CTBBC (Children’s Brain Tumor Tissue Consortium) Q&A with Monica Angel, Co-Founder, Executive Director of the Cure Kids Cancer Now! Foundation

Q: Cure Kids’ Cancer Now! was founded in honor of your daughter, Emmi Grace Angel. Can you tell us a little about her?

Monica Angel (MA): She laughed the most infectious and beautiful belly laugh that sounded as if it should be coming out of a very large old man. She had spectacularly red hair and brilliant blue eyes set on ivory white skin. We were chosen to adopt her only a few weeks before her birth but were lucky enough to be present for the delivery.

How did you come to find out Emmi Grace was suffering from an Atypical Teratoid Rhabdoid Tumor (AT/RT) brain tumor? What warning signs appeared that let you know something was wrong?

Emmi Grace was thought to be a colicky baby for her first three months. She cried quite a bit. She also had what we thought were some digestive issues in her second month. In her third month, she seemed to have some neck strength issues that were dismissed as being from sleep positions, so we were working with her to strengthen the other side of her neck.

When she was 3 ½ months old, she changed quite drastically over the course of a few days. Seemingly, she could not keep anything down- even tiny amounts of water or Pedialyte as prescribed by her doctors. She also lost her smile and did not appear to be tracking me any longer. I took her to the pediatrician in desperation knowing something was not right. Thankfully, he very quickly diagnosed hydrocephalus noting that her fontanelle was not as soft as it should be for her age. He also measured the circumference of her head which had grown exponentially since the last visit. He immediately asked us to head over to our local hospital for admittance. Upon arrival, we learned that she would require a placement of shunt to relieve the fluid buildup but first would need an MRI to ensure she had no abnormalities prior to surgery.

The following morning, we received the news. Her amazing pediatric neurosurgeons entered the room and told us that she had a tumor on her brain stem which needed to be removed immediately. I do not remember much of that conversation. I did ask for a second opinion and was told that we did not have time for that. I asked what it was.

They said it. She likely had cancer. My 3-month-old had cancer. My 3-month-old had brain cancer?!  I heard static.

Trying to recall what came next is like trying to see through a storm. It is unclear. One surgeon handed me a post-it note. It had the names of three kids’ cancers that I had never heard of before. Medulloblastoma. Ependymoma. AT/RT.

What? What are these? I asked what we should be hoping for. They answered- anything but AT/RT.

What did Emmi Grace’s treatment journey look like? How did your family navigate that journey?

She immediately went downstairs to pre-op. We waited. We held her. We sobbed. We prayed. She was taken to surgery. I begged God to let her survive. I bargained. I pleaded. Anything. I will do anything. Take me instead.

She survived. We praised God for this blessing and got to work on determining our next steps.

We sent slides of her tumors to multiple hospitals to confirm her diagnosis. They did not all agree. First, we were told that she had an Ependymoma. Oh no! Wait…  At least it isn’t AT/RT. Ok. Let’s go. We learned as much as we could about Ependymoma as quickly as possible.  I did not sleep for days. A few days later we found out that two of the labs had mistakenly diagnosed Ependymoma. Three others including Dana Farber, Duke, and CHOP all confirmed our worst nightmare… AT/RT.

We trudged forward.  She had a Broviac placed and a GJ tube. We then transferred her care to an ATRT specialist at Arnold Palmer Hospital in Orlando. The transfer was tough. There was the physical toll it took on Emmi, the hassles of insurance approval, the struggle to decide where to take her or if to stay in our hometown, the pain of moving to another city with one child when our other child was to stay at home with my parents. Like I said, it was tough.

We trudged forward. Upon arrival at Arnold Palmer, we learned that her hydrocephalus had returned. She had lost vision. She would likely not make it through the night. They thought that perhaps we should not have come. I did not sleep that night.

The following morning, she had another MRI. No new tumor growth. Phew. They moved forward with placing an Omaya Reservoir to serve as both a shunt and a delivery mechanism for intrathecal chemotherapy. She survived again.

