November and Thanksgiving are a time when we reflect on things for which we’re thankful. For Mara Norton, a senior at Orono High School, the feeling of gratitude reaches into a completely different hemisphere.
I asked Mara at what moment she knew things weren’t quite right. “When I was five years old I was in gymnastics. I remember my coaches getting frustrated with me because I couldn’t put my arms over my head. My coach said to my mom, ‘There’s something wrong with Mara’s shoulders, we don’t know what it is, but you should take her to a pediatrician.’”
Mara’s mom, Tina, explained, “When I took her in, they couldn’t see what was wrong from her x-rays, so we were referred to an orthopedic specialist at Gillette Children’s Hospital. When the specialist saw Mara’s hands, that was her first clue ~ her hands appeared somewhat clawed. Because her soft tissue and joints were also affected, the initial thought was she had an extremely rare disease called Hurlers mucopolysaccharides (MPS-I). Back in 2006 Mara was only the 36th person to have been considered to have MPS-I. It’s a recessive genetic disease that takes over your body: it affects your brain, heart, eyes, organs, eventually putting people in a wheelchair. We were devastated. Later the diagnosis changed to MPS-VI, which was still bad, but at least does not affect the brain and can be better managed.
“Mara was born without an enzyme that helps your body function normally. An enzyme replacement therapy was recommended where once a week for life, Mara would go in for an IV treatment and have an artificial enzyme placed in her body. On her first surgery, a port was placed and every Thursday after that she received the infusion treatment at the University of Minnesota’s Masonic Cancer Center.”
This was the beginning to a life that exceeds challenges most could handle. This brave young girl would endure over 80 surgeries, consisting of, but not limited to, a VP shunt placed in her brain, two bone marrow transplants, a kidney transplant and excruciatingly painful months upon months living in the intensive care unit at the University of Minnesota Hospital. Mara coded four times ~ but came back to tell her story. It is apparent she has more to accomplish in her lifetime.
Back to the start of her journey in 2006. “I was super, super allergic to it (the enzyme),” Mara said. “I would get really high blood pressure, hives and I was sick to my stomach. They pre-medicated me with Benadryl and steroids, which we weren’t crazy about because my body was pumped with all those meds, but it did help alleviate the symptoms. I missed a lot of school because after the weekly Thursday treatment, Fridays I was too sick to attend school, I needed to recover.”
Tina continued, “After 1 ½ years of weekly treatments at the U, we ended up talking with Dr. Orchard, our pediatric bone marrow transplant doctor for metabolic diseases. Mara was the first case of enzyme replacement therapy failure, she was chronically getting sick because of her allergic reactions and weakened immune system. Before Mara, prior to the enzyme replacement therapy, the only available treatment was bone marrow transplant. Dr. Orchard asked us to consider it, and it was a hard decision because there are tremendous risks ~ some children have died from complications from the process of bone marrow transplants.
“In 2008 Mara had her first bone marrow transplant surgery. It was an intense process ~ we tried it because the alternative was to watch the disease progress, eventually putting her in a wheelchair and possibly losing her vision. It began with a lot of chemo and radiation prior to the transplant surgery to wipe out all the bone marrow from Mara’s body. That process made her very, very sick.” The bone marrow came from an umbilical cord, with the transplant ultimately failing, and from 2008-2009 Mara couldn’t leave the hospital due to multiple complications. In Mara’s Caring Bridge, Tina outlines almost daily the various symptoms Mara’s body developed and the multitude of ways the doctors and medical staff valiantly tried to help her. The children who were at the University of Minnesota’s Children’s Hospital on Mara’s floor were all battling serious metabolic diseases, blood disorders and cancers. Tina’s entries in the Caring Bridge often referred to hearing through the walls the heart wrenching grieving of parents who had just lost their child. “It was like a war zone. Out of the 14 patients on Mara’s floor, Mara and another girl were the only two to live. We felt lucky to have brought her home to let her body heal, albeit still with MPS-VI.
“A year later we met with the genetic doctor who informed us the options were to try the enzyme replacement therapy or try another bone marrow transplant. We moved forward with her recommendation to put her in the ICU once a week for 24 hours where Mara was heavily pre-medicated as the enzyme slowly dripped into her body.” This treatment started November 7, 2011.
“We gave it a fair shot once a week for eight months, but Mara ended up really really sick. She ended up in the intensive care unit for two months, and over Christmas.” Day after day, new crisis cropped up with Mara in ICU. The medical team continuously attempted to alleviate Mara’s pain to allow her body to heal, but new issues arose seemingly daily.
An excerpt from Mara’s CaringBridge, written by Tina:
I am going to make this short, as I am emotionally, mentally, and physically drained.
