Oh my.  Just got this today – we understand the newsletter also goes to pediatricians and cardiologist around the country.  So proud of our little Eve.  She has done some wonderful things in her short time here…thank you University of Minnesota Amplatz Children’s, Mayo Health System, Children’s Hospitals, Regions Hospital and Minnesota Department of Health for all you have done to make sure babies don’t go home with undiagnosed heart defects.  Rockstars.  All of you…

file:///Users/annamarie/Desktop/UMACH_CVHeartCenter_v5.pdf

In this first year of Eve’s life, we have come to this epiphany.  In our daughter’s cardiologist, we found medical nirvana.  He has been smart, savvy, pigheaded (as needed), caring, proactive, collaborative.  Many days, we would see this man at 7 am – and again at 7 pm.  If we emailed or called or paged at 11 pm, we’d still get an immediate response.

Most importantly, it seemed like he treated our baby as he would treat his own child.  I never asked if that’s how HE really felt (that would be weird…), but it’s how he made US feel.

That is a very special gift.  Not every pediatric physician possesses it.  That’s why it’s so special to see glimpses of that pervasive caring as people name their 1in100 Rockstars http://1in100.org/rockstars/.

I will say this.  When it was time to schedule surgery, Eve’s heart only had a few weeks left.  The team at our hospital was outstanding.  And that’s why we were able to bring Eve to another hospital 2,000 miles away.  Because in their caring, compassionate, collaborative way – they knew Eve’s case was unique.  And that she was in the best hands with one of their peers.

Accolades aren’t enough.  That takes not only special doctors, but a special hospital.  Just this week, I wrote a letter to the University of Minnesota Amplatz Children’s Hospital.  We never formally acknowledged the amazing, selfless decisions that allowed our baby to live and thrive.  I hope they know how important this is.  That they are the model for how hospitals can, should and WILL be practicing in years to come. I might add that the doctors we’ve come to know during this journey are still committed allies.  Now joining together to tackle such challenges as universal pulse oximetry screening, pediatric heart device development and real-time clinical information exchange for the youngest patients.

When it comes to saving and protecting lives, there should be no hospital borders, no state borders, no country borders.  The medical community is just that – a community.  Neighbors, keep helping each other.  That’s what makes a Rockstar.

We knew our baby’s heart would be stopped while the surgical team at Children’s Hospital Boston repaired it.  It’s a damn freaky thing.  If your heart isn’t beating, you are…well, dead.  Right?

It occurred to me that we – the families of pediatric heart patients – are rather unique in having experienced this with our kids.  There are certainly alot of horrible diseases and disorders that plague kids, but how many others require the heart to stop beating to get better?

The National Library of Medicine has a rather clinical explanation of what bypass heart surgeries entail, the risks, the preparation and the aftermath.  Sorta glad I didn’t see that page one year ago today as they carried my baby into the OR.

http://www.nlm.nih.gov/medlineplus/ency/article/007363.htm

More than 50% of all children born with congenital heart defect will require at least one invasive surgery in their lifetime.

That means a whole lot of people, with increasingly successful heart surgeries will get to celebrate rebirthdays.  Eve’s surgeon, Dr. del Nido introduced us to this phrase.  In essence, when her heart started beating again at 12:40 on April 13, 2009 – she was re-born.

So at the Saarinen house, we get to celebrate December 12 AND April 13 every year. In fairness, we are letting our other kids celebrate two special days each year as well.

I’ve decided my second celebration day is April 23.  It’s the day we got to bring Eve home.  It’s also the day I found out we were pregnant with her.

Both seem like excellent reasons to party.

As if we didn’t have enough to give thanks for, we learned this week that a local medical device company completed a prototype of what we hope will be the first FDA-approved pediatric heart valve brought to market in the US.  That happened within 8 short months of the first conversations about it last summer.

And the newborn screening pilot program in Minnesota has had a ripple effect in other communities and states as hospitals have started implementing pulse oximetry screening even in advance of the official recommendation for universal screening that we intend to see from Health and Human Services before the end of this year.

Somehow Eve has rallied an amazing roster of medical professionals and public policy leaders around common sense initiatives that help families coping with the world’s most common birth defect, affecting 1in100 newborns. CHDs are responsible for one third of all birth defect-related deaths. And 20 percent of CHD children who make it through birth will not survive past their first birthday.  Those are mind-boggling statistics.  There are good people adding to their plates every day to change these numbers.

This isn’t easy stuff.  And we never thought we’d be living it.  But for now, all I can hope for is that every rebirthday Eve celebrates brings with it a sense of repurpose, recommitment, and renewal.  We’re in.

