Whirlwind Week

Through our Mended Little Hearts group's contact with the American Heart Association, I had the opportunity to share Sofia's story and how it related to current pulse ox legislation for local Cleveland news last week.  I was interviewed on Thursday by Channel 3's health correspondent and the story aired that night.  In the Ohio State Senate, Bill 4 is up for debate, which would mandate pulse oximetry testing in newborns.  Because Sofia's defect was not discovered prenatally, we were lucky hers was discovered in the hospital through pulse ox screening.  I have posted the video link here of the story:

http://www.wkyc.com/news/story.aspx?storyid=287708

In even more amazing news, on Friday the American Heart Association asked me if I would provide testimony for the Ohio state Senate Health and Services Committee on the bill.  I knew I had to do this and it would be an amazing opportunity, so I drove down to Columbus yesterday and gave my speech today at the state house.  So glad my sisters were there!  Dr. Rosen, a pediatric cardiologist, at Nationwide Children's Hospital also provided proponent testimony.  He did a great job fielding questions from the Senators on why pulse ox testing on every newborn should be mandated and not optional.  We didn't ahead know that there was another parent who also was offering testimony today.  She was called first to speak and as soon as she started speaking and getting emotional I knew that her baby did not have the same positive outcome that Sofia had.  The parallels were a little uncanny.   Her baby's name was Sophia and she was treated at the Cleveland Clinic also.  Her baby lived for 15 days and her story was heartbreaking.  What a courageous person it takes to get up in front of a group and talk about something still so fresh and painful.  I knew it was over for me, and the calm and thoughtful speech I had planned went out the window.  It was hard for me not to get emotional, and part of that was definitely out of gratitude that Sofia is thriving.  

Thank you American Heart Association!  They have been instrumental in making this happen and we so helpful and fun to work with.

                                         I have the best sisters in the world! 

My speech:

Senate Medicaid, Health and Human Services Committee

Proponent Testimony for Senate Bill 4

Chairwoman Jones, Ranking Minority Member Cafaro, and Committee Members, 

Good morning.  My name is Anne Curwen and I am from Lake County.   Thank you for giving me the opportunity to speak before you today.   I don’t know a mother who doesn’t like talking about her children, but not many get to do so in front of the distinguished audience of the State Senate.   I found out just 4 days ago that I was going to be able tell you about my special daughter Sofia, but in many ways I’ve been preparing for this for 4 years, which is when Sofia and congenital heart disease entered our lives.  

I had a normal and healthy pregnancy with Sofia and when she was born a week before Christmas in 2008, we were beyond excited to have our first child.  We found out she was girl, something we had waited 9 months to discover. And she was perfect.   She was born at 8 in the evening on
December 18th at a community hospital in Willoughby, Ohio.  I didn't think life could get much better than this.  What I didn't know is that things were going to get much worse before they got better again.

After spending a few hours with Sofia after she was born, in the late evening a nurse had offered to let my husband and me get a little sleep, take Sofia to the nursery to do her standard hearing screen, and bring her back when she was hungry again.   When the nurse returned to our room a few hours later, she did not have Sofia with her, and she told us that they thought Sofia had a congenital heart defect and would require surgery.   We were in shock, and thought they could not mean our Sofia, who a few hours earlier was born seemingly healthy. The nurse said that they had noticed Sofia looked a little dusky in the lips and they put a pulse ox monitor on her, and with that they knew pretty quickly she was in distress.   What they didn’t know yet, was that Sofia was born without her tricuspid or her pulmonary valves (2 of the heart’s 4 valves) and now that she was out of the womb, she was not getting enough blood and oxygen to her lungs.  The nurses acted quickly and were able to reach the neonatologist on-call, who came in to start treatment on Sofia.  They began a medication called prostaglandin which acted to deliberately prevent her main blood vessel that was open in utero from closing off, as this became a lifeline for her, which stabilized her until she could be transported to the Cleveland Clinic.  Sofia needed heart surgery at just 4 days old.  
 

It did not take long for my husband and me to realize that a series of things that went remarkably right for Sofia saved her.  She was only 1 of 2 babies in the nursery that night, which led to an observant nurse to put the pulse ox monitor on her.  We struggle when we think about what could have happened had Sofia gone home before her heart defect was discovered.  Sofia could have gotten very ill, could have been rushed to the hospital from home because was had gone into distress,  it could have been too late to administer the prostaglandin because that blood vessel would have already closed off, and she could have faced surgery in a much more dramatic fashion.   We live about 35 miles from the 2 major Cleveland hospitals that treat congenital heart defects.  There might not have been enough time.  
 

This is why it is critical to catch babies with undetected heart defects in the hospital.  In talking with other families, I have learned that not having a prenatal diagnosis of a heart condition is not that uncommon .  I had routine prenatal care andultrasounds during my pregnancy with Sofia, and her heart defect, which is considered a complex defect,  was undetected.  The pulse ox screening was a safety net that literally saved her life.  

