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READ MOREPublished on September 17, 2024 in Pediatric Brain Tumor, Educational Resources
Diffuse intrinsic pontine glioma (DIPG) gained notoriety in the 1960s when astronaut Neil Armstrong’s 2-year-old daughter passed away from the devastating disease seven years before her father’s infamous first steps on the moon. Despite advancements in medical research and treatment for other cancers over the past six decades, DIPG and diffuse midline glioma (DMG) survival rates tragically remain relatively unchanged.
This Childhood Cancer Awareness Month, let’s talk about the unique and formidable challenges faced by children diagnosed with DIPG or DMG and their families.
DMG tumors predominantly occur in the brainstem, thalamus, and spinal cord. DIPG tumors are only found in the pons area of the brainstem. Surgery is generally not a viable option for DIPG and DMG tumors due to their location in areas responsible for essential life functions, such as breathing, heart rate, and blood pressure. These diffuse tumors also invade surrounding brain tissue rather than forming a distinct mass, which makes it difficult to surgically remove the tumor without damaging healthy brain tissue.
“One of the key features [of DIPG/DMG] is that you can’t take it all out, and that’s what makes it so challenging,” said Lauren Weintraub, MD, Director of the Pediatric Neuro-Oncology Program at Albany Medical College. “The molecular features we know are associated with such an aggressive tumor. We don’t have any curative treatments right now. Even though there is a lot of active research in that area, unfortunately, nothing has been shown to have a sustainable effect.”
These tumors are notoriously resistant to conventional therapies because of their location and molecular features. Radiation therapy is the existing standard of care treatment, but it only offers short-term relief of symptoms. According to a Brain Tumor Research and Treatment article, “Radiotherapy is effective as a palliative treatment, providing transient symptomatic improvement, and improves the overall survival duration by several months.”
Jacob M. was diagnosed with DIPG at four years old, less than two weeks after his mother, Mia, noticed his head tilt and balance difficulties. Mia shared, “When they told us that it was DIPG, my first question to the doctor was, ‘If we do radiation and it goes away, how long do we have after it comes back?’ He told me that it would come back, and it would be under one year. It shocked me and made me realize that our lives just completely changed.”
In some cases, radiation therapy may provide a window of time during which the child is stable enough to participate in clinical trials.
“When I first started, we would get a patient with DIPG, I would say we don’t need to get a biopsy because we know it’s DIPG, and we’re going to treat it the same,” Dr. Weintraub explained. “And, now, I pretty much insist that patients get a biopsy because we need to know more about them — not only for the patient but for the community — and it opens doors for clinical trials. We’re not going to get anywhere without clinical trials.”
DIPG and DMG are complex and aggressive brain tumors. Specialists regularly treating DIPG and DMG have more experience with the latest treatment protocols and clinical trials. A second opinion can provide insights into options that the initial treatment center might have yet to consider or have available.
Obtaining a second opinion also empowers families to take an active role in their child’s care, ensuring that all possible avenues have been explored and that the chosen treatment plan is the most appropriate.
“Early on, second opinions become huge,” Dr. Weintraub said. “Especially being at a smaller institution where a lot of patients end up being treated, I think that encouraging families to get second opinions, or at least have their physician get a second opinion or just talk to people, makes them feel like they’re doing everything they can.”
Unfortunately, the prognosis for children with DIPG or DMG remains poor. A Neuro-Oncology Advances article states that DMG “tumors are the leading cause of brain tumor-related death in children, with the median survival time for those diagnosed being 9 to 11 months.” Long-term survival is rare, with fewer than 10% of patients with DIPG surviving beyond two years.
Christine S. vividly recalls learning of her 6-year-old son Devin’s DIPG diagnosis from a group of neurosurgeons and neuro-oncologists: “‘Your son has DIPG, and he has eight months to two years to live if you’re lucky.’ And we’re like, ‘I’m sorry. What?’ I don’t remember what exactly happened, but I remember just feeling like I was on the ground. Whether I was on the ground or not, I have no idea.”
Families facing a DIPG or DMG diagnosis often find themselves torn between two competing priorities: securing the best possible treatment for their child and making the most of the limited time they have together.
“I think sometimes it’s hard for people to understand that there are different ways to approach [treatment],” Dr. Weintraub said. “I think the general public hears about a 3-year-old with a brain tumor, and they’re like, ‘OK, well, they have to go to St Jude. I like the docs at St. Jude. They’re great, but that’s not the only place you can get your treatment. And sometimes, it’s much better [for quality of life] to be at home.”