Before we had Eve, I thought I knew about giving.   Paul and I have been fortunate enough to be involved with a number of wonderful people and organizations over the years.   We’ve been inspired to give more through Smile Network International (smilenetwork.org), Children’s HeartLink (childrensheartlink.org), Adopt A Room (adoptaroom.org), Artspace (artspace.org) and others.

But in my 4+ decades on this planet I have never seen giving like that in the CHD world.   We are closer to many people we have come to know through this journey than friends we’ve known our whole lives.  Many of them we have never met in person.  Yet the bond is just as strong.  Stronger maybe.  Because we are tied together through the gifts of our children and the experiences most of us never imagined we would have.

Last Easter, Paul and I had just flown Eve to Boston for her ablation and open heart surgery.   She had about one week left to live.  It was a time of uncertainty and prayer.  Virtual strangers (who we met through another virtual stranger, online a couple months earlier) offered to pick us up at the airport and take us to Children’s Boston to get settled.   Four days later, the day before Eve’s OHS, that same couple drove back to into the city, picked us up and brought us back to their home to spend Easter Sunday with them.

I find it difficult to even write this without crying.  Ron and Isabelle Ouimette and their little Andre not only gave us hope that Eve could come through her operation and start thriving – but they GAVE.  In every way that matters.  They opened their hearts and their homes to us.  They made us feel “normal” that day, so far away from our other children, friends and families.  They came to visit again twice before we left Boston – as did the “other” strangers Kelli Carlson and her family. (P.S. Kelli too is an amazing advocate, mom to Will and founder of Rhode Island’s Helping Hands, Healing Hearts, www.riheartgroup.com.)

We will never forget the gift they gave us that day.  And I cannot imagine a day when we won’t think of them as friends.  Thank God Isabelle is as passionate as they come in spreading CHD awarness (CHDspeaks.blogspot.com) – it’s easy to keep in touch with her and continue to be inspired.

It’s funny – Easter means alot of things to alot of people.  For us it has become the day before Eve’s life-saving heart surgery.   I don’t have any fancy baskets or dyed eggs around the house this year.  Part of that is just how crazy the last couple weeks have been.  But it’s also because I want tomorrow to be about THE day.  Exactly one year ago we prepared Eve’s 5 medications before bed and got ready for 12 hours of NPO so a team of doctors could open her chest and fix her heart.

Today, we are grateful.  For the true significance of Easter.  And for the other things that make us smile – Sunday dresses, family, friends, cakes, cookies, stuffed bunnies…all the simple stuff that the Ouimette’s went out of their way to remind us of last year.  Thank you CHD friends everywhere.  You give so much more than you know.

I am happy to share the good news from the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children (ACHDNC) meeting last week in DC.  This committee makes national recommendations on which disorders should be universally screened for in newborns. (http://www.hrsa.gov/heritabledisorderscommittee/).

The committee voted to accept the nomination of critical congenital heart disease, using pulse oximetry, to be considered for recommendation to the Secretary.  A favorable subcommittee report on pulse oximetry was presented by Dr. Piero Rinaldo from the Mayo Clinic, who is a voting member of the committee (as well as a MN Advisory Committee member and counsel on our MN pilot). As a next step, the ACHDNC has an Evidence Review subcommittee that reviews those nominations.

This was one of two nominations to be approved (the other being screening for severe jaundice). The consensus of the committee was that pulse oximetry was the higher priority of the two – and the committee voted to make screening for CCHD their #1 priority moving forward. Both nominations have been forwarded to an evidence review group, which is directed by Dr. James Perrin at Harvard.  I spoke to Dr. Sanghavi (Chief of pediatric cardiology at U Mass) about this today…he has long been a vocal advocate for pulse ox screening (http://well.blogs.nytimes.com/2009/04/09/saving-babies-with-broken-hearts/) and would be happy to share his insights with Dr. Perrin.  I am still learning how the Evidence Review committee is formulated, but it seems there is some strong support readily accessible.

The workgroup will obviously be looking at the AHA evidence review on pulse oximetry – and seeking additional information. There seemed to be a clear consensus that there is sufficient evidence that CCHD is a serious condition, that early identification leads to better outcomes, and that pulse oximetry is a suitable screening test. The most important remaining issues that need to be addressed would appear to be logistical: in particular, how can universal pulse oximetry be applied in a wide range of birthing centers, many of which lack pediatric cardiology expertise to interpret neonatal echos.

