Getting the Diagnosis

Once Matt's parents began to notice the strange symptoms of balance/coordination loss and mood changes, they took him in to see a doctor where he received a scan of his brain. Typically, if after a MRI or CT scan there is a tumor noted in the lower back portion of the brain, a biopsy will be done to determine what type of tumor it may be. There are three different ways this biopsy can be done:

1) surgery: the patient will have an operation called a craniotomy in which all or most of the tumor will be removed and then analyzed to determine what type of tumor it is
2) sterotactic (needle) biopsy: a hollow needle is placed into the tumor to remove a sample of tissue from the tumor; this technique is usually used if the tumor is difficult to reach
3) lumbar puncture: a small amount of cerebrospinal fluid (the fluid around the brain and spine) is removed with a needle and then analyzed

In Matt's case, he underwent surgery to have most of the tumor removed and then it was determined that the tumor was a medulloblastoma.

Once the sample of medulloblastoma is obtained, it will be diagnosed as either standard-risk medulloblastoma or high-risk medulloblastoma. The criteria for both are:

standard-risk: less than 1.5cm of tumor is left after surgery, cancer has not spread anywhere else, the patient is between the ages of 3 and 21
high-risk: more than 1.5cm of the tumor is left after surgery, the cancer has spread to other parts of the body, the patient is between the ages of 3 and 21

From this point, the tumor can be placed into one of several different subtype categories, which can help determine the type of treatment that will be best.

Receiving the diagnosis of a medulloblastoma can tip a person's world upside down. The impact does not only affect the patient, but also the patient's family. It is important that the family and the patient receive a tremendous amount of support, both from the medical team and from their friends and family. Luckily, Matt received this support and was able to begin his journey on The Road to Awesome. It is important to remember that the person is so much more than their diagnosis. Amidst all of the different tests, procedures, and treatments, it is easy to only see that person as their disease. Maintaining a sense of normalcy and continuing to partake in as many activities of daily life as possible can have a huge impact on the person's ability to cope with and manage the disease. Matt's family seemed to hold this idea very close to heart during the course of Matt's diagnosis and treatment. They strove to never stop having fun and continued to view Matt as "Matt" instead of "Matt with Medullblastoma".

References:

http://www.mdanderson.org/patient-and-cancer-information/cancer-information/cancer-types/medulloblastoma/diagnosis/index.html

http://www.cancer.net/cancer-types/medulloblastoma-childhood/stages

The Twists and Turns on The Road to Awesome

Over a period of a few weeks, Matt began to experience symptoms of balance issues, mood changes, and weight loss. Because he was always so active and constantly jumping around from one activity to the next, the balance changes were obvious to both him and his family. The mood changes stood out as well since they were such a change from his usual constant state of being outgoing and fun-loving. After it was determined that he had a brain tumor of some sort, surgery was scheduled. The turn-around time was fast - he was diagnosed on April 1 and was in surgery by April 8. After a very long 6-10 hour surgery, they were able to determine the type of tumor and Matt was officially diagnosed with a maligntant medulloblastoma. After the surgery, there was a long recovery time in the hospital. Matt's strong spirit was evident immediately after the surgery. Just two days after, the breathing tube was removed and he said his first word post-surgery: "Matt". The next day he said two more words: "Hi, mom." Three days after surgery, he stood up outside of the bed. The next day, he walked 10 steps. Despite the fact that the hospital staff was in amazement at how quickly he was healing post-surgery, it remained difficult for Matt and his entire family. Just 2 weeks earlier, he had been skiing black diamond runs. And now he was being cheered on to take 10 steps next to a hospital bed. 11 days post-op, he moved out of the ICU and laughed for the first time. 

Once he was fully recovered from surgery, about a month later, he began the long process of chemotherapy and radiation. This involved the entire family picking up and moving to Boston for a little bit. A hospital in Boston was able to offer a unique form of radiation that his hometown of Boise wasn't able to offer. After completing his radiation treatments in Boston, he came back to Boise for his chemotherapy. Throughout the entire process, his entire family was right by his side.  He has continued to slowly make progress and recover from all of the radiation and chemotherapy. It has involved multiple physical therapists, occupational therapists, doctors, nurses, friends, and family to all be there supporting him. 

Matt with his parents

Here he is with his little sister, Maggie, who shaved her head for him.

Matt and big brother Josh 

Mama's Boy

It is still unclear exactly how medulloblastoma arises. Some say that it comes from multiple different cells of origin. Other studies have indicated that it arises from a problem in both the Wnt/beta-catenin and the sonic hedgehog (Shh) signaling pathways. When these pathways are inhibited, the tumor is able to begin growing and spreading. It has been hypothesized that sonic hedgehog signaling pathway problems account for 25-30% of all cases of medulloblastoma. As the tumor begins to grow, cerebrospinal fluid is prevented from getting to the fourth ventricle of the brain, which can cause internal swelling of the brain (hydrocephaly). Then, the tumor can begin to spread. It is even capable of spreading through the cerebrospinal fluid down to the brain stem. Medulloblastoma is a unique pediatric brain tumor because it able to spread more quickly than most other tumors. 

References: 

http://reference.medscape.com/article/987886-overview#a0104

http://www.abta.org/brain-tumor-information/types-of-tumors/medulloblastoma.html

Zinke, J., Schneider, F., Harter, P., Thom, S., Ziegler, N., Toftgard, R., . . . Liebner, S. (2015). β-Catenin-Gli1 interaction regulates proliferation and tumor growth in medulloblastoma. Mollecular Cancer, 14(17), 1-9.

How common is medulloblastoma??

