June 4, 2009 – February 3, 2015
Diagnosed: March 20, 2014
Written by Emma’s family
We noticed early March 2014, that Emma began tilting her head a little more than normal when watching TV. When she would look at us, sometimes her eyes did not seem completely aligned and her head tilt continued. A few years back her brother Anthony developed a lazy eye, so we were convinced she had the same thing that he had and needed glasses. By March 7th I called the eye physician and made the appointment. I asked the teachers if they noticed and no one else did. When the ophthalmologist looked at her eyes he thought that her sixth nerve palsy wasn’t right. He said it could possibly be from the strep throat she just got over but to be safe we needed to take her for an MRI. The MRI was done the week following and there was no alarming need or concern that we worried about.
March 20, 2014
Emma had her MRI in Winter Park, FL at an MRI building. It was spring break and we all went as a family. About an hour after we got home we got a call at 5pm from Emma’s pediatrician giving us the horrible news that as I fell to the floor my entire body went numb and weak. The call started off as “Donna I am sorry to hear about Emma…” those words will always haunt me. “Sorry?”… I was told Emma had a mass in her brain. We were scheduled at Arnold Palmer hospital at 8am the next morning with the pediatric neuro surgeon and pediatric neuro oncologist. Hearing the word oncologist for a 4 year old did not sound right. We were scared. I was numb. That night I stayed up the whole night and watched my world turn into a nightmare. As I researched, I feared from Emma’s symptoms that it was a brain stem glioma (DIPG) but I hoped with everything that it was not that one after searching the different brain cancers.
March 21, 2014
We walked into the office at Arnold Palmer’s Children’s hospital and no neuro surgeon came in. It was the pediatric neuro oncologist and her team members. The kids were taken outside the room by nurses and after the doctor reviewed the MRI she came in. I will never forget the way she stared at us and then she said: how much do you know? The doors closed and our worst fears came true, it was a “diffuse intrinsic pontine glioma.” In her words, there are no survivors in the world and most children succumb to this between 9-12 months. We were given very little hope and our entire worlds collapsed in front of our eyes. Since this type of brainstem tumor would be catastrophic if surgery was attempted, we were offered local treatment that we were told would consist of 6 weeks of radiation which would temporarily shrink the tumor and symptoms to buy us more time. Another option was we could travel to St Judes to try a clinical trial with radiation and a chemo inhibitor drug called “MK1775” which “could” help the radiation work better. We were told we caught it early but unfortunately since this is inoperable it does not change the terminal diagnosis. We were told we had to decide that day. We went home and never looked at our little girl the same way. Her voice, touch and smell was and is so precious and every moment has counted since. We decided to fly out and by Sunday we were at St Judes in Memphis Tennessee.
ST JUDES – Clinical Trial in Memphis, TN
March 23 – 16, 2014
We arrived in Memphis and got settled at the grizzly house which was fully funded by St Judes. The next morning at 8am we checked into the hospital. Emma walked up to the St Jude statue and said “Dear St Jude thank you for helping fix my eyes, Amen”. We hadn’t filled her in from the eye appointment to the meeting at Arnold Palmer enough but started by telling her she had a bo bo in her brain. We could not believe how many children were suffering from cancer. Many of the kids had no hair. Brain tumor kids were in wheelchairs and many of them were swollen all over from the steroids. We felt like there was a big mistake and that we were not meant to be part of what we were in… but we were.. and emotions continued to run high. How? Why? Was it our move to Florida? I was pregnant when we decided to leave Toronto and move to Florida. We had no idea what caused this. Emma was our super healthy active child. She would eat salad and salmon with me for dinner at 3 years old.
Emma got to meet her neuro oncologist there and HOPE was all over the hospital. The love, support and faith from this hospital is what helped us all get through this emotionally and mentally. I don’t think I could have asked for her to be in better hands. This place is unreal. Emma completed her radiation and chemo drug by May 16th and were on our way home after 2 months being away.
May 16, 2014
Our families were helping us research long term survivors of DIPG and a homeopathic protocol from a doctor in India, Dr Benergi, came to light so we started it right away. We also consulted Jeanne Wallace, Phd from Nutritional Solutions and her team of nutritionists to help us with Emma’s plan of supplements and alternative medicines. They refer to her lab reports to help us along the way. Emma takes Aloe Arborescens, vital-zyme, therBiotic, selenium, alkaline water, boswellia, omega, zinc and various herbal supplements. These are all customized to her blood/lab work which are drawn from the hospital and a support planner is created for her. Typically chemotherapy is not known to help DIPG because it tends to not be effective crossing the blood brain barrier.