Many families prioritize making as many happy memories as possible, whether through special trips, family gatherings, or simply cherishing everyday moments together.
“Losing Devin was the worst heartbreak of our lives, but we also made the most amazing memories,” Christine shared. “The things we did in eight months and three weeks, most people don’t do in a lifetime.”
DIPG and DMG are highly aggressive, rapidly growing tumors. The cells multiply quickly and spread diffusely, often leading to a rapid decline in neurological function.
“DIPG, by definition, is in the pons, so kids with that tumor — because of its location — tend to have a lot of balance and vision issues,” Dr. Weintraub said. “You have to radiate that area, so that can make those issues worse. When the tumor starts progressing, those are the issues that get so bad. For the other DMGs, it depends on the location, but we see that most of our patients tend to have significant neurologic issues with some exceptions. Most kids have some significant degree of weakness on one side or trouble talking.”
The symptoms, such as difficulty swallowing, speaking, and moving, profoundly impact the child’s quality of life.
“I felt that if I knew enough to be able to acknowledge and understand the symptoms that were coming, then I would be better prepared,” Mia said. “For example, I knew he wasn’t going to be able to speak again. So knowing that ahead of time, I would think in what ways can we start acting now to make it easier for us to communicate with him.”
Providers frequently recommend palliative care after a diagnosis as it focuses on relieving the physical, psychological, social, and spiritual concerns, troublesome symptoms, and suffering that patients with life-changing diagnoses and their families may experience.
“We have a palliative care team that gets involved right from the beginning when it comes to those kinds of cases,” Dr. Weintraub said.
Steroid treatment, commonly prescribed to patients with DIPG and DMG to reduce inflammation and manage symptoms caused by the tumor, can create significant challenges for both the patient and their family.
“In addition to whatever type of therapy you’re trying to use, you have to use steroids to try and control symptoms,” Dr. Weintraub said. “And the side effects of steroids are pretty significant. I think that’s hard for kids and families because then kids look really different.”
Steroids can cause significant weight gain and a puffy face, which can be distressing for both the child and the family, as the child may no longer look like themselves.
Steroids often cause an increase in appetite, which can lead to constant requests for food.
“They are starving all the time,” Christine shared. “Devin was waking us up hangry at 4 a.m. demanding cheeseburgers.”
Additionally, steroids can cause significant mood swings, including increased irritability, aggression, and emotional instability. These changes can be distressing for families, as they may alter the child’s usual behavior, making them harder to care for and more emotionally volatile.
“The steroids were the worst thing ever,” Mia explained. “They completely changed him. He gained tons of weight. He was angry all the time. He didn’t want to play … Once we knew that the tumor was growing, we decided that we did not want him to continue with the steroids because we wanted his quality of life to be a little bit better than it was.”
The entire family dynamic can shift after a diagnosis, with the needs of the sick child taking precedence. This change can alter routines, disrupt normal family activities, require role changes (e.g., an older sibling of driving age now has to run errands), and lead to feelings of resentment and jealousy.
“Families focus so much on the sick child,” Christine said. “The siblings can feel displaced because all the attention was on that one child, and then that child doesn’t survive, and it’s the others who are left. It’s really hard. If you can keep your family together as much as possible after the diagnosis, I think that’s honestly equally as important [as seeking treatments].”
According to the National Cancer Institute, about 300 American children will be diagnosed with DIPG each year. The rare nature of this disease can be less incentivizing for pharmaceutical companies to invest in research, leaving it up to nonprofit organizations like NBTS and the government to fund best-in-class research.
“That’s where money and policy is needed,” Dr. Weintraub said. “There are some promising things out there, but they’re just not going to go anywhere without more funding for kids.”
This September, our community comes together for Childhood Cancer Awareness Month to recognize, advocate for, and honor children who have been affected by cancer and the families who care for them.
Since the DNA Damage Response Consortium launched in 2022, the team at St. Jude Children’s Research Hospital has received $2 million for their work from NBTS to tackle the most aggressive pediatric brain tumors like DIPG and DMG.
But we need your help to continue this important work. Join NBTS in driving discoveries forward for the pediatric brain tumor community by making a gift today. For everyone with a DIPG or DMG diagnosis, it’s time for a better chance.
TAGGED WITH: pediatric cancer, DIPG, CCAM, Childhood Cancer Awareness Month, DMG