I was very specific in addressing this in my comments – stating that while every facet may not be in place from the outset, telemedicine can and will be a viable the solution to this challenge.  The president of the March of Dimes is particularly interested to see what comes out of evidence review in this regard – stating concerns that babies that test low may or may not get an echo promptly – and even if they do, it won’t be read by a qualified pediatric cardiologist in the same timely manner as in major medical centers.  This is a valid concern…and one we have brought up in our discussions.  I mention it in terms of helping proactively address it in our study (to the degree possible).

Some other folks in attendance stated that they think one of the the major hurdles in terms of a favorable recommendation of universal pulse oximetry is the lack of large-scale pilot studies needed to demonstrate feasibility and practicality on a community-wide basis.

The committee is aware of our pilot – and is very enthusiastic about receiving the latest information.

Moving forward, the recommendation would goes to the DHHS Secretary, who is required by law to respond within 120 days.  The chair, Dr. Howell stated that she has been moving much faster on previous interactions….and has generally accepted the recommendations sent her way.

The committee can and will look and domestic and international data – and there is a solid collection to put forth and the Minnesota study will help address not only some screening consistency issues, but the outstate diagnostic and treatment challenges as well.

Finally, I was very encouraged by Chairman Howell’s commitment to “not let this one sit on the shelf”.  Dr. Rinaldo did an excellent job of bringing this nomination forward and presenting the information in a way that was unanimously embraced by the committee.  It was actually quite overwhelming when the two votes actually happened.

This is historic work…thank you to all that continue to work to make this happen.  Now the pressure is really on. 

Presentation to the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children

January 21, 2010

Dr. Howell, Ladies and Gentlemen of the Committee,

Thank you for the opportunity to be here today. My name is Annamarie Saarinen. My daughter Eve was diagnosed at 2 days old with a severe mitral valve defect and enlarged heart. She was very nearly sent home – and was in complete heart failure at 5 days old.  24 hours later, she was also diagnosed with SVT and Wolf Parkinson White Syndrome.  In other words, she would never have made her one-week well-baby visit.

During those first weeks, I learned how fragile life is.  I learned that 1in100 babies are born with a heart defect – the world’s most common birth defect – and that nothing I did caused us to become that 1in100.

I also learned to rely on a small monitor that stayed wrapped around Eve’s foot.  It was a pulse oximeter – measuring the oxygenation in her blood.  The phrase “what are her sats today” became part of our vernacular.  Above 95%, we’re good.  Below, her heart wasn’t working the way it needed to.

While most textbooks identify nearly 40 different heart defects, Dr. Jack Rychik, at CHOP says there are probably more than 100 different defects.  Many congenital heart problems are very difficult to identify by fetal or neonatal ultrasound. These issues are even more relevant in hospitals outside major medical centers or in rural areas.

A recent study showed that less than 1/3 of CHDs are diagnosed prenatally.  That leaves 2/3 that are NOT.  Of these, data indicates that routine newborn exams fail to detect at least half (50%) of CHD cases.*

Murmurs often indicate a heart defect, but many serious defects do not present with a murmur immediately after birth. Even with a murmur and a careful exam, additional measures can help increase early detection of life-threatening cardiac disease.

Pulse oximetry – done at the appropriate interval between 24-48 hours after birth – can indeed detect otherwise “silent” heart defects. More than 60% of defects can result in mild desaturation (like Eve’s) not detectable with the eye, but readily detected by pulse oximetry. Simply put, pulse oximetry DOES increase detection of true CHD over exam alone. And the earlier CHD is detected and treated, the more likely a child will survive and have fewer developmental delays and long term health complications.** A baby coming back to the hospital in heart distress is PROVEN to have increased chance of death and a worse neurological outcome that those diagnosed before discharge.

False positive rates have already proven to be quite low – about 1/3 of one percent (.034).  New generation pulse oximeters may result in even greater specificity and fewer false-positives…in addition to catching any number of other respiratory and lung issues that can also go undiagnosed.

I believe the nomination process will clearly show that postnatal screening to identify CHD should be a vital part of normal newborn care.  There are many fine institutions in this country that already realize this and are screening without mandate, including Regions Hospital in Saint Paul, Mary Bridge Pediatric Heart Center in Tacoma, and Children’s National Medical Center right here in Washington DC.

Pulse oximetry testing is non-invasive, easily performed, inexpensive, and requires little training of nursery personnel – even we have one at home. It can simply be done in conjunction with other in-the-nursery screening, such as hearing…and will require very few unnecessary echocardiograms.

I understand the role of this committee in ensuring that suitable newborn screening tests are developed and safe, effective treatments are available for implementation.