By the end of May we were in NYC at Sloan Kettering, meeting with a team for the CED trial.
The CED (convection enhanced delivery) clinical trial was on hold because of a new dose level that they were applying for. By July the FDA approved the trial and we were the first ones on it for the new dose level. Emma was approved and scheduled to be the 15th patient on the trial with a one time dose of 2.5 mcg scheduled on August 16th.
August 8 – Sept 10, 2014
We headed to Sloan Kettering in NYC to begin preparations for the convection enhanced delivery (a tiny tube surgically placed into the brainstem) that releases an antibody over 8 hours or more. Emma’s dose level was double and a half the last patient so we were very nervous. Our hopes were that this trial would help shrink the tumor and stabilize it longer. Emma had the procedure on August 16th The MRI showed slight shrinkage and a stable tumor.
September – October 2014
Emma had a follow up stable MRI at Arnold Palmer and her white blood cell counts went back to normal. After 30 days, we were able to start back on homeopathic and herbal medicine.
We headed back to NYC’s Weill Cornell Hospital in Manhattan (which was right across from Sloan Kettering) for trial #3 which was an Intra Arterial Infusion involving 2 chemo drugs avastin and Erbitux. It is injected in through the femoral artery. Emma was first injected with mannitol to disrupt the blood brain barrier. Emma spent a week in NYC during this trial and quickly recovered. The downfall with such a rare brain cancer is that there are not many doctors that know all the clinical trials out there so it is up to the parents to research them all and then ask. This was one that we were able to find out more about from the NY team after doing the CED trial.
Emma had an MRI locally in Orlando. It was the 30 day follow up MRI to the infusion from Weill Cornell. Her MRI read stable with overall improvement of the pons! Emma completed a bucket list of an amazing year and continues to battle this disease with strength and courage. She enjoys life to the fullest and has been blessed by so many amazing people and organizations helping her in this journey.
Emma went in for a follow up scan, and she continued to have little to no symptoms. We were shocked and filled with sadness when the Oncologist at Arnold Palmer came in the door that day and told us the tumor is progressing. At the end of the visit it ended with “I wish there was more I could do.” At a later visit we were given the option of a clinical trial, but it got put on hold for safety reasons and was no longer an option. Other options were chemo’s but we were told chemotherapy did not have a positive track record of helping DIPG. It seemed the risks outweigh any benefits. I knew the clock was ticking, and I spoke to key DIPG researchers and neuro oncologists across the country. The team in NY scheduled us to come back right away.
The teams in NY came together with a solid plan so we flew up to New York and met with Dr Souweidane and Dr Greenfield at Weill Cornell. From there we did a full extensive MRI and after they confirmed the enhancement of her tumor they offered us the option of an open biopsy, Emma has an atypical area of the tumor that extends outside of the pons so this option was less risky and very unique to her tumor as they cannot normally do this type of biopsy for DIPG. We were confident in the team and the ability to do this custom planned procedure for her outside of a clinical trial. Emma was biopsied on March 17th and stayed in the ICU for 2 nights. The initial findings of the biopsy was that we were looking at a grade 2 DIPG and the progression rate was not as aggressive as most DIPGs. The team at Weill Cornell will continue to study which drugs/antibodies will work best for her tumor on a custom drug approach. These researchers are not looking at the one size fits all approach to DIPG as they know it cannot help. Instead they will study many different types of drugs including immunotherapies and antibodies. This approach may also require FDA approval as adult drugs cannot always be used for children.
Emma is continuing to recover well. The incision area has healed better than we thought. We are blown away by her courage and ability to be very brave throughout all these treatments. She does 90 minute MRIs at the age of 5 without sedation, she takes needles without complaints. She wants one thing in life and that is to be a doctor. I think that part of not giving up is having a child that has the courage and strength to feel the same way.
Our next steps are that we plan to start a clinical trial next month (hoping she falls under the exclusion criteria) after that, we move into the custom drug approach to help her fight the progression. We are fully back on the holistic medicine hoping something will help in the
interim. Her next MRI is in May in NY. She is excited to get back to swimming, she is loving school and her recent cruise vacation!
Sloan Kettering Hospital – Re-radiation in New York, NY
We decided that we felt comfortable going back to Sloan Kettering in NYC, to have the experts care for her treatments of re-radiation since it came with high risks. We had no other option. Currently even with all the clinical trials out there nothing but radiation was known to effectively help DIPG, so we moved quickly. Emma enjoyed the city and was excited to be back, but losing many of her functions was also very hard for her. She did get to complete a few wish list items being back in New York. An afternoon at the Plaza Hotel, including time in the Eloise store, her first Yankees game, which she loved and another prom Memorial at Sloan Kettering. Most importantly, she partnered with Books that Heal to work on her first book called “Emma the Brave”. It will be out this summer. She loves the team of doctors there, which made the experience for her better than expected. Emma completed 12 rounds of radiation, with no sedation, which was amazing for a 5 year old.
