Updated: Mar 7
Updated: March 7, 2023 ( I will keep on updating and add more info as I come across it)
I am not an expert and thus will share the best summary from experts that I've seen after 3 months of reading and educating myself.
Source for the data and image: https://4sqclobberscancer.com/reference/dipg-suggested-best-practices-guide/ There you can find a link to the printable pdf with the best practices and copy of the text below:
A quick note on DIPG vs DMG (see historical graph below). DIPG is a subset of DMG: DIPGs are the same tumors that start and/or are located in the Pons (see pic above). DMG is a wider definition that cover the whole CNS (Central Nervous System) and up to the Thalamus, including the Pons. Both terms are often used interchangeably as the DMG is a new classification (just a couple of years old)
"NEW BEST PRACTICES TO KNOW UPON DIPG DIAGNOSIS
Dr. Sabine Mueller
The most important first step is radiation to start relatively quickly (to preserve as much function as possible during the honeymoon period that many patients have, or to improve any functional loss before its permanent) -for some children with less symptoms this might be less critical.
·There is no documented difference between proton or photon radiation for DIPG/OMG, so whatever can be offered sooner should be considered. Radiation is standard of care used for many years and can generally be performed at most hospitals safely.
Steroids should be weaned as quickly and safely as possible-when and how much to use depends heavily on the clinical presentation and should be discussed with your team. Consider with your team whether it is necessary to start steroids proactively. Steroids do not inhibit the tumor from progressing, steroids help alleviate symptoms caused by inflammation surrounding the tumor. Learn the pros and cons of steroids early.
Biopsy should be performed by someone who has experience with the procedure. If a patient is critically ill and needs to start radiation therapy quickly, a biopsy can also be performed after completion of radiation therapy. Biopsies are necessary to confirm the diagnosis and determine the specific DIPG subtype. Having tissue available is becoming more and more important as many trials require information from the biopsy or actual tissue for enrollment. Be sure that the tissue is used for relevant testing - often the tissue is fairly limited. Data generated from the tissue can easily be shared with others. [Note: Other sources have shown data and claim that DIPGs without a biopsy can be miss diagnosed in 10 to 30% of the cases}
Some clinical trials start WITH radiation, but most clinical trials start after radiation - all trials are listed on clinicaltrials.gov but be sure to discuss these options also with your team. It is challenging to sort through the different options alone.
Ask for a second opinion if you want to- it is highly recommended to get second opinions from different centers with experience treating DMG, as centers have different options to offer. Some trials are only available in certain places and providers have variable levels of comfort taking care of ALL diagnoses, including DIPG.
If your insurance is not covering all needs, talk to your social worker about secondary insurance options (i.e. Medicaid).
Palliative care is often a helpful resource in the beginning to coordinate care, provide emotional support, etc. and palliative care can be activated WHILE on therapy in pediatric and plays a different role than classic "HOSPICE" end of life care in adults. Palliative care teams can really make your life much easier
We will make more progress when working as a team- data sharing for a rare disease like DIPG is critical. One example is the DMG ACT. This group combines researchers around the globe to develop the best next therapies for children with DIPG. Anyone can join and we aim to make data available as soon as possible to enhance research further.
Consenting to share data with the Children's Brain Tumor Network allows researchers to learn from DIPG patients to drive treatments."
If you have 7 more minutes: this is a short Video where Sabine Mueller, MD, PHD, MAS explains what to do and prepare for first.: https://youtu.be/YyaG4XaIkZk
If interested in the history of how DIPG and the DMG terms have been classified and diagnosed since 2007. It explains a bit why there are still many misdiagnosis, especially in centers not specfilied in pediatrics and DMG. Image and data from: https://www.frontiersin.org/articles/10.3389/fonc.2022.1082062/full