Tag Archives: heart defects
It’s Father’s Day. Where’s the Baby Book?

It’s Father’s Day. Where’s the Baby Book?

I remember our daughter Eve’s first haircut.  It happened on Saturday night, at 2 AM.  She was 9 days old.

Paul and I were in the usual half-sleep that comes with having a child in an ICU.  We were down the hall from the PICU at the University of Minnesota Children’s hospital, and had just heard the dreaded “code blue” come over the intercom system.  This time was different.  About 2 minutes later, my cell phone rang.  It was the ICU attending physician asking us to come down.  I flew out of our makeshift floor-bed and RAN like hell the 50 or so yards to where Eve was.  “Is it Eve??”  No, calm down, they said.  Apparently all her tiny veins were blown out from days of fluids and meds being pumped into the body of 5.5 pound newborn…the only place left to place the IV was in her head.  They needed to shave some of her beautiful dark hair

Eve Saarinen, with Elle and Jack, 10 days old, U of M Children's PICU

- and didn’t want us to freak out when we came in the morning.  They saved her locks of hair for us.  We still have them to this day.

What we don’t have is the proverbial Baby Book to put them in.  There’s nothing on paper historically documenting Eve’s first coo.  Her first smile. Her first babble, first tooth or first roll-over.  This, I suspect, is a sad reality for too many of the families whose babies face critical illnesses. There are simply more important things to focus on when your kid is fighting to survive. I just told my mom this morning that I cannot recall being at Eve’s baptism. I know we had one…it was at our church even.  But it wasn’t long before we were prepping to go to Boston Children’s Hospital for her heart surgeries and our brains must have been mush. Gratefully we live in the era of cell phone cameras, or we probably wouldn’t have any photos of Eve. Frankly, it’s a little tough to be in “photo shoot” mode while your baby is hooked up to a dozen lines and monitors.  While these images may be hard for others to look at, we treasure those grainy pictures.  She was so serene and lovely – yet tough as nails.

Eve Saarinen, April 2009

We also live in the era of Facebook, Twitter and blogs. Eve is fortunate to have a daddy who qualifies as an “early adopter” of these methods of communication and historical documentation. Random fact: Eve’s older sister Elle’s birth was hailed by the Sydney Morning Herald as the first live-tweeted delivery).

Paul was a champion at keeping our close friends and family posted on what was happening with Eve in the hospital.  It’s hard to express how grateful I am for this.  He took photos, he blogged. He DID the baby book.  Virtually.  We can still access most of it.  And may go through the process of pulling Facebook and blog posts into a more permanent format, so Eve can read her own story someday, as so many others have told it too.  Some of Paul\’s posts were so profound (still are, even this week)  that I still revisit them now and then for a moment of thankfulness and grounding.

Eve and Paul, Children's HeartLink gala, 2009

We think she’s a pretty cool kid – with a pretty cool story (minus the actual baby book).  For now, the locks of hair, the pulse ox wrap and her ankle bracelets can stay in a keepsake box.  Her life continues to be full of firsts.  The dates don’t really matter.  What matters is that she came to us, she powered through, and she is here to ask her daddy all about it.

It’s Awareness Day…and??

It’s Awareness Day…and??

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.

The Birth Announcement (That We Didn’t Want to Send)

The Birth Announcement (That We Didn’t Want to Send)

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

Early Detection of CCHD – Pulse Oximetry Advocacy

Early Detection of CCHD – Pulse Oximetry Advocacy

Pulse Oximetry Advocacy Temporary Toolkit:

Interactive screening map with current state legislation, legislation pending and hospitals screening for CCHD:

http://www.cchdscreeningmap.com/

I. INITIAL OUTREACH TARGETS

State Departments of Health

Newborn Screening and/or birth defect surveillance divisions

Hospital Medical Staff – contacts

Pediatric Cardiology
Neonatology
Pediatrics
Nursing
Newborn nurseries/Labor & Delivery

Hospital Administration

CEO
Chief Financial Officer
Medical Director
Nursing Executive Leadership
Patient Safety/Patient Care

Organizations

AAP – State Chapter leadership  – AAP Chapters

Hospital Associations by State

America Heart Association (state chapter)

Pediatric Heart Organizations /Sites (some with State Chapters)

Mended Little Hearts
It’s My Heart
Lasting Imprint
Congenital Heart Information Network
Bless Her Heart
Children’s Heart Foundation
James’ Project
CHD Speaks
BabyCenter Community: Babies and Children with Heart Problems
Helping Hands, Healing Hearts
CHD Babies
II. INITIAL TALKING POINTS

Website and address of every hospital in the US, by state: http://hospitalandmedicalcentercompare.com/by-state

