1in100

I had an opportunity to be in Boston a few days ago – and to visit Children’s Hospital Boston.  A fellow Minnesota family is there right now with their 8 month old baby girl, recovering (beautifully) from a heart surgery at the hands of Eve’s surgeon, Dr. del Nido (their cardiologist at the University of Minnesota Amplatz Children’s Hospital is also ours, Dr. Kochilas.)  Baby Maddy’s room was two doors down from our old room – 8E, right across from the nurses station.  It all came flooding back.

It was 3 years ago this week that my husband and I flew our 3.5 month old baby from Minnesota to Boston to fix her heart.  She had just a couple weeks left to live.  I don’t remember being anxious or afraid. I remember being confident.  We were doing the right thing. She was going to come home with us and grow up to be a happy, healthy girl.  Somehow I knew this to be true.

I’ve told people many times since, that it was our mission at that time to never bring fear or pain into Eve’s ecosystem.  No matter how tired, discouraged or nervous we were along her difficult newborn journey – we were always positive and upbeat around her hospital bed.  Always.

There was always a scarf in her little isolette that smelled like mommy or daddy.  We must have recited Brown Bear, Brown Bear a thousand times.  We played the Disney Lullabies CD over and over.  And that musical seahorse.  I still wake up hearing that sometimes…

Even during pokes and painful procedures, we would just tell her how we were sorry, but that she was so very brave and strong. She needed us.  That is certain.

Sometime when we took a walk outside her room, it was all we could do to stand.  There’s no way to explain this type of sheer exhaustion unless you’ve been there.  We needed others – to lean on when we left her room.  Our hearts shine from the friendships we have gained because of Eve and her broken heart.  For every lost friend (unfortunately there were some), we have gained 10 new ones.

One of the great human tragedies of pediatric disease is the loss of friends, family, support systems. Again, no one can understand unless they have been through it. Nearly every day, I hear a heartbreaking story of how a best friend never came to visit the hospital.  How parents, brothers, sisters…and yes, even spouses, just bail. Sick infants are not the things of story books and fairy tales.  Tubes, wires, monitors, cuts, stitches, blood.  Congenital heart disease is an abomination.  No child should have it.  Period.

But until the day when children suffer disease no more, reach into your soul to BE THERE for those you know going through a traumatic health experience with their child.  BE THERE for those you barely know.  And BE THERE for those you don’t know. Suck. It. Up.

Eve is 3 now. She is the happy little girl I promised her she would become.  And someday soon, I hope we can share her amazing story with her – and teach her one of the most valuable word in the human vocabulary: compassion.

February 14  is Congenital Heart Defect Awareness Day.  It’s also the day our 2 month old almost lost her life on the way to a hospital emergency room.  Not a great memory or reason to celebrate.  But is is a reason to share, which is what thousands of families are doing on this day.

When Eve was diagnosed at 48 hours old, the doctors said she had unknown “congenital heart defects” and possible pulmonary hypertension of the newborn, resulting in a diagnosis of Congenital Heart Disease.  What is “congenital”??  Is that like “congestive” heart failure?  Is it a disease?  Or a defect?   Tip: do not Google “pulmonary hypertension of the newborn” from your hospital bed at 2 AM after they have taken your baby away to the ICU.

She would struggle to make it past one week old.  But after she did – through a cocktail of 7 round the clock medications to keep her heart functioning – we started digging into what CHD really meant.  Fact: we had no clue.  And I had been around heart stuff…serving on advisory committees for the American Heart Association and Children’s HeartLink…and had personal friendships with some of the pioneers in cardiac medical devices.  My dad had Rheumatic fever as a child – and had subsequent heart issues.  His mother – my grandmother – was a blue baby, and in the 1950s was among the first patients on Dr. Walt Lillehei’s heart lung machine.  That surgery extended her still abbreviated life by almost 5 years…long enough to see one of her 4 boys graduate high school.

Still, with all this, I was a complete idiot on CHD.  This deer-in-the-headlights response recurs almost daily when we use the term (or the acronym) to explain what happened to our daughter Eve.  People just don’t get it.  Lumping Critical Congenital Heart Disease (CCHD) in there – and seriously, it’s almost time to throw in the towel.

The simplest way around this so far has been this: Eve was born with heart defects that caused her heart disease – which she will have for the rest of her life.

I think what’s unique to the CHD community is that we are dealing with a BIRTH defect.  Explaining that’s what “congenital” means is important, but not required.  I think great strides can be made when we start talking about “heart defects” and “heart disease”.  People CAN get their heads around that.  And they can talk to others about the prevalence, the need for research and the astounding progress that has been made to improve the long-term survival rates.

