Friday, February 29, 2008

Leap Day: Last Day of CHD Awareness Blog-A-Thon


What a month it has been! It's hard to imagine that only a month and a half ago, I found out for the first time that Feb. 14 was the official "CHD Awareness Day," and that Feb. 7-14 was "CHD Awareness Week." One month ago I decided to start this little attempt at educating myself, and along the way maybe a few friends and family members, about the issues facing CHD families and survivors today.

Today I'm a bit emotional about this whole thing. I have met a wide variety of new people through this blog - adult CHD survivors who are worried about finding adequate health care and professionals experienced with adult CHD issues; parents of children who have been lost due to CHD; a teenager on a crusade not to "be a freak" anymore; and bunch of parents who share the same concerns I do, and who are ready and willing to step up to the plate and find ways to work together to get the word out to our government and the public at large. It's been an overwhelming, inspiring, sometimes heartbreaking, joyful, somber and overly intense month.

I've debated what to do with this blog... continue? Let it go? I added some analytics to watch the number of page views per day, and I'm surprised at how many of you have hung in there, who read regularly and who continue to come back. Thank you!

Better yet, the blog is coming up in web searches more and more frequently! This is great news to me, personally. When I was in the hospital while my daughter, Sadie, was fighting for her life, I sought the Web relentlessly for links to groups, people and answers about TGA (her specific diagnosis) and heart defects. I never thought to look for Congenital Heart Defects, and missed out on the support I could have found through that avenue. In fact, my doctors never even mentioned CHD as her diagnosis. Hopefully a few other parents new to the diagnosis will stumble across this site and find the links they need for support, facts and answers.

To answer my own question about what to do with this blog, I finally came to the decision that though it will be work, I'd like to keep the blog going. But in order to do it, I'll need to find new data sources, keep abreast of the news, share new stories and insights and get input on future directions. One month of entries was a huge task to take on. I won't be as regular, that's for sure! I guess what I'm trying to say is, that your help is appreciated and needed - if you stumble across items of interest, stories you'd like to share, newly published facts, articles, etc., I would love to share it with this reading community!

To all who have come and visited, and all of you who will continue to, thank you. I wish you and your families much health and happiness in the year to come!

Best wishes,

Kim, mom to Katie (5, HH) and Sadie (17 months, TGA, ASD, VSD)

Thursday, February 28, 2008

Information from US NIH

I found a fabulous cross-referenced website I'd been missing last night - the National Institute of Health (US) has a webpage chocked full of information and news on CHD. Check it out!

This has been the most helpful page I've found so far... they have links to videos, clinical trials, current research, recent news and advancements, treatment options and more.

Why is it so hard to find info on CHD? Maybe I just wasn't looking in the right places before...

Wednesday, February 27, 2008

In the News: Experienced Hospitals are Better

Babies With Heart Defects Do Best at Hospitals With Most Experience
Centers with a high volume of corrective surgeries show higher survival rates, study found

URL of this page: (*this news item will not be available after 03/16/2008)


Robert Preidt

Monday, December 17, 2007

MONDAY, Dec. 17 (HealthDay News) -- Babies with congenital heart defects are more likely to survive if they're treated at a hospital with the most experience in treating such cases, U.S. researchers conclude.

Researchers at the University of Michigan analyzed figures from the 2003 Kids' Inpatient Database, which is sponsored by the U.S. Agency for Healthcare Research and Quality and includes information on children hospitalized in 36 states.

The study authors focused on two of the most severe congenital heart defects: transposition of the great arteries (TGA), in which major blood vessels leading between the heart and lungs are reversed; and hypoplastic left heart syndrome (HLHS), in which the left side of the heart does not develop properly.

If heart surgery isn't done within a few weeks of birth, both conditions are fatal. An arterial switch operation is used for TGA and an operation called the Norwood procedure is used to correct HLHS.

Reporting in the online edition of Pediatric Cardiology, the team found that the risk of an infant dying in a hospital during or after one of these operations varied greatly depending on the number of these procedures performed at a hospital. Death rates ranged from less than 1 percent to more than 10 percent for the arterial switch operation, and from around 10 percent to more than 35 percent for the Norwood procedure.

"The relationship between hospital volume and risk of dying was significant across the spectrum for both defects, though in the case of arterial switch operations, the difference dwindled among hospitals that performed about 15 or more in a year," study author Dr. Jennifer Hirsch, a pediatric cardiac surgeon, said in a prepared statement.

Copyright (c) 2007 ScoutNews, LLC. All rights reserved.

Tuesday, February 26, 2008

The Sibling Factor

I often think that siblings have the toughest task of any family member when a family is faced with CHD. Not only do they lose much of their parents attention during "high-stress" periods, they fear for their sibling, and they are often left out of the loop, but realize something is wrong.

I can see substantial changes in my oldest daughter, who is now five. She was a few weeks shy of turning four when her sister was born with TGA. Her world crumbled around her - instead of going to the hospital and meeting her new little sister we'd all been so eagerly anticipating, she came to the hospital and waited a few hours, until we realized something was wrong and sent her home with her grandparents. It was several days before she saw us again, and her new baby sister was hooked up to machines, swollen and blue. I didn't realize at the time how bad her new baby sister looked - but looking back at the photos, I'm shocked that I let my older daughter see her in that condition. I guess we just do the best that we can at the time.

My oldest daughter was bounced between family members while we stayed in the hospital that first month. It was also the first month of school, and she missed the crucial bonding period in her new preschool. By the time we came home, she'd fully regressed in her potty training, and had stickers covering her babies "to monitor their hearts."

The next few months didn't let up for her. We faced a flurry of doctors appointments every week, the challenge of fitting into a preschool with kids she didn't know (who by that point all knew eachother), and a crying, frantic bundle of energy that she so desperately wanted to cuddle with, but couldn't stand to be touched. Our oldest daughter tried so hard to love her baby sister, to hold her during "good times" and she was fiercely protective of her. I remember one time I had to take both girls to the doctor, and she got very angry with a nurse who was going to give Sadie a shot. She just wouldn't stand for it.

Over the past year and a half, the baby has gradually calmed a bit, and now will even give her big sister hugs from time to time. Just the other day, the eldest was hurt, and the youngest came running to give her a hug and kiss. It melted my heart - finally my big girl is getting the baby sister she's always dreamed of.

Since those early days with the baby I've seen a lot of changes in my oldest daughter. She's more self-reliant. She's confident and more outgoing. But at the same time, she panics if anyone is sick or hurt. She is scared of the doctor. And she's incredibly clingy with the people she is closest to. It's not a surprise in anyway that we've noted these changes... but it is sad that she's gone through these changes so quickly. I miss her innocence.

I'm so proud of my oldest daughter. She has been such a good girl throughout the entire ordeal, and she's so incredibly compassionate and strong. There were many times this past year during this whole ordeal that I simply would not have made it another day without her. She's a shining light and I'm so thankful she came first, to light the way for her special little sister.

Monday, February 25, 2008

Somewhere... Someplace...

Somewhere…someplace… today…
A family is waiting to hear…
Is something wrong with their baby?
The answers aren’t quite clear…

This family has entered an unwanted world…
And they just don’t know what to expect…
Somewhere…someplace… today
They first heard the words: heart defect.

And how they hoped this was not true…
And thought… this cannot be…
I too… know just how this feels…
For one day…this was me.

A man and a woman embrace…
Their baby is in surgery…
They long to see her face…

They haven’t got to hold her yet…
Without…a cord or line…
They pace the room awaiting news…
And hope she’ll be just fine.

Prayers fill this busy waiting room…
And mom and dad are scared…
The tiniest hearts are repaired.

A child’s growing fast…
His mom thinks…can this last?

It’s almost easy…to forget…
That anything is wrong…
Her child seems so strong.

Somewhere…someplace… today…
A little boy fights…just to live
A father holds his tiny hand…
His love…all he can give…

The doctor’s are all baffled…
They fear that he might die…
A family says goodbye…

Somewhere…someplace…each year..
More than 40,000 families will see…
What it means…when something’s wrong…
They’ll face a CHD.

Today…for just a moment…
Make time to tell someone you know…
“I’ve been changed by a heart defect.”