Draft Hospital Outreach Letter

Click here for the Word document file.Letters sent 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

 

III. FEDERAL RECOMMENDATION – NEWBORN SCREENING FOR CRITICAL CONGENITAL HEART DISEASE (using Pulse Oximetry)

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.  The Secretary responded on April 21, 2011, by asking the newly convened Interagency Coordinating Committee (ICC) to review the recommendation and specifically address implementation and infrastructure gaps associated with state by state adoption of this screening.  The ICC has been tasked with providing a full action report within 90 days (which would be before the third week of July, 2011 or sooner).
Secretary Sebelius
The interim period is still an excellent time to weigh in with Secretary Sebelius in support of early detection of heart defects.  Advocates may use their own experience, share a screening update from their state or advocate broadly for national screening.
To reach the Secretary of Health and Human Services, call 202-690-7000.  After hours, there is VM comment line: 202-205-5445 

Or 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”

Congress
Advocates may also weigh in with the Congressional Members in support of saving lives through screening for CCHD.

Use this link to find your delegation, with emails and phone numbers:

http://www.contactingthecongress.org/

Sample Letters

Use the following sample letters for talking points – or customize however you’d like.  The more personal the better.

Sample letters – one for Secretary Sebelius: Family letter_HHS_NBS CCHD

and one for a Congressional member:  Family letter_Congress_NBS CCHD

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

Outreach to State Elected Officials/Government

Directory of Federal, State, Local Officials and Government Agencies

SHARE

The online CHD community is powerful.  Utilize the following resources to mobilize, gather and share information:

Cora’s Hopes and Dreams

Children\’s National Medical Center Pulse Ox Program

Facebook Pages:

Pulse Oximetry

Pulse Ox Please

Check Their Hearts: Support Pulse Oximetry Screening for Heart Defects

Pulse Ox Pennsylvania

Pulse Ox Mississippi

CHD Babies

1in100

 

 

Goodbye 2010

Goodbye 2010

2010, you have been good to us.  Our family thrived.  We celebrated.  1in100 made a difference.  Thank you for that.

But I wish you could have been better to others.

To the 11 families who had to say goodbye to their babies – EVERY SINGLE DAY of 2010 – because of heart defects that were beyond repair.

To the thousands of lives that were shattered because burying a child in a coffin the size of a shoe box is WRONG.

To the families that fell apart because their pain was too deep to bear.

To those that languished in hospital rooms, often for months at a time.

To physicians that tried, but sometimes failed to mend broken hearts.

A good friend just sent me a note this afternoon – hoping this for 2011:  that fewer patients die; fewer clinicians endure the traumatic impact; and more patients and physicians are brought closer together through adversity and success.

2011, I don’t know you yet, but I already aspire great things for you.  May you fulfill the best wishes and brightest hopes of those you touch.

“These are not isolated incidents”

“These are not isolated incidents”

This gorgeous little girl is Taryn Kennedy.  She was happy, healthy, growing until almost one month old.  Her parents didn’t see it coming, and she was lost to an undiagnosed heart defect – TAPVR – at 29 days old.  I stood next to her mom, Vi Kennedy (blessherheart.org), just two weeks ago at the national advisory committee meeting evaluating newborn screening for Critical Congenital Heart Defects.  She is eloquent and brave…and she is not alone.

I sent this via email to the members of our Minnesota pulse ox pilot team about 6 weeks ago.  Thought it was worth posting here.  I am amazed by the continuity and consistency of the data that comes in regarding newborn screening for CCHD.  I get that we have a bias…but the facts are simply becoming far to difficult to argue with.  As Dr. Martin put it to the national committee “these are not isolated incidents, babies are missed all the time…”   Feel free to add your comments or feedback – the armor gets stronger every day.  Here’s the email:

Wanted to share this with you.  They are the top 8 defects – in order of prevalence – that are the most often missed during routine newborn exam alone (these come from Dr. Hoffman’s recent paper and gathered study data).  Below that are some of the responses heart families posted on what their undiagnosed defects were…on our Facebook page alone, we got over 2 dozen responses in a matter of a few hours.  I may post again to see what additional feedback comes in.  Would be interested to hear if anything hear strikes you as unusual (other than an HLHS baby going undiagnosed for 2 months!)   Annamarie

1. Coarctation of the Aorta (COA)

2. Interrupted aortic arch

3. Aortic stenosis

4. Hypoplastic Left Heart Syndrome (HLHS)

5. d-TGA

6. Truncus arteriosus

7. Tetralogy of Fallot (TOF)

8. TAPVC

RESPONSES:

1. Marlee had an Interupted Aortic Arch and a VSD and wasn’t diagnosed until she was 2 days old and her PDA was closing!

2. My son had d-TGA with a VSD. Dx at 5 days old!!!

3. The defects listed are critical when the children’s lives depend on the ductus staying open. This is why I would like to follow the pulse ox push before discharge with a pulse ox at the pediatrician’s office on day three. There will be many, many more caught if we can do both (posted by a former NICU nurse in Tennessee)

4. They didn’t catch Lauren’s HLHS in utero. We didn’t know something was wrong until about 19 hours after she was born when she stopped nursing. The surgeon told us later they may have missed it because her left ventricle is 60% the size it should be, which is large for HLHS. At one point, they even considered trying to let her use the left ventricle, but they decided against it.

