The Journey Continues

Throughout this blog, I have tried to put into words some of the experiences that Matt has gone through on The Road to Awesome, both medical and emotional. While the medical parts of this journey can be easily explained with words and online sources, I have found it difficult to adequately put into words the emotional journey that Matt, his family, and his friends have been on.

On April 1, 2015, 2 years after Matt received the diagnosis, his Dad wrote this on his Caring Bridge site:

Yes, it was 2 years ago today that we learned about Matty's tumor.  It's a weird day.  I can't decide whether it's just a yearly reminder of the worst day of our lives, or a day to celebrate another big milestone on The Road to Awesome.  So I'm choosing the latter.  As my good friend, Scott (aka "Huerdy", as the Champ refers to him) says, it was the worst day - but now it can be the best, cancer free and moving forward.  And though it remains slow, Matt continues to move forward.  In fact, he moves forward a lot.  Around the house, he's always on the go - walking around the backyard, going upstairs, or downstairs, or out to the garage - I think in part just because he can.  He's made incredible progress in the past year - ditching the crutches, mostly ditching his walker (except at school, when he's reminded - right, Mrs. B?), skiing fast enough to break a collar bone - but some days, progress is just not fast enough for a kid who loves to go fast, and that really frustrates him.  "Dad, I just want to run" he told me just the other day.  I know you do, Champ.  We all want you to run.  And when I watch the video I found on my phone last week that Matt made of himself jumping up, charging across the room, and launching himself into a flying tackle of a giant stuffed panda bear (thanks, Niles), I think to myself he's getting closer, each day, slowly but surely.  I also think to myself that flying tackles of giant panda bears are maybe not the smartest move for a kid with a recently broken collar bone.  But he led with the non-broken shoulder, and if you heard the laugh and saw the smile afterward (on the kid, not the panda), you'd agree it was worth it.

His Dad has done a truly magnificent job chronicling the journey on The Road to Awesome and it has been an absolute honor and a joy to be a part of his journey. On January 22, 2015, Matt had a clear MRI. Shortly before this, he broke his collar bone while skiing, which I see as proof that his vivacious spirt and love for life will never cease.

I wish you all the best on your continued journey down The Road to Awesome, Matt!!

Nursing Diagnoses - What are they and what ones should I consider?

Nursing diagnoses are a unique kind of diagnosis in the medical world and should not be confused with medical diagnoses. NANDA (the North American Nursing Diagnosis Association) does an excellent job explaining what a nursing diagnosis is and what it is is not. A nursing diagnosis should not focus primarily on the patient's medical condition (because it is not a medical diagnosis) and it should not simply be a way to reword the medical diagnosis. A nursing diagnosis should not act as a specific label for a patient in an attempt to verbalize what you are seeing in the patient.

NANDA defines a nursing diagnosis as, "A clinical judgment about individual, family, or community experiences/responses to actual or potential health problems/life processes. A nursing diagnosis provides the basis for selection of nursing interventions to achieve outcomes for which the nurse has accountability."

A nursing diagnosis should always be formulated after a comprehensive head-to-toe nursing assessment. When a nursing diagnosis is based off of a comprehensive assessment, more effective and safer patient care can occur which often leads to a improved patient outcome.

Below are some nursing diagnoses that commonly arise when working with a pediatric patient with medulloblastoma. It is important to remember that not all of these nursing diagnoses may apply and that a nurse should thoroughly assess the patient and their family members to determine the appropriate nursing diagnoses.

