Wednesday, February 21, 2018

Heart Month Spotlight- AJ HLHS

Happy Heart Month!

I've been saving this story because I wanted to be sure that I could do this little guy justice. Miracles exist, and y'all, AJ is proof. His parents have been through so much and have fought for him and with him every step of the way. They are also the nicest people! To follow AJ's journey, 'like' his Facebook page, Advocating for AJ.

Antonio Jordan Garay or AJ as he is know was born on October 17th 2016 with Hypoplastic Left Heart Syndrome (hlhs), Restrictive Atrial Septum(ras) and Left Ventricle dependant Coronary fistulas/sinusoids. He also had two true knots in his cord. Despite all that he was born pink and needed no resucitation! We received his initial diagnosis at 19 weeks during a routine anatomy scan. We were told he had HLHS, a congenital heart defect that is fatal without a series of palliative surgeries.
We prepared for his birth, at home in San Antonio. The hospital decided his 1st open heart surgery  would happen at just 10 days old. Surgery was abruptly cancelled the night before, they found that his heart was more complex than they thought. We later found out his heart is truly one of a kind. His only option at life would be to go to Texas Children's. Hospital in Houston, 3 hours away from home in San Antonio. Once the decision was made he was life flighted 3 hours later at just 13 days old. We packed up and prepared to leave our other 3 children for an uncertain outcome and an unknown amount of time.

On 11/2/2016 AJ had his first open heart surgery called the Hybrid procedure. He recovered well. His aorta began to narrow and he had a stent placed on 12/9/2016. Then a g-tube placed 12/21/2016. He again did great and continued to recover.
On 1/13/2017 they decided to address his RAS. They placed a stent to open it. He came out and did great, then shortly before midnight he coded. They emergently reintubated AJ and worked to get his heart rate down. He stabilized that night and we spent the next 13 nights sleeping in recliners as AJ continued to decompensate. The next day on 1\14\2017 AJ developed an extremely dangerous infection called Necrotizing Endocolitis(NEC) it has an extremely high mortality rate especially for someone that was in his unstable condition. The next 48 hours were critical, but AJ prevailed.
Then on 1/18/2017 everything came crashing down. His heart was having terrible arrythmeia that was affecting his blood pressure. They spent 9 hours pushing drugs into his body desperately trying to counter act what the arrythmeia was doing. We watched our baby turn grey and cold and were told to start making end of life decisions for him. We had several nurses and doctors come bedside and cry with us for our son. They believed his heart was failing and he wouldn't make it through the night.
The next morning they decided he needed an emergent trip to Cath lab to get a better look at his heart. By now his heart had been in a fatal rhythm for almost two days, taking to Cath would mean be extremely high risk best it was our last option. Before they could take him back a wonderful nurse whom we have grown close with gave us a very special rosary. I put it in his hand and read the story of Fr. Seelos for whom the rosary is in honor of. AJ's heart went into normal sinus rhythm for 6 minutes straight! The cath was postponed until he was less critical. Over the next several hours his heart was more and more in normal rhythm. The next day they did the Cath and found no additional issues with his heart. It was not in failure and his arrhythmias stoped. In less than a week he was off the ventilator and back to his old self.

They decided now was the window of opportunity to perform his next open heart surgery the Norwood. On 2/2/2017 he underwent his 2nd open heart surgery, he flew through it like a champ in the first 36 hrs. Then he began to decompensate. On 2/12/2017 he coded and was back on a ventilator. He went back to the Cath lab days later and found his lungs weren't getting enough blood flow.
On 2/16/2017 AJ went for his 3rd open heart surgery, the Glenn procedure. On 2/23/2017 AJ once again developed NEC this time it caused his bowel to perforated resulting in emergency surgery and ileostomy. He struggled to recover from the the Glenn and developed plural effusion needing chest tubes to drain them. He had multiple trips to Cath lab and was diagnosed with pulmonary hypertention and also underwent a thrombectomy. He had been on the ventilator for nearly two months and started to make plans for a trach for him.
Then on 3/29/2017 we were called, AJ's heart had stopped and they were doing CPR. The did compressions for 12 mins and he came back. We later learned it was do to massive pulmonary embolisms that completely occluded his lungs, it is a miracle he survived. The clots were removed in the Cath lab. It was decided to take his Glenn down as his body was not accepting it. A Glenn take down is extremely risky and not ideal. He underwent his 4th open heart surgery on 4/3/2017 at just 5 months old. He came out getting the odds again. A few days later he had his chest closed. For the first time in months he was able to be extubated! Unfortunately due to all his complications he was habituated to massive amounts of narcotics, because of these he would go apenic. After of few episodes of apnea his lung collapsed several times. We decided the best thing for AJ would be a tracheostomy to have a secure airway and allow him to progress on 5/12/17 AJ had his trach placed.

Within a month for the first time in his life AJ went to the step down unit and we were preparing for discharge. AJ was going home on hospice but we were excited none the less. The excitement only lasted a week when AJ began having complications. I knew something was wrong and demanded we be sent back to the CVICU. We were initially hoping it was a quick fix but once again my mommy instinct told me different, I knew it was his bowels again. The next night we received a call that AJ had went into pulseless vtach, he once again had CPR and this time they used a defibrillator as well. Once again AJ prevailed, pulseless vtach in usually not survivable especially for a heart baby who we also found was in septic shock.
After many tests and monitoring AJ continues to decompensate over the next few days. He became so swollen his ears folded over and he couldn't be touched without massive amount of pain. I knew it my heart what the issue was and fought for the surgeons to take him back even though the tests showed nothing. They finally agreed and prepared us for him to not come out of the OR alive. A heart baby in septic shock doesn't survive bowel surgery. They closed the blinds gave us privacy to say goodbye until they took him back. A few hours later we got the word he pulled through! Further more they discovered it was in fact his bowel, it had flipped on itself causing an obstruction. If I didn't push for surgery he would have died. The days leading up to this were the worst we have ever seen AJ my heart broke for him and questioned if I was being selfish by wanting him to stay. I asked God to not leave my arms empty and fill them how ever he saw fit, I became pregnant that week!

Over the next few weeks AJ made very very slow progress and of course some steps back. Some set backs were bad including another code and CPR again. Many doctors questioned AJ's ability to recover enough to ever get out of the hospital and what his nuero status would be after all the trauma. Eventually thanks so a dedicated primary team AJ stated having slow steady progress. Days turned to weeks, which turned to months. Finally the day came we didn't ever think would happen!
After 353 days inpatient on October 5th, 2017 AJ was discharged! Remember that special rosary AJ received? Well as it turns out October 5th is also Fr. Seelos' feast day!!! AJ came to our new Houston home (the doctors recommended we permanently relocate). We have since adjusted to out new normal life and focus on living very much in the moment! His siblings have been absolutely amazing and are very active in all his care. We feel so blessed to be apart of his story and that God would trust us to be his parents. We enjoy celebrating every 1st he has, big and small! Next month AJ will have another big 1st as he becomes a big brother!