Nathan watches a Disney video during EKG testing at Children's Hospital in New Orleans.

Annual checkups are an important part of maintaining Nathan’s heart and back health.  Recently, Bryan, daughter Michelle, and I traveled with Nathan to Children’s Hospital in New Orleans to see how his heart looks four years after surgery to correct Partial Anomalous Pulmonary Venous Return (PAPVR), a problem associated with his scimitar syndrome.

The echocardiogram seems to be a challenge for the technician each year.  His heart is positioned strangely in his chest (dextrocardia), so it takes a little patience to capture the desired images.  It doesn’t help that Nathan is extremely ticklish, especially when the transducer is placed at his neck.

During Nathan’s heart surgery, a baffle (tunnel) was created to redirect oxygenated blood from the right atrium to the correct chamber, the left atrium.  The last time the technician was able to capture an image showing the baffle was a few days after his surgery in 2006.  So it was a pleasant surprise when the cardiologist spotted it and reported that it appears to be unobstructed and working fine!

Nathan poses by a whimsical seahorse painting while he waits for one of his doctors.

In addition to his congenital heart defect, Nathan was born with a hemi-vertebra which was diagnosed as congenital kyphosis three months after his heart surgery.  We visited Nathan’s pediatric orthopedic surgeon on the same day.   I wish I had snapped a photo of the radiographs Dr. King presented to us.  One showed his spinal column right after spinal fusion surgery; a piece of cadaver bone replaced the hemi-vertebra and was easily discerned from his natural vertebrae.  The latest x-ray showed the cadaver-bone vertebra and the two vertebrae directly above and below had transformed into one solid piece, just as it was supposed to do.  There was concern, however, that the fusion was curving about 40 degrees.  If it continues to curve, reaching 50 degrees or more, it may be necessary to place rods in his back.

I hope, I hope, I hope… it will hold.

We pushed that thought out of our minds and went to Copeland’s after our day at Children’s.  We had their fabulous Spinach and Artichoke dip with fried bow-tie noodles for an appetizer (Nathan only eats the noodles).  Bryan had barbeque with cornbread.  Michelle and I had a delicious pecan encrusted fish.  Nathan had his usual–chicken tenders with fries.  Dessert:  Cheesecake Napoleon with praline sauce for us big kids, Big Al’s Chocolate Fudge Cake for Nathan.

Sometimes, you have to look at life’s challenges with the mindset of that famous philosopher, Scarlett O’Hara:  “I’ll think about that tomorrow.”

This interests me because Nathan has dextrocardia, too.  Hopefully, he will never need a heart transplant, but it certainly would pose a challenge if he ever did.  It was a relief to see a happy ending for the gentleman on GMA–his transplant was a success.

Dr. Edward Bove is experienced in the treatment of complex congenital heart defects.

Back in April, I attached this photo to a post I wrote about Dr. Edward Bove (the surgeon who performed our Nathan’s surgery in 2006).  I learned from Phil Eppard, that the little fella’ pictured with Dr.  Bove is Phil’s son, Aidan.  The photo was taken in 2005 after Aidan’s first heart surgery and has been used in “The Michigan Difference” campaign.

That’s pretty cool, but it’s not the reason I’m saying “Hooray for Aidan.”  Apparently, under the excellent care of Dr. Bove, Aidan had his second heart surgery yesterday, and it went great!  Aidan is out of PICU and in a regular room now.

The photo has been reproduced as a giant banner that hangs at the hospital for all to see.  So Aidan is famous at the University of Michigan Medical Center. 

Update:  9-19-09  Aidan has been discharged and is on his way home.  Best wishes to the Eppard family.

In case you missed this Oprah episode, watch as Dr. Oz explains why “the stem cell debate is dead.”

Click here to view the embedded video.

In our situation, surgery was far enough into the future that it seemed premature to burden him with information that could wait. Our approach was to refer to appointments with pediatric cardiologists as “check ups.”  Both of us were present at these appointments so one of us was able to engage him in distracting conversations or games while the other spoke with the doctor about the particulars of his condition or the details of surgery.  When we finally secured a surgery date, our instincts proved correct as the surgeon recommended we postpone talking to our five-year-old son until two weeks before the surgery.  Presenting information too far in advance can potentially create unnecessary, prolonged anxiety.

Tips for speaking with your child:

Nathan takes his first walk down the hall after heart surgery to correct PAPVR, Scimitar Syndrome. His surgery was performed at C.S. Mott Children's Hospital, an excellent facility for the treatment of congenital heart defects.

• Gather your thoughts before you speak.  It may help to write an outline of what you need to discuss with your child.
• Keep the conversation simple.  Let them know a doctor is going to make their heart work better so they will grow strong and healthy.
• Assure them that you will be there for him/her during their hospital stay.
• Ask them if they have any favorite toys, books, or DVDs they wish to bring.
• Books such as Franklin Goes to the Hospital can be great tools for introducing unfamiliar hospital practices to your child.

