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.

Little Heroes

Congenital Heart Awareness Week comes with some mixed emotions.  My life changed forever when CHD entered it.  How can you not look at this and feel pride and hope? 

(a poster and write up about Sofia and other CHD kiddos were on display at the Clinic this week.     so awesome )

As a mom, I also can admit that I feel little sad and a little fearful at this time of year.  Sad for parents who have lost their babies, and sad knowing I am all too close to it and I could put myself in that place, yet at the same time couldn't even imagine the grief.  I think you cannot be a mom of a child with CHD and not feel at least a little bit of those emotions too.  We don't know what the future brings and for planners like me, that can be scary.  I have decided though that I am mostly happy during CHD week this year.  In my mind, Sofia is a CHD success story.  She is one of the lucky ones and we do not take that lightly.  Sofia may have been born with a broken heart, but she is not broken.  It hasn't broken us.  She is happy, energetic, and so full of life.  I once read that as a parent, you are only as happy as your sickest child.  So I won't hesitate to happy and enjoy my girls!

Four years ago, I was just learning what congenital heart defects were, having a new 6 week old baby who had just had surgery 3 days before Christmas and had only been home for a few weeks.  It was a surreal whirlwind, and that I am glad time has erased some of the details.  We have come a long way in four years, and now are active in meeting and supporting other families with children with heart defects.  At the time, I didn't think I would ever to get this point in my life.  There is much to be celebrated in that.

CHD awareness to me is about sharing the stories of remarkable children (and adults!) who are literally modern miracles, and hoping that with increased awareness, that legislation will be passed (especially in Ohio where it's lacking) for newborn pulse oximetry screening.  This would help diagnosis many children, including those with Sofia's type of defect, early. 


In honor of CHD week and Valentine's Day tomorrow, here is my special sweetheart on her very first Valentine's Day. 

                                                         (Sofia - February 14, 2009)

And here are just a few more because I can't resist cute pictures

first tea party at friend's birthday party

 

love, love this one

 

 

                                             sweet Adelaide wearing red for Sofia this week

Christmas 2012

Ahhhh, Christmas.  I do love this time of year.  The lights, the colors, the tree, the cheer, the giving, the excitement.  Even wrapping presents.  I feel as if a Christmas hangover is going to be imminent.  We still have Christmas with Oliver's parents starting tomorrow as they are driving down from Toronto to spend a few nights with us, so the hangover will be delayed.  It is hard not getting caught up in the build-up to Christmas, and then suffer the withdrawal symptoms afterward.  There is always New Years to perk us back up!

We had a really nice Christmas.  We spent Christmas Eve with my family, at my parents house with all of my siblings, nieces, and some extended family too.  Christmastime at their house, our childhood home, has always been the norm for us, with family always coming to us.  It has been harder the last few years, as we all have families of our own now and for the last few years haven't all been together for Christmas Eve and Christmas Day.  But this year we did and it was nice.  My parents do so much to host these events and I just can't imagine Christmas any other way!  I love some traditions we have and we definitely owe it my parents for keeping up with them.  The 4 granddaughters were together and they were crazy together, but it was fun.  I hope they will have wonderful memories of these holidays.  I know I do!

The girls loved opening presents (of course) and playing with new toys.  I think they will keep busy with them for a long while.  I would love to purge some old toys.  It is amazing just how much stuff accumulates after just 4 years of having children. 

My brother Chris and I took on a project this year for Sofia and Camryn's presents.  He grudgingly went along with my plans and the result was perfection!  He admitted it later too.  We bought kind of a bare bones dollhouse bookshelf and decked it out with all the upgrades - carpet, flooring, a loft, furntiure.  I know the girls will play with this for years and I love that it has our personal touch on it.  Adelaide was so cute walking around the little puppy that my sister Kate got her.  She loves walking Kate's bulldog, Dexter, and now she has her own little puppy that walks on leash.  When I was tucking her into bed tonight, her last request was for her puppy.  So sweet.

We will never forget 2008 when Sofia was born exactly one week before Christmas.  I was due 12/20 and I was kind of relieved to be induced on 12/18,  so that we could spend our first Christmas at home as a family.  And as I learned, there are things you can't plan for, or you can try to plan all you want, but some things can't be predicted.  Sofia had surgery on 12/22 and was in the hospital well into the new year.  It was a hard holiday season, but somehow we made it through.  I think we have come to love this time of year even more and appreciate the good years we've had since then.

We are very lucky everyone was in good health this year and we truly had a holiday season to remember! 