Author - Stephanie Husted

Sunday, February 24, 2008

References on Prenatal CHD Diagnosis Research

It looks like there are others out there who, like me, want to absorb all they can about CHD, incidence rates, diagnosis rates and other information. Often that info is hard to find.

The following references were uncovered by MaryAnn Kuzio, mom to a son with CHD. Thanks for sharing this information MaryAnn!

Trends in Prenatal Diagnosis, Pregnancy Termination, and Perinatal Mortality of Newborns With Congenital Heart Disease in France, 1983-­2000: A Population-Based Evaluation
Babak Khoshnood, MD, PhD*, Catherine De Vigan, MD*, Véronique Vodovar, RN*, Janine Goujard, MD*, Anne Lhomme, MS*, Damien Bonnet, MD{ddagger} and François Goffinet, MD, MPH*
Paris Registry of Congenital Malformations, Epidemiological Research Unit on Perinatal and Women's Health, INSERM U149, Villejuif, France
Service de Cardiologie Pédiatrique, Hôpital Necker-Enfants, Malades, Paris, France

Published online January 3, 2005
PEDIATRICS Vol. 115 No. 1 January 2005, pp. 95-101

Objective. To examine population-based overall and malformation-specific trends in the prenatal diagnosis, pregnancy termination, and perinatal mortality for congenital heart disease (CHD) during a period of rapid
progress in prenatal diagnosis and medical management of CHD and to explore the impact of prenatal diagnosis on early neonatal mortality for specific (isolated) cardiac malformations.

Methods. A total of 1982 cases of CHD, which were not associated with a known chromosomal anomaly, were obtained from the Paris Registry of Congenital Malformations. Main outcome measures were trends in the proportions diagnosed and terminated before birth, stillbirth, and early (<1 day, 1-week) neonatal mortality for (1) all cases; (2) all cases excluding isolated ventricular septal defects; and (3) malformation-specific trends for transposition of great arteries, hypoplastic left heart syndrome, coarctation of aorta, and tetralogy of Fallot. Analyses included cusum and binomial regression models for analysis of the trends during 1983­2000.

Results. Prenatal diagnosis rates for CHD increased from 23.0% (95% confidence interval [CI]: 19.0­27.4) in 1983­1988 to 47.3% (95% CI:43.8­50.8) in 1995­2000. Termination rates increased between 1983 and 1989 (9.9%; 95% CI: 7.2­13.2) and 1989 and 1994 (14.7%; 95% CI: 12.3­17.4) but seemed to remain stable thereafter. Other than for hypoplastic left heart syndrome, pregnancy termination was exceptional for the other 3 specific malformations examined. Early neonatal mortality decreased to less than one third in the period 1995­2000 as compared with 1983­1989 (risk ratio, first-week mortality: 0.31; 95% CI: 0.18­0.53). First-week mortality was significantly lower for cases of transposition of great arteries that were diagnosed before birth (risk difference: 15.4%; 95% CI: 4.0­26.7).

Conclusions. Progress in clinical management, together with policies for increased access to prenatal diagnosis, has resulted in both a substantial increase in the prenatal diagnosis and considerable reductions in early neonatal mortality of CHD in the Parisian population.


Prenatal diagnosis of six major cardiac malformations in Europe ­: A population based study
Ester Garne and THE EUROCAT WORKING GROUP, From the University of Southern Denmark, Odense C, Denmark
Members of the Eurocat Working Group:Bianchi F, CNR Institute of Clinical Physiology, Pisa (I); Garcia-Minaur S, Hospital de Cruces, Baracaldo (E); Gillerot Y, Institut de Pathologie et de Genetique, Loverval (B); Riano Galan I, Registry of Asturias, Orviedo (E); Nelen V, Provincial Institut voor Hygiene, Antwerp (B); Nevin N, Queens University of Belfast (UK); Philip N, Hopital d¹Enfants de la Timone, Marseilles (F); Stoll C, Hopital de Hautepierre, Strasbourg (F); Stone D, Yorkhill Hospital, Glasgow (UK); de Vigan C, Inserm, Paris (F); de Walle H, University of Groningen (NL).

Aim. To present data on prenatal diagnosis of six major cardiac malformations in low-risk European populations.

Methods. Data from 12 Eurocat registries on congenital malformations. All registries have multiple sources of information and use the same methods of data collection and coding. The six cardiac malformations included were hypoplastic left heart, tricuspid atresia, single ventricle, Tetralogy of Fallot, transposition of great arteries and common A-V-canal.

Results. There were significant differences in the proportion of cases diagnosed prenatally, with the highest detection rate in France (91% for single ventricle in Paris) and the lowest detection rate in countries without prenatal ultrasound screening (no cases diagnosed prenatally in the Danish registry area). Prenatal detection rate was significantly higher for the three malformations affecting the size of the ventricles (hypoplastic left heart, tricuspid atresia, single ventricle) compared to the other three malformations (46% versus 24%, p<0.001). Time of diagnosis was late, with only one third diagnosed before 24 weeks of gestation. The risk of fetal death seems to be low.

Conclusion. There are significant regional differences in prenatal detection rate of major cardiac malformations in Europe.

Abstract Fetal echocardiography has impacted the fetus with congenital heart disease in many important ways. Advances in fetal echocardiography have allowed for more accurate and earlier detection of cardiac abnormalities. In turn, the prenatal diagnosis of cardiac abnormalities has improved the care and outcome of selected fetuses with severe
cardiac malformations or arrhythmias. Fetal echocardiography has improved the understanding of the development and evolution of congenital heart disease in utero, and it may serve a role in identifying candidates for prenatal intervention. The prenatal diagnosis of congenital heart disease has allowed for better counseling and preparation of families regarding the anticipated prenatal development of the fetus as well as the expected postnatal management plans and prognosis. This article reviews the impact of fetal echocardiography in these and other areas.


Importance of timely diagnosis of congenital heart disease
Houssem Masmoudi & Martial M Massin
Queen Fabiola Children¹s University Hospital, Department of Paediatric Cardiology, Free University of Brussels (ULB), Brussels, Avenue J. J. Crocq, 15 B-1020, Belgium.

Most important types of congenital heart disease can be recognized early during prenatal or neonatal screening. However, a substantial proportion of cardiac defects are not diagnosed in due time. Such delay may lead to serious complications or even death. This article reviews the impact of screening strategies upon the outcome in pediatric cardiac patients.

Objective: About 1% of live born children have congenital heart defects (CHD). Knowledge of the true incidence of CHD is important because of the risk of bacterial endocarditis in patients with heart defects. This knowledge could also serve as a basis for research on the etiology of CHD. The aim of our study was to investigate the incidence of CHD in
children born in Iceland during a ten year period, from 1990 to 1999. A similar study on CHD was carried out in Iceland for children born 1985-1989. The incidence of CHD in the present study was compared to the previous and to similar studies from other countries. Material and methods: Information about the patients were obtained from medical records from two hospitals covering the whole country, a private clinic of pediatric cardiologists, echocardiography database, autopsy reports and death certificates. Distribution of specific heart defects, age at diagnosis, symptoms leading to the diagnosis, referral pattern, treatment and quality of life was investigated. Other congenital defects, chromosomal defects and syndromes in the patients were studied.

Premature children with patent ductus arteriosus (PDA) and children with patent foramen ovale (PFO) or atrial septal defect (ASD) smaller than four millimeters were excluded. We also studied the incidence of bicuspid aortic valve (BAV). All diagnoses were confirmed with echocardiography and in some cases cardiac catheterization.

Results: Between 1990 and 1999 there were 44,013 live births in Iceland, 740 children were diagnosed with a CHD or 1.7% of live born children. Yearly incidence varied from 1.04% of live births in 1991 to 2.34% in 1997.
Male/female ratio was 1/1. The distribution of the defects was following: ventricular septal defect (VSD) 338 (45.7%), ASD 90 (12.2%), PDA 85 (11.5%), valvar pulmonal stenosis 48 (6.5%), BAV 38 (5.1%), coarctation of the aorta 28 (3.8%), tetrology of Fallot 22 (3.0%), transposition of the great arteries 14 (1.9%), aortic stenosis 11 (1.5%), common atrioventricular septal defect 10 (1.4%), mitral valve regurgitation 9 (1.2%), sub-aortic stenosis 7 (0.9%) and hypoplastic left heart syndrome 5 (0.7%). Other defects were less frequent.

