Monday
Sep272010
Cora's Story: The hidden disease of congenital heart defects
Monday, September 27, 2010
Today I am welcoming my friend Kristine as a guest poster. I have been following Kristine (@KristineBrite) on twitter for quite a while and have been inspired by the way she turned her personal tragedy into incredibly important advocacy work that will save the lives of infants. Kristine blogs and shares important information on her cause on her website Cora's Story. I'm pleased to share her story here and I hope you will help spread the word too.
I gave birth to my daughter Cora in November 2009 after a healthy pregnancy and a delivery free of medical complications. I took her home and spent the next three days cuddling and rocking.
Breastfeeding Cora made me feel so happy, just so content. Early one morning, everything changed. Cora latched and was feeding happily. I looked up for a split second to tell my husband how cute she was, looked back down, and Cora was dead.
The coroner called a few days later and told me Cora died of a congenital heart defect. I’d never heard of it and thought it must be rare. While pregnant, I read everything I could get my hands on. I scoured websites for evidence-based safety recommendations and read about other birth defects and conditions.
Congenital heart defects, or CHD, are not rare. They strike 1 in 100 babies in the US according to the CDC, as reported by the March of Dimes. This means congenital heart defects are by far the most common of all birth defects in the United States.
Sadly, Cora was not alone. Congenital heart defects are one the most common causes of death in infants under one year of age in the United States. Birth defects remain the leading cause of death, and the majority of those deaths are from congenital heart defects in particular. Every day, 9 babies die from a congenital heart defect.
I’m still in shock that I found out about this from the coroner. I wish for even more awareness for other conditions that also kill or affect babies such as SIDS or Down’s Syndrome, and don’t want to take away from those efforts. I’m thankful I could read up about both and that other conditions are so well known. But, congenital heart defects affect more babies than many other conditions I did know about. The letters “CHD” need to be known and recognized by everyone, especially parents and parents-to-be.
I thought maybe I’d somehow just overlooked information about congenital heart defects. But, so often others find out about CHD from Cora’s Story as well. And, I read notes from other mothers that learned about heart defects as their child was rushed to emergency surgery or in heart failure almost every day. I’m not the only one blindsided.
Thankfully, even though CHD remains a leading cause of infant death, most infants survive. Of the 40,000 infants born with congenital heart defects each year in the United States, a majority live to adulthood.
I was even more shocked to learn that as a pregnant woman and mother, knowing about CHD could have saved Cora’s life. We’ll never know for sure, but through prevention efforts, more screening, and knowing the symptoms of congenital heart defects, I might be nursing her now rather than saving others.
I’m working closely with other organizations, and started one of my own, Cora’s Story, to make sure no other mother ever finds out about the most common birth defect from the coroner.
We have an easy way to all work together to save babies, and improve outcomes. Early diagnosis is important and can save babies from developmental delays, organ damage, or death.
The exact cause of congenital heart defects is not known. Research has linked a gene to some defects, although CHD can happen to anyone. No one in my family or Ben’s family has a known heart defect. I say known, because heart defects can go undetected into adulthood.
Researchers have found links to CHD, folic acid, not smoking, and maternal weight to name a few. The heart develops in early pregnancy before a mother knows she’s pregnant so taking extra folic acid if trying to conceive can be helpful. I take extra folic acid all the time, even though we aren’t trying, just in case, as recommended in this medical journal article.
Detection is like a puzzle because nothing finds these defects all of the time. Ultrasound, especially the routinely conducted around 20 weeks finds some of them. Other in utero testing not routinely done like fetal echo and a level ii ultrasound also catches some of them some of the time. I’m going to insist on both with my next pregnancy. Knowing family history is key so you can speak to your health care providers about testing. Sometimes, you might not be aware a relative has a CHD. Over 30 types of CHD are known and relatives might call the condition by something else.
