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.

 

My son Jason Hoffman was born June 9,2009 When he was born he was blue all over. After staying at the hospital for the required 4 days we was released to go home. I knew something was wrong with him but the doctors all kept telling me he was fine just had a little jaundice and was had to go into the doctor the next day to test his jaundice levels and see of they had gone down. When we went to the doctor the next day they said his levels was still up and we needed to return back the following day for another test. That night he was not much for eating and still had a blue tint to him and in the middle of the night he started choking on his own salivia. I told the doctors that they needed to do more test because something was not right. They finally checked him out and told me that he had a slight heart murmor but was nothing to worry about they go away on there own but they was gonna go ahead and do a echocardiogram just to make sure but they was sure he was fine. The next evening around 4:50 pm I recieved a phone call from Arkansas Childrens Hospital saying that I needed to have Jason there at 8 in the morning. I ask the lady if there was something wrong since I had yet to hear from the doctor where I live and she said no Dr. Best just wants to get to know your son. The next morning we got up at 5 am to start our drive to ACH. After arriving to ACH they did another echo on him and 3 different doctors came in to review it then they sent us to another room to wait for Dr. Best. I will never forget the words out of Dr. Best as he entered the room the first time I ever met him he says “There is something wrong with your son but I can fix it” my heart sank into my chest and I asked what is wrong with him? He says no one told you before you got here? I said no. (the doctors where I lived was supose to tell me but they didn’t instead they refused to see him anymore) So he proceeds to tell me my son has a VSD, ASD and His pulmonary valve was narrow due to only having 2 sleeves instead of 3. They place him on some lasiks and our goal was to et him to 6 months of age so he could gain some weight and be better prepared for the surgery. We went for checkups to ACH every 2 weeks and he made it to 2 1/2 months then they had no choice but to do the surgery he was not gaining weight he was losing weight. September 1, 2009 he went in for his surgery and was in surgery for 7 hours. He was out of surgery for approx an hour and we got to go back and visit with him for the 1st time. We was back there visiting and hadn’t been back there 5 minutes and the paged the surgeon 911 in panic I asked what’s wrong and one of the nurses said his chest is filling with blood. The surgeon comes in and tells me that is was probably a stitch or something they will take him back and fix it. They came into the waiting area to update us after he was out for the 2nd time and he said the pacemaker wires that they had placed in him was cutting a hole into his aorta and the hole was getting bigger everytime the heart would beat. He was on the vent for 7 days, then day 8 we went to the step down unit! Today he is a healthy child they have to watch him and make sure the patches grow with him and since they stretched the pulmonary vavle instead of cutting and patching it they have to make sure

it doesn’t narrow back out. So far so good. God works miracle and so does ACH!!

 

Kayla Hoffman

 

Congenital Heart Defects (CHD) are the most common birth defect, impacting 40,000 infants each year.  Twice as many children die from CHD than all forms of childhood cancer combined.  Families who have children with CHD are often in crisis and need the support Mended Little Hearts (MLH) provides.

Mended Little Hearts currently has 50 groups in 22 states, providing peer-to-peer interaction, educational programs, hospital visiting, and creating awareness using media and other instrumentality.  We will increase these services by creating more volunteer-led community support groups and by generating awareness in communities and in the nation.  This will enhance medical care and result in more children and families having access to much needed resources and emotional support.

Deliverables
*At least 25 new community support groups
*Production of 10,000 heart defect awareness packets
*At least 5 press mentions about congenital heart defect impact
*150+ hospital care packages