Has it really been a year since my last post? Wow...

Well, the unfortunate news from me here today is that my beloved mother died last Saturday (1115/2014). She was so strong and positive throughout her 20-month battle with this atrocious disease, but the widespread metastasis produced multiple systems failure which her precious body couldn't combat any longer. My heart is broken. Today will be the first of many Thanksgivings without her, and her 60th birthday would have been 1 week from today.

The usual things people say to me are that at least she's no longer suffering; now she's at peace; and she'll always be with me...all this is true and encouraging, but the pain of her loss is still very acute.

For what it may be worth to future readers of this post, here's a brief history of my mom's process. She wanted her "case" to be used to help support the medical community, others who might learn from her experience at all, and people making decisions about treatment options. I'm relaying this in that spirit.

*March 22, 2013: Diagnosed with Stage IV cholangiocarcinoma; mets to liver, lung, bone and lymph
*April, 2013: Neurosurgery procedure to remove diseased C4 vertebrae, replaced with titanium cage
*May-Aug, 2013: Radiation on spine to treat bone mets to thoracic and lumbar regions
*May-Jul, 2013: Started first chemo: Gem/CIS -- completed 6+ rounds; minor liver tumor shrinkage.
*Jun-Sep, 2013: Chemo-embolization and ablation treatments.
*Aug-Sep, 2013: Started second chemo: 5-FU...also completed 6+ rounds. Didn't respond well.
*Sep-Nov, 2013: Started third chemo: FOLFOX...toughest one yet. Lung mets became aggressive.
*Nov, 2013: First Y-90 procedure. Results showed dramatic reduction in tumor markers.
*Jan, 2014: Second Y-90 procedure. Sustained liver tumor shrinkage but caused stomach perforation.
*Jun-Jul, 2014: Prescribed Tarceva for continued tumor shrinkage. Lungs worsened.
*Jul, 2014: Began routine paracentesis for abdominal ascites drainage. Averaged 3 Liters, 1-2x week.
*Jul-Sep, 2014: Prescribed Irinotecan to combat lung mets. Didn't respond well. Declined quickly.
*Oct-Nov, 2014: Constant coughing from lung fluid buildup. Thoracentesis drained 750mL 1x/week.
*Oct, 2014: Took a break from treatments/procedures to contemplate next move.
*Nov, 2014: Met with dr. to discuss immunotherapy. Not proven for CC, only melanoma. $75k/trtmt
*Nov, 2014: Admitted to ER with shortness of breath; oxygen levels 94 (out of 100). Put on O2.
*Nov 9, 2014: Declined to pursue further treatments and elected hospice care (in-home).
*Nov 14, 2014: Hospice was approved and came in to provide a hospital bed, morphine, etc.
*Nov 15, 2014: Died at home, surrounded by her family. She was 59.

I've left out the many, many MRI's, x-rays, CT scans, blood transfusions, blood tests, and visits to specialists, hospital and ER teams. Often her hemoglobin was so low that she required blood transfusions in order to qualify for her next round of chemo. She was greatly fatigued, but never lost optimism or hope right up to the very end. Mind-body connection was always positive and strong. She was a devoutly faith-filled Catholic and this sustained her. She prayed daily for all those afflicted with cancer and chronic pain. She ate very carefully...was essentially on a vegan diet (no meat, soy, dairy, gluten, sugar, or alcohol). She also juiced regularly.

Her care team was primarily supported by:
-Dr. Saeed Sadeghi (Hematology Oncology, UCLA)
-Dr. Lisa Chaiken (Radiation Oncology, Providence St. John's Health Center)
-Dr. Edward Lee (Interventional Radiology, UCLA)
-Dr. Antoni Ribas (Immunotherapy, UCLA)
-Dr. Timothy Weiss (Hospice/Palliative Medicine, UCLA)
-Dr. David Wallenstein (Hospice/Palliative/Pain Management, UCLA)
-Skirball Hospice & Palliative Medicine

If there are any detailed questions or further inquiries that may assist others, I would be happy to share anything that might be of help.