About 47% of children with CHD were diagnosed either before birth or before discharge from the delivery institution. A cardiac murmur on examination was the most common symptom leading to the diagnosis of CHD, 631 patients (85.3%). Extracardiac anomalies were seen in 89 patients (12.0%). Chromosomal abnormalities were seen in 36 patients of whom 28 had Down's syndrome. The majority or 499 patients have no symptoms but are still in follow-up, 20 patients have daily symptoms and/or receive medical treatment and 27 patients have died.

Conclusions: Annual incidence of patients diagnosed with a CHD has increased during the study period. This is observed in minor CHD but the incidence of major defects does not alter. This yearly incidence (1.7%) is higher than in
the previous study, where it was 1.1%. The difference can partly be explained by the BAVs, which were excluded in the 1985-1989 study. But the number of CHD diagnosed each year has increased, this being most pronounced in the last three years. The annual incidence in our study is also higher than in other population studies. The most likely
explanation for the higher incidence in our study is the fact that access to pediatric cardiologists is very good in Iceland. Diagnosis, registration and follow-up is conducted by only a few cardiologists for the whole nation and takes place at a single pediatric cardiology center. Of 740 patients diagnosed with CHD in the study period 713 are alive. The outcome of the therapy is good and the majority of the patients has no symptoms.

Saturday, February 23, 2008

Insurance Info

I received two replies that I thought may be helpful to share concerning the insurance issue previously posted. Hopefully they might be helpful for families like ours that struggle to find insurance answers.

Also, I found the following organization, Coalition for Affordable Health Care, that has data, facts and info about current insurance initiatives, bill failures, proposals and more in the US. According to them, 60% of persons in small businesses is uninsured. Other uninsured majorities include minorities, self-employed and high-risk individuals.

We became pregnant with our son right after my husband had graduated from college, so we were on the state health care plan. The entire pregnancy we were unemployed as my husband searched desperately for a job. Two weeks
before Jacob was born He found employment with a great company with decent benefits.

Being on the state health plan, if you are pregnant when you are on it they are obligated to cover all the pregnancy, birth and one year after. So, we got lucky, even though we had other insurance by the time he was born - his
surgery, everything was covered 100% - we never saw a bill. We realized how fortunate we were as we visited with other families who were still trying to pay their hospital bills.

A year later my husband switched companies and there was to be a period before benefits would start - everyone would be covered under cobra - except our son. Luckily the new employers used the same insurance company, so we were able to keep from breaking coverage. It is a hard reality to us that my husband would need to stay employed with a company that offered great benefits becuase we wouldn't be able to find insurance for our son otherwise. We looked at other supplemental things and it is always the same - no coverage for our son. We considered
doing some of those life insurance things for children - no coverage for him. We've been more fortunate than most - I just don't know how they manage it.

There is a lot of pressure. Any time my husband considers looking for new employment, benefits are a MAJOR factor - as well as not wanting to move away and leave our awesome doctors who know us and our situation so well. I can't help either, we have 5 children (our fifth born in December - almost 2 months old now) and I stay at home with them and teach piano lessons, so like in your family, the responsibility to ensure our son lies on my husbands shoulders. I feel guilty too not being able to do more to help.

California Children’s Services will pay for your child’s cardiac care until she is 21 years of age. She should also be eligible for MediCal as well or maybe Healthy Families. I see middle income and lower income families that have the same problem. Check out the webpage of to see if you are eligible.

NOTE: Norene has been kind enough to offer to look further into this with other states. To my knowledge, the state in which I reside (Virginia) does not have a program under which we can be covered, as a family of 4 (which is what we are) must make $42k or less to qualify.

Thank you, ladies, for sharing your information and stories!

Friday, February 22, 2008

What we need to convey

Our cause is lost in a sea of cause-related campaigns. But ours is equally as important as breast cancer and AIDS.

I came across a company called Ethos JWT, today, that specializes in social responsibility for corporations and, of course, advertising. Here is a sample of what they do.

Can you imagine what we can do if we can get our message as part of a corporations national campaign? Forget the PSAs, let's dig in to our employers (or our husbands or whoever) and get them onto our campaign. Kids causes are the #1 "seller" cause, so we should be a shoe in. Or, let's come together ourselves (maybe through CHIN?) and get something like this done and on the air.

Thursday, February 21, 2008

Moms Will Find the Answers to Save our CHD Kids

Today, I've been inspired and revitalized on the whole CHD front. My day was full of outstanding presentations at the Consumer Trends Forum here in Boston. I've learned a ton, and will have more take-homes than I think I've ever had from a conference. But this is CHD month, and I'm on this mission to promote awareness, and thus one thing is stuck out in my head.

These past few weeks I've been mulling over the whole CHD awareness thing. Why don't people know about it? Why is it a "silent" epedemic? Why can't we get more research dollars?

You know why? Our children do not have a vote. Most CHD survivors don't realize they are part of an overall picture. And MOMS are not strong enough advocates. Sure, we tell our close friends and family, maybe even a congress member, but we aren't using our greatest strengths to change the face of this daunting task.

Don't get me wrong. As parents of children with CHDs, there is nothing more we want than to find treatments, answers, cures. There is nothing more we want than to save another mother from the pain we have seen ourselves and watched our children endure. But we are discounting ourselves.

One of the speakers at today's meeting was Maria Bailey, founder of a mom-based marketing group called BSM Media. A lot of things she talked about struck a chord with me (e.g. finding out I'm a mompreneur, how moms will decide who the next president is and how much major brands want to woo us). I left with the new reality that more moms are coming who are connected, who share my values, and who work in a similar manner that I do, yet share my family-first values. We, the mompreneurs, soccer moms and SAHMS, have more power than I ever imagined.

Why does this relate to CHD? Because I think we're underselling ourselves as moms. I myself have said, "I'm just a mom" when referring to my role in CHD awareness. I've made a weak attempt at awareness. And I discount my desire for change (I want it, but have felt that I can't achieve it on my own).

Here's the reality, though, and it's a kicker. I'm part of a $2.1 TRILLION dollar buying power who can influence my favorite brands. My vote will change our future. My voice does matter, because it's not a voice of one, but of thousands. We have POWER, and it's time to tap into it.

Tonight I learned that we have more influence than I ever imagined. Now it's our responsibility to tap into that power to bring attention to the matters we care about most - the health, well-being and future of children and adults with CHD. It's time to erase the "silent" part of the epidemic we face and find answers. I'm fired up and ready for action. Now for the next step...

- How do we come together to accomplish changes in legislation?
- How do we join forces and change the pitiful funding for the research that will change our children's lives?
- And how do we use our buying power and leverage to influence major companies to join our fight for our children and future generations?

Wednesday, February 20, 2008


I'm wondering how many other families there are out there who have heart children that have been deemed "uninsurable." Here is our family's story...

Our daughter was born in September and had open heart surgery almost immediately. My husband's employer was AWFUL about giving him time off to be with his tiny newborn, and he soon began to look for another position elsewhere. Fast forward to April of 2007, and he took a contracting job for a major financial firm. Because he is not an employee, he does not get benefits. I am a small business owner, so we made the assumption that we could still find somewhat affordable insurance.

The reality is that we can not find ANY insurance that is willing to provide coverage for our "heart" baby, who had the ASO switch in September 2006. We have been told that it will be a total of five years before insurance will be considered for her, but that the rest of our family may be covered.

It's a sad state that our healthcare system is in. I would consider our family to be "middle class," yet we have no access to insurance for our daughter. We have been told time and again that we would be better off in terms of health care for her if we were on government assistance (welfare). Every time we walk into the pediatrician's office (which, of course, is often) it's a huge hit.

Has anyone else faced this dilemma? Email me at

Tuesday, February 19, 2008

CHD Info From the March of Dimes

The March of Dimes is a one of the several reliable sources of information on Congenital Heart Defects. Below are some excerpts from their website.
Congenital Heart Defects

About 40,000 infants (1 out of every 125) are born with heart defects each year in the United States (1). The defect may be so slight that the baby appears healthy for many years after birth, or so severe that its life is in immediate danger.

