Tetralogy of Fallot and MY Kid

Like most parents, you live your child's lives. Not live through them, but live for them. Meaning if they're going through something you read about it, learn about it and guide them or console them if possible.

Right now my focus is on Cole. He did extremely well, but now that his heart's been 'messed' (their words not mine) again, it can be thrown off and forget what to do. His sternum has been cut open once again, so this will make future surgeries more fun for the surgeons because each one can add scar tissue and adhere the heart to the sternum. The very reason they try to find alternatives to limit the number of open-hearts one person has.

A lot of people have asked what he has. Tetralogy of Fallot sounds so huge and technical. It's really:

1) Pulmonary Infundibular Stenosis A narrowing of the right ventricular outflow tract.

2) Overriding aorta An aortic valve with biventricular connection, that is, it is situated above the ventricular septal defect and connected to both the right and the left ventricle.

3) Ventricular septal defect (VSD) A hole between the two bottom chambers (ventricles) of the heart.

4) Right ventricular hypertrophy The right ventricle is more muscular than normal, causing a characteristic boot-shaped (coeur-en-sabot) appearance as seen by chest X-ray.

Cole actually had a pulmonary valve at birth, but due to #1, it was removed and a patch put in it's place making it a monocusp instead of bicuspid. Cole had 2 large holes instead of 1. These holes are the actual reason tet babies turn blue or have cyanotic spells. Once the holes are fixed the baby should no longer turn blue unless the patches fail. After this, not having a pulmonary valve or one that doesn't work right, the blood will regurgitate back into the right ventricle instead of flowing to the left. This causes an enlarged right ventricle and low blood flow to the rest of the body. It can also cause failure of the right muscle and it can forget how to work or loose elasticity and not work even after repair, requiring transplant.

They had been watching Cole so closely that while the upper leakage at his pulmonary valve had caused such enlargement that another lower leak developed, they did surgery before it really (or they think as of now) began to loose it's function and elasticity. Because of his condition and where the repair is, open heart will always be necessary over going through a side rib. However, it's possible now that he actually has a bovine valve with a stent already in place, the next surgery may be prolonged by installing a Melody Valve via cath instead. It's new, so they do not yet know how long this will put him off for full valve repair via open heart surgery. Their goal as of now is to make it 10 years on this one.

The reality is that in the support group I belong to for adults and children with tet, some do not survive, some require transplants, some develop rhythm problems. All of these are extremely life threatening, but many can do well with the right interventions and great physicians. Ours will be one.