Since the initial resection, she had damage to her vocal cords which cause breathing issues and swallowing issues. She did not eat or drink. She did not cry. We did not hear her voice.

She agreed to a very intensive protocol beginning with systemic chemotherapy. We had no idea what that meant, “intensive protocol”. We only saw it as something that had worked before. We had to try. Maybe we were wrong?

The next few weeks were spent in the PICU or the Special Care unit at Arnold Palmer. She went, once, to the oncology floor but that only lasted about a day and we were transferred back to PICU. We were quite lucky to have some of the most amazing nurses I have ever encountered in my life in those units.

She was able to tolerate the intrathecal chemo well. However, once we began systemic chemo, everything went downhill quickly. Starting with bladder and kidney problems, then liver, then lungs and heart. Within only two months, she went into septic shock.

The adult chemotherapy (her best possible option) was destroying her organs.

Were there any insights that you gained – either good or bad – while going through this illness with your child? (These could include insights about your family, about childhood cancer care, about your medical team, about your community, etc.)

 Our family and friends were tremendous during Emmi Grace’s treatment. We were surrounded by love and kindness.

We all were shocked to learn about how children with cancer are truly treated. That the standard of care is to treat them with adult medicines?  How can this be? We reformulate something as small as Tylenol to avoid toxicity but are regularly pumping adult chemotherapy into our children? Our babies?

We wanted so desperately for it to be someone else’s fault — but it wasn’t. She had a remarkable and loving team of physicians, nurses, and techs. She was treated well. Unfortunately, the tools at their disposal were (and remain) terribly outdated and toxic.

What has Emmi Grace’s legacy been? Can you share more about the work of the Cure Kids Cancer Now! Foundation?

After Emmi Grace’s death, well-meaning people told us all sorts of silly things. One we kept hearing, “things happen for a reason.” What could that reason possibly be?! We could not see it, so we chose to create one. We started a foundation in her memory and honor.

We originally aimed to provide family support to newly diagnosed families to pay forward the love we had received. Over time though, we realized that there were already other spectacular organizations in our area who were already providing these. We wanted to make as big of an impact as we possibly could with the resources that we had. We took an informal poll, “what would you want most?” was asked of parents of children with cancer. “Time” was the resounding answer. But how can we give time?

We worked with our local hospital to develop the “Home for the Holidays” project after learning that there are children who are medically cleared to go home but their insurance denied coverage for their medicines and necessities at home. We now go to the hospital just before Christmas (on Emmi Grace’s birthday) to deliver a check to send these children home for Christmas. We could give them TIME together for Christmas.

In 2018, we also worked with local schools to collect items that the hospital requested for kids with cancer including pillowcases, pajamas, and baby soothers. We could give them TIME not having to go to the store to purchase these items.

In 2019, our focus on research grew quite a bit.  TIME? TIME!  We need these children to survive.  We can give them TIME.

What would you most like to share with families who’ve learned their child has an AT/RT or other type of brain tumor? What would your message be to clinicians, hospitals, or medical scientists?

For other families hearing, “your child has a brain tumor,” for the first time… I am so sorry. This should not have happened. One of Emmi Grace’s oncologists said it best, “When a child gets sick, people get struck stupid.” People will say ridiculous things to you. Most of them mean well but still it can be maddening. Offer grace and let it go. You are in for a hard battle and you do not have time to waste on anyone or anything that is not helping your child. It is not your fault. It wasn’t the breast milk or the formula. It wasn’t that one time you stood too close to the microwave. It wasn’t the Pop-tarts you ate every morning in college. It wasn’t you.

Listen to the physicians. Learn about the condition and possible side effects. Make the best decisions you can with the information available to you. Stave off the desire to pop it into “Google” and instead request valid sources from your medical team.

Let people help. If you struggle with this like we did, remember, it is not for you. It is for your child. Let them be surrounded by love.

Reach out to local organizations that may be able to help. Reach out to your social worker or child life specialist for recommendations.

And love — love your children and yourselves.

Most importantly, you can do this!

Read the full article here…