Mara went down to surgery at 8:30 am and came back up to the PICU at 3:15 today. Long day for her. Neurosurgery got her shunt back in her head and tunneled down to her chest. Her doctor said things went well with the placement, however she is not going to be happy for the next 48 hours due to chest pain. (He had to cut into the muscle between the ribs to get the line in.) Next general surgeon came in and placed her new port in her chest. He managed to use one old incision, but needed to make two more to get everything in place. Lots of other details to go with this….but just can’t put it all down now.
Mara has been in HORRIBLE pain. They now have added different meds and increased some to help her through these next few days. Trying to keep her sedated and calm, as vomiting and crying strains those chest muscles, and her belly that is healing and spirals lots of pain.
What started as a routine enzyme replacement treatment December 6 turned into a long, painful ordeal with admittance into Children’s Minnesota Hospital that wouldn’t end until February 5, 2012 when she went home, albeit fragile and weakened by the toll placed on her body. “After that enzyme treatment, I decided I didn’t want to go through that again,” Mara softly interjected. “I could have let my disease go, but I didn’t want to end up in a wheelchair or blind, I wanted to do something with my life. I decided I wanted to try a second bone marrow transplant.” With the wisdom of Dr. Orchard plus advances in bone marrow transplants, a year later Mara and her parents decided to move forward with a BMT, this time donated by a 46 year old male who was a very close match.
The second bone marrow transplant worked, but after many many more complications, for which Mara paid a price. After 60 days in the hospital, she was home for only a few hours before Mara’s body rebelled.
An excerpt from Mara’s CaringBridge, written by Tina:
Definitely not the homecoming I was expecting for Mara, that is for sure.
We got home around 5:30 pm. We got Mara comfortable in her newly decorated room, monitors hooked up so she could call to me while I was in the kitchen starting to get her bags of medications laid out and organized.…….Mara and I settled into her bed for the night, I set my phone alarm for midnight to check her blood sugar and give an insulin shot. At 11:30 Mara was crying her head was really hurting so I gave her a dose of her pain medication, we checked her blood sugar and gave her a unit of insulin. We layed back down and the next thing I woke up to Mara sitting up and saying, “I think I just had a stroke, mom.” I sat up immediately and rubbed her back and said, “Its ok, I think you were dreaming. Try and relax and let’s get some rest.” I knew we would need to be up at 4 for another blood sugar check and insulin shot so I wanted her to get as much rest as she could. She didn’t want to lay back down, expressing she felt very funny…..Mara then started looking off to the side and saying she felt really funny and was scared. I was trying to figure out what was going on and asking her who I was she knew I was mom, I then asked her to hand me her blanket and she couldn’t. At that point I knew something was seriously wrong and yelled for Christian to get me the doctor’s phone number off the kitchen counter immediately. I no more than came back from the door….Mara was seizing in the bed. Catherine came running in to help roll Mara on her side for me while I called 911 and paged Dr. Mitchell. The doctor called me back and we talked through me running down stairs to draw up Valium to get in her J tube right away, keep her on her side to prevent her from choking which Catherine was helping me do. Mara was turning blue and continued seizing for 8 minutes (which felt like a lifetime, let me tell you all). Christian got Landin and the dog next door to Lisa and Jeremiah’s during all of this. Paramedics got to our house, I finished talking with Dr. Mitchell and handed the phone off to one of the paramedics so the doctor could briefly fill him in on Mara. She was slowly coming out of the big seizure but was continuing to have small ones and difficulty breathing. Dr. Mitchell finished talking with the paramedic, they got her ready for the ambulance, I talked to Dr. Mitchell one last time and he said he would be meeting us in the emergency room. Off I went with Mara in the ambulance…..
As you can see in the most condensed version given, it has been scary and awful for our gracie girl. It saddens me beyond words at what she continues to go through….I just don’t understand it.
Today, four years later, Mara agreed it was terrifying. “I will not lie. But it all (treatments and surgeries) was worth it,” Mara explained. “With what choices I had, I would try what we did all over again. Since the kidney and second bone marrow transplants, I still need minor surgeries here and there, but I can deal with that compared to how sick I was with the enzyme treatments.”
Tina’s journal entries on the CaringBridge site read like a book, outlining just how long, agonizing and confusing Mara’s journey has been. Through it all, Tina talks of gratefulness and the underlying word that has carried them through: HOPE.
Mara is in her senior year at Orono High School and has big plans for her bright future. “After graduation I will start with two years at Normandale Community College, and from there I’d like to attend St. Catherine’s in St. Paul. They have a great nursing program ~ I’ve had the best care through all of this and would love to give back.”
Not many people survive the trauma that Mara Norton has experienced with such grace and positive outlook. “There’s a lot I want to do!” Mara emphatically states. Mara knows how to work through challenges to get to an end result. She understands things don’t necessarily come easy, but she knows how to get there with that gutsy attitude, love and hope.
Mara vehemently states that without the phenomenal medical team at the University of Minnesota, she wouldn’t have this opportunity to pursue her dreams. “I am extremely grateful for all my doctors have done and continue to do for me.”