My name is Tiffany and at the age of 30, I was catapulted into the world of being a mother to a medically complex child following the birth of my 4th child, AJ. During our anatomy scan at 19 weeks, we discovered AJ would be born with a complex heart defect called Hypoplastic Left Heart Syndrome. We were devastated and in total disbelief as we had 3 other healthy children, how could this happen to us? What did I do wrong?
As it turns out, this congenital heart defect is often just a fluke that happens usually before you even know you’re pregnant. Once the high-risk doctor confirmed his diagnosis everything began moving at warp speed. That day in the office I had to make choices like whether I wanted to continue this pregnancy and if so to do an amniocentesis or not. I ended up choosing yes to both, I knew I wanted to give him the best chance at life.
From that point on our world became consumed with appointments and trying to mentally prepare ourselves and our children for what was about to come. I did all the things that other parents and experts recommended, met with the hospital, surgeon, and team that would be taking care of him. The one recommendation I didn’t take was to relocate for care to a better facility. I couldn’t fathom being away from my family for at least 6 months while he had the necessary surgeries or how we would finically be able to have two households on 1 income as I wouldn’t be able to work. I also wanted so much to keep some normalcy for the other children. Overall, we also felt very confident in the team we had locally, after all they had nothing but positive things to say and assured us they were capable of performing all his needed heart surgeries. In fact they did eight of them the year before! Well, you know how sometimes things seem too good to be true, and you have that feeling in the bottom of your stomach you keep trying to ignore? I had that the entire time and it was a feeling I eventually learned to never ignore again with AJ. On Monday, October 17th,, my water broke at 5 am, we immediately went to the hospital where I was put in a room and left mostly unmonitored despite his known complexity. Finally, at 4:18 pm AJ came into the world crying and breathing on his own despite his heart and the two true knots he had in his cord! I was able to hold him for 1 minute before he was whisked away to the NICU.
As prepared as I thought I was everything came immediately crashing down in that hospital. As I was wheeled back to my room I was assured they would take me to see him and keep us updated on his echocardiogram as post birth is when they can see the heart clearly. Neither of these things happened and finally at 1 am I went into momma bear mode and demanded to be taken down or I would rip out my catheter and crawl to him, it was a little dramatic but it got the job done and I finally was able to see him and get our 1st update. Over the next few days we were only told how amazing he was doing and to expect him home around Thanksgiving. This was shocking to me as it went against all the research I read, as did the way they handled his care. For example, all the research we read said the babies should not be fed until their heart surgery and that surgery should be done within the first week of life. For us AJ was allowed to eat as much as he wanted to “gain strength” and no surgery date was set until he was 10 days old because “he didn’t show signs of needing it yet”. It wasn’t until AJ was 9 days old and the night before his scheduled surgery that we found out the truth, AJ’s heart was much worse than they thought, something they knew immediately after birth but never told us. In fact it was so bad they couldn’t even perform surgery on it. The next few days were consumed with fighting the hospital to get him transferred to a higher lever hospital and basically having to barter with the surgeon there. He wanted to do more invasive testing and wouldn’t release him for transport without us agreeing. This was one of the first huge lessons I learned on advocating for care for AJ. If I had not been in contact myself with hospital advocating our wishes to the hospital and the insurance company AJ would have never made it out of that hospital. I kept pushed back and eventually with the help of another doctor we had AJ life flighted the night of October 29th, to Houston.
As I waited for the crew to arrive for transport I was terrified for AJ, our family, and all the other babies in that hospital we were leaving who didn’t have a voice. In just the short time there the disparity between the babies who had someone to advocate for them and those who didn’t or had additional barriers like a language barrier was so blatant. It sickened me and ignited a deep passion to do everything I could to help other families like ours. For the next 341 days the CVICU inside Texas Children's Hospital became our home. We quickly saw the difference between good and bad care (now versus where we were) and that not everything was going to be positive for AJ. So many things were different, we were actually a part of rounds and his plan of care every day, decisions were discussed with us and we also learned just how bad his heart was. The need to continue to advocate was still very much there just in a different way, after all no one knows your child better than you. For example, when I noticed minor changes and pushed the team to take x-rays, it was because of that we caught his bowel perforation early. When he went septic and no one could figure it out and I again insisted on looking at his gut, after a few days they reluctantly did and again, I was right. When he had a stroke and began displaying odd symptoms, I compiled the data and showed them he checked all the boxes for neurostorming and that the seizure medication was having an adverse effect.