Congenital heart disease accounts for the majority of deaths from congenital defects in childhood – six times more common than chromosomal abnormalities.

Nearly twice as many children die from Congenital Heart Defects in the United States each year as from all forms of childhood cancers combined

By any standard, this is a public health need.

I am very respectful of the need for fiscal responsibility in health care  – but have little patience for more babies struggling or dying needlessly.  In the past 3 months alone, I personally know several families who have buried their babies due to undiagnosed heart defects.

Eve’s heart had about a week left when we flew her to Boston for open heart surgery 9 months ago.  Today, she is proof that medical professionals can work their magic on babies with severe heart issues – BUT ONLY when they are given the timely opportunity to do so.  I look forward to the opportunity to keep you informed as our own Minnesota study moves forward supporting the other clinical data pointing to the efficacy of pulse ox screening.  On behalf of the 40,000 US families whose babies will be diagnosed with heart disease this year – and the 4,000 who will not see their first birthdays, I sincerely thank you for your commitment to the health of newborns and children.

~Annamarie Saarinen, 1in100

———————————————–

*Although many of the missed cases will be only a ventriculoseptal 2 defect (VSD), an additional study showed that only 38% of patients with IAA, HLHS, COA, and aortic stenosis (AS) became symptomatic or were clinically diagnosed prior to discharge.7 Some types of CHD have complete mixing of venous and arterial blood, with resulting pulse oximeter readings (SpO2) in the low 90s, and many observers cannot recognize arterial desaturation until the SpO2 drops into the mid- to low-80s. Therefore, mild desaturation may indicate serious CHD and not be recognized.

** March of Dimes

On this day last year, I finally was able to talk about Eve.  I sent my first message to friends and co-workers since Eve’s delivery on December 12, 2008.

“Sorry to have missed you all over the holidays….and that it has taken me a while to write this email.

Eve Isley Saarinen was born the evening of Friday, the 12th  – 6 lbs, 9 oz, 20 inches….and lots of beautiful dark hair.

At 2 days old, Eve was diagnosed with congenital heart disease. It’s a broad category.  Her biggest problem is a severely leaking mitral valve, which puts strain on the whole heart function, enlarging the heart and putting pressure and excess fluid on the respiratory system.  She was transported by Lifelink to the University of Minnesota NICU on the 14th.  Paul drove through the blizzard that night to be at her side, but since I had a c-section I could not be discharged until the next day.  Took a cab over there on the 15th and we hunkered down for the next 5 days.  The medications seemed to be working at first, but within a couple days her heart was continuing to get larger, her breathing more labored.  After an episode of SVT – which is basically super-fast heart arrhythmia, she was immediately transported to the U of M Children’s Hospital pediatric intensive care unit – closer to the full time cardiology staff and cardiac surgeon.

She was immediately started on a new, stronger drug to alleviate some of the pressure and workload on her heart.  We were warned that her situation was precarious.  The surgeon and team spoke to us of a possible transplant (her heart had already been greatly damaged by her defect and the tachycardia).  Yet with all this, she has started to turn a corner and improve.

With only a couple setbacks, Eve has been getting stronger every day.  It is slow progress – and it’s hard to be patient.   We don’t know how long we’ll be here as the medicines do their work…though they seem to be stabilizing her.

The tentative plan is to get her bigger and stronger for a heart surgery at about 3 months old.

There are very sick children all around us here.  It’s the holiday season and there shouldn’t be any sick children.  Anywhere.  So we pray for all these babies and continue to be grateful for how well Eve has been doing.

Blessings to all of you in the new year – cherish your new arrivals.  They are the greatest gifts.  We’ll do our best to keep you posted (Paul’s Eve blog updates can be found at:  www.tweetandmeet.com)

Annamarie, Paul, Jack, Elle and baby Eve”

I can tell you writing about this today – as I try to finally get the 1in100.org website up and running – nearly brought me to my knees.  Those days were a fog.  A never-ending series of rounds, drugs, dozens of nurses, sleeping on floors, praying and praying some more.  We wouldn’t see our other children for days on end, for fear of what might happen if we left Eve’s side.

None of this is news to heart families.  They’ve seen it.  Lived it.  And then some.

The blessing of Eve is her journey.  I can honestly say that I would not be doing any of this if we’d lost her.  The pain would be too intense.

But today – one year after Santa actually cried at her bedside – we are mobilizing.  We give thanks for our gifts and will never, never forget that each day thousands of families are living it.  It is with the greatest honor to those comrades in arms that I make my first official 1in100 post.  Know more.  Do more.  #1in100.