Emma turned 6 years old on June 4th, 2015. Surrounded by close friends and family, the weekend could not have been more special. Her balance was not the same and cognitive ability was not what it used to be. She takes every day as it comes and fights with all she can. Emma completed Avastin infusions at Florida Hospital every other Monday, to help prevent hydrocephalus and necrosis (water/swelling and dead tissue…good tissue). Radiation has its side effects, especially twice, but we were not ready to lose her. We will never be, so the balance between quality of life and love has us on a teeter totter.
Emma visited her family in Canada to celebrate Canada day and enjoy her baby cousin’s baptism. We finally got the full genome sequencing report back from Weill Cornell in NYC, to start the drug plan that can possibly prolong her life. In constantly speaking with doctors all over the world, there is still no cure and it is harder to find hope. With help from a DIPG researcher at Duke University and her oncologist at Florida Hospital, we hope that she will be able to finally start the drugs soon. They are off label and not FDA approved for DIPG, so it may be challenging. Her symptoms sadly are progressing, loss of ability to walk, slurred speech, swallowing issues, loss of hearing on left side…. we can only pray for a miracle.
Emma made her First Holy Communion and discovered a new favorite restaurant called The Melting Pot, on Mommy’s birthday. All while continuing to battle symptoms of a tumor that is overcoming her body. Emma started two targeted drugs, “dasatinib” and “Everolimus”. These are considered “off label drugs” since they are not approved for DIPG. They have had recent positive results from a medical publication study. Also a clinical trial in Europe is testing them at higher doses than here in the U.S. We took the recommendation from a DIPG researcher at Duke and the dosing from a researcher in France, under his recommendation. As long as her lab work is cleared every week, the doses can be increased. Sadly, Emma has completely lost her ability to walk this month and her left side from head to toe has also become weaker. Emma’s book was also launched this month “Emma the Brave” and is available on Amazon. Emma was very sad to not be able to start grade 1 at her school, Annunciation. But we were able to set up a book signing there, so she could sign/stamp her books for the students.
Emma was able to continue her targeted drugs at increased doses and her MRI this month had shown that the tumor was “stable” in comparison to July 2015. This was positive news for us, but at the same time this has been the most heartbreaking month since diagnosis. She has lost a lot of her speech abilities, it has become slurred and it is harder to understand. Her right eye does not blink, so she needs eye drops. She cannot walk or stand and drools often. Drinking is also a challenge, so her water gets thickened to help her. Her swallowing and hearing improved near the end of the month, which we were grateful for any improvement in the midst of progression. Her coloring is not the same, because her right hand is weak. Emma loves sea shells, so we went on a trip to Captiva Island, FL. She loved doing arts and crafts with all the seashells she collected. Visits to Crayola have also helped keep her creativity up. Emma also got to watch Disney on Ice and go back to Disney for another visit.
Emma’s symptoms are still very real and hard for her every day. To help her during tough day’s, we have taken her to American Girl, Disney’s Epcot Food and Wine Festival and the Pumpkin Patch. She continues to take the two targeted chemo drugs, in efforts to slow down the growth of the tumor that is taking over her body.
In November we had to increase Emma’s steroids, as her head control and speech worsened. Many day trips to Disney were taken and we were given a wish trip by the Kira Spedale Foundation to stay at Disney overnight for a few days. The whole trip brought so much joy to her life. We were also granted another wish in November, for a private Cinderella horse and carriage ride. We are blessed to have continuous wishes and dreams filled, as we enter the difficult stage of DIPG.
December brought on tougher decisions for us, as we started hospice and Emma’s breathing began to get heavier and worsen. Her speech, inability to walk and weakness on both hands have had her spirits down, but Christmas brought the magic back, especially with her new iPhone, polaroid camera, electronic pet kitty and love from all her family.
January has brought on many scares as Emma’s health has completely declined, now she is unable to swallow well and her breathing has become more of a challenge. Hospice is helping with keeping her comfortable, using an oxygen cpap machine. As parents, we watch with fear… of the future. This month Emma was able to meet her new baby cousin James from Canada, who she absolutely loved to hold.
Our beautiful daughter Emma Marie Marques, went to be with God at 12:55am. Our hearts are forever broken. Please keep our family close in prayer, as we grieve the worst loss – our beautiful princess.