences and concerns but also joys and accomplishments is an inexplicable relief! Each family has their own story and each is an inspiration and a wealth of knowledge. But most of all, watching these amazing hearts, from babies to teenagers, reminds us of what is most important in life!
Eli Smith
Eli is an energetic and strong willed soon to be four year old. His heart defect was detected during a routine prenatal ultrasound. Although the news was devastating it gave us time to learn about his condition, make choices regarding his surgery and plan for his birth.
As we were making our final plans for Eli's arrival and care for our two older children he decided it was time for him to make his way into the world, a month early. He was born in Virginia via c-section and when he was two days old he was flown to Pennsylvania where he would have his surgery. He was only six days old when he underwent open heart surgery for transposition of the great arteries with ventricular septal defect, arch hypoplasia, coarctation of the aorta and hypoplasia of the aortic valve annulus. The most amazing part of it all is that he had his surgery on a Monday, was helicoptered back to a VA hospital on Friday and was at his home on Wednesday. It was very scary bringing home a baby that had just gone through all of this and weighed barely five pounds. Each day Eli seemed to get stronger and stronger and we really felt our prayers had been answered.
Eli has been doing great and sees his cardiologist every year for a heart ultrasound. He is not on any medications and leads a wonderful little life. He may need another surgery when he is about twelve but we can't worry about that now because Eli has taught us how to live. His zest for life is astounding. To see him run and play reminds us how precious life really is.
Jennifer and Greg
Madeline Sadie
On the morning of September 4, Madeline "Sadie" Thies made her entrance into this world. Sadie had a very rapid birth, arriving only seventeen minutes after her parents entered the labor and delivery room. Upon birth, she cried, and her mother was relieved - her first daughter had been born with the umbilical cord wrapped around her neck so tightly that she had to be revived for several minutes. Little did her parents know that, though her mother had several "abnormal" ultrasounds, her OB-GYN had failed to detect that Sadie had a life-threatening heart defect.
Sadie's parents watched with growing concern as the nurses tried to "pink up" their tiny daughter. After a few minutes, a nurse brought the baby over for her parents to see briefly before whisking her away to the nursery for intervention.
Minutes turned into hours before, finally, the doctors came in to tell Sadie's parents that she had a heart defect called Transposition of the Greater Arteries, or TGA. TGA results in the aorta and pulmonary vein being transposed, causing the blood to flow in a parallel circuit, preventing oxygenation. Open heart surgery was required to save little Sadie's life.
Sadie was rushed to the nearest location to undergo treatment and surgery. On the way out of the hospital, the transport team let Sadie's parents see her and hold her hand through the incubator, tubes and machinery that were keeping her tiny body alive. Already she had began to turn blue and swell and was barely recognizable from the baby they had seen for only moments earlier that evening after delivery.
After her arrival at the UVA PICU, it took 11 days to stabilize Sadie enough so that she could undergo an arterial switch surgery. During that time her family was constantly prepared for the worst case scenario. Many different doctors throughout those trying days tried to prepare her family when it appeared that her kidneys were likely failing, that her pulmonary hypertension would be permanent, that she may be retarded as a result of lack of oxygen to the brain, and even that she may lose toes or an entire foot due to the poor circulation. On more than one occasion nurses told her mother that they had never seen a baby with TGA that was "so sick."
Finally, 11 days after her birth, Sadie underwent surgery. The day before the surgery Sadie had opened her eyes for the first time that her mother had seen. For her mom, it was the reassurance she needed that the surgery would be successful, and that Sadie was alive and intelligent behind the veil of her drug-induced coma. Four hours later, Sadie was in recovery - the operation was successful.
After Sadie's surgery, she made a remarkable recovery - stunning the doctors, nurses and caregivers alike. On one of the last days of her stay in the PICU, Sadie's mother finally summoned the courage to ask one of the doctors what the long-term prognosis and implications of the defects would be for Sadie. He said that after Sadie healed, her family could expect to have "a normal baby."
A little more than two weeks after surgery, Sadie was released to go home. Though she faced several months of severe withdrawal symptoms from narcotics (similar to the reactions a heroin addict has when trying to break their addiction), she gradually became the normal baby that the PICU doctor predicted she would be. No ill effects have been found to date in regard to heart, coronary, kidney, brain, lung or muscle damage from those early days of heart failure.
Today, Sadie is a healthy and active one-year old. She is extremely bright, inquisitive, feisty and headstrong, and development is considered normal for a one-year old. She loves music and playing with her big sister, Katie.
In the future, Sadie is expected to lead a normal life. As she grows, her heart will be closely monitored through annual checkups with her cardiologist to ensure that it is growing properly, and that the transversed vessels grow with it. Sadie is expected to live a normal life, with only minor modifications to accommodate her heart condition (such as avoiding contact sports and scuba diving). Only time can tell if additional surgeries will be needed, though her outlook is bright.
Sadie's family's motivation to join Mended Little Hearts stems from their experiences in the hospital and in the first year of Sadie's life. "Those days in the hospital, watching your child hover at the brink of death are the longest and most terrifying thing that any parent can face. I really wished I could have someone to talk to that could relate to our situation. Family and friends were wonderful support, but no one can relate to an experience like this until you have been in those shoes," says Sadie's mother, Kim. "Through Mended Little Hearts we hope that one day we can fulfill that need for another family.
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