Oliver and Sofia on Christmas Eve

4 Granddaughters

Sofia with her ornament she made in preschool

Adelaide and me on Christmas Eve

Dollhouse Before

 

Dollhouse After

Decorated for Christmas

 

London

I've been thinking about updating for awhile, but knowing it would be a bit of a lengthy post, I wanted to find time to sit down and write.  First things first, we had an amazing time in London!  We had basically a 10 day vacation visiting Oliver's brother in London and in Wales for his wedding.  The girls were good on the flight over and we had Oliver's parents to help us on the flight. When we arrived in London we went into the city and they spent the week in Bath.  We did lots of sighseeing in London and the girls enjoyed being strolled around the city and shown the sights.  It was on the cold side, but not very rainy, so we managed.  We had a great apartment that we rented, which was in a good location and had 2 bedrooms, and a kitchen.  The wedding was beautiful and it was an all around great trip.  It was nice to be able to enjoy a real family vacation.

Germ season is clearly upon us as both of my girls have had stomach bugs in the past 2 months, but luckily Oliver and I have escaped it.  Sofia's was particularly persistent over 14 hrs. and involved a trip to the ER as she wasn't keeping anything down .  The next day she bounced right back and was fine.  She is doing well heart-wise.  She has done well since her heart cath and is doing well on coumadin.  We have now gotten her at a good dosage and we were able to get a home testing device and monitor her levels at home.  She now only has to be tested once per month at home, so the frequent trips to the lab are going to come to an end, which is nice.  She had a cardiology check up this week and Dr. Edwards said how good she looks, which is what we like to hear.  Her echo was good and it was uneventful, which is great. 

She is being followed by a gastroenterologist ever since it was discovered she had gallstones back in June.  One of the reasons we took her to the ER when she had the stomach bug was to rule out that her gallstones weren't what was causing her to be sick.  And over the past few months, when her liver is palpated, it seemed to be larger than normal, but her blood work had been normal.  She then had one result that showed a slightly elevated AST and ALT.  The gastroenterologist recommended that we recheck, do an ultrasound and then meet again in January.  Thankfully, her bloodwork and ultrasound were both normal in October and November, so I'm hoping for a good appointment in January with her.  I'm hoping her liver had just taken its time to recovery from her heart surgery in May and that these issues are behind us at this point.  I also know that Fontan patients can develop liver problems due to their elevated venous pressues, so I would expect she is always monitored for these issues.  I also hope that they know more about these problems and have a course of treatment by the time Sofia reaches an age where this could be a real problem for her and patients with similar anatomy.

There is big cause for celebration tomorrow because it is Sofia's 4th birthday!  I truly cannot believe she is going to be 4.  When she was born I couldn't imagine what things were going to be like when Sofia was a toddler and preschooler and into the future, and I am so very proud of this sweet, beautiful girl.  I can't wait to see her excitment on her big day and celebrate Christmas with her, Oliver, and Adelaide this year. 

Adelaide celebrated her 2nd birthday in London.  She is becoming such a big girl and has a vocabulary to rival her sister's at that age.  She is sweet and sassy and still a mommy's girl.  She loves her big sister and it can be very cute to watch them together.

I am so thankful to have these beautiful girls and my heart just breaks for the parents who had their children taken from them this week in Connecticut.  I just cannot imagine life without my girls, and I pray for these families because their lives will never be the same.  So, so sad.

The four of us at Thomas and Selena's wedding

                                                           Adelaide on her 2nd birthday

                                                                 

Coumadin

Sofia is doing great and she's back to feeling like herself after her cath. She has been on coumadin for 11 days now and INR has been tested twice.  After the first time, she wasn't at a therapeutic level yet, and she was just checked yesterday for the second time and the results weren't back yet.  She went back to gymnastics yesterday, which she is allowed to continue.  She follows up with Dr. Golden, who did her cath, on Tuesday and she also has an appointment with the gastroenterologist.  I think forgot to mention in August that she went to see her for the first time because it was discovered back in June when she was in the hospital that she has gallstones.  Dr. Hupertz, the gastroenterologist, will follow her.  She's not having symptoms so we will just hope that she continues to not be bothered by them.  However, if they do cause a problem they will take out her gallbladder.  Hoping it doesn't come to that!  Her liver was also enlarged, so they are going to keep an eye on it.  Dr. Hupertz's opinion is that if there was some liver congestion after the surgery it could take a few months for it to return to normal.  She will have an ultrasound on Tuesday.

Only 3 more weeks until our trip.  Have a lot to do before then!  

