Crazy Heart Mama

Colman was born with hypoplastic left heart syndrome and a mild tricuspid valve regurgitation.  The diagnosis was made in utero at 32 weeks gestation.  Kevin and I were told that we had three options:

1. Three open-heart surgeries to be performed in stages during the first three years of life with the first surgery, the Norwood, being shortly after birth.
2. Heart transplant, but he would probably die waiting since there are not enough neonate hearts.
3. Palliative care.  We could take him home, but he would probably not survive the week.

Colman had the tricuspid valve regurgitation which complicated his situation.  If his tricuspid valve was too leaky, he would not be a candidate for the three surgeries and we would have been left with the dreaded third option.

Colman was born March 30, 2004, and underwent his Norwood procedure on April 2, 2004 at three days old.  We came home from the hospital April 20, 2004 just 21 days later.  We were back at Santa Rosa Children’s Hospital to undergo the Glenn on August 6, 2004, when Colman was four months old. Then on May 16, 2007, Colman had his third and final surgery of the HLHS series, the Fontan.  It was during his Fontan surgery that his cardiothoracic surgeon noticed that his tricuspid valve leak had increased to the moderate to severe range and managed to tighten up that valve so that it was back to only a mild leak.

Other than hospitalizations for heart surgeries, Colman remained healthy and active.  In the Spring of 2012, his tennis team won All-City and Colman finished the season almost undefeated.  He tied his last game.  Colman said, “I should have won it, but that guy was such a whiner.”

In July of 2012, we took a family trip to Hawaii.  It was on this trip that Kevin and I noticed Colman had developed a nasty cough and was very tired.  For the seven months leading up to that point, Colman had a regular cough that sounded very dry.  I brought it up to his cardiologist, and we attributed it to the Fosinopril (ace inhibitor) Colman took on a daily basis.  A dry cough is one of the side effects. The couple of times we were able to coax Colman into the ocean, he would immediately turn blue and ask to get out.  Then he would sit on the beach in the sun and play in the sand with a pained expression on his face.  His cough worsened, and I took him to a clinic on Kauai.  The doctor listened to his lungs and said they sounded clear.  Kevin and I moved up our flight home by a day, and we came back to San Antonio.

Colman’s coughing continued once we returned home.  We were going into the weekend, and I was planning on calling his pediatric cardiologist on Monday.  On Friday evening, I told Colman, “If you cough anything up, don’t throw it away.  I need to see it.  Okay?”  He asked me what I thought he might cough up, and I told him that I wasn’t sure.  I did show him a couple of pictures of bronchial casts off google images because I had been warned plastic bronchitis, a very rare and deadly disease, sometimes occurs in children who have a Fontan circulation, along with Protein-Losing Enteropathy, liver failure and a myriad of other awful things.

Late Sunday morning, Colman came running down the hall and said, “Mom, I coughed up that thing just like you said I would!” and he plopped a very soft and pliable bronchial cast into my hand.  I said, “Good job!” and my heart sank.  I called the pediatric cardiologist on call, and he said that as long as Colman wasn’t in any respiratory distress, they could see him on Monday.  I hung up the phone, placed the bronchial cast on a piece of cardboard and put it in a ziploc bag to take to the hospital.  Then I took a few pictures of it next to a ruler with my iPhone and texted the picture to our pediatrician.  He loves to get picture texts from me.

I went to check on Colman, and he was sitting on the sofa ramrod straight.  He looked so tired.  I asked him if he wanted to lie down or sit in my lap for a while and he said, “No” to both, that when he did anything other than sit exactly like he was sitting at that moment, he felt like he couldn’t breathe very good and he would start coughing again.  I called cardiology back and told them we were on our way to the emergency room.

Once we arrived at the emergency room, we were back in a room in less than five seconds.  I didn’t even fill out paperwork.  And, Friends, that’s what happens when you use the phrases “complex cardiac” and “respiratory distress” in a sentence together. The ER doctor immediately sent Colman for an x-ray, which showed he had a huge pleural effusion on the right side of his chest and a partially collapsed right lung.  We were immediately admitted to the PICU, where I handed off my ziploc bag to the nurse and watched her carefully label it for the pathology lab for testing.  Colman had a small surgery that evening to place a chest tube, which drained a little over 1200 cc’s overnight.

After further evaluation and testing by our heart team, we were told Colman was in heart failure.  His tricuspid valve, which had held steady at a mild leak for the past five years, was leaking severely and Colman’s heart was enlarged and not pumping efficiently.  With a diagnosis of heart failure and plastic bronchitis, we were told there was nothing more they could do at that time and Colman needed to be taken to a center to be evaluated for a heart transplant.  We were taken by air ambulance to Texas Children’s Hospital, where Colman underwent testing, as well as a six-hour heart catheterization, and was stabilized with new medications.  We were released two weeks after our arrival.

Colman is now being followed by the Heart Failure and Transplant team at Texas Children’s Hospital in Houston.  He is on ten medications and is currently stable.  He is not listed for transplant at this time, and we are hopeful that we can keep him stable for a while longer.