Lisa, I can feel your anxiety.  My son, Nathan, also had PAPVR, but his was complicated by his dextrocardia (his heart was severely rotated backward).  He was successfully treated at C.S. Mott Children’s Hospital in Anne Arbor, Michigan.  I am so grateful that we live in a time of great medical advances for children with Congenital Heart Defects.   Prayers for Gage and to you and your family.

Nathan regains consciousness after surgery to correct Partial Anomalous Pulmonary Venous Return (PAPVR), Scimitar Syndrome. This was taken in PICU at C.S. Mott Children's Hospital in Michigan.

As Nathan regained consciousness after heart surgery, I wanted to snap a photo to record the moment.  Partially out of habit, and partially out of pure joy at seeing his eyes flicker, I blurted out, “Smile!”  Instantly, I felt ashamed of myself given the ordeal he had just come through.  But ever-obedient, his lips slowly assumed the position.

He’s smiling for real these days – the surgery to correct his congenital heart defect was a great success.

[See also:  About, Nathan's Prayer, Dr. Edward Bove, Before the Back Surgery, The Principle of Finders Keepers,  Body Cast, Three Year Anniversary]

Three years ago today: Nathan and sister Michelle at the Med Inn (C.S. Mott Children's Hospital, Michigan) playing the night before surgery to correct PAPVR, Scimitar Syndrome. On the outside, we tried to keep things light-hearted. On the inside, we were crazy afraid.

The Sea Climb at Lynn Meadows Discovery Center; Gulfport, Mississippi

“This is great, Mom,” Nathan said as I pulled in at Lynn Meadows Discovery Center. “It reminds me of my childhood.”  Nathan is only eight-years old, but seems to believe he is no longer in his childhood.  In a way, I understand his perspective.  After all, he was a regular at “Meadows” (Nathan-speak for LMDC) when he was three and four-years-old.  That’s half his life ago.

Due to reasons beyond our control, our trips to LMDC stopped abruptly. First, Hurricane Katrina swept an 11-foot storm surge through the first floor, destroying the exhibits, and causing catastrophic damage to the supporting structures:  the education building, the gymnasium, and the extensive tree houses built among the live oaks.

Nathan in the Sea Climb; Lynn Meadows Discovery Center; Gulfport, Mississippi

Then, as the community and corporate sponsors pulled together to rebuild LMDC, Nathan’s life became dominated by surgeries. The first surgery occurred in Michigan’s C.S. Mott Children’s Hospital to repair his congenital heart defect (he has Scimitar Syndrome with PAPVR).  After heart surgery, we learned he had Congenital Kyphosis and needed spinal fusion surgery to prevent paralysis.  He was in a body cast for several months afterwards. So we stopped going for a while, and  Nathan seemed to have forgotten his old stomping grounds until I drove into the parking lot two weeks ago.

He quickly recollected his “childhood” and began reenacting scenes from that era.  He scrambled up the Sea Climb, pausing briefly for me to take a couple photographs.  He hopped out at the History Hotel on the second floor  and sat down for “tea” and a plate full of “pastries.”

Nathan rings up groceries at Lynn Meadows Discovery Center; Gulfport, Mississippi

He visited Mexico at Celebrate the World We Share and shopped for groceries at To Market, To Market (chocolate milk, a baguette, an orange, Orville Redenbacher Popcorn, and a crab).  Then he executed a flawless cooking show as if he were Emeril Lagasse in the LMDC Television Studio.  He also performed about a half dozen experiments in A Matter of Science (and he thought he was just playing).

After a kayak ride down the bayou, Nathan made his way down Sea Climb to check out the first floor exhibits.  He hopped a train at the Dolan Avenue Depot, and spent some time at the spacious train table.  After riding the rails, he took to the seas at The Port, manning the helm and ringing the bell on a shrimp boat.  Feeling a little creative, he stepped down the hall to the art room where volunteers showed him how to do “scratch art.”

Bubblemania at LMDC

When Nathan’s masterpiece was completed, we decided to take a shortcut through Bear Camp Bayou (for children under four) to check out Bubblemania, an outdoor summer exhibit.  By pulling a long cord, he made a huge bubble sheet at one station.  Then he tried out a machine that formed hundreds of tiny bubbles between two glass panes.  Mostly, he liked making large bubbles with the assorted bubble wands.  No one was looking, so I made a few myself (if the Executive Director, Cynthia Minton, can do it, so can I ).

