Diffuse Intrinsic Pontine Glioma (DIPG) is an aggressive brain tumor found in a part of the brain stem called the pons, located in the center of the brain. Children with DIPG commonly experience double vision, reduced eye movement, facial weakness or asymmetry, and arm and leg weakness. They also have problems with walking, coordination, speech, chewing, and swallowing. As the tumor progresses, it also interferes with breathing and heartbeat, which ultimately results in the child’s death.
DIPG affects children almost exclusively. Approximately 1,000 (possibly up to 4,000) children are diagnosed with DIPG each year. These children are typically between the ages of 4 and 11. DIPG accounts for roughly 10-15% of all brain tumors in children.
DIPG is a disease which strikes at the heart of childhood and it is in desperate need of a cure. It is even suggested that a cure to DIPG might result in a cure for almost every other type of cancer. For this reason, we have to start now.
What causes DIPG?
No one knows what causes a child to develop a DIPG tumor. Some cancers are caused by particular genes that a person inherited from his parents, but there is no evidence that links DIPG to any specific inherited genes. Other cancers are caused by environmental factors (such as smoking or exposure to chemicals or radiation), but there is no evidence that links DIPG to any environmental factor.
What is the treatment for DIPG?
Surgery - Surgery is not an option for two reasons. First, the tumor is located in the pons, which is in the center of the brain. A surgeon could not get to the tumor without damaging the surrounding portions of the brain, likely causing severe neurological damage and even death.
Second, even if the tumor could be accessed, total removal would be impossible. A DIPG tumor is not a solid, well-defined mass like some tumors. Instead, the tumor cells spread out between the healthy brain cells. Because the tumor cells could not be entirely removed without also removing the healthy cells, even if surgery were performed, the remaining cells would continue to divide and the tumor would soon return.
Radiation - Radiation is part of the standard course of treatment for DIPG patients, as it is the only form of treatment that has proven benefits. For roughly 70% of DIPG patients, radiation causes the tumor to shrink, which provides relief from many of the symptoms associated with DIPG.
The benefits of radiation, however, are only temporary. Even for those Patients whose tumors shrink during radiation, the treatment does not increase their chances of survival, and the tumor begins to grow again within months. Because DIPG Patients who do not undergo radiation have a median survival length of roughly 5 months, radiation therapy typically extends a Patient’s life by about 3-4 months.
Chemotherapy - In the past 30 years, DIPG patients have participated in more than 250 clinical trials. These trials have involved a wide array of chemotherapy drugs – sometimes alone and sometimes in combination with other drugs. None of these studies have shown any benefit in either the likelihood of survival or the median length of survival.
The brain is in its own "bubble" protected by the Blood-Brain-Barrier. This defense mechanism keeps out infections and protects the brain from anything attacking it from the outside. Ironically, it also locks out the drugs that are trying to penetrate it to fight the tumor within the brain. Chemo drugs' molecular makeups are too large to consistently pass through the Blood-Brain-Barrier, making the dosages needed to ensure enough penetrates the barrier too high for a body to withstand its toxicity - especially a child's body.
What is the prognosis for a child diagnosed with DIPG?
Medical advances in the past 40 years have greatly improved the survival rates for children diagnosed with most types of cancer. For some cancers, the medical advances have been extraordinary. For example, the survival rate for children with acute lymphocytic leukemia has increased from less than 10 percent in the 1960s to nearly 90 percent today. Overall, the survival rate for children with cancer is about 83 percent.
However, children diagnosed with DIPG have had no increase in their chances of survival. The typical prognosis is 5-9 months, with "treatment".
Brain tumors are now the most common cancer and cause of cancer-related death in children less than 15 years of age. They are the most common solid tumor in children – accounting for approximately 25% of all childhood cancers. DIPG tumors are the most common brainstem tumors in children, representing approximately 75-80% of all pediatric brainstem tumors.
Approximately 200-300 Patients are diagnosed with DIPG in the USA per year. Worldwide it is in excess of 1,000, though it is thought it could be as high as 4,000 due to the fact that many Patients die before proper diagnosis.
The median age of Patients with DIPG is approximately 6-7 years old.
The male/female ratio of DIPG patients is 1:1.
Median overall survival for DIPG patients is < 1 year – ranging from 8-11 months.
2-year survival is approximately 10%.
5-year survival is <1%.
This is a horrible disease and one that urgently needs a new research approach. When you consider the current treatments for this disease, you realize how horrific the current approach is. Presently they are trying every type of Chemotherapy they have, and are basically “just giving them a go” – this is not science. This is a business model to generate income for the Pharmaceutical Companies and the Researchers.
The Patients are very young – in most cases less than 6 years of age – and they are being hit with the most aggressive and toxic Drugs on the market. They have virtually every toxic drug thrown at them – each of which have no proven benefit in prognosis or quality of life. We had one mother tell us that by the time the doctors had finished with the treatments, she could not physically recognize her own daughter – these drugs had disfigured her so much and had no benefit at all.
Our approach is different - very different - as our Product produces no side effects at all. We have shown that we can reduce the tumor, and most importantly we increase the life expectancy and quality of life of the children.
Our first DIPG patient has been with us for over 8 years and is well, and although she has some problems because of the Radiation given when she was first diagnosed, she is going to school, living an active life, and get stronger every day. She was given a prognosis of 6 weeks to 9 months to live – and that was 8 years ago. Remember, less than 1% live 5 years past diagnosis, and this little girl is beating those odds with no treatment other than our Product for the last 8 years.
These results are unheard of in the DIPG field, and this is why we need to make this treatment option available to other Patients. With zero side effects, proven results, and increased quality of life, more research on this treatment could change the course of the DIPG disease and help the thousands of children dying every year.
Until now, there was no help, or hope, for these kids. This is truly ground-breaking research we are doing and with continued success, this could be a massive breakthrough – not only with DIPG, but in every other form of Cancer Treatment.