At 15 and 16 weeks old, Eve would undergo two surgeries to repair her heart defects.  Her recovery after was nothing short of miraculous. Today, she is a boisterous 3-year-old with only the remnants of the scars from her open heart surgery.  1in100.org and facebook.com/1in100 began with the purpose of connecting families and impacting health policies that can improve outcomes for pediatric heart patients.

The first priority was early detection of critical heart problems that kill and compromise babies.  Heart defects are the leading cause of infant death from birth defects, claiming the lives of more than 4,000 babies in the U.S. each year.  Fortunately, a simple, non-invasive test in the newborn nursery can help clinicians detect possible heart problems, ensuring earlier intervention and better outcomes.

It was a two-year crusade.  One that reinforced the utter lack of understanding of what CHDs are and how often they occur.  One percent of newborns are impacted. The population surviving with congenital heart disease is growing 5 percent each year.

This month, I have the sincere honor of presenting hospital Grand Rounds with Eve’s cardiology team in Minnesota, and will be addressing the topic of newborn screening for heart defects at Cardiology 2012, attending by clinicians and medical experts from around the world.  I’m safe with the CHD and CCHD acronyms among these folks.  It’s the rest of the world that needs to understand.  And if kicking the acronyms to the curb works, then let’s roll with it.

Eve Isley Saarinen was born the evening of Friday, December 12th  – 6 lbs, 9 oz, 20 inches… 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 leaky 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 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.

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.  We’ve told our parents to come now…want them to know her if God takes her from us now.  Just writing this is almost unbearable.  For now, we are only positive around her…so we cling to a tentative plan 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

Published in the journal Pediatrics: Endorsed by the American Academy of Pediatrics, the American Heart Association and the American College of Cardiology, a federal advisory panel recommends nationwide screening for critical congenital heart disease using pulse oximetry, a probe placed on a hand and a foot that uses a light source and sensor to measure oxygen in the blood. Low oxygen levels signal the need for further testing to look for a heart-related problem.

View the report here: Newborn Screening for Heart Defects Using Pulse Oximetry Recommended

There are few words to describe the excitement.  Thinking back to Eve’s Impact, her diagnosis and surgeries – and on to that day in the fall of 2009, when the team in Minnesota agreed to pursue a pilot project, to discussing a federal nomination with the committee member, to providing comments to the federal advisory committee and participating in this federal workgroup that yielded this report..it’s been a whirlwind.  Now, all this news coverage in the past 24 hours.  It’s clear the time has come to use all the tools available to support the early detection of heart defects in newborns.

http://www.cbsnews.com/video/watch/?id=7377689n&tag=mg;earlyshow

http://articles.latimes.com/2011/aug/21/health/la-he-heart-screening-20110822

http://www.webmd.com/parenting/baby/news/20110819/blood-oxygen-test-finds-heart-defects-in-newborns

http://www.medscape.com/viewarticle/748382

http://www.baltimoresun.com/health/boostershots/la-he-heart-screening-20110822,0,1119997.story

http://seattletimes.nwsource.com/html/health/2015978538_webheart22.html

http://www.smartplanet.com/blog/rethinking-healthcare/could-a-new-screening-help-identify-heart-problems-for-newborns/6335

http://health.usnews.com/health-news/family-health/heart/articles/2011/08/22/steps-outlined-to-screen-all-newborns-for-heart-defects

http://blogs.wsj.com/health/2011/08/22/screening-newborns-for-congenital-heart-disease/?mod=WSJBlog

Surreal Day Part 1: Today marks Eve’s 2-year rebirthday.  April 13, 2009, Dr. del Nido and his team stopped our 4 month old baby’s heart for 6 hours, repaired it, and started it again.

Surreal Day Part 2: Following some weird arrhythmia this week, I canceled my DC trip and headed in for morning echocardiograms, EKGs and evaluations for a very crabby 2-year-old (needless to say, these exams don’t exactly get easier past infancy) at U of M Amplatz Children’s, (where Eve spent 4 months of her life in as a newborn). Results were awesome – echo and EKG looked great…just keeping an eye on the heart rate moving forward.

Surreal Day Part 3: Then it was off to Fairview Southdale Hospital in afternoon for a late day in-service training session on pulse oximetry screening for heart defects.  This was attended by administration and newborn nursery directors from 5 large hospitals.  The Minnesota rollout continues.  Dr. Kochilas and Dr. Thompson presented screening protocols and educational materials. We also talked about health IT and how much easier this will get to report results over time.