This was just the tip of the iceberg over the 6.5 years that AJ was with us where I had to advocate for him from a clinical standpoint. The side though that most of us medically complex parents have no idea about is how hard it is once you are home, NO ONE prepares you for that! The day finally came when AJ made it home, it was simultaneously the best and scariest day of my life. We had worked so hard to get to this point as excited as I was to have him home I also kind of wanted to send him back, lol. I did all the training, all the research, and had his team, appointments, therapy, nursing, and DME in place. However, just like after his birth immediately things fell apart, except this time everything was solely on me to figure out. In addition to raising three other children, we had to live off of one modest income as we had to permanently relocate to Houston and maintain a lower income so he could have access to Medicaid while we waited for his waiver, balance our marriage, and oh and I was 5 months pregnant. As you can imagine the stress was all encompassing. Reality hit when we experienced immediately how ill-equipped and ill-trained these private duty home health nurses were. Most of the time being vent/trach trained meant they tried it on a mannequin doll one time. They showed up if and when they wanted with no recourse. Often I was training them, and many were nervous to even touch AJ. I had many who fell asleep on the job, would lie about the care that was performed, unexplained broken bones, and even one who refused to put his trach back in when AJ pulled it out “because he doesn’t look like he needs it”. As helpful as PDN (private duty nursing) was supposed to be it felt like a full-time job to oversee and manage it. Out of all the years AJ was home and approved for 168 hours a week of nursing I don’t think we ever had all those hours filled.
On average we received between 96-60 hours a week, leaving the other 72-108 hours a week for myself or husband as the main caretakers. That was often more than a full time job for each of us before trying to make a living and manage everything else. His siblings were also trained and helped when possible, most of the time they proved more knowledgeable than the nurses. I still can’t understand how an industry like Private Duty Nursing has no standard state-wide regulations or reporting requirements when it comes to training or hours to report (similar to DOT for drivers) and many of the mistakes we encountered through these nurses were in error of them being exhausted from working multiple shifts with multiple agencies that were back-to-back. For many of the people, my son was seen simply as a paycheck and not a person. DME, Medication, and Therapy were an additional stressor. At the hospital everything is in endless supply at your hands. Once we got home that all changed, we often had to ration items and reuse disposable items. So many times, I had to get creative and swap with other parents or spend endless hours on the phone trying to get orders from the doctor to the DME company or find out where the disconnect in communication was. Each month I was holding my breath hoping that everything AJ desperately needed would show up. Which is exactly what it was like for Medications and infusion meds. Meds were especially tricky if they were controlled, it always seemed like AJ was just short a dose or two, and trying to get anything refilled on time is a nightmare. We had constant worry as many of these meds are life-dependent.
Getting codes and overrides from the insurance companies to and from the pharmacy over time became second nature and again was something I learned from necessity. I had no idea any of these obstacles would be continuously part of AJ’s care. So many times, I would be blindsided as no one would notify me prior that medications, supplies, or therapy could not be provided for one reason or another. Throughout the 6.5 years of AJ's life, the constant stress and trauma became a relentless part of our existence, pushing our limits of endurance. I would keep thinking the stress would pass and the truth is it never did, you just learn to live with an ever-rising ceiling of what you can (and can’t) handle. Our entire family's life revolved around AJ's care, a decision that, while full of love, had significant consequences. My other children, homeschooled due to AJ’s condition, matured beyond their years, losing some of their childhood innocence. They were acutely aware of the fragility of life, having witnessed their brother's struggles and understanding the reality of his condition. This experience made it difficult for them to relate to their peers. My marriage, too, felt the strain of our circumstances.
Despite our strong bond before AJ's birth, the relentless stress took its toll, There is a reason that the divorce rate with a special needs child is a staggering 87%. My marriage also became a casualty of the high-stress lifestyle. AJ's life, though marked by challenges, was a source of immense learning and joy. His contagious smile brought light even to our most challenging days. The journey with AJ, along with meeting other families facing similar struggles, deepened my resolve to make a difference. Our children's different diagnoses and medical needs may vary, but our shared experiences of struggle and the lack of adequate support systems unite us. AJ's legacy is not just the memories we cherish, but the drive he instilled in me to advocate for change and support for families navigating the complex world of caring for a medically complex child. Tiffany G