Diffuse

The tumour is not well-contained as a solid mass. It grows out into other healthy tissue like a spiders web.

Intrinsic

Sometimes ‘intrinsic’ is replaced with the word ‘infiltrating’ and indicates how the cancer infiltrates healthy cells. In short, ‘I’ means ‘in’.

Pontine

Where the tumour is located in the pons, located in the lower brainstem. Responsible for vital functions such as breathing, sleeping, balance, blood pressure and heart rate.

Glioma

A term for tumours originating from glial cells that are found throughout the brain and support and protect the brain’s neurons. Glioma tumours can form in different areas of the brain.

DIPG tumours are 'primary', meaning they have not spread from elsewhere. The grow quickly; a child will typically have one month or less of symptoms before they are diagnosed, and then worsen rapidly. Caused by the growing tumour compressing anatomic structures in and near the pons.

The most common symptoms caused by pressure around the cranial nerves include:

• Difficulty controlling eye movements; such as a sudden lazy eye, the eyes not tracking with binocular vision, vertigo that causes nausea and vomiting.

• Changes in facial expression control and numbness.

• Changes in speech, such as slurring and sense of having a fat tongue.

• Issues chewing and swallowing.

• Sense of weakness in the arms and legs.

• Problems with walking and general coordination.

DIPG is normally diagnosed through advanced imaging, such as MRI.

Recent advances in neurosurgical techniques, such as stereotactic biopsy, means it’s more ‘safe’ to biopsy DIPG tumours.

The purpose of biopsy is to take a small tumour sample and have it tested for gene mutations — that may (and may not) help with treatment options (of which there are currently none, so I’m not sure why they do the biopsy).

Around the world, approximately 3,000 children are diagnosed with DIPG every year.

It normally strikes children between the ages of 4 and 11, however, it can occur at any age in childhood, and sometimes, rarely — into adulthood.

It occurs in males and females equally.

So, actually — no one knows why, or exactly how how DIPG occurs.

There is no scientific evidence that DIPG is a result of exposure to any environmental, lifestyle, inherited and/or hereditary factors.

Because it rarely affects adults scientists believe it’s linked to the developing brain of kids. NSS.

What is known is that something goes wrong with the process of cell reproduction, and a 'mutation' occurs. Which is typical of most cancers.

A number of chromosomal and genomic abnormalities have been reported for DIPG, including

• Histone H3 genes (H3F3A, HIST1H3B and HIST1H3C)

• Activin A

• PDGFRA amplification

• TP53 deletion

In DIPG, all current treatments (or Standard of Care — which is a term I’ve come to detest) is palliative.

Which is to mean, designed to preserve quality, and/or the extension of life — there are no current treatments designed to cure.

• Radiation is a first line of treatment after diagnosis with the hope to damage or knock back the tumours growth. While producing positive responses in more than 90 percent of children the reprieve is short-lived, lasting 6 to 9 months on average, before the tumour starts growing again.

• Steroids are really good at helping with the swelling that occurs around the brain with DIPG. Terribly addictive. Cause of Cushings Syndrome if used for too long at high doses. Don’t get me started, but concede them as a necessary evil in the treatment toolbox.  

• THC / CBD is experimental and frowned on by most doctors. In New Zealand we used The Cannabis Clinic to gain a prescription to the Tilray 10:10 product, which at the very least, helped with sleep during the ‘Living with Cancer’ chapter. At it’s best, it helped with brain swelling and staying off Dexamethasone (steroids).

• Experimental chemotherapy Multiple clinical trials have demonstrated that routine chemotherapy does not increase survival rates for this condition. Neat.

• Surgery is very rarely a treatment option because of the diffuse nature of DIPG. It would be like cutting a net curtain out of raw bread dough and hoping you don’t damaging anything on the way.

• Clinical Trial a total can of worms. And no trials open in New Zealand which makes the decision of whether to participate (or not) a relatively easy one. Even though you will look out to the world in hope and envy that other families can access trials — I will say that they don’t all appear to be created equally, and are designed to be of scientific value (eg: providing data), not all with the outcome driving treatment or survivorship.

With no known effective treatment, once the 30 rounds of radiation has finished, families are left to clinical drug trials, and/or using drug combinations that are unproven and natural supplements and changes in diet — often lead by parents desperate to help their kids.

Brain tumours remain the most common cause of cancer-related death in children.

DIPG is the leading cause of death from paediatric brain tumours.

A child diagnosed with DIPG today faces the same prognosis as a child diagnosed 50 years ago.

There is still no effective treatment and no chance of survival.

Only 10% of children with DIPG survive for 2 years following their diagnosis, and less than 1% survive for 5 years.

The median survival time is 9 months from diagnosis.

This section is not of facts. It’s just a brain dump of my thoughts. If you’re a parent dealing with DIPG in your family, I stress that your experience will be different to ours, and to the next kid, and the next.

Just like your unique kid before this, and during the climb up the DIPG mountain; so will their days and moments before passing.

Having a vague idea of what to expect is perhaps helpful. But IMHO, reading a parent’s experience in detail isn’t.

I’m not an authority on DIPG in a clinical sense, only what I experienced with Jemima. Sample size of one. So I’m not going to regurgitate clinical signs and symptoms in this section. This is also likely a form of self protection.

The death of a child is so very challenging (I’ve never written more inadequate words in my life); but having you by their side, and helping them will be the most meaningful thing you can do for them. This is a time when palliative care doctors can actually be quite helpful — even if other doctors been largely ineffective with treatments in the past.

You’ve already walked through the fire with your person in this life, and walking them to The Gates is the hardest request life can ever make of you.

But, you can do it - even when you absolutely think you can’t. And absolutely as much as you shouldn’t have to. You will. Because you love them so much.

On the practical side — hospice care can be invaluable in supporting the end of life choices, and maintaining quality of life.

Psychosocial support can also provide support if you feel like you’re the only person in the world who hurts this much.