This is also the hospital where I gave birth to Eve (and our other children) and where she was first diagnosed with heart failure.  Surreal.

Surreal Day – Part 4: It is coming down the wire.  On October 15, the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children sent a letter to Secretary Sebelius recommending all newborns be screened for Critical Congenital Heart Disease using pulse oximetry.  She has 180 days to respond – which means until the end of this week.

Over the next 24 hours, it seems like a great idea to rally.  That means contacting the Secretary, and asking congressional members to reach out to the Secretary on the impending federal recommendation to screen all newborns for CCHD.  The next 24 hours are critical.  This is the path to saving lives.

Here’s what individuals, families and organizations can do, if possible.

1) Reach out to Secretary Sebelius at Health and Human Services to let her know of your support for screening for CCHD.

During business hours, call 202-690-7000.  After hours, there is VM comment line: 202-205-5445

Email your personalized letter/note to the Secretary at: Kathleen.Sebelius@hhs.gov

You can copy and paste your letter right into the email.  Use the subject line:  “Support Newborn Screening for CCHD”

2) Reach out to your members of Congress to share your support and ask them to weigh in directly with Secretary Sebelius.

Use this link – it’s quick and easy way to find your delegation, with emails and phone numbers:

http://www.contactingthecongress.org/

3) Email the appropriate version of these letters – one for Secretary Sebelius: Family letter_HHS_NBS CCHD

and one for a Congressional member:  Family letter_Congress_NBS CCHD

Or just use them for talking points if you are calling (or do both!)  You can customize however you’d like.  The more personal the better.

Here’s the background briefing we’ve been using with leadership as well.

4) Share with your contacts.  The online CHD community is powerful and beautiful thing.  When it mobilizes, it rocks.  If you need more, Cora’s mom has compiled one of the best online resources for pulse ox info at Cora’s Hopes and Dreams:

5) Let me know what you are hearing from your outreach at Facebook.com/1in100.  I suspect this is not on the radar of most elected officials – use it as an opportunity to make them aware of CHD and what can be done to save lives and improve outcomes through early diagnosis.

P.S. Since this is mission critical, I don’t have the time (or talent) to include the links to every online site and Facebook page that can help with this…but there are many.  Grateful for every single one.

Today in 2009, we were far from Minnesota – in Boston.  Eve underwent surgery to ablate an electrical pathway responsible for rampant episodes of SVT (supraventricular tachycardia).  Diagnosed with Wolff-Parkinson-White syndrome a week after birth, these episodes of 280+ beats per minute were wreaking havoc on her already fragile heart.  Any repair to her defective and damages mitral valve would be in jeopardy without taking care of the SVT first.  It was successful.  Few hospitals in the country have EPs that will do infant ablations – only 3 actually at that time, perhaps a few more now.  We are confident this helped save Eve’s life and give her a fighting shot at a lasting heart repair.  She looked like hell afterward.  But in context – we were just so grateful the procedure was a success.  We settled in for the night, looking forward to meeting Dr. del Nido the next day in preparation for her next surgery – in 4 days.  Eve would turn 4 months old, the day before her open heart surgery.

More about SVT: http://ow.ly/4x8hn

Children’s Hospital Boston Cardiology: http://ow.ly/4x8hV

Two years ago this week, our 9 week old daughter Eve was lying peacefully on our bed.  It was one of those rare, but happy weeks when she was home from the hospital.  Her mitral valve leak was “moderate” when we were discharged….down from “severe” during those first few weeks.  We were just so desperate to bring our new baby home….relatively speaking, we were very, very new on our CHD journey.  I was going to start changing her, when my husband and I thought she was breathing a little heavy.  As had become our routine, we grabbed the stethoscope to check her heart rate.  No need to start the stop watch.  It took only a couple seconds to know she was in SVT – Supraventricular Tachycardia. During episodes of SVT, the heart’s electrical system doesn’t work right. In her case, her heart rate would soar up to 280 beats per minute.  Most episodes of SVT are caused by faulty electrical connections in the heart.  In Eve’s case, in addition to her mitral valve defects, she had Wolff-Parkinson-White syndrome – and that was causing her chronic SVT, happening 20 times a day at its worst. We had learned in the hospital what “tricks” could be used to help pull her out of these heart-racing episodes.  Pushing her knees up to her chest, ice across her eyes and nose area.  These would usually work, although in her early hospital bouts with SVT, she was almost always given the drug adenosine which ran right into her line – a straight shot to jolt the heart back into rhythm.  We had seen SVT often enough that we were quite calm about it.  Even when we couldn’t get her heart rate to come back down, we calmly got our coats and shoes on, grabbed a bag and headed for the hospital.

