Celebrating McKenzie

McKenzie Bailey was born Feb. 25, 2008. She was born with neonatal Marfan syndrome, a genetic, connective-tissue disorder affecting the heart and lungs. Neonatal is the most severe form of Marfan syndrome and is considered terminal. Some of McKenzie’s doctors did not think she would make it to her first birthday let alone her second, but she did. She fought hard, always smiled and loved to play with her toys. 

She died March 24, 2010, just one day shy of turning 25 months old. Through her parents, Jennifer and Ashley Bailey, McKenzie touched many people’s lives and taught us many lessons about life, with one of the most important one being to make the very most of every moment.  Jennifer and Ashley knew their time with McKenzie would be short, so instead of having annual birthday parties, they held parties on the 25th of every month, no matter where they were.

Here, McKenzie’s mom, Jennifer Bailey, tells about her daughter’s earliest months…

Happy Birthday, McKenzie
After a three-day (yes, three!) induction, McKenzie arrived on Feb. 25, 2008. After we settled in our room in the mother/baby unit, one doctor commented about McKenzie’s fingers — they were very long and skinny, almost spider-like. 

The doctor said this could be a sign that McKenzie has a genetic disorder and we may want to look into having genetic testing completed. Marfan syndrome and other connective-tissue disorders were mentioned, but nothing concrete. And, of course, that’s not the first thing you want to hear just a day after having your precious baby.

The Long Road to Diagnosis
About 2 months after her birth, McKenzie was seen at Shands pediatric genetics clinic. The genetics doctors and staff looked her over, noting everything from head to toe. They did a skeletal survey — full head-to-toe X-ray — along with lab work to test for genetic disorders. At that point, the geneticist did not think McKenzie had Marfan syndrome. The only true identifier she had were the long skinny fingers. And so, the Marfan lab test was not ordered. Disorders that are caused by a deleted or additional gene were the disorders they tested for, because these do not have to have a specific gene identified.

McKenzie’s skeletal survey showed she had hip dysplasia, which is common among infants, so there was still nothing concrete pointing to Marfan.

In the meantime, we noticed McKenzie’s eyes were a little different. Her pupils seemed to bounce around when she looked at things and blinked. So, our next stop was the eye doctor. We saw a specialty eye doctor at Tallahassee Memorial Hospital. He did not know what to think about her eyes. All he knew is that she did not have nystagmus – a condition that causes the pupils to move involuntarily, usually horizontally, but sometimes they are vertical and/or rotary. McKenzie’s pupils would bounce when she moved her eyes, but then it stopped.

We were referred to a pediatric eye specialist at Emory in Atlanta. When he saw McKenzie, he said would almost bet money that she had Marfan because the lens in her eyes appeared to be dislocated, a Marfan’s trait. He also noted that she was nearsighted and suggested contacts because it was so severe and the degree of nearsightedness was very different in each eye.

Contacts for a 4-month-old?! WHAT?! But we did it. We learned a unique way to place contacts in an infant. So, every morning and every night, McKenzie’s contacts went in and came out.

While we were at Emory, we saw a pediatric orthopedist for McKenzie’s hip. He suggested a hip brace that would push her hip deeper into the socket. He did not think our daughter had Marfan, and he said if he were wrong, he would eat his words. Boy, was he wrong.

So many doctors, and McKenzie was only 4 months old. At this point, we also noticed that her head was flattening on one side and it was not reshaping itself. Add a neurosurgery consultation and a CT scan. Neurology discovered she had mild plagiocephaly (flattening of the skull) and slight craniosynostosis (a condition in which the sutures close too early, causing problems with normal brain and skull growth). We were set to try using a special helmet to try to reshape her skull.

It was also at this appointment that someone mentioned hearing a heart murmur. Of course, we all thought, how could we have been to all these other specialists and appointments and no one heard a murmur until now.

When McKenzie was 6 months old, we were sent to Tallahassee Memorial Hospital for an echocardiogram (an ultrasound of her heart), and then we followed up with a pediatric cardiologist in Tallahassee. The echo report said it was incomplete because the patient was “uncooperative.” How cooperative can a 6-month-old be?! Especially one who was just laying there playing.

The echo was not complete, but it did not appear to show anything; it was assumed that McKenzie must have an atrial septal defect (a hole between the atria) or a ventricular septal defect (a hole between the ventricles). It was also assumed that the whole must be closing and getting smaller, causing the murmur to be present – the smaller the hole, the louder the murmur. We were scheduled for follow-up when McKenzie was a year old – six more months.

But McKenzie never made it to that appointment. In October 2008, when McKenzie was 8 months old, she was being admitted to Shands Children’s Hospital.

Closing in on Answers
We didn’t know Oct. 15, 2008, was the beginning; we simply thought McKenzie was sick, she would get well and we would be home soon. She had been sick the previous few days, running a fever, coughing, throwing up. She lost her appetite, which, for her, wasn’t that unusual. But not eating much turned into not eating anything at all for more than 15 hours. She’d always been a little underweight, so not eating for half-a-day was a BIG deal.

We were admitted to the TMH pediatric unit, where they discovered she had pneumonia. The next day, her breathing had become so labored, we moved to the hospital’s pediatric intensive care unit. The doctors said if her breathing did not improve quickly, they would intubate her. Soon after, she was intubated. Everything was so new to us. We were very, very scared.

Once she was intubated, the doctors decided to do another echocardiogram because of the heart murmur and the suspicion of Marfan. She was sedated during this echo so that they could complete the exam. It showed that her aortic root was enlarged, she had mitral valve prolapse and mitral valve regurgitation – all classic cardiac signs of Marfan syndrome. This was the first diagnosis, but genetic testing would be the only way to identify it for sure.

Getting the Best Care

In those nine days at TMH, my husband Ashley and I had already learned so much. We knew what the ventilator setting were and what some of them meant. We knew when they changed a setting whether it was a good sign or a bad sign.

After a few days at TMH, McKenzie was not improving. Were the doctors at TMH out of ideas or options? We started asking if we should be transferred to another hospital. We knew when we needed to ask to move on.

By Friday, Oct. 24, we were transferred to Shands Children’s Hospital’s pediatric intensive care unit. McKenzie went by the Life Flight ground crew because the rain that night made it to dangerous to fly.

If you are concerned about your child’s care, or you believe your child would be better served somewhere else or by someone else, do not be afraid to ask for another opinion or to be transferred. If we offended someone, sorry, but when we were talking about our McKenzie, we didn’t care who we offended. We wanted the best care possible for her.

That’s a lesson we all learned: Do not be afraid to ask, ask and ask again. Whether it’s your child, another family member, or yourself, you have a right to know and to understand what is going on, and to insist doing what’s best for your loved one.

After many months in the hospital, McKenzie was able to come home with around-the-clock care from her parents, who had learned to do everything they needed to do, and from a home-health nurse. Jennifer plans to write more about McKenzie’s journey, so be sure to check in again.