Heart Cath and Heart Walk

While it's still fresh, I wanted to write about Sofia's heart cath.  She went in on Oct. 1st for what was her 4th heart cath.  As I've written before, the primary reason she was having this cath is because her left pulmonary artery (PA) has looked narrow in one area.  The secondary reason is if there were any collateral vessels, they would be coiled.  Her left pulmonary arterty has been worked on before in her Glenn surgery at 6 months old.  In her pre-Fontan cath in April, Dr. Golden pointed out a narrow section on 3D imaging, but it was thought to be in a good position that would be right where her Gortex conduit was going in and that would hopefully take care of it.  However, in the last few echos in August and September it looked narrow again.  Dr. Edwards said she would need a cath to put a stent in it to keep it open, and that we could have it sometime in the winter.  We opted to have it done before November, which is when we are going on vacation.

Dr. Hill and Dr. Golden told us on the morning of the cath that it was possible that they could get in there and decide that the PA looked fine and they wouldn't need to do anything.  Dr. Golden also said that if he saw collateral vessels he would coil them, but he would not go looking for them.  The reason is that there is not definitive research that coiling them is the best thing go do, and there could be a benefit to the body creating this natural "top off."  Therefore, if big ones weren't evident, he would leave them alone.

Sofia did well in the morning.  Her cath was pushed back and we didn't even have to be there until 9.  She mentioned being hungry a couple times, and we promised something to eat when she woke up.  She requested French toast.  We were able to be with her and take her to the cath lab.  She was given versed first, which made her very relaxed.  She was under at 11 AM.  It was a really long wait.  She was extubated at 4:55, so the entire cath took 5 hours.  We did get a few updates during the cath, and had learned that she would need the stent.

Dr. Golden came out to talk to us right afterward and gave us the details.  Her Fontan pressures were on the higher side at the beginning of the cath, which suggested something was going on.  At the end of the cath when the stent was placed the pressures decreased (we were told that pressures tend to be higher at the end due to being under anesthesia that long and the use of the dye), so it was good that her pressures came down.  It was obvious the stent was needed and the timing was probably good.  There were no collateral vessels that were coiled.  Dr. Golden described himself as a perfectionist, and there was one thing that didn't please him.  Apparently, the stent can moved when it is being placed.  But once it is place, it cannot move.  The only way to remove is through surgery.  Hers did move when it was placed and it sticks out slightly.  We saw it on video.  Really wish I could draw a picture here to show it.  Essentially, it is not completely off blocking anything, and it is mesh so blood can travel through it, but because of it sticking out he told us she would probably need to be on Coumadin.  This wasn't expected, as Dr. Edwards favors aspirin, and we thought we'd have a lot more time before she'd need to be on coumadin.  However, we understood the need to for coumadin due to the stent placement, just not thrilled about it. 

About half of cardiologists prefer that Fontan patients are on coumadin, so if we had a different cardiologist she might have already been on it.  So she is going to have weekly blood draws until a therapeutic level is set and then hopefully about once per month to monitor.  I did find out that my insurance company will cover an at home testing kit.  I had a long talk with Dr. Edwards on Thursday and felt better about it after talking to him.  He has a reassuring effect and are thankful that after things happen that we can call him and have a good explanation of things.  She can continue to do gymnastics.  We are still going to go to London.  I just worry about how active she is and the risk of injury due to falls.  We will need to make sure the preschool and our babysitter know that it is important for her to avoid physical contact and notify us of any injuries.

I'm glad that Sofia is recovering well from the cath.  When she woke up after surgery, she was so hungry and it definitely made me smile that the first thing she said was request for breakfast.  She proceeded to eat a ton of spaghetti and meatballs.  She stayed one night at the Clinic and Oliver spent the night with her.  Thankfully, they had a private room.  It was really hard for me to work the next day and it would have been nice to have one day more off.

We had a nice weekend, and we had the big Congential Heart Walk on Saturday.  We really enjoyed it last year and were glad to be doing it again.  There were double the walkers this year and the last number I heard was that it raised $70,000, which is great.  Mended Little Hearts also had a table set up and we raised some money selling some merchandise.  It was a cold, rainy Friday, but it stopped raining for walk day, and then resumed today.  The walk reminds me of how lucky we are that           1) Sofia has continued good health, and it is not lost on us that there are lots of families that have more struggles than we do, and 2) that the CHD community is a very caring and close one.  Because when things happen that have you down, there are always people to talk with.  There were lots of families there that I have come to know over the past year or so, and it is nice that everyone comes together for this annual event.  This has been a big heart year for Sofia having 2 heart caths and her Fonan surgery, so we have become more involved.  It was also nice to see a good turn out from the health practitioners too - we recognized lots of nurses and doctors there.  The support of our family and friends is so touching and it means a lot that we had a lot of support and a lot of walkers for Team Sofia at the walk!  Great weekend!

Just one of the Hazel siblings missing... Next year the Hazel 5 will walk together again!