ABOUT DIPG (Diffuse Intrinsic Pontine Glioma)
When Eva was diagnosed with DIPG, we had no idea what world we were about to enter. Naively, many assume that brain tumours can be treated with standard therapy or surgically, but this isn't the case.
DIPG stands for Diffuse Intrinsic Pontine Glioma.
Diffuse means to spread out through an area
Intrinsic means to be inside
Pontine stands for the area of the brain which is effected by DIPG – the Pons.
Glioma is the type of tumour which grows
The Pons is the area of the brain which sits at the top of the brainstem; the area of the brain which acts as a bridge from the brain itself, and the spinal column. The Pons is responsible for many of our most important bodily functions, including breathing, swallowing and heartbeat.
Pontine gliomas are highly aggressive cancerous tumours which spread very quickly throughout the area. Unlike other brain tumours, they form as a series of small tumours which intertwine within the nerves in the Pons, which means they cannot be surgically removed as surgery would lead to immediate loss of life.
Biopsy & Diagnosis
Most DIPG diagnoses are done via MRI, however occasionally, doctors will perform a biopsy. Though surgery isn’t possible, it has become more common for a biopsy to be performed due to recent discoveries of certain gene mutations in tumours, which may in turn effect treatment decisions when considering clinical trials.
Facts about DIPG
DIPG primarily effects children, with the average age at diagnosis being between 2 and 10 years old.
The prognosis for children diagnosed with DIPG is very poor, with the average survival time post-diagnosis being less than 11 months. Of all children who are diagnosed, less than 10% survive for 2 years, and less than 2% survive 5 years. There is no cure.
Between 100 and 300 children are diagnosed with DIPG every year in Europe and a similar number in the US. In the UK, 20 to 30 children are diagnosed each year, meaning that every 12 days, a child and a family begin this terrible journey.
Upon diagnosis, in the UK, the only offered treatment is high doses of Dexamethasone to reduce swelling, and standard Radiotherapy. In some cases, Radiotherapy reduces the overall mass of the tumour, which provides temporary respite from symptoms, however in ALL cases, the tumour returns, often more aggressively.
Chemotherapy is not considered effective as traditional chemo drugs fail to cross the protective barrier which surrounds the Pons (known as the Blood Brain Barrier).
Outside of the UK, there are 70 active clinical trials, across 17 countries, however none of these are accessible via the NHS, and so accessing these trials is costly, and often therefore out of reach for most families. For example, a current trial in the US using Car T-Cell therapy costs a family in the UK in excess of $1 million to access.