Heart defects are among the most common birth defects and are the leading cause of birth defect-related deaths (2). However, advances in diagnosis and surgical treatment have led to dramatic increases in survival for children with serious heart defects.

What tests are used to diagnose heart defects?
Babies and children who are suspected of having a heart defect are usually referred to a pediatric cardiologist (children’s heart disease specialist). This doctor will do a physical examination and often recommend one or more tests. These tests often include a chest X-ray, an electrocardiogram (which records heart rate patterns) and an echocardiogram (a special form of ultrasound that uses sound waves to take pictures of the heart). All of these tests are painless and non-invasive (nothing enters the child’s body). Some children with heart disease also may need to undergo a procedure called cardiac catheterization. In this procedure, a thin, flexible tube is inserted into the heart after the child is given medications to make him sleepy. This test provides detailed information about the heart and how it is working.

What causes congenital heart defects?
In most cases, scientists do not know what makes a baby's heart develop abnormally. Both environmental and genetic factors appear to play roles.

A few environmental factors are known to contribute to congenital heart defects. Women who contract rubella (German measles) during the first three months of pregnancy have a high risk of having a baby with a heart defect. Other viral infections also may contribute, as may exposure to certain industrial chemicals (solvents) (1, 3). Drinking alcohol in pregnancy also can increase the risk of heart defects, as may cocaine (3).

Certain medications increase the risk. These include the acne medication isotretinoin (Accutane and other brand names), lithium (used to treat certain forms of mental illness) and certain anti-seizure medications (3).

Certain chronic illnesses in the mother may contribute to the risk. For example, women with diabetes are at increased risk of having a baby with a heart defect (3). However, women with diabetes can reduce their risk by seeing their health care provider before pregnancy to make sure their blood sugar levels are well controlled. Women with an inherited error of body chemistry called phenylketonuria (PKU) also are at high risk of having an affected baby, unless they follow a special diet before and during pregnancy (4). Some studies suggest that women who do not consume enough of the B vitamin folic acid before and during the early weeks of pregnancy may be at increased risk of having a baby with a heart defect (3).

Scientists are making progress in understanding the genetics of heart defects. Since the 1990s, they have identified several gene mutations (changes) that can cause heart defects. For example, a March of Dimes grantee discovered a series of genetic steps that appears to contribute to a common, important group of malformations affecting the heart’s outflow tract (5). He also identified a gene that can cause a heart defect called an atrial septal defect (a hole between the upper chambers of the heart) and one that may contribute to hypoplastic left heart syndrome (underdevelopment of the heart’s main pumping chamber) (6, 7).

Heart defects also can be part of a wider pattern of birth defects. For example, about one-half of children with the chromosomal abnormalities Down syndrome (mental retardation and physical birth defects) and Turner syndrome (short stature and lack of sexual development) have heart defects (8). Children with Down syndrome, Turner syndrome and certain other chromosomal abnormalities should be routinely evaluated for heart defects. Heart defects also are common in children with a variety of inherited disorders, including Noonan (short stature, learning disabilities), velocardiofacial (craniofacial defects and immune deficiencies), and Holt-Oram (limb defects) syndromes (9).

What research is under way on congenital heart defects?
Scientists funded by the March of Dimes are among many who are trying to learn more about the causes of heart defects, so that they can develop better ways of preventing and treating them. For example, a number of March of Dimes grantees are studying genes that may underlie specific heart defects. March of Dimes grantees also are looking at how environmental factors (such as a form of vitamin A called retinoic acid) may contribute to congenital heart defects. Other researchers are exploring the possibility of prenatal surgery to correct heart valve abnormalities and help prevent serious heart problems (10).

For more information

1. American Heart Association. Congenital Heart Defects. Accessed 7/8/05.

2. Kochanek, K.D., et al. Deaths: Final Data for 2002. National Vital Statistics Reports, volume 53, number 5, October 12, 2004.

3. Mone, S.M., et al. Effects of Environmental Exposures on the Cardiovascular System: Prenatal Period Through Adolescence. Pediatrics, volume 113, number 4, April 2004, pages 1058-1069.

4. Michals-Matalon, K., et al. Nutrient Intake and Congenital Heart Defects in Maternal Phenylketonuria. American Journal of Obstetrics and Gynecology, volume 187, 2002, pages 441-444.

5. Yamagishi, H., et al. Tbx1 is Regulated by Tissue-Specific Forkhead Proteins Through a Common Sonic Hedgehog-Responsive Enhancer. Genes & Development, volume 17, January 15, 2003, pages 269-281.

6. Garg, V., et al. GATA4 Mutations Cause Human Congenital Heart Defects and Reveal an Interaction with TBX5. Nature, volume 424, July 24, 2003, pages 443-447.

7. Garg, V., et al. Mutations in NOTCH1 Cause Aortic Valve Disease. Nature advance online publication, July 17, 2005.

8. Hoffman, J.I.E. Congenital Heart Disease, in Rudolph, C.D, and Rudolph, A.M. (editors): Rudolph’s Pediatrics, New York, McGraw-Hill Medical Publishing Division, 2003, pages 1780-1783.

9. Pajkrt, E., et al. Fetal Cardiac Anomalies and Genetic Syndromes. Prenatal Diagnosis, volume 24, 2004, pages 1104-1115.

10. Ohye, R.G., and Bove, E.L. Current Topics in Congenital Heart Surgery, in: Allen, H.D., et al (editors), Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, 6th Edition, volume 1, Philadelphia, Lippincott Williams and Wilkins, 2001.

Monday, February 18, 2008

Detecting CHDs

How are CHDs detected at birth? Are they even tested for? Of course every doctor listens to a baby's heart, but time and again they do not report murmurs (which are frequent and can often be harmless in small children). Many times babies "blue" but are sent home anyway. The following is an article from 2005 posted recently on a list serv on CHIN that tells about the latest trends in detecting CHD.

The evolution of diagnostic trends in congenital heart disease: A
population-based study
* V Grech
1Paediatric Department, St. Luke’s Hospital, Guardamangia, Malta
Correspondence to: V Grech
Senior Registrar, Paediatric Department of St. Luke’s Hospital,
Guardamangia, Malta. Fax: 240176; email:

Objective: To analyse historical trends in diagnosis of congenital heart
disease (CHD) in all diagnosed cardiac malformations born between 1945
and 1994 in a population-based study, the first of its type.

Methodology: Retrospective analysis of age and mode of diagnosis was
carried out in 953 patients with CHD, in the setting of a regional
hospital providing diagnostic and follow up services for all of Malta.
Main outcome measures were age at diagnosis, cumulative percentage
diagnosis by age and mode of diagnosis.

Results: A significant negative correlation of age at diagnosis with
time was found for both lesions not requiring intervention, and
requiring intervention (P < 0.0001). Cumulative percentage diagnosis by
age increased progressively with time. Echocardiography increased the
birth prevalence of definitively diagnosed defects, particularly of
lesions not requiring intervention. The annual number of cardiac
catheters for these conditions has decreased with an increased number
and proportion of interventional catheters. The current catheterization
rate for CHD is 6.1/100,000 population.

Conclusion: Echocardiography has led to an increase in the diagnosis in
the birth prevalence of CHD, and provided early diagnosis, supplanting
cardiac catheterization as a primary diagnostic tool.
Published online January 3, 2005
PEDIATRICS Vol. 115 No. 1 January 2005, pp. 95-101

Sunday, February 17, 2008

When Time Stands Still

"When Time Stands Still"
by Debbie Hilton-Kamm

It starts with news impossible to hear
It conjures up your every fear
It's when they say your child is ill
That's when time just stands still

In that moment, that suspended time
A thousand thoughts run through your mind
Will he ever laugh and play?
Will I see his wedding day?

