Facing the unknown with PBD

The story of Makenzie

I want to share the story of our beautiful daughter, Fynlee. I hope to share what life is like when faced with a disease you or the doctors don’t know much about.

My name is Makenzie, I am 23 years old and my husband, Christian, is 27. We welcomed our first born into this world in 2013 when we were only 17 and 21. A beautiful, healthy baby boy. We named him Kash. He grew on time and developed beautifully. We had no doubt he was going to be a healthy child. Through this whole process he has matured and understands his little sister needs more attention than he does. He is a rockstar who helps me tremendously every single day. He’s my little man and his little sister thinks the world of him.

In December of 2015, we found out I was pregnant with our second child and SHE would be coming in July. I had a rough pregnancy with her. I was very sick and had a constant, very high heart rate. The doctor thought it would be good to induce me at 39 weeks. 

On July 27, 2015, we welcomed a beautiful baby girl into this world. Her name is Fynlee. She developed beautifully every single month, hitting every milestone as she should. After her 1st birthday she started to walk. She was very clumsy and it was very unsettling to me. I mentioned this to my husband almost every single day. I decided to take her to her pediatrician for him to tell me, “she’s fine, she will start walking just fine as she grows and gets more control.” It just didn’t feel right.

I am a stay at home Mommy so I studied her walking every single day, all day, for the next couple of months. I decided to take her to my family doctor. He checked her out and made her walk and immediately set her up with a neurologist. We went to see the neurologist close to her 2nd birthday. 

She had an MRI in August of 2016. The MRI confirmed she did have brain damage and it was probably cerebral palsy that the doctors had missed. In the meantime, we waited to see a geneticist and her walking started to get worse and worse. Her right foot started to turn inward and I had to deal with no knowing what was wrong with my beautiful baby girl. 

We eventually got an appointment with her geneticist who ran multiple tests. Before we left her office she expressed to us she thought Fynlee had a disease known as metacromatic leukodystrophy (MLD for short). She didn’t tell me much other than that so I immediately googled this after leaving her office and as I was reading the prognosis, my whole body went numb. It read that my daughter wouldn’t make it past 5 years of age, if she were lucky. I felt like I was in a terrible nightmare. My days were full of tears and anxiety as I had to wait on the results to come back. No parent should have to endure anything like this. 

On my 22nd birthday, November 14th, 2017, I got a call from her geneticist that her blood and urine came back with a clear indication that MLD was Fynlee’s diagnosis. I fell to my knees and cried until I was sick. My husband held me and we both cried together as we were thinking of what we were about to face with our two year old angel. She would lose her sight, all of her mobile function, would have to be tube fed and her body would shut down soon after.

A week passed and we got a call from a stem cell doctor from Duke University. He wanted to bring us down to meet Fynlee but first he would like a second test done as the symptoms Fynlee had didn’t match anything he had ever experienced with MLD. By this time, Fynlee couldn’t walk at all. She had lost all ability to use her precious legs. He ordered blood and urine at our local hospital and had it sent to him immediately. We waited 24 hours. 24 hours of our precious community praying for us as we begged God to take this from our daughter. The next day we waited for him to call back and tell us there were no traces of this disease in her blood or urine. God showed us mercy and performed a true miracle for our precious daughter. 

Shortly after, her geneticist called and expressed that Fynlee’s blood work for her long chain fatty acid level wasn’t right. This soon began the talk of PBD. They retested her long chain fatty acid levels and they came back abnormal. She suggested that my husband and I get genetically tested. It came back positive for a mutation on PEX 16 in both our blood work as well as our daughter’s. We then received the diagnosis of PBD, even though it is not clinical. 

My precious daughter can’t walk and her orthopedist thinks surgery is the next option. Even though she struggles daily with this disease, it does not define who she is. She is a beautiful, bright light who shines on every single person she meets. She is a rare warrior and even though I don’t know what the future holds for my daughter, I pray one day there can be more done to find a cure for this disease as no child of family should have to spend life battling it or having to face the unknown. 

I hope this brought some understanding to you as it’s all I can do. Share my beautiful daughter’s story and never lose hope in finding a cure for my daughter and all the other daughters and sons who are faced with this disease.