5. They didn’t catch Brayden’s TOF in utero. Diagnosed at 3 days old.

6. None of Caylen’s defects including heterotaxy, dextrocardia, TGA, av discordance (just some of the major defects) were seen in utero. It took them three days just to figure out all her defects and diagnose her.  I love the pulse ox campaign, though, to catch all the ones who don’t show up as dramatically as hers did after she was born. I push it to everyone. My brother is a respiratory therapist in a NICU and he’s pushing the idea, too.

7. My daughter’s TOF was diagnosed at three days old…we knew something wasn’t right because her murmur was SO loud but, nothing was ever picked up in utero.

8. My son was born with a vsd, pds, asd, and an extra mass in his heart.. and he was born with heterotaxy and polysplenia syndrome and we didn’t find out until he was 2 months old because he was breathing fast. I took him to the emergency room and that’s how we found out…we were in total shock.

9. My daughter’s TOF was caught by a nurse who heard the very loud murmur AFTER the doc wrote our discharge orders for home. We were literally 10 minutes away from going home. An OB/GYN did our US at 18 weeks and couldn’t get the blood flow. I will always wonder if someone else would have detected the TOF. I’m all for the pulse ox and blood pressure check on upper and lower extremities.

10. I had 5 sonograms – and none caught Dom’s CHD (coarctation of the aorta, biccupsid aortic valve, mitral valve stenosis). The only reason why it was caught before we went home because we were in NICU because they thought he had an infection and he was getting antibiotics. They heard the mumur when he was 4 days old and did the echo just as procedure. I was told that if it wasn’t caught then, I would have been back in the hospital before 2 weeks was up with Dom in heart failure. So I am thankful that we happened to be in the right place at the right time.

11. Maddy’s TAPVR went undetected for two weeks after birth.

12. My son had critical aortic stenosis that was not caught until a few hours after he was born. He turned blue shortly after he was born. He ended up having a heart transplant when he was 6 weeks old. He will be 7 yrs next week! I love the idea of the Pulse Ox for all new borns!

13. Alex’s HLHS wasn’t diagnosed until he was 2 months old. It’s a miracle he’s still with us. An unusually large left ventrical was the difference.  Hopefully we can work together to make sure more heart defects are detected earlier so others won’t have to worry like we did.

How Cinderella made a grown woman cry…

How Cinderella made a grown woman cry…

Some of you know the Saarinen’s took our first vacation as a family of 5 last week.  It was an Orlando extravaganza.  The whole Disney thing.  I know our babies won’t remember a blessed thing.  And our teenager is probably outgrown it, but we were all onboard with it.

Our first full day was slated for the Magic Kingdom.  It was gorgeous.  75 and sunny.  Coming from Minnesota, you don’t realize how tropical that really feels until you are in it.  We ventured into the park with our “We’re Celebrating” buttons.  We hit the castle just in time for a musical number with all the big-time Disney characters.  Mickey, Minney, Goofey, Princesses, Buzz Lightyear…all of em.  We were strolling the two little ones up for a closer look and out of nowhere, I start bawling.  Like serious breakdown.  I kneel down next to Elle, our 2 year old…partly to avoid too many stares.

The enormity of the past year, I think, just hit me at that single moment.  I realized that if we had lost Eve that I could never be standing there feeling the joy of being in such a wonderful place with all of my children.  Something would have been forever missing.  With this, instantly comes the thought of all the families we have come to know (and many we don’t know, but realize exist) that HAVE lost their children to killer heart defects and heart disease.  Many of them have other children – and must TRY to go on and have family vacations and such, right?  But how could you look up at Cinderella and not think of the child who wasn’t there to be part of it??

I have no doubt that the tears that day were indeed tears of joy.  Complete, overwhelming joy.  My husband and I were able to afford a real vacation with all three of our children in tow…to this very magical place.  We were happy.  And we were healthy.  And that is something I will never have enough gratitude for.  Cinderella, you made me cry.  And I think I needed that.  Thank you.