11 Things I Would Tell Parents Dealing With a Diagnosis of a Right Side Heart Defect...

Laurel's daughter was born with an overall diagnosis of Hypoplastic Right Heart Syndrome. You can read her story here.  She is back with some amazing advice to those who have just learned their baby will be born with a right side heart defect. 

1.  Give birth at the best children’s hospital you can get to.

Your baby will still be able to be born normally.  They tolerate labor and delivery well, and don’t have problems until after they are born.

2.  You may not be able to nurse your baby right after birth, or at all.  

Babies with heart defects get tired more easily, and can have problems gaining weight.  It is very common for them to have a feeding tube in the hospital and to be supplemented with extra calories.  After you go home from the hospital, your baby may still need extra calories per ounce.  Nursing is hard work for a baby, and bottle feeding can help them get the calories they need.  

3.  Recovery times are not exact, and vary greatly.  

My daughter was in the hospital about four weeks after her BT Shunt, less than a week after her Glenn shunt and two weeks after her Fontan.  

Many times the recovery from the BT Shunt is the most difficult.  After her surgery her right pulmonary artery collapsed, causing her oxygen levels to drop below 50%.  They had to put an emergency stent in her artery to keep it open.
Recovering from Fontan Surgery 
4.  Surgical outcomes depend greatly on surgical volume.

The best outcomes are done by surgeons who have performed the type of surgery your child needs several times.  If they do not have enough experience treating defects similar to your child's, you may not have as good an outcome.

5. While she was recovering from the BT shunt, she was put on ECMO     (Extracorporeal Membrane Oxygenation). 

ECMO is a machine that functions in place of a patient’s heart and lungs.  Two tubes or “cannulas” are placed in the arteries of the neck, blood is pumped out, oxygenated, and pumped back in.  This is a VERY serious thing, and luckily no one told me just how serious it was.  She was on this for a week while her lungs and arteries recovered enough to function on their own.  

Your children’s hospital should have one of these machines and the necessary staff trained to run them.  We had our ICU nurse AND a dedicated ECMO nurse caring for her while she was on ECMO.
6.  During the winter months while they are 2 or younger, they will need a shot every month to prevent RSV known as Synagis.  

11 years ago it was $1500 per dose.  I cannot even imagine how expensive it is now.   Thankfully, insurance should cover this. 

7.  After they leave the hospital they may still be on a feeding tube.

There is no way to tell how long a child will need a feeding tube.  We had to deal with it for a few weeks; some children need it for months or even years.
8.  Between the first and second surgeries, children with single ventricle defects are classified as “medically fragile”, and should not be in a day care situation around other babies.

There will need to be a stay at home parent during this time, and possibly beyond.

9.  Babies who have been hospitalized for a length of time may have developmental delays requiring physical and/or occupational therapy to get over.  

While in the hospital, they are not learning to nurse or bottle feed, getting “tummy time”, or doing all the things other babies do.  In the case of heart defects, it can cause them to not hit developmental milestones at the same time as other babies.

10.  As they grow, they will still be able to run and play like any other child.  

Will they be an elite athlete?  No, not at all.  But they will still be able to have a normal childhood.  Some children are even able to tolerate competitive sports.  
11.  They are discovering that children who have had the Fontan develop fibrosis and cirrhosis of the liver to some degree.  

Several years after the Fontan, make sure your doctor is ordering blood work, and performing a biopsy and/or ultrasound of the liver to determine the extent of the damage.  
This is a pretty new finding and not all doctors are really aware of this.

This can seem overwhelming but I have some good news! It gets better! I promise you.  I found the first two years or so were the most difficult due to all the doctor and therapy appointments.  After the age of 2 these decreased to the point I almost felt I was parenting a normal child. 

Sadly, not all doctors, even now, are well versed in heart defects, and have been known to unnecessarily frighten parents into thinking that their child will not survive, or will have a poor quality of life. 

If your doctor is doing this, GO FIND A NEW DOCTOR.  

Sometimes, yes, the defects would be severe enough that this might be true, but more often than not your child will be just fine after some bumps in the road.

Here are some resources for you:

Children's Heart Foundation
American Heart Association - Common Types of Heart Defects  

The American Heart Association focuses most of their efforts and funding on acquired heart disease, and does not send a lot of money to fund research into CHD’s. 

If you wish to donate to a cause, I recommend donating to the Children's Heart Foundation.


Books to read about heart defects:

The Parent's Guide to Children's Congenital Heart Defects: What They Are, How to Treat Them, How to Cope with Them

It’s My Heart 
Obtained through the Children's Heart Foundation.

Heart Warriors: A Family Faces Congenital Heart Disease 

The author of this book is the Founder of the Hypoplastic Right Hearts support group, which now has a thriving Facebook community.

King of Hearts: The True Story of the Maverick Who Pioneered Open Heart Surgery 
This is an amazing look at the beginnings of heart surgery and one of the great doctors who dared to even try it.

Walk on Water: The Miracle of Saving Children's Lives

There are many more out there, but these are a few that I have read myself or know who wrote it.

Also, I highly recommend watching the movie “Something the Lord Made”, which originally aired on HBO, but now can be purchased on Amazon. It’s only available on DVD, not Blu Ray.  Our surgeon recommended it when my daughter was born.

Today's guest poster is Laurel Warner.  She is a wife and mother of two beautiful girls! If you would like to connect with her you can contact her at lellenn@gmail.com. 

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