It was about 10 minutes into that 20 minute drive.  Even though it was dark in the van, I could tell her skin had changed color.  She stopped moving.  I put my hands on her couldn’t feel her breathing in her carseat.  I started to panic….telling my husband to drive faster.  I unbuckled her and held her in my arms – in a car going 80 mph down the frozen highway.  If she was going to die, I was going to be HOLDING her, not WATCHING her strapped in a carseat.  I prayed.  Hard.  Just let her be OK.  PLEASE…I will do anything. Quietly, I started whispering that mommy was there and everything was going to be OK.  I said “I’m sorry”….over and over.

Once we got to the ER, the docs started working on her.  They tried the ice.  No.  Then they tried putting her whole head and upper body into a big tub filled with water and ice.  No.  Then again.  Still 260 bpm.  Then they started trying to get a line in her to do the adenosine.   She was always, always horrible to get a line in.  Tiny veins…most of which had been annihilated by all the IVs and PIC lines during her ICU stays.  They handed her to me with a towel, she was freezing cold and limp…and I started whispering again.  This time: “Mommy’s still here.  It’s not time to leave.  Slow your heart down and come back home with us.”  I started reciting “Brown Bear, Brown Bear” in her ear…her favorite.  I can picture all of this like it happened yesterday.  After about 30 seconds, like magic, we watched the heart rate monitor pop back down to 130.

We actually ended up going home that morning.  She started to get her color and vitality back – and docs didn’t think we needed to stay since we were scheduled for our cardiology appointment 2 days later.  It was at that appointment we found out that Eve’s heart had plummeted back to a 4+ leak and was summarily admitted to the ICU for an extended hospital stay.  She was in heart failure.  During the following 7 weeks, she would be home with us a total of 9 days, including the few days leading up to her trip to Children’s Boston for her ablation and open heart surgery.

Since we had to stay strong for Eve and our other children, I’m not sure Paul and I ever truly absorbed what happened that night – or really much else leading up to and through her surgeries.  It’s survival mode.  Heart families everywhere get that.

But this past weekend, when I looked at the calendar and realized it was the 2nd anniversary of Eve’s christening – and approaching two years since that horrific car ride to the hospital – I had another meltdown.  I prayed that we would never experience that fear again. I prayed others who know this fear may have peace in their journeys.  And I prayed that we never forget the debt we owe.

Eve’s life – and the talents of her medical teams have given us the unique opportunity to know more and do more.  It is because of her that 1in100.org and Facebook.com/1in100 were founded. She has even helped ensure that soon every newborn in this country will be screened for heart defects before leaving the hospital.  We are profoundly grateful…

Two years ago today was Diagnosis Day (or D-Day). Eve was 48 hours old. We thought the murmur was innocent and the echo was just a precaution. An hour later, a cardiologist was standing in the doorway telling us something was wrong with our baby’s heart and they needed to transport her to a NICU at another hospital…. Our world changed on December 14, 2008.

The only thing we knew is that we didn’t know anything.  Paul drove in a blizzard to the University of Minnesota Amplatz Children’s Hospital.  I stayed behind.  My stomach had been cut open two days earlier, but I packed my bags and the clothes Eve was meant to come home from the hospital in, and set them by the door.  We didn’t call our family, but did call the people we knew in cardiology and pediatrics….friends of ours who surely would know more than we did.   Still, there was little to go on.  It was midnight before Paul could call and share what he was hearing from the team in the NICU.  But what they were saying wasn’t good.  Pulmonary hypertension of the newborn.  Don’t Google it.  Severe mitral valve regurgitation.  Enlarged heart, displaced kidneys, liver.

I tried to rest.  But the other babies were tucked in their bassinets, crying their newborn cries for the moms that were right next to them.  Where was my baby?  It took everything in me to stay put until the OB/GYN rounded in the morning.  He tripped over my bags on his way in.  “Leaving so soon?…you really should stay another day to recover.”   Yeah.  Don’t think so.  Here’s what I need: discharge papers signed, pain medication prescriptions called over the the U of M Pharmacy, and a taxi waiting downstairs in the next 15 minutes to take me to my baby.   What could he say?

The follow up to D-Day continues…to this day.  There were the darkest of days – and days of pure bliss.  And now, milestones.  Somehow the “first everythings” seem sweeter now.  We remind ourselves that every day is D-Day for 110 more babies and their families.  It would be weeks later before I knew 1in100 babies were born with this most common birth defect.  CHD requires a damn good fight.  It’s time for the victories to outnumber the defeats.

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