Does your child have a brain tumor like this? (Diffuse Intrinsic Pontine Glioma - DIPG) Shall we talk about this?

It's normal to feel a great deal of stress when thinking about your little one's health. Sometimes, we get really worried when we hear about illnesses that affect children. Today, we're going to talk about a brain tumor that's a bit more serious, but it's important to be aware of. It's called diffuse intrinsic pontine glioma, or ``DIPG'' for short.

What is DIPG? What does this name mean?

Although this name may seem a bit long, knowing its meaning can give you some idea about this disease. Let's look at the meaning of its parts.

• Diffuse: This is not like a lump in one place. The cancer cells are spread out and mixed with healthy cells. It's like when you put a drop of dye in a glass of water, it spreads throughout the glass. That's why it's so difficult to remove with surgery. Because it's impossible to remove these spread out cells without damaging the healthy parts.
• Intrinsic: This is located within the child's brainstem. That is, it is connected to it and goes deep inside.
• Pontine: Pontine refers to the part of the brainstem called the pons. This pons is a very important place. It controls the most important things in our body to live, such as blood pressure, heartbeat, and breathing. Not only that, but it also controls things like vision, hearing, speech, and balance. So this is one of the reasons why these `(DIPG)` tumors are so dangerous.
• Glioma: A glioma is a tumor that develops when glial cells become cancerous. These glial cells are a type of cell that protects the nerve cells, or neurons, in our central nervous system (CNS).

Simply put, DIPG is a malignant tumor that arises from glial cells in the pons, a part of the brainstem of a child. It is considered a fast-growing, high-grade glioma .

What is the difference between DIPG and DMG (Diffuse Midline Glioma)?

You may have also heard of `(Diffuse Midline Glioma - DMG)`. In the past, the terms `(DIPG)` and `(DMG)` were used interchangeably. `(DIPG)` also belongs to the `(DMG)` category. The `(DMG)` type develops in the middle parts of the brain. That is, in places like the `(Pons)`, `(Thalamus)` and `(Spinal Cord)`. However, `(DIPG)` develops specifically in the `(Pons)` area.

Both are high-grade gliomas. However, there are some differences between the two. When treating your child, your doctor will consider these differences to decide which treatment is best for you.

Who gets DIPG? How common is it?

These tumors can develop in children of any age, regardless of gender. However, they are most often diagnosed in children between the ages of 5 and 9.

Adults can also develop DIPG, but it is very rare.

This is actually a very rare condition. Even in a country like America, only 150 to 300 children are diagnosed with this type of tumor each year. Most gliomas that develop in children are ``low-grade'', meaning they can be treated and cured. However, only about 10% of childhood brain tumors are this dangerous type called ``DIPG''.

What are the symptoms of DIPG?

Because this tumor, called ``DIPG'', grows very quickly, symptoms begin to appear quickly. Keep an eye out for these signs in your child:

• Eye problems: blurred vision, double vision, uncontrolled eye movements, drooping eyelids.
• Headache: Headaches, especially in the morning. Sometimes, this pain subsides after vomiting.
• Walking difficulties: Difficulty walking, loss of balance (ataxia), and decreased muscle coordination. Like being unable to keep your feet on the ground.
• Sagging or weakness on one side of the face .
• Difficulty chewing and swallowing food.
• Feeling of weakness in the limbs . It feels like a sudden loss of strength in an arm or difficulty in moving a leg.
• Nausea and vomiting.
• Feeling like your speech is slurred , unable to get your words together properly (slurred speech).

If one or more of these symptoms appear suddenly and increase rapidly, it is very important to see a doctor immediately.

What causes DIPG?

Simply put, what happens in DIPG is that cells that are supposed to develop into glial cells become cancer cells. These cancer cells then grow rapidly and uncontrollably.

Unlike most cancers, this condition called ``DIPG'' does not appear to be linked to environmental risk factors such as cigarette smoke or radiation. That is, it has not yet been determined whether it is caused by something the parents do or something in the environment.

However, if your child has a rare condition like Li-Fraumeni syndrome, the risk may be slightly higher. Also, certain genetic changes, called mutations, in cells can cause DIPG. It has now been discovered that a mutation in a protein called H3K27M can cause these glial cells to become cancerous.

How is DIPG diagnosed?

Usually, a biopsy, which is a sample of tissue taken to test for cancer, is required. However, this is not always possible in the case of DIPG. This is because the location of the tumor is very sensitive. Therefore, doctors often diagnose DIPG by looking at the child's symptoms along with special imaging studies.