Nursing Diagnosis	Related to	Plan/Outcome	Interventions
Disturbed body image	Hair loss and other body changes	Short term: Patients express a change in body image Long term: Patient reports having an positive self-image	Facilitate therapeutic communication with the patient, educate patient on what kind of body changes to expect, education caregivers on coping strategies to discuss with their child, observe the patient in a social interaction
Impaired skin integrity	Chemotherapy and radiation	Short term: skin remains intact, no signs of redness/breakdown Long term: patient’s skin integrity is maintained	Assess skin integrity every 4 hours, keep skin clean and dry, turn the patient every 2 hours, encourage the patient to consume adequate amounts of liquids and nutrients
Acute pain	Side effects of treatment	Short term: Patient reports no discomfort (no visible grimace/cry if not able to self-report), vital signs are stable, patient is able to participate in activities Long term: pain management remains consistent with no peaks/troughs in pain levels	Assess characterisitics of pain (OLD CART = onset, location, duration, characterisitics, alleviating/aggravating factors, any radiating pain, treatment options), try to establish a set schedule for administering pain medications, provide comfort to patient (distraction, repositioning, etc.), anticipate pain with activity and pre-medicate patient before activity
Risk for fluid volume deficit	Chemotherapy and radiation	Short term: Intake and output is balanced, skin turgor is present and mucous membranes are intact/moist, electrolytes, Hb, HCT, and vital signs are all within normal range Long term: Patient understands risks associated with fluid volume deficit and maintains hydration status.	Continually assess: skin turgor, mucous membranes, patient’s thirst level, blood pressure, HR, serum electrolytes, albumin, and CBC Monitor intake and output, insure adequate intake of fluids (checking IV pumps, etc.),
Anxiety	Unknown future	Short term: patient will express willingness to discuss anxieties, patient will develop coping strategies to manage anxiety Long term: patient will report a reduction in overall anxiety	Suggest relaxation/distraction techniques to reduce anxiety, maintain a relationship of therapeutic communication with the patient, encourage the patient to verbalize his/her anxieties as they arise, allow the patient time alone to rest

Some other nursing diagnoses not mentioned above but that can be important to consider include: risk for infection, risk for caregiver role strain, risk for ineffective coping, and readiness for enhanced learning.


References:

http://www.nanda.org/What-is-Nursing-Diagnosis-And-Why-Should-I-Care_b_2.html

http://nandanursing.com/nursing-care-plan-for-brain-tumor-intracranial-tumor.
html

http://wps.prenhall.com/wps/media/objects/737/755395/brain_tumor.pdf

Nursing Care for Medulloblastoma

Nurses have a vital role in the care of pediatric cancer patients. They are able to assess the coping mechanisms of not only the patient, but also of their family and loved ones. Based on the coping mechanisms observed, the nurse can provide emotional support and encourage the family to find coping strategies that work for them. The nurse should also encourage loved ones to continue to provide self-care for themselves. Often, parents of a child with some form of cancer will neglect all self-care for themselves and fully invest themselves in the care of their child. This is not sustainable and can lead to a lot of parent burn-out. Therefore, parents and caregivers should be encouraged to actively make their own self-care a priority. Nurses can foster this through small things such as offering to sit in the patient's room while they go get coffee and go on a walk.

Nurses are also responsible for all medication administration to these patients. Often, drugs used for chemotherapy can be very harmful to the veins and can cause a lot of pain when they are being administered. It is the nurse's responsibility to put interventions in place that can either help reduce this pain or distract the child from the pain so that their medications can be appropriately delivered. This can often involve recruiting help from loved ones at the bedside who may be able to provide input into techniques that have worked in the past.

The specific nursing care for a child with a brain tumor will depend on a lot of outside variables including: the age of the patient, the specific type of tumor they had, the family dynamics, the treatments that they will be undergoing, and current neurological symptoms they are experiencing. The nurse will have to continually assess where the patient and their family is at and adjust the care that they are providing appropriately.

No matter where the patient is at, education will be a huge part of the nursing care. Both the patient and their family will need to be educated on what kind of symptoms they can expect, what different treatment options will look like, what kind of side effects may be observed, and how they can best care for themselves when they are outside of the hospital. The nurse will also play a role in helping the family and the patient reenter the world outside of the hospital. They should be educated on supplies to have at home/on them at all times and should be emotionally supported as they prepare to reenter school. Often times, children may struggle with how their classmates will view them as they go back to school with some neurological deficits. No kid wants to seem "different" and it is very hard to not be different when you are coming back to school with a walker after being away from so long. The nurse should be prepared to address these difficult feelings as they come up.

Being a nurse in a cancer care unit requires a lot. It is a job that asks you to be knowledgable, compassionate, friendly, and empathetic. Nurses in these environments truly do some amazing work and can have a huge impact on the lives of their patients.

References:

http://nandanursing.com/nursing-care-plan-for-brain-tumor-intracranial-tumor.html

Cartwright, C. (2007). Nursing Care of the Pediatric Neurosurgery Patient. Berlin: Springer. 

