Philly Summary

Is the search over? Lord, is this the place where our son should have his next surgery? We were both asking ourselves the same question. We have been able to take Caleb to The Hospital for Sick Children in Toronto, Miami Children's Hospital, Children's Hospital in Boston and recently The Children's Hospital of Philadelphia (CHOP). Taking our son to Boston and Philly, which are the top two cardiac programs in the nation, was a fleeting thought since our state insurance would not cover such medical ventures. We are still so amazed at how God granted our unspoken desire so naturally and easily. The bible says the Lord knows our needs even before we ask (Mat 6:8).

So far CHOP has offered us the most information, research, and opportunity for Caleb and our family. We still don't want to rely on our understanding, but on God's leading. This is where there is peace even in the midst of difficulty. During our visit we got some of our unanswered questions answered. Why is his head so large? Why the developmental delay and complications after the 1st surgery? As well as some answers about life after the Fontan.

The Fontan is the next surgery Caleb will be having. After his cardiac echo (ultrasound of the heart), vital signs, EKG, meeting with Dr. Spray (the chief cardiac surgean at CHOP) and Dr. Wernovsky (one of the top cardiologists at CHOP) we were assured that Caleb is a good candidate for the Fontan circulation. He definitely needs the operation and it needs to be done soon while he is strong and stable. Right now this is technically considered an elective surgery, but if he does not have the surgery soon, his oxygen saturations will continue to decrease and he will become very sick.

So what is the Fontan? It is the last stage in a series of three repairs done to children with one or more congenital heart defects that cannot be corrected. Caleb has seven heart defects and two of them cannot be corrected. We say that he has half a heart. He is missing the right ventricle and right valve called the Tricuspid Valve. Consequently the right side of the heart is basically missing or useless. The Fontan is achieved after three stages of surgery. They have to do it in three steps because babies have high pressures in the lungs when they are born. The Fontan circulation does not work when the pressure in the lungs is high. The first surgery in the palliation involves opening up the upper heart wall (atrial septum) and putting a shunt from a part of the aorta to the right pulmonary artery which replaces the Patent Ductus Arteriosis (a connection between pulmonary artery and aorta that all babies use in the womb and are born with but closes quickly after birth).

The next surgery called the Glenn or hemi-Fontan is done at around six months when the pressures in the lungs decrease and the babies have grown into or out of the shunt. At this point, they disconnect the shunt from the first surgery. They also disconnect the superior vena cava from the right atrium of the heart and connect it to the right pulmonary artery. The superior vena cava carries all the blue blood from the body to the right atrium so it can go to the lungs via the pulmonary artery with the pumping from the right ventricle. This is what Caleb is missing. They also disconnect the main pulmonary artery. This is an easy surgery to recover from because the blood flow goes with gravity since it falls from the head to the chest area.

When kids are about Caleb's current age, about 3 yrs old, they complete the Fontan circulation by disconnecting the inferior vena cave (blood flow from the lower body, similar to superior vena cava from used in Glenn sugery) from the right atrium of the heart, extending it with a tube and connecting it to the bottom of the right pulmonary artery branch. Because the blood from the lower part of the body travels against gravity and depends on the pumping of both ventricles of the heart, the recovery is long and includes complications with fluid building up around the lungs (plural effusions). Because Caleb's body has developed many extra veins (collateral veins) to try to compensate for his lack of oxygen, he is at higher risk for the effusions. This is a minus for him. But thankfully it is the only minus he has.

We asked the Nurse Practitioner who is the liason for out of town patients, Katie Dodds, to submit Caleb for surgery scheduling for June. We are waiting for the appt. date and will post again as soon as we have it.

We would also ask that if you are reading this post that you really begin (or continue) praying for Caleb and us, as we have a renewed sense of the risks involved in this surgery.

Thank you!!