• MRI (Magnetic Resonance Imaging) Scan: An MRI can produce very clear images of your child's tumor. It can also show if the tumor has spread beyond the pons. A DIPG tumor looks a little different on an MRI. An experienced radiologist can tell it apart from other brainstem tumors.
• Stereotactic Biopsy: Sometimes, if it is safe to do so, the doctor may use a small, thin tube-like instrument to take a small piece of the tumor for testing (a biopsy). However, in most cases, taking a piece of tissue from the pons in the brainstem is very risky because it can damage the brainstem. If you are having a biopsy for DIPG, it should only be done in a hospital that regularly does this type of procedure and has expertise in the area.

How is DIPG treated?

This is a bit sad to hear, but the current treatments cannot completely cure `(DIPG)`. However, these treatments can extend the child's life for a while, reduce symptoms and provide relief to the child. The brain stem is a place made up of nerve tissue that controls very important functions in our body. So if you try to remove the tumor surgically, these essential nerve structures may be damaged.

Therefore, doctors recommend other non-surgical treatments:

• Radiation therapy: This is the main treatment for (DIPG). In this, a machine uses a beam of radiation to destroy the cancer cells. It also tries to minimize damage to surrounding healthy tissue. Radiation therapy shrinks the tumor, kills cancer cells, and reduces symptoms. Your child will likely receive radiation therapy every day for about six weeks.
• Corticosteroids: Your doctor may give you a type of medication called corticosteroids before or after your child's radiation treatment. These medications can help reduce swelling in the brain and help control symptoms. They can also help reduce swelling after radiation treatment. However, corticosteroids can have side effects. These include increased appetite, weight gain, and mood swings. Talk to your doctor to understand the benefits and risks of these medications.
• Chemotherapy: Treatments like chemotherapy for common cancers are not very effective for DIPG. However, sometimes your doctor may recommend that your child participate in clinical trials that combine chemotherapy and radiation therapy.
• Palliative care: Palliative care professionals can work with you and your doctor to create a care plan that maximizes your child's quality of life. They can provide the medical, psychological, and spiritual support you, your child, and your family need when dealing with a diagnosis like DIPG.

Your doctor may also suggest that your child be enrolled in a clinical trial . A clinical trial is a study that tests how safe and effective new treatments are. Researchers have identified certain characteristics of (DIPG) cancer cells. New treatments that target those characteristics – for example , molecular targeted therapy and immunotherapy – may be available through a clinical trial.

For example, researchers know that many cancer cells have a mutation called (H3K27M) in the gene ``DIPG''. New, experimental therapies that target cancer cells with this mutation (and others) are already being developed and tested in clinical trials.

Can DIPG be cured?

No, Diffuse Intrinsic Pontine Glioma (DIPG) cannot be cured. The most effective treatment currently available is radiation therapy. Radiation therapy for DIPG can prolong a child's life and temporarily improve symptoms and quality of life. Newer, more effective treatments are needed to improve the prognosis of this serious cancer.

How long can a child with DIPG live?

I know this is hard to hear. Without treatment, most children live for about six months after diagnosis. Radiation therapy can usually extend this time to nine to eleven months. Less than 10% of children live beyond two years. It's normal to feel sad when you hear these statistics, but it's important to remember that every child is different.

What questions should I ask the doctor?

Work closely with your doctor to understand your child's care plan and the factors that will affect their future. Don't be afraid to ask any questions you have. Here are some questions you can ask:

• What treatments do you recommend? What can we expect from them?
• How often will my child need treatment?
• How often will my child need to come for follow-up appointments to monitor the progress of the cancer?
• How do we manage the side effects of cancer treatments?
• Can you connect us to palliative care services and support groups?
• Are there any new research or clinical trials my child can participate in?

Finally, things to remember (Take-Home Message)

Finding out that your child has Diffuse Intrinsic Pontine Glioma (DIPG) can be one of the hardest things you can hear as a parent. The pain, fear, and anxiety you feel can be overwhelming. But remember that you are not alone in this time.

As you make treatment decisions in the coming months, take advantage of all the help and resources available to you. Talk openly with your child's doctors about what to expect with this disease. If you can, reach out to professionals who provide palliative care. Ask for help from friends and family. Above all, stay connected to your child. Talk to him, play with him, and give him love. Let him know that you are with him every step of the way.

In these difficult times, it's important to think about yourself and take care of your mental health. If you stay strong, your child will be strong too. You are not alone, we are all with you.

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