When I think back to when Sofia was being transported from Lake West Hospital to the Cleveland Clinic in snowy December, I remember what the neonatologist said to the transport team.   In a stern, and almost mother-like way, she told them “do not speed, do not take risks.  This baby is stable.”  How lucky we were Sofia was stable.   With that stability, came the opportunity for us to meet the cardiologists and the surgeon that would operate on our tiny baby, and go over a plan for Sofia’s surgery.   That probably would not have happened if Sofia’s defect had not been caught while she was still in the hospital.

Once we learned what Sofia’s specific heart condition was, we knew she would face 3 heart surgeries to give her optimal circulation. She has had her 3 surgeries and she is an active, energetic, and bright 4 year old. We really are the lucky ones and we don’t take that for granted because we know of too many who were not as fortunate.

I am speaking to you today on behalf of not only Sofia, but also babies who could be saved by standard pulse ox screening ,which are estimated to be approximately 250 per year.   I’m also not speaking on just my own behalf, but for the moms, like I was at one point, that are naïve about the the test that could save their babies.

 

Thank you

Neurocardiac Clinic

Along with opportunity to meet new families through Mended Little Hearts, we have also been introduced to some great healthcare providers.  We've had a few presentations in conjunction with our regular meetings, which have been very informative and practical.  We had a pediatrician speak to us in the fall, and last week we had a presentation from a pediatric neurologist, Dr. Neil Friedman (who has an interest in neurology and congential heart patients) from the Cleveland Clinic. 

As a parent of a child with heart disease, I know that developmental delays are not uncommon.  I've always attributed this to surgeries, being on bypass, long hospital stays, etc.  I have always thought we were lucky with Sofia because always been developmentally on track despite having three surgeries.  When she was an infant we had Help Me Grow follow Sofia, and when at age 2 they told us we didn't need them anymore, I insisted they keep following up with her until she was 3 (which is as long as the program follows these kids).  I have always been amazed with how bright Sofia is.  She is smart,  she understands 2 languages, and she is always amazing us. 

After the presentation by Dr. Friedman, the neurologist, I didn't expect to feel less confident about my own beliefs about Sofia's development.  The presentation was very interesting, as it detailed the study of pediatric heart patients and neurology.  Since surgery began on these babies (not all that long ago), the developmental delays that were noticed (psychomotor delays) were believed to occur  because of surgery and the amount of oxygen that was getting to the brain.  During the 1980s and 1990s there were studies that focused on different techniques, but it was learned that none of these correlated to a lower indicence of developmental delays.

In the last decade or so, a shift to studying of brains of babies in utero took place.  Dr. Friedman showed scans of a full term baby with a heart defect, a full term heart healthy baby, and a premature baby.  The conclusion was that one could not tell the difference between the full-term heart baby and the premature baby (essentially they both had white matter injury).  It was kind of a blow to learn that the abnormal circulatory patterns for heart babies in utero affects babies before they are even born, so the only way to restore better flow would be to do surgery in utero.  Which, if you're like me and Sofia, did not know about her defect ahead of time, and I'm not sure anything could even have been done differently had it been diagnosed prenatally.  It boiled down to not really mattering when your child had a particular surgery (like and old Fontan or new one) because the developmental implications were set in utero, not in surgery.

So you can see why I felt somewhat uneasy after this presentation, especially because the develomental delays were more common in children with complex congenital heart conditions (like Sofia's).  The types of developmental delays that occur are behavior difficulties (ADHD), visual/spatial dysfunction, fine motor, and language difficulties.  She doesn't exhibit any of these delays, but that doesn't mean she will be without any of these problems in her life time.  ADHD is generally not diagnosed until kids are school aged (selective attention and inability to change focus, impulsivity, hyperactivity).  And ADHD medications often have cardiac implications, which can make these patients harder to treat.  Dr. Friedman also termed one condition as the "Fontan personality," which is observed autistic-like traits in patients who have had the Fontan surgery.  So, more unsettling.

I am glad I attended, and I ended up making an appointment for Sofia for an assessment with Dr. Friedman.  The neurocardiac clinic at the Clinic seems like a good place for that (they also can diagnose genetic conditions, provide PT and OT if needed, nutrition, etc.).  I figure that an extra set of expert eyes on Sofia can't hurt.   I also now know that you have to be proactive about these things and we will keep an eye out on her as she is in preschool now, but especially when she starts kindergarden.  However, I will not obsess about how pokey she is...how long it takes her to put her shoes on, get her coat on, and get ready to leave the house because she is 4 and this does not mean she has ADHD. :)

I'm thankful we have the MLH group and am learning things everyday.  The one thing I often take away from these types of presentations is that Sofia and kids like her are on the cutting edge of medicine.  There is so much unknown and they are products of refined surgeries.  There is much to be learned, but I am so grateful she is here and doing amazingly well.