Below is Mackenzie`s story provided by her mama.
I want to explain things from the beginning, because her journey is an important one. During pregnancy with Mackenzie, I (mom) was very sick the entire time. I had to have a home health nurse and a zofran pump 24/7 due to sickness. As the pregnancy progressed, the high risk OBGYN became concerned and transferred us to a fetal cardiologist because she "couldn't get pictures of all of it." Once we saw the fetal cardiologist we found out that something was up with Mackenzie's heart. We were told her right atrium was enlarged. I was also diagnosed with Cholestasis (for a second time) and PUPPS. Both of these made for a miserable pregnancy. At 35 weeks, I went to the high risk OBGYN becuase my feet were swollen and I was seeing black spots. This was the start to preclampsia. Doctors decided to wheel me over, frorm that appointment, to the triage for labor and delivery. They said we were having the baby that night. Fast forward a couple hours and we were told that the "team" was not ready. We had no idea what that entailed, at the time, but we ended up admitted and were going to deliver in the morning. In the morning, at 9:30 am, doctors starting entering our room and explaining things, but the doctors kept coming. They were shoulder to shoulder. There were cardiologists, residents, pulmonology, neurology, other doctors, nurses, students, residents and so many people. Mackenzie came into this world at 10:08am. Weighed in at 6lbs 12oz. 19 inches tall. She was taking from the room by 10:10am and was rushed upstairs to the Level 3 Nicu. Grandma and Nana followed her and were met with the hallways filled shoulder to shoulder with doctors waiting to see her. They did not know what they were facing when she was born so they had everyone there. One by one they started dismissing the doctors who were not vitally needed at that time. From that moment on, our long journey started. Mackenzie was born with Scimitar Syndrome. Scimitar Syndrome is a congenital (present at birth) heart defect. It's a type of partial anomalous pulmonary venous return (PAPVR). In scimitar syndrome, people have: Hypoplastic (underdeveloped) right lung. Hypoplastic (underdeveloped) right pulmonary artery (the blood vessel that carries blood to their lungs). Mackenzie's form of Scimitar caused her to be born with an underdeveloped right lung and her right pulmonary artery and vein missing completely. She also had an ASD (a hole in the heart between the upper chambers (atria)). She was intubated at 5 days old, and was on Nitric, oxygen and vent. From this time until they figured out what the main issue was, she was fighting for her life everyday. There were times were I would wake up to her being bagged (resuscitated). Her heart rate would spike up to 200 and then drop to 40 within a matter of a minute. This time would be a series of nearly losing her every day. She then was told that she would need to switch from the NG tube to the G Tube. This was her first surgery. After that, she continued having severe ups and downs. She then was told that she would need a trach because her breathing was not improving and they needed a more stable airway. This was her second surgery. A week and half later, this trach was false tracked. The tracheostomy tube can be displaced into a false passage, usually in the pretracheal space. Signs of a displaced tracheostomy tube include difficult ventilation, difficulty passing the suction catheter, subcutaneous emphysema or pneumothorax. This happened to be the ENT's first false trak in 25 years of practice. This alone could have killed her. Luckily, she did not have the more serious complications. This was her third surgery, she needed to have the trach replaced and her trachea was sutured to ensure that the trach did not move. This forever altered her trachea. It is now curved. At three months old, after numerous sleepless and scary nights, she was scheduled for a VQ scan. A VQ scan, also called a Ventilation (V) Perfusion (Q) scan, is made up of two scans that examine air flow and blood flow in your lungs. The first scan measures how well air flows through your lungs. The second scan looks at where the blood flows in your lungs. Prior to our field trip don to the VQ scan, the doctor that was in charge of her that day called the pharmacy and ordered 14 different medications. These meds were rescue meds, pain meds, sedatives and meds to reverse those meds. She, and everyone else knew to not underestimate this tiny little human. The VQ scan showed that the increased blood flow through the remaining pulmonary artery was increasing in size everytime she would get upset. The aorta was enlarging with every whimper and was pushing down and compressing her left bronchus. This was the only bronchus that she had and everytime she would get mad/ upset that bronchus would decrease down to a 2/3mm hole. In other words, the only functioning breathing straw she had was 2mm in diameter because it was being compressed. The doctor came in, sat down on my bed and said, "She needs heart surgery." That was on a Thursday. Friday morning they transferred her to the CVICU and ahd to sedate her that entire weekend with the major sedatives. Vecuronium and Rocuronium, and our baby was not allowed to be awake becuase with every cry it could be her last. Monday morning was her open heart surgery. They went in and did a life saving, unheard of surgery called an aortopexy from a genius doctor. They tacked back her aorta and provided immediate relief from her bronchus and she was able to breathe again. The nect couple months, our only goal was to get home and get back to life. There were many horror stories that many in the CVICU remember, even to this day. On one of our attempts to go home, we got down the hallway and she turned grey, so we had to come back. After three or four admissions and discharges we were kept in the hospital until she was 6 months old. At 6 months old, that same genius doctor said, "Take out the trach, she doesnt need it." Everyone was unsure of this plan, but we went with his lead and on her 6 month birthday she was trach free and that much closer to being a normal kid. From 6 months until 3.5 she ahd various admissions an er visits and probably close to 1000 doctor appointments. At 3.5 years old she began getting a series of Rhinovirus, Pneumonia, and Salmonella. The doctors struggled becuase she was quickly depleting the antibiotics that she could take and she was also getting sicker with every illness. The reason after testing was found to be that pneumonia had gotten into that underdeveloped lung and sat there, so everytime she would get sick, it would compound on top of that already sick piece of lung. This is when they decided that her most recent surgery would be needed. At one of our pre-surgery appointments, the risks were laid out to us that the doctors last case, of a similar case did not survive. We also had a similar situation where during pre-op doctors kept coming in and showing up, more and more and more. We let go of our "healthy" baby girl with nurses and doctors, not knowing if that would be the last time we would see her alive. She fought through this surgery and came out successfully. This is her story. This is her journey. This is her mountain that she is continually climbing with our support and your support.
