DIPG Survival: Understanding the Statistics and Treatment Options

April 9, 2023

Introduction to DIPG: Understanding the Disease and its Statistics

DIPG, or Diffuse Intrinsic Pontine Glioma, is a rare and aggressive type of brain tumor that primarily affects children. It is located in the pons, a part of the brainstem that controls important functions such as breathing, heart rate, and blood pressure. DIPG is considered one of the most challenging forms of cancer to treat, with a very poor prognosis.

Statistics

  • DIPG accounts for 10-15% of all brain tumors in children
  • The average age of diagnosis is around 6-7 years old
  • DIPG is more common in males than females
  • The 5-year survival rate for DIPG is less than 1%

Causes

The exact cause of DIPG is unknown, however, research suggests that it may be caused by genetic mutations in the cells of the pons. These mutations lead to the uncontrolled growth of cells, forming a tumor.

Symptoms

The symptoms of DIPG can vary depending on the location and size of the tumor, but some common symptoms include:

  • Weakness or paralysis on one side of the body
  • Loss of coordination
  • Difficulty speaking or swallowing
  • Vision problems
  • Headaches

Diagnosis

Diagnosis of DIPG is typically made through a combination of medical imaging tests, including:

  • Magnetic Resonance Imaging (MRI)
  • Computed Tomography (CT) scan
  • Biopsy

A biopsy, which involves taking a small sample of tissue from the tumor, is usually performed to confirm the diagnosis and to determine the type of tumor.

DIPG is a rare and aggressive form of brain cancer that primarily affects children. It is located in the pons, a part of the brainstem that controls important functions such as breathing, heart rate, and blood pressure. DIPG is considered one of the most challenging forms of cancer to treat, with a very poor prognosis. The causes of DIPG are still not well understood, but research is ongoing to find new treatments and improve the survival rates of children with DIPG.

Current Treatment Options for DIPG

Treatment options for DIPG are limited and typically involve a combination of radiation therapy and supportive care. The goals of treatment for DIPG are to control the growth of the tumor, relieve symptoms, and improve the patient’s quality of life. However, despite the best efforts of medical professionals, the prognosis for DIPG is poor, and most patients succumb to the disease within a year of diagnosis.

Radiation Therapy

Radiation therapy is the primary treatment for DIPG and is typically given in a series of daily treatments over the course of six to seven weeks. The radiation is delivered to the brainstem and surrounding areas in an effort to shrink the tumor and control its growth.

Chemotherapy

Chemotherapy is not considered a standard treatment for DIPG, however, some children may receive chemotherapy in combination with radiation therapy. Chemotherapy drugs are used to target and kill rapidly growing cancer cells, but the effectiveness of these drugs in treating DIPG is limited.

Stereotactic Radiosurgery

Stereotactic radiosurgery (SRS) is a type of radiation therapy that uses high doses of radiation to target the tumor while minimizing exposure to healthy tissue. SRS is a non-invasive procedure that is typically performed on an outpatient basis. However, the effectiveness of SRS in treating DIPG is still under investigation.

Supportive Care

Supportive care is an important aspect of treatment for DIPG. This may include physical therapy to help with weakness or paralysis, speech therapy to help with difficulty speaking or swallowing, and occupational therapy to help with coordination and vision problems. Supportive care also includes palliative care to help manage symptoms such as pain, nausea, and fatigue, and to provide emotional support for the patient and their family.

Treatment options for DIPG are limited and typically involve a combination of radiation therapy and supportive care. Despite the best efforts of medical professionals, the prognosis for DIPG is poor, and most patients succumb to the disease within a year of diagnosis. It is important for patients and their families to have a thorough understanding of the treatment options available and to work closely with their medical team to make informed decisions about care.

Clinical Trials and Emerging Therapies for DIPG

Despite the poor prognosis of DIPG, research into new treatments is ongoing. Clinical trials are an important way to test new therapies and to improve the understanding of the disease. Participation in clinical trials can also provide access to new treatments that are not yet widely available.

Immunotherapy

Immunotherapy is a type of treatment that uses the patient’s own immune system to fight cancer. This is done by either stimulating the immune system to attack cancer cells or by engineering immune cells to target specific cancer cells. Some early-stage clinical trials are currently testing the effectiveness of immunotherapy in treating DIPG.