All the planning, the hopes and dreams
Are put on hold -- just what does this mean?
His crib is empty, his toys alone
For now, the hospital will be his home

This is a place where time stands still
Where the void's too large to ever fill
For in a hospital's intensive care
Children lie, some unconscious, some aware

And time is measured by a new yardstick
Every second marked by a monitor's tick
Noting every breath the child takes
And every beat his tired heart makes

Just a moment of watching a child writhe or strain
In sedated confusion, or fear or pain
Or pleading for a drink to which you can't oblige
Seems like far more than an entire lifetime

And the children who live far too long
In hospital gowns, trying to be strong
They have old souls, that's what they say
Because in each moment they've lived a thousand days

For those who say time goes by too fast
Sit with an ill child, and see just how slowly time can pass

Saturday, February 16, 2008

CHD Awareness Poem

Your help is needed
And that’s not all,
we need awareness,
for the little and small.

We need your help,
to spread the word ...
of congenital heart defects,
for little have heard!

This is a deadly defect,
affecting many that are born,
turning lives upside down ..
leaving dreams shattered and torn.

Congenital heart defects,
known as CHD's,
are the #1 birth defect,
but a hidden disease.

Doses of medicine,
many times each day,
a little child,
that can't run or play,
his little heart,
can't take the stress,
his little body
just needs to rest.

A little baby
that struggles to eat,
with tiny blue lips,
and hands, and feet.
A mother stands by
her baby's grave,
praying through research,
others will be saved!

Written by Angela Brooks

Friday, February 15, 2008

Makenzy's Story

What is it like to be a big brother or sister to a kid with a heart defect? Makenzy, age 8, has a sister who is one and has a HLHS. Here' s what she wrote...

When my mom had Jameson, I was very sad. My Aunt had to lie in bed with me sometimes at night and talk to me about it. My Aunt and uncle were watching me and my sister while my parents were gone. When I came to see Jameson at the hospital, I was sad when I saw the stuff they had on her. But I was very happy to see my parents and Jameson.

I remember the first time Jameson came home. Me and my sister really wanted to hold her but we couldn’t. My parents told me that she was very sick and thought it might hurt her. The first time I saw her scar I started to cry. My parents told me everything was OK. WE have to wash our hands before we touch Jameson because it is important we don’t get her sick. We do not go out much because Jameson might get sick.

I am very happy that my sister is here with us today.

Thank you for sharing your story, Makenzy! Lots of other kids, like you, have brothers and sister's with CHD. I'm sure they will like to hear your story, because they have a lot of the same feelings you do. Jameson is lucky to have you as a big sister!

Thursday, February 14, 2008

7 For 7: Advice for CHD Parents

Happy Heart Day! Valentines is the international day for CHD awareness. Thank you to everyone who has checked into this blog, who has made submissions, and who is out there spreading awareness for our heart children and adult survivors!

This final "7 For 7" entry for this year's blog-a-thon is provided by Christy, mom to Aubrey (who has a healthy heart) and Sydney (who has TA). Thanks, Christy, for sharing your advice!

~ ~ ~ ~ ~ ~ ~ ~ ~ ~

IF I was to ever give advice, to family member and/or caregiver of a child with a congenital heart defect, it would be 7 things:

(1) Don’t be afraid to ask for help
(2) Lean on people when they offer. People want to help!
(3) Be your child’s advocate.
(4) Always trusts yourself. You know your loved one more than anyone else.
(5) Cherish every moment and not sweat the small stuff.
(6) However difficult may be, treat your child like a "normal" child. I did not save my child's life, just to keep her from experiencing it.
(7) Find a support group, so you are never alone. I am so lucky to have friends, family, a wonderful community and Mended Little Hearts support group.

Remember how special these children are. They have so much strength. My little Sydney, despite her medical problems, amazes me. She is full of love and life. She always has a smile, no matter what is going on. I thank God for her every day, and despite the fact it is hard being a “heart parent,” there is NEVER a day, or a moment, I would change, if it meant not having Sydney in our lives. She belongs in this world--our little Angel.

Wednesday, February 13, 2008

7 For 7: Strangers Who Saved My Daughter's Life

On September 4, 2006, my daughter Sadie entered this world with a bang - 17 minutes after we arrived at the hospital. She came out screaming, but immediately turned blue. Six hours later, she was diagnosed with Transposition of the Greater Arteries, ASD and VSD (holes in the heart) and pulmonary hypertension. During her first days, the actions of complete strangers were what kept her alive. So today I'd like to share a personal 7 for 7...

7 Strangers Who Saved My Daughter's Life

1. The Unknown Organ Donor.
Someone's life was lost, and because of him or her, my daughter's life was saved. A piece of his is now beating as part of Sadie's heart. It's almost too much to think about.

2. The Ambulance Driver. He was supposed to get off hours before he took my daughter on her life transport from the hospital she was born in to UVA, where she had a atrial septotomy that would keep her alive until she was viable for surgery. Before he left, I had to sign papers for him. As I signed them, I asked him to get her there safely. He squeezed my hand, and with tears in his eyes said he would. And he did.

3. The PICU Nurse. Actually, there was an entire parade of nurses that held my daughter's tiny life in their hands and saved her time and again. From the first nurse I met, who had been up all night with her, trying to get her vitals on an even keel, to the last one I said goodbye to, who hugged Sadie and wished us luck... they don't get enough credit for all they do.

4. The Unknown Blood Donors. Sadie had so many transfusions during her stay in the hospital that I lost track of them. I know there were a minimum of six. Because the blood bank requirements would not allow us to donate blood (we would need to have it screened prior to its use, and there was no time for it), we relied on the life that flows through other people.

5. The PICU Residents. Overworked, underpaid and sleep-deprived, they save lives every day in pursuit of their careers. I once watched 6 of them surround Sadie's incubator, trying to determine what cocktails they could concoct to save her life "this time" and was scared that there wasn't an attending present. I did not know a single person's name. I still don't. But they poured their hearts and minds into saving that little girl that night.

6. Dr. Albrecht, our cardiologist. No longer a stranger to us, he happened to be on call the night she was born. He diagnosed her, and sent her on her way to the life-saving procedure that she needed. It just so happened that his specialty is TGA. It just so happened that he was on call that night (we didn't have a NICU at the hospital she was born in). Even he is astounded by the way that it turned out that he was the person there, and right in the nick of time. Every time he sees Sadie, and comments on how the stars were aligned that night so that she could be with us today.

7. Jatene and Mustard. In 1975, Dr. Jatene succeeded with the first arterial switch operation for the type of defect Sadie had (Transposition of the Greater Arteries). The Jatene procedure, or arterial switch, was pioneered by Canadian cardiac surgeon William Mustard and it was named for Brazilian cardiac surgeon Adib Jatene, who was the first to use it successfully. Twenty five years later, my daughter is "fixed" because of their research. This is why advocacy is so important - because awareness of heart defects will lead the public, government and corporations to fund research. And research can and does lead to finding ways to repair more hearts.

There are so many other strangers out there, but these are the first 7 that came to mind. Want to share your story, comments or list of strangers? Email me at and I'll be happy to post for you!

Tuesday, February 12, 2008

7 For 7: FAQ From the AHA

7 Frequently Asked Questions
Taken from a fact sheet provided by
American Heart Association

  1. What is a congenital heart defect?
    Congenital heart defects are structural problems with the heart present at birth. They result when a mishap occurs during heart development soon after conception and often before the mother is aware that she is pregnant. Defects range in severity from simple problems, such as "holes" between chambers of the heart, to very severe malformations, such as complete absence of one or more chambers or valves.
  2. Who is at risk to have a child with a congenital heart defect?
    Anyone can have a child with a congenital heart defect. Out of 1000 births, 8 babies will have some form of congenital heart disorder, most of which are mild. If you or other family members have already had a baby with a heart defect, your risk of having a baby with heart disease may be higher.
  3. How many people in the United States have a congenital heart defect?
    Estimates suggest that about 1,000,000 Americans have a congenital heart defect. Approximately 35,000 babies are born with a defect each year. (EDITOR's NOTE: According to the CDC, the number is approximately 40,000. See link above.)
  4. Why do congenital heart defects occur?
    Most of the time we do not know. Although the reason defects occur is presumed to be genetic, only a few genes have been discovered that have been linked to the presence of heart defects. Rarely the ingestion of some drugs and the occurrence of some infections during pregnancy can cause defects.
  5. How can I tell if my baby or child has a congenital heart defect?
    Severe heart disease generally becomes evident during the first few months after birth. Some babies are blue or have very low blood pressure shortly after birth. Other defects cause breathing difficulties, feeding problems, or poor weight gain. Minor defects are most often diagnosed on a routine medical check up. Minor defects rarely cause symptoms. While most heart murmurs in children are normal, some may be due to defects.
  6. How serious is the problem?
    Congenital heart defects are the most common birth defect and are the number one cause of death from birth defects during the first year of life. Nearly twice as many children die from congenital heart disease in the United States each year as die from all forms of childhood cancers combined. Over 91,000 life years are lost each year in the US due to congenital heart disease. Charges for care exceed 2.2 billion dollars, for inpatient surgery alone.
  7. Are things improving?
    Definitely. Overall mortality has significantly declined over the past few decades. For example, in the 1960s and 1970s the risk of dying following congenital heart surgery was about 30% and today it is around 5%.

Monday, February 11, 2008

7 For 7: Why I'm Happy

7 Reasons I am Happy to be a Mom to a Child with CHD.

No parent is happy to receive a diagnosis of CHD, but CHD is not a death sentence. For today's post, Karen Ward, mom to a beautiful little girl who has HLHS (half a heart) shares 7 reasons she is happy.
  1. I don't sweat the small stuff any more (or as much)
  2. I snuggle a little longer at night when I tuck my kids in
  3. I have met some WONDERFUL people
  4. I have a stronger faith and better relationship with God
  5. I am more thankful for what I have and have found that I want less
  6. I have found strength that I never knew I had
  7. I appreciate life so much more
Thanks for sharing your 7, Karen!

Sunday, February 10, 2008

7 For 7: 7 CHD Stories

Today's 7 For 7, features just a few of the stories that have been shared with me by my newfound CHD friends, through this blog, or online. You will not stand up from reading this post without your heart feeling lighter!

Cynthia's Story. I am a 60 year old survivor of Tetralogy of Fallot. I had the Blalock-Taussig Shunt when I was 3 and a total correction at 15 years. In 2004, I had to have my pulmonary valve replaced. In addition, an aneurysm had formed in the teflon that was used to expand the pulmonary artery during the total correction. This was also repaired in 2004.

Throughout my life, I've always worked to overcome shortness of breath, becoming tired, and all of the other symptoms that go along with this defect. I realize that I push myself further than anyone else pushes me.

I have discovered an organization, the Adult Congential Heart Association, that I would like to share with you. People like me who have lived longer than anyone expected them to live can present a problem to doctors. We're sort of leading the way for the children who are just being born with CHD. By the time some of these children reach my age, I hope that the things that are learned now in managing my health and the health of other adults will be standard procedure for the management of their health.

Parents, prayers are answered every day. As you all have indicated, your children are already a blessing and, in many ways, are blessed. I would not have lived if Dr. Blalock and Mr. Vivian Thomas had not worked diligently for years to develop the surgery that saved my life. Others are working equally as hard to find ways to save the lives of children born today and tomorrow. My thoughts and prayers are with all of you and all of the children as you move through life.

Misty's Story. My CHD story begins on New Year’s Day, 2005 when I found out I was pregnant with my 3rd child. My pregnancy was pretty normal. All of my ultrasounds were fine and there was no cause for concern, so we thought. Sydney was born on August 24, 2005. Her APGAR scores were 9 and 9. Everything looked good until the pediatrician came in and checked her out and heard a murmur that was “slightly louder than what we like to hear” and called in a pediatric cardiologist, Dr. Albrecht.

At 4:30 pm that afternoon, Dr. Albrecht came into my room with a nurse. I was there by myself taking a nap and waiting for my newest little girl to be able to come to my room and get to hold her. I’ll never forget what he said. “Your daughter has a problem with one of the valves in her heart and I have called a transport team from UVA Medical Center to come and pick her up and transport her there. She will have surgery tomorrow morning.” Sydney has Aortic Stenosis (AS). Dr. Albrecht advised me that her Aortic Valve was a bi-cuspid, instead of a tri-cuspid, valve and was barely functioning and letting very little blood out of the valve to the Aorta. After he went over everything, he sent the nurse to bring Sydney to my room so I could say my “hello’s and goodbye’s”. My husband called his parents to go pick up our other 2 children and bring them to the hospital to meet their new little sister before they took her an hour away to prepare her for the surgery the following morning.

The next morning my husband called to let me know that they were getting ready to take her back and that she had so many tubes and wires coming out of her that the only place that you could touch her was on her head. At that point, he started to break down, again, and I began falling apart, again. Two hours into her procedure, we received the news that everything went well, she had had two blood transfusions, and she was going to be ok.

When I was finally released from the hospital and able to go see my daughter two days after her birth, I found her in her little bed with an intubation tube and many tubes and wires attached to her. Since that day was my birthday, Steve’s parents brought our two older children up to the hospital to see their sister. I didn’t want them to see her with all of the tubes and wires, but someone brought them back before I could say anything. What was amazing was that when she heard them start talking, her heart rate stabilized, her respiratory rate normalized and her blood pressure returned to a normal state. It was incredible.

Today my daughter looks like a normal 23 month old, but she is not a normal little girl. There are things that she will have to go through that ‘normal kids’ won’t have to do. Besides the future surgery(ies) to open and eventually replace her aortic valve, she won’t be able to do some sports that she may want to do and she will, most likely, have to take medicine every day of her life. Her heart will never be normal.

Joshua's Story. Joshua is an active 4 year old. He was born in June of 2003 with hypoplastic left heart syndrome, a rare and fatal defect, unless treated. Basically, he was missing his left ventricle and his aorta was very narrow. Parents of children with this defect are given three options: they can take their child home to die, they can try for a heart transplant in hopes that a heart will become available soon enough, or their child can undergo three surgeries that will reroute the blood so that the child can survive. Jodi and Mark, Joshua's parents, opted for the surgeries.

Joshua developed severe blood infections after his first surgery, but miraculously pulled through, and his parents were able to bring him home almost two months later. His second open-heart surgery took place when he was 5 ½ months old, and he went home five days afterwards. He had his third surgery, the Fontan, when he was three. This surgery was particularly hard on the family as they had had over two more years to learn to love Joshua, and they knew there was a possibility he might not come home. However, Joshua also made it through this surgery with a few post-surgery complications called pleural effusions. Though it kept him in the hospital longer than anticipated, Joshua did fully recover.

We do not know what is in Joshua's future. The doctors cannot "fix" his heart. We are hoping that Joshua will not need any other procedures for a long time. In the meantime, he has fun playing with his brothers and making his parents laugh. Every day with him is a blessing.

Sharon's Story. Saving the life of infants us everyday work for Dr. Karen S. Rhueban, a Pediatric Cardiologist at University of Virginia Hospital. She was interrupted from an interview to perform an emergency cardiac catheturization on a newborn 4 1/2 pound twin whose blue-tinged skin indicated her blood was not circulating properly to receive oxygen.

In the cardiac catheturization laboratory, Dr. Rheuban inserted a plastic tube in the baby's leg and laced it through blood vessels to the heart to record circulation of the blood and make an accurate cardiac diagnosis. The end of the tubing was blown into a nickel-sized balloon and passed through a hole in the upper chamber of the baby's heart to allow blood to circulate. Known as a balloon atrial septostomy, this procedure saved her life. The baby had to have open-heart surgery at 9 months old in order to survive.

Today, Sharon is 23, and a marine wife. She leads a normal life with a daughter of her own, who has a clean bill of health. Two adults have told me in the last week that they had never met any one else with CHD. Sharon is one of them!

Christy D' s Story. My husband were excited when we found out I was pregnant with my second child. Then our world came crumbling down at our 21 week ultrasound; Steve and I were told our little baby girl had a severe and life threatening heart condition. Instead of joy, we were expecting, our hearts were filled with pain, fear and grief. The baby was diagnosed with Tricuspid Valve Atresia (TA)---meaning her tricuspid valve did not form. Without her tricuspid value, her blood was unable to flow into her right ventricle, enabling growth; meaning she has a 3-chambered heart.