Other defects are found at birth or shortly after birth. The baby might present with one of these symptoms:
Sometimes a baby has no symptoms until it’s too late. I don’t remember Cora having any of these symptoms. But, my mother says she breathed “funny.” Being a first-time mom, I didn’t think anything of it.
Of course echos and EKGs after birth are a great tool for diagnosis, but aren’t routinely done, and are expensive and a bit more intrusive for some parent’s liking. I advocate strongly for pulse oximetry screening. Pulse oximetry measures oxygen saturation, and I had one on most of labor, so it’s already used quite commonly. Babies in the NICU or that present with health problems are routinely monitored. It costs less than a diaper change and is non-invasive. Efforts are away in the United States to make pulse ox part of the newborn screening panel. I also like this type of testing because it doesn’t interfere with bonding. A parent can even hold the baby while it’s being done. For best results, it should be done after 24 hours of life.
I believe this screening will become routine soon, a big step forward happened earlier this month when the Secretary's Advisory Committee for Heritable Disorders in Newborns and Children (SACHDNC) unanimously agreed to recommend the addition of screening for Critical Cyanotic Congenital Heart Disease to the panel for universal screening of all newborns in the United States. The type of defects picked up by pulse oximetry are generally the most lethal. Some refer to pulse oximetry as the “fifth vital sign.” Until it’s routine, asking for it to be done on your newborn after 24 hours of age while baby is calm could save your child’s life.
…
Thank you Annie for helping me give Cora a voice, and helping her save lives. I know it’s not an easy subject. I know Cora’s story is exceedingly sad, but I hope you’ll all remember it, and share it. Help us save lives.
Cora's Story
I gave birth to my daughter Cora in November 2009 after a healthy pregnancy and a delivery free of medical complications. I took her home and spent the next three days cuddling and rocking.Breastfeeding Cora made me feel so happy, just so content. Early one morning, everything changed. Cora latched and was feeding happily. I looked up for a split second to tell my husband how cute she was, looked back down, and Cora was dead.
The coroner called a few days later and told me Cora died of a congenital heart defect. I’d never heard of it and thought it must be rare. While pregnant, I read everything I could get my hands on. I scoured websites for evidence-based safety recommendations and read about other birth defects and conditions.
Congenital heart defects, or CHD, are not rare. They strike 1 in 100 babies in the US according to the CDC, as reported by the March of Dimes. This means congenital heart defects are by far the most common of all birth defects in the United States.
A hidden disease
Sadly, Cora was not alone. Congenital heart defects are one the most common causes of death in infants under one year of age in the United States. Birth defects remain the leading cause of death, and the majority of those deaths are from congenital heart defects in particular. Every day, 9 babies die from a congenital heart defect.
I’m still in shock that I found out about this from the coroner. I wish for even more awareness for other conditions that also kill or affect babies such as SIDS or Down’s Syndrome, and don’t want to take away from those efforts. I’m thankful I could read up about both and that other conditions are so well known. But, congenital heart defects affect more babies than many other conditions I did know about. The letters “CHD” need to be known and recognized by everyone, especially parents and parents-to-be.
I thought maybe I’d somehow just overlooked information about congenital heart defects. But, so often others find out about CHD from Cora’s Story as well. And, I read notes from other mothers that learned about heart defects as their child was rushed to emergency surgery or in heart failure almost every day. I’m not the only one blindsided.
Thankfully, even though CHD remains a leading cause of infant death, most infants survive. Of the 40,000 infants born with congenital heart defects each year in the United States, a majority live to adulthood.
Learn about CHD, save lives
I was even more shocked to learn that as a pregnant woman and mother, knowing about CHD could have saved Cora’s life. We’ll never know for sure, but through prevention efforts, more screening, and knowing the symptoms of congenital heart defects, I might be nursing her now rather than saving others.
I’m working closely with other organizations, and started one of my own, Cora’s Story, to make sure no other mother ever finds out about the most common birth defect from the coroner.
We have an easy way to all work together to save babies, and improve outcomes. Early diagnosis is important and can save babies from developmental delays, organ damage, or death.