Targeted Therapy

Targeted therapy is a type of treatment that targets specific molecules within cancer cells, with the goal of stopping the cancer cells from growing. Targeted therapy is based on the genetic makeup of the tumor, and it is considered to be more precise than traditional chemotherapy. Some early-stage clinical trials are currently testing the effectiveness of targeted therapy in treating DIPG.

Oncolytic Virus Therapy

Oncolytic virus therapy is a type of treatment that uses a virus to infect and destroy cancer cells. The virus is engineered to specifically target and kill cancer cells while leaving healthy cells unharmed. Some early-stage clinical trials are currently testing the effectiveness of oncolytic virus therapy in treating DIPG.

Despite the poor prognosis of DIPG, research into new treatments is ongoing. Participation in clinical trials can provide access to new treatments that are not yet widely available and can also improve the understanding of the disease. The emerging therapies such as immunotherapy, targeted therapy, and oncolytic virus therapy are showing promising results and are being tested in clinical trials for DIPG. It is important for patients and their families to be aware of these new treatments and to consider participation in clinical trials as part of their treatment plan.

Palliative Care and Quality of Life for DIPG Patients

Palliative care is an important aspect of treatment for patients with DIPG. The goal of palliative care is to improve the patient’s quality of life by managing symptoms, providing emotional support, and addressing the needs of the patient and their family. Palliative care can be provided alongside other treatments, such as radiation therapy and chemotherapy.

Symptom Management

Palliative care focuses on managing symptoms caused by the tumor or the side effects of treatment. This may include managing pain, nausea, fatigue, and difficulty speaking or swallowing. Medications, such as pain relievers and anti-nausea drugs, can be prescribed to help manage these symptoms. In addition, physical therapy and occupational therapy can also help to improve the patient’s function and quality of life.

Emotional Support

Palliative care also includes emotional support for the patient and their family. This may include counseling and support groups to help cope with the diagnosis and the emotional impact of the disease. Social workers and chaplains can also provide support and resources to help with practical issues, such as financial assistance and end-of-life planning.

Caregiver Support

Caring for a child with DIPG can be physically and emotionally taxing for the family. Palliative care team can provide support and resources for the family to help them cope with the stress of caregiving. This may include counseling, respite care, and support groups for family members.

Palliative care is an important aspect of treatment for patients with DIPG. The goal of palliative care is to improve the patient’s quality of life by managing symptoms, providing emotional support, and addressing the needs of the patient and their family. Palliative care can be provided alongside other treatments, such as radiation therapy and chemotherapy. It is important for patients and their families to understand the importance of palliative care in managing DIPG and to work closely with their medical team to ensure that their needs are being met.

Coping with a DIPG Diagnosis: Support for Patients and Families

A diagnosis of DIPG can be devastating for the patient and their family. Coping with the diagnosis and the challenges that come with it can be difficult, and it is important for patients and their families to have access to support and resources to help them navigate this difficult time.

Support for Patients

  • Counseling: Patients may benefit from counseling to help them cope with the emotional impact of the diagnosis and the challenges of treatment.
  • Support Groups: Joining a support group can provide patients with an opportunity to connect with others who are going through similar experiences.
  • Child Life Specialists: These specialists can provide support to children and their families by helping them to understand their diagnosis and treatment, and by providing activities and resources to help them cope.

Support for Families

  • Counseling: Families may benefit from counseling to help them cope with the emotional impact of the diagnosis and the challenges of caregiving.
  • Support Groups: Joining a support group can provide families with an opportunity to connect with others who are going through similar experiences.
  • Respite Care: Respite care can provide temporary relief for caregivers, allowing them to take a break and recharge.
  • Hospice Care: Hospice care can provide support and resources for families during the end-of-life stage of the illness.

A diagnosis of DIPG can be devastating for the patient and their family. Coping with the diagnosis and the challenges that come with it can be difficult, and it is important for patients and their families to have access to support and resources to help them navigate this difficult time. Support is available to help patients and families deal with the emotional impact of the diagnosis, and to provide practical assistance with the challenges of caregiving. It is important for patients and their families to work closely with their medical team to ensure that their needs are being met and that they have access to the support and resources they need to cope with DIPG.

Sources & references used in this article: