Pediatric Cancer: Diffuse Intrinsic Pontine Glioma

Categories: Cancer

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Kids all over the world are suffering from some form of cancer. However, because of its rarity, not as many children are suffering from the deadly brain tumor called DIPG. DIPG stands for Diffuse Intrinsic Pontine Glioma. DIPG is the most deadly and aggressive pediatric brain tumor.

Pediatric cancer is the leading cause of death in children. More children die from cancer in the United States than any other disease or sickness. Before the age of 20, approximately 1 in 285 children in America will have cancer (“About Childhood Cancer”).

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Brain tumors are the most common solid tumor found in the pediatric population. Every year approximately 3400 children are diagnosed with a primary tumor of the central nervous system - brain and spinal cord, with only about 300 cases a year being from DIPG. Brain tumors are the leading cause of death in the pediatric population. Over the years pediatric cancers have had many great medical advances. There are new treatments to help cure cancers and help the effects caused by the cancer.

Medications have developed over the years to help combat treatment side effects. According to an article from St. Baldricks Hospital, “For kids diagnosed with a rare and fatal type of brain tumor called DIPG, or Diffuse Intrinsic Pontine Glioma, there is no cure and treatments are scarce” (Jessop). Less than 1% of children with DIPG survive 4 years after diagnosis and most pass away before the first year. All in all, it is becoming easier to cure children with cancer. The unfortunate news is that DIPG has had no medical advances.

It is the same as it was 100+ years ago. There are treatments to lessen the pain and symptoms but nothing has offered a permanent cure.

Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive type of brain tumor found in the pons, a part of the brainstem. The brainstem controls many important functions, like breathing, heart rate, and blood pressure and as the tumor grows it puts pressure on the nerves that control those functions (Mosier 1). This glioma tumor invades the normal brain tissue which makes it difficult to treat. Most pediatric cancers are caused by genes, however, that is not always the case. Even tho some cancer may be genetic, just because a parent or grandparent had cancer doesn't allows mean their child will. It is sometimes due to genetic mutations that come together in a certain way that will then form cancer (“Why Do Kids Get Cancer”). Unfortunately, there is no known cause of the formation of the DIPG tumor, but some research has shown that it may be linked to early abnormal brain development (Mosier 2)

DIPG affects all sexes, race and age, although you are more likely to be diagnosed with this tumor if you are between the ages of 5 to 10. Within the past 2 years in Volusia County, two children under the age of 18 have been diagnosed with DIPG. The first young man was Kayne Finley. Kayne was diagnosed in November 2016 at the age of 17. He was senior at Spruce Creek High School and was also on the swim team. His dream was to attend Louisiana State University (LSU) after his graduation. He was able to attend the fall semester of 2017 until he developed more severe symptoms and was forced to go back home. He passed away November 24, 2017. The other boy in Volusia County who was diagnosed with DIPG was Anthony Franzini. Anthony was 11 years old at his diagnosis. He loved basketball, playing with his little sister and like most 11 year old boys, playing video games. Anthony passed away at the age of 12, only four short months after his diagnosis. They both fought hard but ultimately lost the battle. Most Americans know that Neil Armstrong was the first man to walk on the moon. What many people don’t know is that he had a daughter, Karen “Muffy” Armstrong, who was diagnosed with DIPG at the age of 2. Her parents decided she should receive radiation treatment to help reduce the size of the tumor, but within months the tumor grew back and her symptoms returned at a quicker rate. She developed pneumonia because of her weakened health and died on January 28, 1962 at the age of 3 (Evens)

DIPG is difficult to diagnosis because the symptoms can mirror many different things. The pontine glioma tumor grows quickly, so symptoms can appear suddenly and progress quickly. The glioma typically affects the cranial nerves causing neurological problems. So many of DIPG’s early symptoms appear in the facial muscles, which can affect eye movements. The most common symptoms of DIPG are difficulty with eye movement, facial weakness, hearing problems, trouble chewing or swelling and even gagging while eating, limb weakness, difficulty standing or walking, headaches, nausea and vomiting from brain swelling.

