Ava is born: 4 lbs. 15 oz. and 18 in.

Ava recovering after the Norwood and TEF/EA repairs at 1 month old.

Ava in Daddy's arms at 2 months

Ava feeling good at 3 months

Ava "waking up" on the PICU 7 weeks after the Glenn at 6.5 months of age

Ava home at last... at 7 months and weighing 20 lbs.

Today is my birthday!

"When we were one, life had just begun"

Ava Lillian was diagnosed in-utero with a 2-chamber heart, pulmonary stenosis and interrupted aortic arch during a surprisingly difficult third trimester. She was born by c-section due to a low heart rate at 37 weeks gestation. Ava was transported to Children's Memorial Hospital of Chicago on her birthday (1/16/05). That day, the diagnosis of esophageal atresia with tracheoesophageal fistula was also confirmed. Three days later, Ava had her first surgery to repair the opening between her trachea and esophagus.

A week after the TEF repair, Ava had her first open heart surgery, the Norwood, done by a team of three surgeons (Drs. Mavroudes, Backer, and Stuart). Post-operatively, her recovery was slow and complicated by chylothorax. We saw her eyes for the first time the day after her due date, Feb 5. Four weeks after the Norwood, we were able to hold Ava for the first time since her birth. She was one month old and weighed about 6.5 pounds. She still needed to remain on the ventilator due to the scheduled esophagus repair (Feb. 28th). My FMLA ran out the following day and I had to return to work full-time. It was sad because it seemed to minimize Ava's importance at a critical time; but I'm sure feigning normalcy was helpful to getting us through the months that lay ahead.

After seven weeks, Ava was finally off the ventilator! A couple weeks later (about 2 months post Norwood), the team noticed that Ava still needed a lot of Vapotherm support to keep her sats up. They had drifted slowly into a barely acceptable range. Occasionally, she was dipping into the 50's and hovered in the upper 60's most of the time. The cardiology team decided to do a cath to evaluate / possibly treat the problem. They felt she might not make it to the Glenn without further intervention. She ended up getting some balloon angioplasty to a narrowed area at the entrance to one of the pulmonary shunts where scar tissue had developed. This helped enough that she was able to get on a standard nasal cannula and wean to a level that was discharge material.

At this point, Ava started feeling good! She even tried drinking from a bottle; however, in the two months that had passed since her esophagus repair, scar tissue had grown and had begun to narrow the esophagus again, leading to spillover into the lungs after oral feedings. An esophageal dilation was scheduled. At this point, she still needed 1/4L oxygen to maintain her oxygen sats in an acceptable range. Nevertheless, her discharge to home was planned to happen within two weeks (if all went well). But alas, things did not go well.

After a very successful dilation, Ava vomited for the first time (she was not on Reglan/Zantac at that time). She got very sick over the next few weeks. She seemed to develop a cold in addition to aspirating fluid into her lungs. She had difficulty managing all the mucus and could hardly breathe. She needed to be intubated again for a week to get over the hump. She seemed to literally be fighting for her life. Everyone, including Ava, knew she was hanging on by her fingernails. During this time, Ava developed "chronic" pulmonary hypertension and high blood pressure. Furthermore, Ava has required continuous feeds by g-tube instead of the boluses she tolerated well before her esophageal dilation. She did recover from this illness (just enough, just in time) for open heart surgery. After some debate about a shunt revision, her surgeons chose to do a Glenn with PA plasty with Ava at 5 months and 14 pounds.

The Glenn was a very long and "tricky" operation (which was performed by Drs. Backer and Stuart) due to her size, pulmonary hypertension, and her complex anatomy. She was, however, surprisingly stable that first night and after a couple days and air of optimism developed. Ava was satting in the low to mid 80's! Then, her stability waned and Ava began to put on more and more fluid. A burgundy discharge continued to run from her nose. Her lungs were congested. She developed chylothorax again. On July 1st, Ava coded shortly after chest drain tube removal. She needed chest compressions to keep her blood circulating. Ava swelled up even more. She looked like she could burst. She was unrecognizable. In fact, did not look like a baby at all. She stayed this way for a couple weeks. They gave her a 50/50 chance to come out of this.

Two weeks later, Ava smiled in her sleep. Then, 5.5 weeks after the Glenn, we were able to hold her again. Within another week, Ava was extubated again (that's 14 weeks total time on the ventilator, but who's counting?) Believe it or not, after 10 months of struggle, we did not feel prepared for her to come home. Her discharge was an abrupt transition for us all. Ava came home for the first time on her 7 month birthday, weighing 18 pounds. After days of Ava crying vigorously with any handling and us scrambling to figure out all the medications and equipment (continuous g-tube feeding by pump and 1 liter of oxygen via nasal cannula), things settled in. In two weeks, she was sleeping through the night. Two more weeks have now passed and everyday she hits another milestone, or two! Today, she played happily on her tummy for several minutes and started banging and mouthing toys! Soon she'll be starting with ST, PT and OT soon to help her catch up developmentally. She is a very easy to please baby who communicates her needs well. She has lots of smiles and a great belly laugh. I am in awe!

Now, if we could just get her unhooked from all the tubes!

February 2006

Well... it has been a year since Ava's birth and "due date".

Ava is a joy. Her dad and I love to play with and cuddle her every chance we get. She is sooooo smiley and soooo happy. Best of all, she is very generous with her delicious laughter!!! I think she knows she's livin' the good life right now! Developmentally, she can sit very solidly to play and stand holding on or being held on to (which she thinks is fabulous)! She tolerates being on her tummy well now that she can pivot a little and roll to and fro. She even prefers to sleep on her tummy at times now, if you can believe it. She still does need help to sit up or stand up or get onto her tummy from being more upright. She loves to sit and kick at any toy in striking distance, but can bend over and grab things when she wants to. She says a lot of "DA" and appropriately at times for DAD and DOLLY (her FAVORITE)! She is able to do much more and is steadily making nice progress on her physical, social, cognitive and play skills. Her occupational, physical, and developmental therapists are very pleased.

Medically, Ava has come a long, long way, but does still have a long way yet to go. Her recent heart and esophagus tests show good post-operative function. However, she still sats about 5 points higher (at 80%) on the 1 liter of oxygen (no change since her discharge) so we are continuing it until further notice. It is a challenge for us to keep the nasal cannula on her. She likes to grab it at her ear and yank it off, especially when she's bored or restless. As far as eating goes, Ava is not particularly interested. She is well fed by g-tube and sees no reason to change. She tolerates us putting most foods in her mouth if we make it fun, but then she tends to hold the puree in her mouth for a long time. At her best, she will open her mouth intentionally for the spoon, lick her lips, and make several good and timely swallows with her rice cereal during a meal. She also likes to take drips of water by eye dropper (imagine trying to catch raindrops as they fall), but she currently refuses a cup or bottle. She weighs over 23 lbs. and is over 29 inches long, her head circumference is in the 75th percentile so undergrowth has not been an issue for her. She takes her toddler formula at a rate of 130-ml/hr by pump in 3 daytime pseudo-boluses with spit-ups at the end of her feed if she's active. She gets only water pumped in at night now. As Ava takes more orally, we'll continue to subtract from her g-tube feeds, until we don't need them anymore. Hopefully, progress with oral feedings and stomach volume limitations will allow us to get off the pump within the year (my goal). Her nutritionist and speech therapist still have their work cut out for them.

We are not currently planning on any more surgeries until the Fontan at this point. They aim for doing it at 3 years of age, if possible, at Children's Memorial of Chicago.