Currently, it is estimated that around 500 children are diagnosed with medulloblastoma annually in the United States. It is the most common malignant brain tumor in childhood, making up about 20% of all tumors of the central nervous system in children under the age of 19. It very rarely occurs after a person is in their 40s (70% of all cases are diagnosed before the age of 10). The highest rates of this tumor occur in children between the ages of 5 and 19. This type of tumor exclusively occurs in the cerebellum. In 2-5% of cases, there are some genetic mutations at play that increase the risk of a child developing medulloblastoma. It has also been found to be more common in males than in females.

One study, from 1984, analyzed epidemiological trends in medulloblastoma cases in children. This study looked at 143 children ages 19 and under with a diagnosis of medulloblastoma. They found the average age of diagnosis in these children was 6.5years-old. All of the patients in study received a variety of treatments, with the majority receiving some form of both surgery and irradiation. They analyzed the probably of survival in the patients they were studying and found: 0.687 probability within 6 months, 0.444 probability within 1 year, and 0.314 probability within 2 years. It is important to remember that this study is more than 30 years old and, therefore, the numbers may not be as accurate as more recent studies. However, this large epidemiological study did help pave the way for others to begin to understand the rates and prognosis of medulloblastoma in children.

A more recent study from 2015 analyzed the incidences of a variety of brain cancers in children under the age of 2. This study looked at a group of 32 patients and found that medulloblastoma tumors made up 18.8% of the tumors (6 out of the 32 patients). Interestingly, they found that, for any brain tumor, those children who treated it more aggressively with surgical tumor resection had an average survival time post-surgery of 67.6 months. On the other hand, patients who were more conservative with their treatment and using medications as their main form of treatment had an average survival time of 25.3 months after starting the medication regimen. An even more conservative form of treatment was undergone by some members of this study. This involved some medication and ventriculoperitoneal shunting to help reduce intracranial pressure. This group had a mean survival time of 10.5 months after beginning their treatment plan.

When reading large studies like these, it is very easy to get overwhelmed and lost in all of the numbers and scientific terms. As I continued to scroll through multiple studies, with multiple numbers flitting past my eyes for how frequently this type of tumor occurs and how long most children survive, it was very difficult to maintain a positive attitude. While it is understandable to want to understand and know everything you possibly can about the diagnosis, I think it is very important to remember that every human life is worth so much more than a number in a study. Take all of these numbers with a grain of salt and recognize that every single patient will have a unique experience and outcome. There aren't a lot of rules on The Road to Awesome, but one of the most important rules it to always, always, always, try to stay positive.

References:

http://www.uptodate.com/contents/clinical-presentation-diagnosis-and-risk-stratification-of-medulloblastoma

http://www.abta.org/brain-tumor-information/types-of-tumors/medulloblastoma.html

Yu, J., Shi, W., Zhao, R., Gao, X., & Li, H. (2015). Epidemiology of brain tumors in children aged two and under: A 10-year single-institute study. Oncology Letters, 9, 1651-1656.

Farwell, J., Dohrmann, G., & Flannery, J. (1984). Medulloblastoma in childhood: An epidemiological study. Journal of Neurosurgery, 657-664.

April Fools?

On April 1, 2013, a 9-year-old boy named Matt was diagnosed with a brain tumor. It was a twist of fate neatly wrapped up into an April Fools joke gone bad that only something as powerful as the universe could deliver. Matt is a blonde-haired, blue-eyed little boy with a voracious appetite for adventure. Physical limits were not something he adhered to; you never knew what kind of crazy athletic feat he would be tackling next. He was wakeboarding by the age of 4 and skiing black diamonds by the age of 5. His relentless optimism and love for life were contagious. It didn't matter if you had spent 5 minutes with him or 5 years with him - he was going to make a huge impact on your life.

Because of his infectious personality, this diagnosis came like a blow to every single person who had ever encountered Matt.  The news sat like a giant rock in the middle of my gut as I struggled to comprehend how this could happen. It's never easy to see a child get sick; it's never easy to see anyone get sick. You ask yourself why this had to happen and why it had to happen to them. You become upset with the world for letting this happen to such an innocent, happy young soul. You cry, you curse, and you come face-to-face with the brevity of your own life.

Matt quickly gained an immense amount of support and love from his family and friends. A Caring Bridge website was set up for him, which you can access here. The first step in his journey was to have surgery to determine the exact type of tumor. After this surgery, he was informed that he had medulloblastoma. In this blog, I will be explaining this diagnosis, the treatment options, the prognosis, and the inner workings of this type of brain tumor. Using Matt's brave story, I hope to shed light on medulloblastoma. Matt paved his way down The Road to Awesome, battling medulloblastoma and inspiring others to join him on this Road.

Medulloblastoma is a tumor that arises in the cerebellum of the brain. The cerebellum is located at the lower back portion of the brain. It is a relatively rare type of tumor and accounts 18% of all pediatric brain tumors. They are usually found early in childhood, with 70% of all cases being diagnosed before the age of 10. It very rarely occurs in adulthood. Some of the most common symptoms noticed are changes in behavior, appetite, and unusual eye movements. Because the tumor takes up space in the brain, pressure inside the brain can increase which can cause symptoms such as headache, nausea, vomiting, drowsiness, and coordination problems.

Receiving a diagnosis of medulloblastoma is an extremely terrifying experience for both the patient and their loved ones. However, it is important to remember that treatment options exist for this and that they are relatively successful. The American Brain Tumor Association states that 60-65% of pediatric patients achieve long-term disease control. The most important thing you can do is stay positive and ask your healthcare provider about any and all questions you may have. The Road to Awesome is a road filled with many ups and downs, but with a positive attitude and support from loved ones a happy outcome can be achieved.

References:

http://www.abta.org/brain-tumor-information/types-of-tumors/medulloblastoma.html