When your child transitions into the teen years, it is important to explain in more detail the type of congenital heart defect he/she has, how it has been managed, and what kind of treatment may be necessary in the future.  According to the National Heart, Lung, and Blood Institute, this kind of frank discussion will help your teen to begin taking responsibility for his or her health and “ensure a smooth transition from care by a pediatric cardiologist to care by an adult cardiologist.”

Nathan enjoys a little father and son bonding.

Remember that childhood surgery is not a “cure” for a congenital heart defect. Make sure your teen understands the importance of followup care throughout adulthood to maintain good health.

Heterochromia

Hands down, Nathan has the most unique anatomy in the Blakeney household. Besides the associated health issues, a component of Scimitar Syndrome is that his heart is twisted backwards in his chest.  Of course, this is, generally speaking, no laughing matter; we were tremendously blessed to have followed him through successful heart surgery.  But we do find our sense of humor about his dextrocardia sometimes.  We point out that he is the only member of the family who should pledge allegiance to the flag with his left hand over his right-sided heart.

Heterochromia: My daughter, Michelle, has a green eye and a blue eye.

So Nathan has the most unique anatomy in the family; but his sister, Michelle, has the most unique eyes in the family. Filling out forms that ask for eye color is straightforward for most of my kids.  Nathan, Adam, and Natalie, have green eyes.  Steven has blue eyes.  Michelle, however, is in a bit of a dilemma when asked to write down her eye color, because one eye is blue and one is green.  It’s a subtle difference, usually takes good lighting to recognize, but she has been grabbed by the face on occasion by the curious observer seeking a better look:  “Hey, what’s up with your eyes?”

David Bowie

Jane Seymour

A difference in the coloration of irises is called heterochromia.  There are two kinds of heterochromia, partial and complete.  In partial heterochromia, the iris has two different colored sections.  Complete heterochromia, which Michelle has, means the eyes are two different colors.

Dan Aykroyd

Kate Bosworth

Certain species of animals are more likely than humans to have this trait. A former boss of mine had a beautiful white manx with a tail that had a striking set of eyes, one blue and one gold, similar to the cat pictured above.  The imperfection had no negative effect at foo-foo kitty pageants; she frequently won awards, including Best of Show.  Other animals that may exhibit heterochromia include dogs (such as Siberian Huskies), horses, cattle, and water buffalo.

Gracie Allen

Even a few celebrities have heterochromia. Take a good look at the eyes of Jane Seymour, Kate Bosworth, David Bowie, and Dan Aykroyd.  The late Gracie Allen also sported two different-colored eyes.

Heterochromia is usually the result of heredity, although it can be caused by a disease or a syndrome (Neurofibromatosis or Waardenberg Syndrome, e.g.).  Occasionally, an eye may change color after an injury, which is the cause of David Bowie’s heterochromia.  Michelle?  She just has a case of funky eyes.

Adam Blakeney culturing stem cells in the Shelby Biomedical Research Building at UAB

My oldest son (Nathan’s big bro’), Adam, has almost completed his undergraduate degree in biomedical engineering at the University of Alabama at Birmingham.  His team has completed a project that has recently been published in Biofabrication, a journal of state-of-the-art research and development.  Much of what those guys do is waaay over my head, but I asked Adam about his work and the kind of innovations we may see in the future.

“I design materials using plastics and protein building blocks, which contain and control the growth of human stem cells derived from adults,” he tells me.  He experiments with the creation of “cell scaffoldings,” attempting to “mimic the body’s natural mesh of collagen and elastin as closely as possible.”  The goal is to fashion the right combination of materials and artistry so that living replicas of human parts (blood vessels and heart valves, e.g.) can become functional replacements for diseased or deformed parts.

How might this technology benefit children with congenital heart defects? “Deformed heart valves in infants usually mean  multiple surgeries over the course of the child’s life as the heart outgrows the implanted artificial valve,” Adam says.  Stem cell researchers hope to eliminate the need for extra surgeries.  “By creating a shell of a valve out of plastics or natural materials, and infusing them with stem cells, the hope is for the cells to overtake the originally artificial valve,” he explains.  “As the heart grows, the old valve would break down and replace itself using the cell’s naturally produced collagen and elastin.”

Adam and Nathan (li'l Adam!)

Adam says it’s difficult to tell how close scientists are to turning research into reality.  “‘How close changes monthly, but I could see applications using stem cells becoming widespread in ten to fifteen years.”  He says there are still challenges to overcome, one of the biggest being “controlling what happens to a stem cell when it is implanted.”  Addressing this problem will involve “a combination of localized drugs, genetic engineering, and the structure which the cells are placed on, each of which have their own set of problems.”

Despite the challenges, Adam sees a positive future for this research.  “Repairing damaged heart muscle and missing vasculature are strong candidates for the first widespread applications of tissue engineering.”

It is no longer unrealistic to envision growing new tissue from our own stem cells to repair damaged organs.  Science fantasy is on the verge of becoming science fact.

heART

• Heart defects are the most common and deadly of all birth defects.
• Approximately 36,000 babies are born each year with a congenital heart defect.
• In 1950, a child born with a congenital heart defect had only a 20% chance of survival.  Today, survival rates have increased to 90%.
• Survivors of successful childhood intervention face life-long risks, including heart failure, stroke, and rhythmic disorders.
• Each year, nearly twice as many children die from CHD in the U.S. than from childhood cancer, yet funding for pediatric cancer research is five times higher than funding for congenital heart defects.
• For every dollar the government spends in medical funding, only a fraction of a penny goes toward congenital heart defects research.