The exact cause of congenital heart defects is not known. Research has linked a gene to some defects, although CHD can happen to anyone. No one in my family or Ben’s family has a known heart defect. I say known, because heart defects can go undetected into adulthood.
Researchers have found links to CHD, folic acid, not smoking, and maternal weight to name a few. The heart develops in early pregnancy before a mother knows she’s pregnant so taking extra folic acid if trying to conceive can be helpful. I take extra folic acid all the time, even though we aren’t trying, just in case, as recommended in this medical journal article.
Finding heart defects
Detection is like a puzzle because nothing finds these defects all of the time. Ultrasound, especially the routinely conducted around 20 weeks finds some of them. Other in utero testing not routinely done like fetal echo and a level ii ultrasound also catches some of them some of the time. I’m going to insist on both with my next pregnancy. Knowing family history is key so you can speak to your health care providers about testing. Sometimes, you might not be aware a relative has a CHD. Over 30 types of CHD are known and relatives might call the condition by something else.
Other defects are found at birth or shortly after birth. The baby might present with one of these symptoms:
- Trouble feeding (falls asleep all the time while feeding)
- Labored breathing or fast respiration
- Turning blue, or dusky coloring
- Frequent respiratory infections
- A heart murmur (Heart murmurs are often innocent, but not always)
Sometimes a baby has no symptoms until it’s too late. I don’t remember Cora having any of these symptoms. But, my mother says she breathed “funny.” Being a first-time mom, I didn’t think anything of it.
Of course echos and EKGs after birth are a great tool for diagnosis, but aren’t routinely done, and are expensive and a bit more intrusive for some parent’s liking. I advocate strongly for pulse oximetry screening. Pulse oximetry measures oxygen saturation, and I had one on most of labor, so it’s already used quite commonly. Babies in the NICU or that present with health problems are routinely monitored. It costs less than a diaper change and is non-invasive. Efforts are away in the United States to make pulse ox part of the newborn screening panel. I also like this type of testing because it doesn’t interfere with bonding. A parent can even hold the baby while it’s being done. For best results, it should be done after 24 hours of life.
I believe this screening will become routine soon, a big step forward happened earlier this month when the Secretary's Advisory Committee for Heritable Disorders in Newborns and Children (SACHDNC) unanimously agreed to recommend the addition of screening for Critical Cyanotic Congenital Heart Disease to the panel for universal screening of all newborns in the United States. The type of defects picked up by pulse oximetry are generally the most lethal. Some refer to pulse oximetry as the “fifth vital sign.” Until it’s routine, asking for it to be done on your newborn after 24 hours of age while baby is calm could save your child’s life.
…
Thank you Annie for helping me give Cora a voice, and helping her save lives. I know it’s not an easy subject. I know Cora’s story is exceedingly sad, but I hope you’ll all remember it, and share it. Help us save lives.
Reader Comments (31)
I have read Cora's story before and shed many many tears over it. It breaks my heart that a family had to face that horror. Kudos to Kristine for working so hard to bring light to this issue.
As a postpartum nurse, I work with over 20 different pediatricians. As of right now only 1 of them routinely orders pulse oximetery after 24 hours of age. One!! Knowing what I have read from Cora's story, I try to check this on all babies before I send them home from the hospital. Thanks for improving my nursing practice. I pray that your story touches many other practicioners!
Kristine,
You are doing amazing things for babies everywhere, you should be so proud of everything that you're doing and for what you've made Cora's legacy. I know that when I have kids, I will absolutely make sure that their pulse ox is checked at birth, because of you and Cora.
Thanks again for doing all these wonderful things for our country.
Karyn
Kristine-
Much love to you and Cora. I continue to be honored to call you a friend and be inspired by your work and devotion as a mother.
Annie, thank you for sharing your space with Kristine and her important message.