Once a child is diagnosed and the family learns of the expected outcome, they then have to make hard decisions on treatment. Because of its location surgery is not usually a treatment option. The brain stem is what controls all of the body’s crucial functions, like breathing. When the tumor pervades into the healthy brain tissue, it doesn't allow surgery to be an option. Surgery could cause death or force the child to be connected to life support for the rest of his or her life. For most cancers the best form of treatment is chemotherapy whether they are injected straight into the infected area or through an IV drip, but for DIPG chemo has not been successful. They have also tried targeted therapy which uses drugs to attack the cancer cells but there has been no success for that as well. In an interview with Laurie Parker, who is a childhood cancer advocate, she stated, “The treatment has been the same for the last 50 years….there are clinical trials available; however, nothing has offered a cure.” The reason most of the treatments do not work is because of the blood-brain barrier (BBB). The blood-brain barrier is defined as “A network of blood vessels and tissue that is made up of closely spaced cells and helps keep harmful substances from reaching the brain…It also keeps out bacteria and other substances, such as many anticancer drugs…” (“Blood-brain Barrier”). So when attempting to inject the viruses or using other treatments, they can not reach the affected part of the brain because the pathways are blocked. In order to be able to get these drugs to be effective we have to first find a treatment for the BBB to ensure the drugs reach the tumor. Again, like all the other treatments, there has been no success. But some parents still put their kids through clinical trials to try and prolong their lives but also to help researchers see what they are doing right and wrong. Kirsten Finley said in an interview, “I believe to treat DIPG it will require a multi-modal drug approach. In other words, not all drugs work the same on different patients and multiple drugs will need to be combined for better treatment and patient benefit…” This is what is happening in Mexico. The dilemma with the trial there is the researchers and doctors are not sharing their findings with any other researchers, nor do they share the details of to the parents of the patients. The worst part is, the treatments can cost between $300,000- $400,000 and even after the treatment they children still die. From this treatment only 2 kids have survived (Finley). The most common treatment path for DIPG is radiation. The use of radiation is to help shrink the tumor. By shrinking the tumor, it is hopeful the child will have dramatic relief from the symptoms for a time. Every child’s response to radiation is different. In Anthony Franzini’s case, the radiation only made him worse. Scar tissue built up around the area of the radiation and caused him to have periods of extreme rage as well his tumor not shrinking. Therefore, his symptoms continued to worsen. In some children, the radiation does provide symptom relief by shrinking the tumor. Although this treatment does not get rid of the tumor it is the only thing that has shown effectiveness towards DIPG. The goal is for the child to have a period of relief from the tumor symptoms but also have little to no radiation side effects. In all cases where the radiation shrunk the tumor it eventually grew back within 3-6 months and that is why it is considered an aggressive palliative therapy.

Once the tumor begins to grow back, the symptoms return at a quicker and more painful rate. When the end nears, the tumor begins taking away many normal day to day functions and making them impossible tasks. Things like difficulty swallowing or inability to eat by mouth may lead to a feeding tube being inserted for nutrition, nausea and vomiting requiring anti-nausea medications, constipation, skin issues, fatigue, breathing problems which can lead to severe anxiety and possibly seizures. Children begin to loose their ability to walk, and use their arms and hands. This requires a parent to care for their child as if they were an infant again. In the final days, most children loose their ability to speak. Communicating seems impossible. Some families are able to use communication devices with pictures to help understand their child’s needs. The care at the end is difficult. Parent’s desire to make their child as comfortable as they can. Most do not understand the emotional strain this puts on the child’s parents. While taking care of the child, the parents life is all about cancer. They eat, sleep, and breath caring for the needs of their child. This is a difficult time for parents. In an interview with Kirsten Finley whose son Kayne recently passed away of DIPG, she stated, “I have been seeing a psychologist weekly for over a year now to help with emotional stability, complicated grief, anxiety and depression.” And for many parents this is the reality they face after their child is gone. Over time his or her body is succumbed by DIPG. It is a slow and horrible death.

Many parents share their perspective on DIPG, their child's days through the battle, and how they coped with it as a parent who is watching their child suffer. One parent wrote this:

Brendan was diagnosed at age 6. Two weeks before diagnoses we noticed several things. His eyes were cloudy and unfocused which we mistakenly attributed to conjunctivitis. He had given up napping when he was a toddler but was very tired and fell asleep in the middle of the afternoon ( Understanding the Journey: A Parent’s Guide to DIPG 38). They continued to share how they thought it was a growth spurt or he was just coming down with something. But then they started noticing he had difficulty chewing and swallowing, his voice was raspy and their once happy kid did not smile anymore. Brendan’s parents took him to the doctor when he began falling and saying he was dizzy. They were told his tumor was DIPG (Diffuse Intrinsic Pontine Glioma). Brendan went through two separate rounds of radiation. Both gave him a honeymoon period where he was symptom free. During his second progression his symptoms began to worsen and the tumor progressed quickly. He passed away within six weeks of the recurrence. This is just one story of many families enduring DIPG.