It’s amazing how fast two hours goes by at LMDC.  I told Nathan it was time to go home, but he managed to talk me into ten more minutes.  He needed to make a run through The Tree House Village for old time’s sake.  I hated to interfere with his quest to reconnect with his childhood.

Nathan waves from the Tree House Village at LMDC.

[The Lynn Meadows Discovery Center is open Monday - Saturday, 10:00am to 5:00pm, during the summer (Tuesday - Saturday beginning August).  Tickets are $7 for adults and children over age one.  Family memberships are also available.  For more information, contact LMDC at (228) 897- 6039 or check out their website at www.lmdc.org.]

Upcoming Events at LMDC:

•The WINGS Performing Arts Center of LMDC presents “The Wiz” at MGCCC, Jeff Davis Campus, Fine Arts Auditorium, July 17-19.  Click here for tickets and details.

•A Circle of Friends presents “Performing Friends” on Saturday, July 25, 2009, 10am to 4pm.  This free event will be held in the WINGS Performing Arts Foyer at LMDC.  This is a family fun day which focuses on disability awareness.

DisabilityConnection.org, an Internet Resource Center for people with disabilities, has partnered with LMDC to bring this event.  Expect a great day of performances, demonstrations, door prizes, games, food, and booths.

Colleen is awaiting test results on her son, Tommy, who has recently received a heart transplant.  Doctors are testing for possible cellular rejection and should know if this is the problem by tomorrow.  Tommy has been returned to PICU; his family is understandably distressed.

Also, remember the family of Lindsay Dean, born last year with HLHS or Hypoplastic Left Heart Syndrome.  Little Lindsay received a new heart earlier this month, but her heart stopped yesterday morning.  She has been placed on ECMO.  Pray for her and parents, JR and Suzie Dean.  [Posted June 16, 2009]  Update:  Lindsey is home!  Follow her progress at http://lindsaysheart.blogspot.com/.

My friend, Donna Miller, is requesting prayer for 9-year-old Hunter, son of her colleague, Cheryl.  Hunter, born with pulmonary hypertension, is autistic and has ADHD. “Hunter has recently developed a ‘butterfly’ rash which is a sign of Lupus,” Donna says.  “They are seeing a Rheumatologist on Monday at Tulane.”  [Posted June 18, 2009]

Pray for three-year-old Brooklyn Keller.  She was rushed to Forrest General Hospital in Hattiesburg with high fever and an abnormal blood count.  [Posted June 23, 2009]

"The Day After My Heart was Fixed" by Nathan Blakeney

This July will make three years since Nathan’s heart surgery at C.S. Mott Children’s Hospital at the University of Michigan Medical Center.  He colored a cheerful picture for me recently that seems to convey instant happiness after his heart was repaired.  That’s not exactly my recollection, so I asked him what he remembers about those days.  Come to find out, not much.

Did he remember being in intensive care?  No.  Did he remember having tubes removed?  No.

What about the good parts?  Did he remember the stuffed animal a nurse gave him?  No.  Did he remember watching new Power Ranger videos on my laptop?  No.  Surely, he remembered the Toys R Us shopping spree when he was released from the hospital…No!

I bring this up because I know that parents of children with Congenital Heart Defects fret over the emotional scars their children are going to have from enduring traumatic surgical events.  We want to find ways to make it better.  And certainly, it is worthwhile to soften the blow by providing entertainment through books and toys and games.

But time definitely has healing power.  The only people in the house with Post Traumatic Stress Disorder are Mom and Dad!  Perhaps, it’s time to step into Nathan’s cheery picture.

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.

Our first look at Nathan after heart surgery

…Jesus called the children to him, and said, “Let the little children come to me, and do not hinder them, for the kingdom of God belongs to such as these.” Luke 18:16

The summer of 2006 was the most terrifying season of my life–Bryan’s, too, for that matter.  The date, July 25, was hanging around my neck like an albatross.  That was the day Nathan was scheduled to undergo open heart surgery at C.S. Mott Children’s Hospital at the University of Michigan Medical Center.  Everything was in place:  our flight from Gulfport, Mississippi, to Ann Arbor, Michigan, was booked; accommodations at the Med Inn were secured; and we had acquired Dr. Edward Bove, probably the best pediatric heart surgeon in the world, to repair Nathan’s congenital heart defect.

As the big day approached, I penned a simple child’s prayer (located at the bottom of the middle column) for Nathan.  He memorized it enthusiastically and would repeat it to anyone who would lend an ear.  Listening to his sweet recitation did not calm my heart, but it certainly warmed it.  Have a child facing surgery?  We would be honored if Nathan’s Prayer brings comfort to you.