-Melissa
Kristine - Watching your journey has been amazing and you are a wonderful advocate. Cora couldn't have asked for a better mommy. Big hugs to you (always).
Made my night Carla. Thank you for being extra vigilant. Nurses are often the ones that notice a symptom, a sign and save little lives. Knowing you're there looking for those signs and running the pulse ox when you can means much.
Thank you so much for this. I'm 6 months pregnant with my second child, and have only just heard about this recently (first came across your story a couple of weeks ago).
One question-- is there a back-end time limit for doing the pulse ox? You say after 24hrs, but does it need to be done *by* a certain number of days? We're planning a home birth and my midwives may be able to get a hold of a pulse oximetry monitor but if not I figured I'll try to make an appointment with our pediatrician in those first days, and would like to know if that needs to happen within a certain specific time frame.
Thank you!
Marcy:
Thank you for bringing up an important point. Since many women do choose homebirth it is important to consider the best way to have this type of test done outside of a hospital environment. I hope Kristine has some information and ideas for you.
I've been brainstorming what to do for home births for months. The actual tests are cheap, but the machine is expensive. The pulse ox can show a problem any time, so would be helpful at the first ped's visit, but here's why sometime around 24 to 48 hours helps, fetal circulation changes/closes then, and that's when some of these problems happen. As a home birth mama, I think signs and symptoms might be a little more apparent to you without the hospital stress. But, that's just common sense talking. I can't wait until there's a foul proof test to catch these all the time.
Kristine,
Thank you for sharing your story with us. Until reading this post, I was unaware of CHD and its impact. If we are able to add to our family again, I will certainly be requesting this pulse ox procedure. Your work is making a difference. Thank you for educating me and many others.
Kristine, you are an amazing advocate and are an inspiration to all of us. Your work to educate expecting moms is so important; thank you Phdinparenting for helping to reach more parents. I also want to thank you for being such a tireless advocate for breastfeeding, even in the immediate wake of your tragedy. We wish you all the best!
I am so humbled by parents who are moved by tragedy to improve the lives of others. Thank you for this story--I'll share it with the new parents I know.
Thank you for sharing. I was familiar with Cora's story, but was not aware the pulse ox test had to be done after the baby is 24 hours old. I am pregnant now and will certainly be asking the pediatrician to do the pulse ox test on my new little one.
Thank you Kristine for being so strong. You are an amazing woman.
Thanks, Kristine, for reminding everyone of this. Our son's duodenal atresia was only recognized by a careful ultrasound tech and a great maternal-fetal medicine specialist, who also noticed a possible CHD. The heart problem turned out to be nothing serious, but we are grateful to everyone we worked with for their support and careful monitoring of him for the first month after he was born. Keep up the good work.
I am touched by Cora's Story. As a maternal/child nurse with experience in labor and delivery and newborn care for over 35 years I have seen some very sad cases that perhaps in hindsight could have been prevented with more vigilance in the newborn period of life. It is my belief that before early discharges of both mother and baby after delivery CHD was more frequently detected in the hospital.
Newborns frequently have heart murmurs as the fetal circulatory system adapts to the extrauterine environment but newborns should not be dusky in color or have difficulty breathing especially while feeding.
It is so important to have close observation of the newborn post delivery for several days or more whether the pregnancy was uncomplicated or not to make sure extrauterine circulation is established. CHD is difficult to detect during this period of life due to the baby's adaptation to life outside the uterus.
I am so sorry that you had to experience the profound loss of your beautiful daughter to CHD. It is my wish that when you have another child that your baby is followed very closely by neonatologists at the hospital where you deliver for several days before being discharged from the hospital.
Thank you for sharing your experience with other woman and making them aware of this sometimes fatal newborn complication.
Kristine,
Thank you so much for sharing your story and helping to educate us. I'm so sorry for the loss of your beautiful daughter, and I'm baffled when I look at the CHD numbers at the lack of awareness about it. I'm troubled, though, as a home birth mama, about how to reconcile the need for awareness and testing with the belief that most mothers and babies are best served by quiet and rest in the first few days. Thank you for thinking of home birth mothers and brainstorming how midwife training and equipment can make a difference for us too.
