Braden’s Story

Braden Cruse Knebel arrived on December 7th, 2009. He was 7 lbs 12 oz and 19 ½ inches long. We found out Braden had multiple problems early on in the pregnancy. He had a Cystic Hygroma on his neck measuring 17 mm, an ASD, multiple pin hole VSDs, Coarctation of the Aorta and probable Noonan’s Syndrome. When he arrived everything looked like it had resolved itself, but when we took him to see the pediatrician for a routine check up at a week old, he had to be sent directly to the Cardiologist for an Echocardiogram to check his heart because something just didn’t seem right and Braden had no palpable femoral pulses. As soon as they checked his heart they called the ambulance and Braden was taken to Children’s Healthcare of Atlanta. He indeed had Coarctation of the Aorta, which is basically the Aorta in his heart was severely narrowed and needed surgery. This is a life threatening condition. He also had pin hole VSDs in between the lower chambers of his heart in his septum, but these need to try to close on their own due to the location, size and Braden’s size. He was admitted on December 14th and had surgery to repair the aorta December 16th They had to use his Left Subclavian Artery to rebuild his Aorta arch because he also had Hypoplastic Aorta Arch. Two days later they attempted to take him off the ventilator, this lasted 16 hours and he had to be reintubated. He stayed on the vent another day and was able to come off once again. Braden was transferred to the Cardiac Step Down Unit on December 19th. Braden stayed in the hospital until December 23rd. Bringing him home for Christmas was the best present we could have ever asked for! He had a wonderful 12 days at home. On January 4th I decided to take him to the hospital because he began to decline, he wasn’t eating well and just wasn’t acting like himself. We remained in the hospital for a week, they told us that it was likely related to reflux and intolerance to his formula so we switched his formula and he started reflux medications. The day before we were supposed to go home they informed us Braden’s pressure in his lungs had changed and he needed a Heart Catherization to see how much blood flow was going to his lungs from the holes in his heart. They decided a second surgery to place a PA band around his pulmonary artery was necessary to help control his breathing and slow down the blood flow to his lungs until the holes close or until Braden was big enough they could attempt to close some of the holes with another surgery. On January 12th he had his second surgery. Once again he struggled off the vent and had to go back on. A week later we brought him home. He was admitted again in March and diagnosed with Parainfluenza which is another form of the flu. After two days in ICU and two days on the floor we finally brought him home! We returned to the hospital on March 5th for oissues with his pulse ox again, it dropped into the 50’s and 60’s. They discovered he was aspirating bits of formula so they did a procedure called a fundiplication where they take the top of his stomach and wrap it around his esophagus so he cannot reflux or vomit. Braden also received a G-tube. A G-tube is a feeding tube in the belly. They take the stomach and bring it up to the wall of the abdomen and stitch it in place and put a tube into the belly. The outside is called a Mic-Key Button. It opens up so we can put formula directly into the stomach. He is allowed to try to eat by mouth but we must thicken his feeds first. On March 29th Braden went into respiratory distress and fluid overload and was admitted to the ICU. He was in the hospital for 5 days ridding him of the fluid. On April 14th he did it again. He spent a day in ICU and 4 days in the Step Down Unit. Another Heart Cath was done and the oxygen in his lungs was only 80% when it exited the lungs. He was sent home with oxygen as we await for Braden to tell us what is next. He has been a mystery to his doctors and surgeons from day one. They have told us that he will likely need to get the PA band removed in the next 3 months. At that time they may attempt to close up some of those pin hole VSDs. His lungs seem to be getting worse even though the holes are getting smaller. We will be following up with a pulmonologist soon to determine if the has any sort of lung disease or if it is indeed all heart related.