DIPG is the most underfunded and least researched of the pediatric cancers. This is devastating to a family who learns of the 0% survival rate. In an article from St. Baldricks Hospital titled, “ An injection of Hope: Researchers Studies Innovative Potential Therapy for DIPG,” The question is asked, “What does a doctor working on DIPG do to help the kids?” The answer is stated, “think outside of the box. Break new ground. Do things differently.” Dr. Mark Souweidane is the Vice Chair of Neurosurgery at Weill Cornell Medicine and Directer of Pediatric Neurosurgery at both New York Presbyterian Hospital and Memorial Sloan Cancer Center. ”The mission of my work is to cure DIPG,” Dr. Souweidane stated. His study is to provide a unique way to treat DIPG patients by slowly injecting cancer fighting drugs directly into the middle of the tumor. He says this approach avoids the blood-brain barrier and targets the tumor with high concentrations of a special drug. He also wanted to create a drug that avoided toxicity that normal chemo causes. The clinical trial was completed in September of 2016. Twenty-eight children were treated on phase one of the trial. None of these children showed any symptoms of toxicity due to the treatment. Some of the kids are stable and doing well. For kids with DIPG and their families, Dr. Souweidnce’s work offers a little bit of hope according to the article (Jessop).

DIPG is a devastating diagnosis for any family. It is a cancer that needs more attention, more research and ultimately more funding. Through Kayne Finley’s foundation, Cannonballs for Kayne, they’ve been able to donate $30,000 dollars to Cincinnati Children’s Hospital Medical Center. This will help the beginning stages of more research as well as funding to find break through treatments. Childhood cancer itself only receives approximately 4% of government funding a year (Cannonballs for Kayne). That means DIPG alone receives less. DIPG advocates are working hard to bring more awareness to this form of cancer. A group of women from Volusia County recently visited Washington, D.C. to stand before Senators and ask them to sign the S.T.A.R Act (The Childhood Cancer Survivorship, Treatment, Access & Research). In May 2018, Congress passed the act. According to Children’s Cause Cancer Advocacy, the act will help to advance pediatric cancer research and child-focused cancer treatments, while also improving childhood cancer surveillance and providing enhanced resources for survivors. Through Kayne and many other pediatric cancer advocates they were able to make May 17th National DIPG Awareness Day in Kentucky, Louisiana, and Florida. They also made September “Go Gold Month” in most of Volusia County ( gold is the official color of pediatric cancer).

Some parents, as hard as it is, choose to donate their child's tumor after they pass away for further research. The Michel Mosier Defeat DIPG foundation works to encourage tumor tissue donations by families. This is essential to further the research of the DIPG tumor. Through the tissue donation, medical researchers gain valuable knowledge about the biology of the tumors. This also allows them to look at the tumor cell, DNA, RNA, and proteins which provides a better understanding of how the tumors form and grow. Many families are eager to defeat DIPG and are are actively working with doctors to arrange tissue donation. However, for most families that reality is too difficult to process while their child is still living. They aren’t focused on what will happen after their child is gone. Their focus is spending as much time with their child as they can. After their child passes away the last thing on their mind is sending their child’s body to a different state to have a tumor biopsy. The unfortunate news is, this is the only way medical researchers and doctors can see how the DIPG tumor works. Researchers must wait for a child to die in order to cure this horrible disease.

DIPG is a gut wrenching cancer that takes away lives of precious children who have not been given a chance to experience life. Children should not have to endure radiation, chemotherapy or prepare for the possibility of death. Most people have no idea how many children are battling for their lives because of a cancer diagnosis. The good news is, researchers say if we can cure DIPG we will most likely have a cure for all cancers because of how challenging it is to find a cure. Diffuse Intrinsic Pontine Glioma is unquestionably one of the most deadly and vigorous pediatric brain cancers of all time.