Sydney was born on December 4th, 2006 and had surgery 8 days later. Without this surgery, which has its own risks, she would not survive. The recovery was a roller coaster ride. Sydney developed seizures 1-2 days post-op, which is a risk to having open heart surgery. Her O2 sats, heart rates, and blood pressures were all over the place. She had so many tubes and lines in her, that the nurses had run out of places to put them. After many prayers and medical assistance, we got to take Sydney home just 3 weeks later, just in time for Christmas. What a great gift!!!

At 4 months of age, Sydney had her 2nd surgery. Just remembering Sydney going through surgery last time, made us sick with grief. We knew she was in the best hands, but there are never any guarantees when it comes to surgery. Thanks to all the Angels above, the 2nd surgery was a breeze compared to the first surgery. After the surgery, Sydney's life has improved in a dramatic way. At approximately 2 ½ years of age, Sydney will have her 3rd surgery, as long as everything continues to progress as planned.

Sydney, despite her medical problems, amazes me. She is full of love and life. She always has a smile, no matter what is going on. I thank God for her every day, and despite the fact it is hard being a “heart parent”, there is NEVER a day, or a moment, I would change it, if it meant not having Sydney in our lives.

Karen's Story. As I lay on the exam table and the ultra sound tech squeezes some warm gel on my expanding belly, I look over at my two daughters who are 4 and 7 and squeeze my husband’s hand. We are all eager to find out the sex of our newest addition to the family. With two girls already, we were eager to find out if Daddy will get a little boy. The baby is uncooperative. The tech is unable to tell the sex. Our excitement elevates. The tech steps out to get the doctor. Soon the doctor comes back. He looks at the screen for a long time. I start to get a sick feeling in my stomach. I squeeze my husband’s hand harder. The sweat is building between our tightly gripped hands. His panic is beginning to flow to me threw his hand as though our bodies were connected. The doctor speaks…. “This is not good. This is bad.” I truly feel like I am going to faint. I look over at my girls who have started to look at a Dr Seuss book and have forgotten about what we are there for. I think to myself that they understand “This is bad.” Get them out of here. I don’t want them to see me cry. Oh my God! Oh my God! The tech takes my daughters out of the room to the waiting room. The doctor tells us that the baby is missing part of its heart. Missing half it’s heart! What does this mean? Is the baby going to be able to live?

We were sitting in the doctor’s office and the cardiologist began explaining everything. Our baby has Hypoplastic Left Heart Syndrome. To this day, I don’t remember that doctor giving us much, if any, hope. But I don’t remember much from that conversation. The one thing I do remember was we were told to think about aborting the baby. After many tears and prayers we decided to continue with the pregnancy and do everything we could for our baby.

Jameson was born on October 13, 2006. When she was four (4) days old, she had her first open heart surgery. The next couple of months were extremely difficult. Jameson was stable but, by no means, was she a healthy new born. She struggled to eat and thrive. She had her second open-heart surgery at 4 1/2 months. The recovery was much quicker this time. The best part was she was crying, crying because she was hungry and wanted to eat! Her third, and hopefully final surgery will be in April.

Every month Jameson has grown stronger. She is one now and is such a wonderful baby. She sometimes gets winded when she is crawling or moving around but other than that, the scar that is hidden under her shirt is the only proof of what this little girl has been through.

Parents deserve to believe their child has a chance. Had we followed the doctor’s advice we would not have our beautiful “Little Champion.”

Christy H's Story.
At 16 weeks pregnant we learned through an ultrasound that our daughter had lung issues. At 23 weeks, we were shocked and devastated to learn of her heart defects. Between the two, she was given a 5% chance of survival. Despite the odds, my daughter, Harlie, was born on September 25, 2006.

Harlie has a CHD called Congenitally Corrected TGA and three other heart defects that complicated matters and forced the surgeon to do a three operation “repair” to re-plumb her circulation. I have been told that her heart will last her about 30+ years and that after that we will be looking at a transplant.

In addition to her heart defects, Harlie also has Goldenhar Syndrome (an asymmetrical craniofacial syndrome). She has an underdeveloped jaw and missing bones, low forward set ears, with a dysmorphic left ear and no canal, many vertebral anomalies, left eye anomalies, misshapen skull, anoperineal fistula, and a lung malformation that required the removal of most of her right lung.

Harlie is now 16 months old and has just started to crawl. She has a 20-word vocabulary in sign language. She breathes through a tube in her throat, called a trach. She eats through a tube that was surgically placed in her stomach. She’s had seven surgeries and has spent over four months in hospitals and she’s just getting started.

Despite all of this, Harlie is amazing! She’s smart and happy and we think that she’s quite cute. She has such an agreeable personality and just seems to go with the flow, whether she’s at home or in the hospital. We are very thankful that we have her.

Harlie's heart, lung, airway and feeding issues are pretty overwhelming. But I try to focus on what’s good and be thankful for those things. I try very hard not to think too much about her heart or her lungs. I see children running and playing everyday and wonder if Harlie will be able to do that. I try very hard not to think about what it will be like when she realizes that she looks different. Or the first time she comes home crying because someone made fun of her. We are a very vain society, and that will make parenting her much more challenging.

But worrying about all that is a waste of energy – energy I need to just get through each day. I do my best. I take very good care of her. I make all the necessary appointments with her 14+ doctors, I got numerous opinions until I found the right doctors with the right plans for her. I give her all her medications as prescribed. I’ve learned CPR and she wears an oxygen and heart rate monitor when she sleeps. But, in the end, despite all my efforts, I cannot control how her heart functions. I just hope that it beats like it’s supposed to and that it doesn’t stop. I hope that she never gets a plug in her trach that blocks her ability to breathe. I hope that she never pulls the trach out (again! – yes that was a close call). I hope I never have to call 911 again. I hope that she will know that her care was never too much for me and that she is worth whatever I have to do to keep her safe and sound. I hope that I can continue to be the strong mother I need to be for her. And I hope that she will know that she has made me the happiest mom on the planet.

Saturday, February 9, 2008

7 For 7: How CHD Changed My Life

This post is a little personal, but something I was inclined to write after the last few weeks of complete submersion in the CHD community. I found that the very same things that have changed so drastically in my outlooks and life are shared by many families with CHD. Here are the top seven things that came to mind as I thought of how our lives have changed forever.

  1. Realizing What a Gift Life Is. The miracle of life is more poignant than ever before. As an emotionally-reserved kind of person, I'm amazed at the overwhelming emotions I have when it comes to babies. I cry in part out of joy, in part out of longing for those moments that were lost with my daughter, part in sadness over the babies I know that were lost, and mostly over the miracle that I see. Life, simply, has so much more meaning to me.
  2. My "Uninsurable" One. I've unfortunately learned the hard way what the real status of health care in America is, and it infuriates, sickens and saddens me simultaneously. When my daughter was first born and crisis hit, we were left with several six-digit "balance" billings that took over 213 hours to get down to "manageable" sums. When my husband switched jobs, we found out our daughter was "uninsurable" from an endless parade of providers.
  3. Learning to Let it Be. With my first child, I spent hours upon hours in instructive play, and when my second child came out with a "defect," was determined to do the same to keep her at or ahead of the milestone charts. Instead, her unique personality and the obstacles she's overcome have made me stop and relax and enjoy those moments of play for the sake of play, snuggling without talking, and letting the house go to hell in a handbasket while we chase each other in circles.
  4. Financial Challenges. I hate talking money, and frankly, have felt ashamed at the toll the entire experience has taken upon our family's finances. Like I should have somehow been prepared, or been able to avoid the impact it's had. The co-insurance costs, counter billing, hotel or hostel stays and travel costs can add up. Not to mention the medication, doctor and specialist co-pays, loss of income from extra time off of work, extra child care... the list goes on and on. It's incredibly hard to talk about but needs to be said. We are lucky - we've taken a hit, but we're surviving. Other families have lost everything and are fighting not only for their child's life, but for their family's ability to survive financially. Every CHD family I have met has been affected financially - whether upper or lower class, or somewhere in between! It's not something you can plan for or prevent.
  5. Learning How Tough Marriage Can Be. My husband and I have definitely had our ups and downs since we got married 12 years ago, but nothing can put a strain on a relationship like having a child in critical condition. You feel closer, yet more alienated from your spouse than ever. Your full attention goes to your child's survival (and, in our case, in worrying about the impact on her sibling), and you lose a lot of yourself in it. Having a partner beside you going through the same thing, but with differing ways of handling things (because none of us are truly alike in how we deal with stress and grief!) is both wonderful and incredibly hard.
  6. Smiling at Tantrums. I used to roll my eyes at parents in stores that had tantruming children, thinking how my child would never behave that way thanks to my parenting prowess. Someone upstairs wanted to take me off my high-horse, and did so with my second child, who has CHD. My daughter has some insanely intense tantrums, which worry me (developmentally) but also reassure me. I'm thankful her heart is strong enough to support them. I'm thankful I get to hear her voice. Most of all, I am thankful for her life. Now I'm the one being glared at, and I often smile, knowing that that person has no idea that this screaming banshee in my grocery cart is an ultimate survivor.
  7. A New Perspective. My friend Christy, mom to Sydney (see the 7 faces entry 2 days earlier), wrote, "Having a child with CHD had totally changed my prespective in life. I do not sweat the small stuff like I used to. I have learned to cherish the moments with my children. For the smiles...drools....tears....temper tantrums....arguments....laughs....for their strength....courage.... and most of all, for how much they teach me about life and unconditional love." Well said, Christy, and ditto to that!!

