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

Another significant journal weighs in on pulse oximetry to help detect CCHD. Nature Reviews Cardiology: Clear benefits of pulse oximetry in neonates—one small step to improving postnatal cardiac care.

The letters, emails and calls recognizing the importance of the U.S. Department of Health and Human Services decision to recommend this simple screening for all newborns has been overwhelming.  I was reminded once again, this week that far too many children still receive a delayed diagnosis of congenital heart disease.  There is still much work to be done…in honor of the 70-plus families who lost their infants to heart defects this week.

SACHDNC Meeting 9/22
Public Comments
Chairman Howell, Committee Members,

Thank you for allowing this participation today.  As of yesterday afternoon, the comments I had prepared have greatly changed.  We are overwhelmed by the Secretary’s positive response to this committee’s vote from one year ago to recommend newborn screening for critical congenital heart disease.

In support of this important screening – over the past few months, the following occurred:

• Meetings with nearly 80 ( 77 ) congressional offices
• Information briefings provided to 12 additional offices within HRSA and HHS, including the Secretary’s office
• Four formal congressional briefings by the Newborn Coalition
• Drafted and delivered dozens of letters to stakeholders, provided fact sheets, cost analytics, and shared updates on the status of the recommendation and response to the CHD community, public health and         health IT committees and leadership
• Working directly with New Jersey’s Dept of Health implementation workgroup
• Established pilot projects that support MU stage 3 care coordination and electronic reporting of pulse oximetry results

In just the year since the committee voted to recommend newborns be screened for CCHD, more than 120 additional hospitals around the country have started screening newborns for heart defects as a standard of care.  Pennsylvania has introduced legislation and New Jersey’s Governor signed their bill.  As of Aug 31, 2011, every newborn in New Jersey is being screened, using the protocol developed by the HRSA implementation working group.  All this happened in New Jersey in less than 8 weeks time…an amazing accomplishment, and a model for other states.

The number of hospitals screening grows every week, including an annual population of 15,000 babies in Minnesota.  We have also translated all our our parent educational materials into 3 languages and have a working IT project with MN Dept of Health to support electronic results reporting – one we hope to open source for every state attempting to set up similar procedures for collecting and reporting pulse oximetry data.

I hope this effort has reinforced something important – that the work here reaches beyond metabolic screening.

Today, 11,000 babies will be born in the United States.  110 will be diagnosed with some type of congenital heart problem.  11 of them will die before their first birthday.  No question – through your important efforts, that number WILL CHANGE.   Fewer lives will be lost to this insidious disease.

My dad was diagnosed with stage 4 cancer last week.  Here’s what no daughter wants to hear: we would have had more options had we known sooner.  It’s also what no parent of a baby wants to hear.  Early diagnosis of hidden diseases and disorders MATTERS.  You help make that happen.

On behalf of my family, 1in100, the CHD community and the Newborn Coalition, many thanks to this committee for the work you do and continue to do.

And thank you Chairman Howell – you have done work that will be recognized by generations.  You leave some very large shoes to fill. Wishing you all the best.

Thank you again.

Annamarie Saarinen, 1in100 / Newborn Coalition

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

This is by far our favorite headline this year.  Please review the full article here: Pulse ox_Lancet2011_Aug4

And share it with your circles of influence – hospitals, department of health officials, legislative offices, associations.  This is an incredibly powerful endorsement of newborn screening for heart defects.  Use it.  Embrace it.  #1in100

The Department of Health and Human Services just held a stakeholder call to update on the status of the recommendation to screening newborns for CCHD with pulse oximetry. Given the 5 recommendations related to this screening, the Secretary has found several items she would like to have further clarification on to move forward with the screening (see the original recommendation letter here). 

It was clearly stated that the Secretary recognizes the critical importance of CCHD, and has requested further efforts be undertaken immediately to work through the issues surrounding the pulse ox technology itself, follow up diagnostics, service infrastructure, and education. The Newborn Screening Saves Lives Act (of 2007) required the formation of a supplemental Interagency Coordinating Committee on Newborn and Child Screening issues. This committee includes directors of the CDD, HRSA, NIH and AHRQ. The committee has been IMMEDIATELY tasked with reviewing the original SACHDNC five recommendations, any gap areas, and the document produced from the Implementation Workgroup – their goal is to close the gaps. It is the committee’s first order of business…and they have 90 DAYS to provide a plan of action.

Here’s what I know. Without the outreach all of you did, sharing your support and personal stories, this important secondary advisory committee step wouldn’t be happening. This was going down the road of being an averted recommendation. Now it’s not.

So while we might not have the green light quite yet, the motor is running and the car is in gear. Please accept my profound gratitude for all you continue to do to support early diagnosis of heart defects.

I can be honest here, with all of you. These last 10 days have been hard. a hundred hours of time, anxiety, nausea, all of it. My first reaction to the 90 day delay was this: in 90 days, 1,000 more babies will die – and realistically, 200-300 of them will be contributed to by late diagnosis.

But I also realize there is solid intent on the part of the federal stakeholders to get this right. In that spirit,  let’s support this effort, continue the work already being done, and look forward to widespread implementation of newborn screening for CCHD.   #1in100

Let there be no mistake.  Early detection of heart disease in newborns is essential.  I mentioned to someone yesterday that sometimes families feel like it falls on our shoulders to advocate.  These letters sent to HHS Secretary Sebelius this week show we’re all in it together.  Inspiring to see physicians, patient safety advocates and hospitals weighing in on this important issue.  Profoundly grateful.  Share these with your own hospitals and docs – they might want to add their voices too.

Letters in support of Newborn Screening for CCHD to the Department of Health and Human Services

Children’s National Medical Center, Washington DC

Darshak Sanghavi, M.D., UMass Memorial and University of Massachusetts Medical School Letter

Patient Safety Advocates Letter

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.