Thanks so much for sharing this; I wasn't aware of the frequency of CHD at all. My grandmother had a baby who died of a heart defect- this has made me plan to take my next baby (home birthed, hopefully) to the pediatrician for a pulse ox test after he/she is born.
Thank you for sharing your story.
I hope that your advocacy results in a CHOICE for parents, though, not a mandate. Many of us prefer to educate ourselves and choose whether to have ultrasounds, testing, Rhogam, eye goop, vitamin K, heel sticks, etc, rather than being made to do it.
A friend of mine just had a baby with a heart defect, but luckily they knew about it before she delivered. The baby had a surgery the day after she was born and then another six months later. A year from now she'll have a third and then she should be totally fine. It was scary when we all first heard about it, but thank goodness that she knew and then was able to takes steps to fix it. My heart breaks for you that you didn't have the chance to fix it. More awareness is really needed, since it can be a treatable problem.
Thank you for sharing this... I am pregnant with #3 and will discuss this with my midwife. I am unsure of whether our family practitioner will have the needed equipment but we're not due until next spring so we have time to figure something out.
I do agree with Kate, above, that this should be optional. We homebirth and while we choose to do the PKU, we make the decision on a case by case basis on Vitamin K, ultrasounds, etc. Especially in the current medical environment, I can envision requiring expensive equipment harming homebirth practitioners, especially in areas like mine where midwifery is strongly contested by the medical establishment.
I am so, so sorry. It can be so hard to detect these things. I had a family history of heart defects (my mother’s first child died from hypoplastic left heart syndrome) so I had a fetal echo at 22 weeks. It showed that everything was normal. My son ended up being born with a heart murmur, detected shortly after birth. Thankfully it has caused no problems other than scaring me to death, but it is still there. He has several tiny holes in his heart wall and visits a pediatric cardiologist occasionally to check everything out. I know that we are very lucky that it ended up being harmless.
Hi Kate,
It's what I work tirelessly to do, to make sure mom's know of the option. I didn't know what pulse ox even was, or that it might have saved my daughter's life.
I think that it's misleading to group it with the interventions you mentioned. All are much more invasive.
Much more accurate, to group pulse oximetry with thermometers, blood pressure cuffs, etc. In the clinical setting, it's a basic vital taker.
Interesting that everything you mentioned, besides ultrasound, also deals with conditions/dieseases much less prevalent than heart defects.
I'm not a fan of intervention myself, and thought much before getting behind pulse oximetry and researched much as well. I had to ask myself, did I want the screening because it might have saved MY daughters or because it might save hundreds of babies. I like you hope and work for it to be presented in the clinical setting to parents as a choice. And, it's important to note, as always, not every CHD is caught through this type of screening.
I absolutely haven't seen anything requiring midwives to have the equipment, wouldn't support such a movement and would work hard against it. I don't want pulse ox to be something that drives midwives out of business. And, again, haven't seen anything indicating it would.
For now, my homebirth solution is to think about those signs and symptoms, and to ask for pulse ox screening at the first ped/health provider visit, whenever that might be. And, congrats! If you have any more questions as you get closer to due date, feel free to find me on Twitter or on my contact page.
Marcy, perhaps you can rent the machine & then have a home health nurse visit soon after birth & administer the test?
We had our son treated for severe jaundice due to ABO incompatibility at home; rented the biliblanket, and hired a nurse who visited every day to take blood tests, check his status (this is obviously unrelated to CHD, but wanted to share our traveling nurse experience). It was expensive, though insurance covered most of it. It CERTAINLY beat staying in the hospital or visiting the Dr.'s office every day. Might be worth looking into...?
My husband and I know much too much about Congenital Heart Disease.
