While the diagnosis of DIPG is difficult, there are steps you can take to improve the quality of care for your child.
DIPG is a type of tumor that starts in the brain stem, the part of the brain just above the back of the neck and connected to the spine. The brain stem controls breathing, heart rate, and the nerves and muscles that help us see, hear, walk, talk and eat. These tumors are called gliomas because they grow from glial cells, supportive cells in the brain.
About 10% to 20% of all childhood brain tumors are DIPG or brainstem gliomas.
They are more common in children between the ages of 5 and 10 years but can occur at any age in childhood. Though rare, they can also occur in adults.
While DIPG tumors may have certain genetic changes (the doctor will call them mutations), this isn’t a disease that children get from their parents. It doesn’t come from exposure to anything in the world around them either, like smoke, chemicals, or radiation. There’s no way to prevent DIPG or lower your child’s risk of getting it.
Doctors think DIPG may be linked to how a child’s brain grows. Tumors tend to appear at an age when the brain is changing fast. During this time, there’s a high amount of a type of brain cell that may drive DIPG tumor growth.
Symptoms usually develop rapidly in most patients because of the fast growth of these tumors.
The most common symptoms related to DIPG include the following:
To diagnose DIPG, a pediatrician first does a physical exam. They’ll go over your child’s medical history and symptoms.
They may do tests like:
Imaging. Brain scans like CT scans help confirm a DIPG diagnosis. Your child may also need magnetic resonance spectroscopy (MRS) test, which looks for chemicals in cells. The doctor could pair it with an MRI scan to look for signs that the tumor comes from glial cells.
The doctor might inject a contrast dye before the MRI to show the tumor more clearly. The tumor’s location in the pons, how it has spread into other tissues, and unclear edges all suggest DIPG.
Biopsy. Children with unusual symptoms or MRI results may need a biopsy to confirm a DIPG diagnosis. For DIPG, a pediatric neurosurgeon doctor can do a stereotactic biopsy test. They’ll drill a small hole in your child’s skull, insert a needle, and remove a piece of the tumor. A doctor called a pathologist examines the tissue for signs of DIPG.
DIPG falls into the Glioma staging system, so they can be classified according to the four stages below based on how the cells look under the microscope. The grades are from the least severe to the most severe.
The main issue with DIPG is that most of these tumors are not classified by grade because surgery to obtain tissue by biopsy or to remove the tumor is not safe because of the tumor’s location. When these tumors are biopsied, they are usually graded III or grade IV tumors, which tend to behave very aggressively. Most of the tumors are diagnosed by their appearance on MRI.
DIPG treatment options include surgery, radiation, and chemotherapy.
Radiation. This is the primary DIPG treatment. The doctor beams high-energy X-rays at the tumor in a series of sessions. Children with DIPG may need five daily radiation therapy sessions a week and up to 30 or more sessions.
Radiation therapy helps to shrink the tumor for most children with DIPG. This treatment may also relieve your child’s symptoms. When radiation shrinks the tumor, it eases pressure on your child’s brain. It can also extend a child’s life by several months.
But the benefits of radiation therapy are usually temporary. Within a few months, the tumors often start to grow again. If that happens, children may be able to repeat radiation therapy to add more months to their lives.
Chemotherapy. Newer, experimental chemotherapy drugs and biologics may be used with radiation therapy to treat DIPG. No medications have shown success in treating this cancer. In one new treatment option for DIPG, doctors inject targeted cancer drugs directly into the tumor to make treatment more effective.
Surgery. Surgery to remove the tumor usually isn’t an option. These tumors aren’t solid masses that are easy to take out. There’s a high risk of damage to healthy brain tissue in surgery. Surgery could even be fatal.
Unfortunately, the survival rate for DIPG remains very low. Only 10% of children with DIPG live for two years after diagnosis, and less than 1% survive for five years.
There is no cure for this tumor. Doctors will work to keep your child comfortable. At this time, medications can’t treat the disease, just the symptoms.
It is hard to prepare for something when you don’t know what to expect. Brain tumors affect children’s abilities differently, but some changes are more common than others and can be anticipated. For example, speech often sounds slurred and can be challenging to understand due to weakness or difficulty coordinating the lips, tongue, and jaw. Children’s abilities to use their arms and hands may also become compromised, making it challenging to write, draw, or point.
Regardless of the difficulties children with diffuse intrinsic pontine gliomas (DIPGs), we interviewed parents who agreed that two essential strategies helped maximize communication.
Learn and practice ways your child can communicate without speech before your child needs to use them. This is not always easy. Children can be reluctant to use communication strategies before they have to, and parents and children often do not want to think about when these strategies will be necessary. This reticence is natural and understandable. However, the patience and concentration needed to learn a new skill may not be present once your child’s energy and abilities decline.
Practice more than one way of communicating without words. If some of your child’s abilities change unexpectedly, she can continue communicating using another familiar means. When practicing other ways to communicate, it is often helpful to find a way to adapt a current communication tool or technique to suit your child’s changing abilities rather than switching to a brand new system. By adopting a strategy that children and families are more familiar with, their experiences serve as “practice,” They may feel more comfortable and confident in their abilities to use it.
We must find a cure to save our children. ❤️