The Road to Recovery: Treatment Options

Typically, the first step in treating medulloblastoma is undergoing surgery, as Matt did. The goal of this surgery is to take out as much of the tumor as possible and to take a sample from the tumor to confirm the diagnosis.

The next step in treatment is usually radiation. If the surgeons were able to remove all of the tumor during surgery, the patient will be treated with a reduced-dose radiation. If some of the tumor is still left behind, a higher-dose radiation therapy will be used. Radiation involves using a high level of X-rays or another type of ray in order kill the cancer cells and slow their growth. Some of the side effects of radiation include: swelling of the targeted tissue (in this case, the brain), mouth and throat sores, and the risk of developing a second cancer. It is important to still encourage an adequate intake of food and water despite the mouth and throat sores in order to prevent dehydration and malnutrition.

In some instances, the patient will also undergo chemotherapy as a last treatment option. Chemo is an extremely powerful agent and will work to to kill cancer cells and prevent them from replicating and spreading. The chemo can either be given by injection or orally. Both of these routes will allow the effects of the chemo to spread throughout the entire body. One of the most difficult symptoms to manage with chemotherapy is the nausea and vomiting that many experience. Also, alopecia, or the loss of hair, is very commonly experienced and can be difficult for patients to cope with.

Generally, all of these treatment options are done over a long period of time. The Road to Recovery is a long road that runs along side The Road to Awesome. These treatment options all demand a lot from both the patient and their family and many will find it difficult to cope with all of the changes. It is important to remember the emotional side effects and consequences of these treatment options and to utilize support resources for the family and the patient.

References:

http://www.stjude.org/medulloblastoma-pnet

Hesketh, P. (2014). Prevention and treatment of chemotherapy-induced nausea and vomiting. UptoDate 1151(47).

Mitin, T. (2014). Radiation therapy techniques in cancer treatment. UptoDate 89528(8).

Symptoms of Medulloblastoma

The most common symptoms that occur with the development of medulloblastoma include:

-behavioral changes (mood changes)
-changes in appetite
-unusual eye movement
-symptoms connected to an increased pressure in the brain caused by the tumor:

• headache
• nausea
• vomiting 
• drowsiness
• coordination problems 

Some of these symptoms can be difficult to recognize, especially in children. It is not uncommon for young children to experience mood swings (happy one second, throwing a tantrum the next). It also is fairly common for young children to frequently change their favorite foods and to refuse to eat what's in front of them. In Matt's case, they were able to pick up on the symptoms of coordination problems and behavioral changes. He was normally always so optimistic and kind and he was very athletic and coordinated, so in his instance they were fairly easy to pick up on.

Additionally, many diagnosed with medulloblastoma end up undergoing some form of surgery and radiation. There are several different symptoms/side effects that one can experience after undergoing radiation. The two most common symptoms experienced are skin problems (dryness, itching, blistering, peeling) and fatigue (constant tiredness/exhaustion that does not improve with rest). There are also some more serious long-term side effects that can happen after undergoing radiation, the most serious of which being developing a second cancer. However, it is important to remember that there is a relatively low risk of this and, often, the benefits of getting rid of the original cancer usually outweigh the risks.

There are also a variety of symptoms/side effects that are specific to radiation in the head/neck. These include:

-difficulty swallowing
-mouth and gum sores
-stiffness in the jaw
-tooth decay
-nausea
-lymphedema (swelling caused by a lymphatic system blockage)

In addition to all of these medical symptoms, there are a variety of emotional symptoms that both the patient and their family experiences. This can include anxiety, fear and depression. Often, there is a lot of anxiety about whether or not treatment options will work. There can be a great deal of fear about the future and the uncertainty of their condition. Both the patient and their loved ones can become depressed and have difficulty coping with all of the changes.

There are also a variety of symptoms that occur on The Road to Awesome. These can include:

-your siblings shaving their heads
-randomly bursting out into laughter
-spontaneous dance parties and crowd surfing with Michael Franti
-the occasional emotional outburst from Mom
-physical therapists being shocked at your motivation to improve
-an outpouring of love from family and friends

References:

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

http://www.cancer.net/navigating-cancer-care/how-cancer-treated/radiation-therapy/side-effects-radiation-therapy

http://www.cancer.org/treatment/treatmentsandsideeffects/emotionalsideeffects/emotional-side-effects-landing

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