1. Normally, the right pulmonary vein enters the left atrium here. 2. In PAPVR, the right pulmonary vein abnormally enters the right atrium.

This is one of the best illustrations I have found for explaining PAPVR.

Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart defect in which blood flow from one or more of the pulmonary veins return to the right atrium instead of to the left atrium.  This is a problem because the right atrium shoots this already oxygenated blood right back to the lungs to be oxygenated again.

Of course, this is a very inefficient way for the heart to function. The heart must work extra hard to provide an adequate amount of oxygenated blood to the body; and over time, this causes a variety of consequences, including arrhythmias, pulmonary hypertension, and right-sided heart failure.

If 50% or more of the pulmonary veins anomalously return, this is considered clinically significant enough to warrant surgical correction. If the patient has an Atrial Septal Defect, the surgeon will close the defect with a patch and redirect blood from the anomalous pulmonary vein to the left atrium.  If there is no ASD, a baffle or tunnel will be created to redirect blood to the left atrium.

PAPVR associated with Scimitar Syndrome has the added complication of dextrocardia. The surgery will likely be performed on a heart that is rotated backwards.  The atrial septum is usually intact.

Our first look at Nathan after heart surgery

My own son, Nathan, had this surgery on July 25, 2006, at C.S. Mott Children’s Hospital at the University of Michigan Medical Center. Our experience included a full day of preliminary testing, surgery the following morning, and a stay in PICU.  After extubation, he was moved to a regular hospital room where he spent the first few days on strong pain medication.  Removal of the chest tubes occurred on about the third day–this was somewhat emotionally challenging but was successful, nonetheless.  By that evening, his personality began to return.  On the fourth day, nurses encouraged him to take a brief walk down the hall.  By the fifth day, he was well enough to be released!

Nathan takes his first walk down the hall after heart surgery to correct PAPVR, Scimitar Syndrome. His surgery was performed at C.S. Mott Children's Hospital, an excellent facility for the treatment of congenital heart defects.

We used a number of child-friendly books to prepare Nathan for surgery.  One of his favorites was Franklin Goes to the Hospital.

Other related posts on this website include:  Scimitar Syndrome, Dextrocardia, Nathan’s Prayer, Remembering C.S. Mott, and Dr. Edward Bove.

Also see my article in Parents magazine:  “Nathan’s Wonder Slide”

If your child has an upcoming heart surgery, consider adding him/her to our Prayer List.

Dr. Edward Bove is experienced in the treatment of complex congenital heart defects.

When faced with a complicated congenital heart defect, confidence in the skill and expertise of your child’s surgeon is of paramount importance.  When our son, Nathan, was diagnosed with Scimitar Syndrome, a rare condition characterized by dextrocardia, pulmonary hypoplasia, and Partial Anomalous Pulmonary Venous Return, we soon learned that our local surgeons had no personal experience performing the required repair.  After reviewing Nathan’s medical records, the closest doctor with a successful surgery under his belt was not willing to proceed due to the “severe rotation of the heart.”

There was tremendous anxiety in our household as we considered our options.  Do we allow a willing doctor to perform the surgical correction even though this would be his first attempt?  Or do we continue to search for a doctor skilled in correcting this rare CHD, possibly allowing Nathan’s heart to weaken in the passing months?

Thankfully, I came into possession of Michael Ruhlman’s book, Walk on Water:  Inside an Elite Pediatric Surgical Unit, which documents Dr. Roger Mee’s dynamic team at Cleveland Clinic in Ohio.  Mee, I discovered (to my initial chagrin), had retired, but the name of another “Walk on Water” surgeon caught my attention–Dr. Edward Bove.

I described our son’s condition to him via email, and was thrilled when he quickly and graciously wrote back that he “would be honored to evaluate” our son for surgery.  Bryan and I were extremely impressed with his credentials.  He had an 85% success rate with Hypoplastic Left Heart Syndrome, a complex condition requiring a series of three surgeries in order to transform half of a heart into a functional heart.  After reviewing Nathan’s medical records, he called one day to inform us that he would perform the surgery.  I took down the necessary appointment information, thanked him, hung up the phone…and shook for the next few minutes, in the grips of an odd combination of joy and terror.

In July of 2006, our family of seven boarded a plane in Hurricane Katrina-ravaged Gulfport, Mississippi, and flew to Ann Arbor, Michigan.  At the C.S. Mott Children’s Hospital, University of Michigan Medical Center, Dr. Edward Bove constructed a baffle out of Nathan’s own pericardium to tunnel through the right atrium to the left atrium, successfully rerouting oxygenated blood to the correct chamber.  That’s not even a Reader’s Digest Condensed version of the story, but suffice it to say, we will forever be grateful to Dr. Bove and his team at C.S. Mott.