Friday, February 8, 2008

7 For 7: 7 Ways You Can Help!

Now that we have some people interested in this site and in creating CHD awareness, I'm getting lots of questions on how to help the campaign for awareness, where to make donations, and what else can be done. For today's list of 7, here are some suggestions of how you can help!

Give Blood.
Before, during and after surgery, our children have multiple transfusions. Sometimes these can come from family members, but many times, if the surgery is not pre-planned, this blood comes from strangers like you. Your gift of blood goes a LONG way for a child with CHD!

Vote on Health Care Issues. In future blog entries, we'll start to delve into the problems that our current health care systems leave families of CHD facing - things like bankruptcy, suggestions for divorce (to qualify for aid), unsurmountable medical bills and general agitation and frustration over the hours that must be spent fighting first for our children's lives, then for the insurance to cover the costs. Don't worry, we'll offer some loopholes and promising information we've found as well. When you see a bill come through that promotes options for "uninsurable" patients (as many CHD patients have been deemed), VOTE, contact your legislator and talk to your community about it.

Support your local group. There are a large number of CHD-related groups out there, each offering services to families... from support to financial aid, medical guidance to research. We have not yet been able to verify the best source to donate funds directly to research of CHD, so I instead encourage you to participate in a fundraiser for a group in your community, such as local chapters of Mended Little Hearts or Its My Heart, make a donation and get involved.

Become an organ donor. Today I read wonderful, yet heart-breaking news that a little girl received her much-needed heart transplant this morning. She had been on the waiting list for over 60 days, and is just over 9 lbs. Through someone else's tragedy, her life has been saved. It's hard to even fathom, but the donation of you or your loved ones body can save one or more lives.

Advocate. You're likely reading this because you, a friend, or a loved one has been affected by CHD. If so, get out there and talk about it. Talk to your doctors, OBs, ultrasound technicians, social workers - anyone who may come across CHD. It's surprising how little they know

Join Forces. If you already belong to a CHD-related group, encourage them to join in first on the Awareness Week campaign, but secondly in finding ways to unite our community. We need to find a common ground that all of our groups can participate in - a way to advocate to the masses and find new sources of revenue for research.

Support a CHD Family.
If you are a friend or loved one of someone who has CHD, simply being a listening ear, taking over meals during the "trying times," sending messages of support, prayers and positive energy all make big differences to a family with CHD. Offer to assist with child care for siblings, to stay and let the parents rest, to mow the lawn, to clean the house, to work overtime so they can have more time off, whatever will assist them. Most won't come out and say they need help, but we all enjoy the help we do receive.

Thursday, February 7, 2008

7 For 7: 7 CHD Faces and Stories

Today is the first, official day of CHD Awareness Week. This week's blog entries will each feature lists of 7s relating to CHD. We hope you will enjoy it, maybe learn something new and pass it on! Quite a few friends are participating in this effort, and we want to thank them in advance for their stories, photos, shared memories and other information.

Today's entry features 7 beautiful people who have broken, mending or mended hearts as a result of CHD. Each has a unique story and has faced many challenges of CHD. Some have more challenges to face. No two have the same results from CHD, but all have the same common goal - making the most of the heart they have been given!

Jameson is a 16 month old with Hypoplastic Left Heart Syndrome. She is a loving and happy toddler, full of hugs and kisses, and an absolute joy to be around. Her first open heart surgery took place when she was 4 days old, and her second at 4 ½ months. After her second surgery, Jameson began to grow stronger every day. Lately, though, she sometimes gets easily winded when she is cruising around, and her stats are chronically lower than normal. Other than that, the scar that is hidden under her shirt is the only proof of what this little girl has been through. Her third surgery (the Fontan) will be in April.

Lauren was diagnosed with Tetralogy of Fallot at birth. Her parents were told that she probably wouldn't make it and I was taken by ambulance to UNC Chapel Hill where I had open heart surgery at a week old. There they performed a temporary repair that lasted until she was 17. Lauren then had another surgery to close up the shunt and perform a homograph pulmonary valve replacement. Her family and friends have always been very supportive and treated her as an equal to her sister, who has a healthy heart. She now is the co-coordinator of Mended Little Hearts in Central Virginia, and is grateful to have been given the opportunity to live and help others. At age 36, there is always a probability that she will have to have another surgery, but for now she is able to do anything I want to do (except be a flight attendant - she's not tall enough!).

Gabe was born in July 2006 and surprised his family with the news that along with a full head of hair, he also had a broken heart. He was diagnosed with D-Transposition of the Great Arteries, minimal Pulmonary Stenosis, and a bicuspid valve. He has undergone two open heart surgeries to repair his heart, and now has pulmonary stenosis due to the nature of his second repair. His family has high hopes that he will outgrow it and that if any intervention is needed, it will be in the cath lab. His mom says he disobeys her with glee, and is a "blur these days... ever moving!"

Sydney was diagnosed with Tricuspid Valve Atresia (TA), meaning her tricuspid valve did not form. Without it, her blood was unable to flow into her right ventricle, enabling growth, leaving her with a 3 chambered heart. She had her first surgery when she was 8 days old and her second at4 months. At approximately 2 ½ years of age, Sydney will have her 3rd surgery, as long as everything continues to progress as planned. She will never be “fixed” unless a way of creating a new chamber is discovered through further research.

Harlie has Goldenhar Syndrome, VACTERAL Association, and Congenital Lobar Emphysema. Harlie’s heart defects are: L-TGA, VSD, intermittent 2nd degree heart block, small right ventricle, and 2 small Superior Vena Cava’s. She was also born with a mass in her chest, and a variety of other physical anomalies. To date, she has had 7 surgeries and spent over 4 months total in the hospital. Despite everything, she is one of the sweetest and happiest babies you could ever hope to meet. In the past two months she has learned 21 signs, and loves show off her new communication skills, which include putting two words together. She hardly ever cries and is absolutely beautiful - your heart just melts when you meet her!

Claire is currently in the hospital with RSV - send positive energy and prayers her way! She was born with TGA and VSD, as well as a faulty valve, which resulted in her having to have a Rastelli Repair, which uses artificial material inside and outside the heart to create the figure eight necessary for normal oxygenation of the blood. If all goes as expected, Claire should be able to act like any other child. Though she will have to have at least a couple of conduit replacements as she grows, there is a bright side there as well. The conduit replacements are not quite as "invasive" as they are at the front of the heart, research on expanding the conduits without open heart surgery to prolong the time before replacement is taking place and there is the possibility through stem cell research that a conduit could be grown from Claire's own cells that would grow with her.

Sadie came into the world screaming, but quickly turned blue and was taken from her parents. She was diagnosed six hours later, when she was critically ill, with TGA, ASD and VSD. She received a catheturization that night, and took 11 days to stabilize enough for the surgery that was required for her survival. Though she suffered drastically from drug withdrawals after surgery, she quickly healed. Today she is a strong-willed bundle of energy. She will continue to receive annual cardiology check ups, but there is a very good chance that no further invasive procedures will be necessary in her life time.