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Living with cancer· Appointment prep

Appointment sheet: Erdheim-Chester disease

One page to bring and write on: your details, the questions for Erdheim-Chester disease plus your own, the words you may hear, what to bring, the treatments the standard of care names, and room for the answers and agreed next steps. What you type stays in this browser. Print it or save it as a PDF. New to all this? Start with the first 60 days. Orientation, not medical advice.

Tick the questions to print

All of this cancer's questions start ticked. Untick what does not apply; ticks are kept in this browser. .

Your own questions

Shared with the prep pack, so questions you add there appear here too.

Print or save as PDF

Use (or Ctrl+P, Cmd+P on a Mac). To keep a copy, choose Save as PDF as the destination in the print dialog. Only the sheet prints; the controls stay on screen. Your typed notes print where you typed them; empty fields print as ruled lines to write on.

Appointment sheet

Erdheim-Chester disease

Prepared with OnCo (onco.cc/prep/erdheim-chester-disease/). Orientation, not medical advice; your team knows your case.

My details

Name
Date of appointment
Hospital and clinician
Who is coming with me

What I know, what is unclear, changes to discuss

Saved in this browser
What I know so far
What is unclear to me
Changes since last time

My questions

17 on the sheet
Newly diagnosed
  1. 1.What is my exact diagnosis, stage, and grade, and which tests established them?
  2. 2.Which biomarkers have been tested on my tumour (for example BRAF V600E in tissue and plasma cell-free DNA, MAP2K1, ARAF, NRAS, KRAS, PIK3CA and kinase fusions on sequencing, CD68 and CD163 positive, CD1a and langerin negative histiocytes with Touton giant cells, FDG-PET/CT for extent and response, Cardiac MRI, brain MRI and pituitary function), and what were the results?
  3. 3.Which subtype is my cancer, and does that change the recommended treatment?
  4. 4.Is germline (inherited) genetic testing recommended for me or my family?
Diagnosis
  1. 5.For my situation (diagnosis), which of the standard options do you recommend and why?
BRAF V600E-mutant symptomatic disease
  1. 6.For my situation (braf v600e-mutant symptomatic disease), which of the standard options do you recommend and why?
  2. 7.Am I a candidate for Vemurafenib, Dabrafenib + trametinib, Cobimetinib, and what side effects should I expect?
BRAF wild-type symptomatic disease
  1. 8.For my situation (braf wild-type symptomatic disease), which of the standard options do you recommend and why?
  2. 9.Am I a candidate for Cobimetinib, and what side effects should I expect?
Second-choice or intolerant
  1. 10.For my situation (second-choice or intolerant), which of the standard options do you recommend and why?
  2. 11.Am I a candidate for Interferon alfa-2a/2b, Cladribine, Methotrexate, and what side effects should I expect?
Asymptomatic bone-limited disease
  1. 12.For my situation (asymptomatic bone-limited disease), which of the standard options do you recommend and why?
Any stage
  1. 13.Are there clinical trials I could join, for example of Cobimetinib, Vemurafenib, Dabrafenib + trametinib?
  2. 14.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
  3. 15.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
  4. 16.I read that “Kinase inhibitors are usually needed for life and relapse follows their withdrawal”. How does that affect my plan?
  5. 17.I read that “Long-term cardiac, skin and secondary-cancer effects of indefinite BRAF and MEK inhibition are unknown”. How does that affect my plan?

The words I may hear

  • Retroperitoneum: The space at the back of the abdomen, behind the gut's lining, holding the kidneys, adrenals, pancreas, aorta and the para-aortic lymph nodes.
  • BRAF V600E mutation: A single spelling change in the BRAF gene that jams a growth switch permanently on.
  • Driver mutation: One of the few mutations in a tumour that actually causes it to grow.
  • Circulating tumour DNA (ctDNA): Circulating tumour DNA (ctDNA) consists of fragments of DNA shed by tumour cells into the blood, detectable with sensitive sequencing.

Tests and results to bring

Diagnosis: Biopsy of an accessible lesion with BRAF V600E testing and broader sequencing; FDG-PET/CT, cardiac and brain MRI, endocrine assessment.

Biomarker results to ask for: BRAF V600E in tissue and plasma cell-free DNA (diagnosis and monitoring), MAP2K1, ARAF, NRAS, KRAS, PIK3CA and kinase fusions on sequencing, CD68 and CD163 positive, CD1a and langerin negative histiocytes with Touton giant cells, FDG-PET/CT for extent and response (long bone uptake), Cardiac MRI, brain MRI and pituitary function, Blood count and myeloid mutation panel for an associated clonal myeloid neoplasm.

Scans and tests linked to this cancer: Active surveillance, Comprehensive genomic profiling, FDG PET, Liquid biopsy (ctDNA), MRI.

Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.

The treatments I may be offered

From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.

Answers and next steps

Saved in this browser
What I was told
Agreed next steps, dates and who to call