Our first son Tristan, was diagnosed with Severe Congenital Heart Disease when I was 5 months pregnant. Though Tristan would be facing open-heart surgeries, his prognosis was very positive. Tristan spent the majority of his short life in CHEO (Children's Hospital of Eastern Ontario) NICU and was home for just 2 days. Tristan passed away suddenly due to a sudden cardiac arrest at only 26 days old.
My husband and I began hosting fundraisers twice a year to help raise funds for CHEO in Memory of Tristan, and to raise awareness of this terrible disease.
Hearing of Cora's story helps me to realize that our family is not alone, and that there are other people who share our grief.
Thank-you Kristine for sharing your story, and helping to spread awareness of CHD.
Thanks, Kristine! I talked to my midwife and to my surprise she does have the equipment! :) She said she felt that in many cases the mother will realize something is wrong, and discussed with me cases she'd had where that happened... but she does have it so I plan on taking advantage of it! Thanks for working to help parents become more aware!
That's great news! And, just to make sure it's clear, many CHDs present with no symptoms, like the National Heart Lung and Blood Institute mentions here: http://www.nhlbi.nih.gov/health/dci/Diseases/chd/chd_signs.html
Detection is really a puzzle. Pulse ox won't always detect a problem either. I can't wait until one day, there's a test that always screens for these. Until them, we'll just use everything we've got. :)
I wonder, for homebirth mothers, if the best way to go might be to develop some kind of directory of either hospitals open to letting homebirth families come in and have the pulse ox done, or of other practitioners willing to do it with their own equipment...
I most likely would not have contemplated this was valuable two or 3 years ago, yet it is fascinating how age evolves the manner you respond to things, many thanks for the blog article it really is great to see anything sensible here and there rather than the typical rubbish disguised as blogs and forums on the web. Regards
I know it's been months so you might not see this, but I think that's a great idea. It's something I'm going to work on after working on getting pulse ox in Indiana hospitals. Thanks for the inspiration!
My Girlfriend was high risk during her pregnancy for gestational diabetes and was never given a level II ultrasound or 3D ultrasound. After birth our son Johnathan was very lethargic and did not want to eat. He rarely opened his eyes and did not cry at all. The hospital told us he may be deaf, and retested his hearing and stated he was not deaf. We told the hospital during their stay something is wrong because he sleeps all the time does not cry and does not want to eat. The hospital assured us he was fine just a very good baby, They came home 2 days later after he was born and he slept constantly despite our efforts to wake him to cuddle , eat , play and to talk to him. The third day at night he began to barely eat at all so we started feeding him half an once every two hours to try to keep his strength up. We called the hospital his fourth day of life about his eating and lethargy, the doctor told us hes normal and he will be fine. Later he started getting cold to the touch and purple splotches formed on his face, all the while he was still breathing. I ran him directly to the ER and they grabbed him and ran him back to a room to work on him. The doctor told us he had low blood sugar and low salts in his blood which caused dehydration which made his heart go out. After the coroners report Congenital Heart defect was released we started researching this. He was four days old and from the symptoms he was experiencing they could have detected this, in utero because she was high risk they could have detected this with a Level II ultrasound. The deafness diagnosis is also a symptom of a congenital Heart defect, why did they do nothing and push us out the door? We are all just a number to the hospital just an invoice to buy someone a new BMW or a bigger house, what about the Hippocratic oath? Oh and to top it all off we received a hospital bill from the ER visit totaling $4,500 because they didn't put him on my girlfriends insurance and didn't not create his own yet. The same hospital he was born in with all his records. What are the policies for the hospitals with checking for birth defects, do they just push you out the door because of the cost of health care and the HMO's not wanting to pay? Something needs to be done, someone needs to stand up and put them in their place so some other innocent baby doesn't die prematurely because a doctor saves a couple bucks so they can buy a new car or Iphone instead of performing a test to save a life.
I'm so sorry that you can relate to my daughter's story. I'm thinking of you and your family, and here if you or your girlfriend need to chat.