I wish all those who struggle with this terrible illness, and those who love and care for those afflicted, the most heartfelt love, strength, courage, and fearlessness as you pursue your own treatment options and work tirelessly for a cure.

~Stephanie

Hi everyone -- just thought I'd give another update on my mom's condition, since I haven't posted in awhile.

Chemo regimen of FOLFOX continues.  She tolerates it reasonably well, but it makes her very cold and very fatigued.  A walk around the block with the dog zaps all her energy reserves.  With every other chemo infusion she requires a 10% strength reduction because of low hemoglobin, and she has had 1 blood transfusion due to low levels.  My sister (blood type match) has begun banking blood.  Mom has begun losing her hair; not in chunks, but it's very thin now in comparison to her norm.  Her doctor gave her a prescription for a human hair wig (couple thousand dollars! yikes!).  I'm planning to get it for her birthday.  Due to almost constant digestive distress, she's on a completely vegan and gluten free diet now (no meat, dairy, soy, or gluten) and she juices regularly.  Weight appears low to me (she was already very thin to begin with), but does not appear visually jaundiced or yellow, so that's reassuring. 

At this time the most apparent "symptom" is really her ever-increasing abdomen.  Fearing this was ascites (fluid build-up) which would need to be drained, and which would be symptomatic of the early stages of organ failure, she received scans to determine how much fluid existed.  These scans showed that there's virtually *no* fluid, but it's instead her extrahepatic tumor that has grown to 10cm in diameter (larger than an adult fist; about the side of a grapefruit) and is causing her belly to protrude.  On her already slight frame, this makes her appear about 4 months pregnant.  About a month ago, the weight of the tumor caused her stomach to collapse.  As a result, she no longer feels hunger pangs, and needs to remember to eat at traditional times.  She also gets full very quickly since her stomach is so compressed by the tumor.

So the question became, what to do about this tumor?  It's huge and growing.  Her interventional radiologist suggested a procedure called Y90.  This is apparently a liver cancer therapy that consists of millions of small glass beads containing radioactive yttrium-90 being injected through a catheter into sites that are immediately local to tumor growth.  The beads emit localized radiation, which is more effective and more targeted than low-dose external radiation therapy.  The beads kill the tumor tissue, while preserving the healthy liver tissue.  It's pretty genius, really.

The last couple of months have been spent battling with the insurance company over coverage denials for the essential Y-90 treatment that was recommended by her medical team (interventional radiologists, nuclear medicine, primary oncologist, etc.).  Talk about stress!  At long last (and on the 3rd and final appeal attempt!) she was finally approved for the Y90 treatment (hooray!).  Preparation required her to be off chemo for at least 4 weeks leading up to the treatment date.  During this time she had various tests and scans to "map" the blood vessel pathway which the millions of small glass beads containing radioactive yttrium-90 would be injected into.  Blood vessels leading to her pancreas and stomach were cauterized to prevent the beads from traveling there.  A "dress rehearsal" procedure was done 2 weeks ago where, after many hours of blood vessel mapping, beads containing contrasting dye were injected along the pre-determined pathways and deposited near the intended tumor sites.  She was then scanned to see if the beads were positioned properly and where they needed to be.  Everything looked correct, so the "real" procedure was scheduled for 11/14 (last Thursday).  It was a success!  The medical team advised that the procedure went "text book" well, and they positioned the radioactive beads exactly where they intended to.  This is an outpatient procedure, and pain was managed with Versed.  Soreness from the catheterization (entry is up through the main artery in the leg/groin area) lasts for about 2 weeks, so she's still quite sore, but has already observed belly size reduction due to the necrosis of the tumor tissue.  This is of course wonderful news; the treatment appears to be working so far.  The tumor tissue is supposed to continue dying off over the next few months, and she'll be scheduled for resection surgery around February.  (She was determined to be unresectable a few months ago, so this is truly meaningful progress).

Another update: the results of her genetic sequencing report indicate that she has NO abnormalities among the current 'known' mutation pathways.  However, she's still going to be tested for Lynch Syndrome to rule out a genetic predisposition to colorectal and/or GI cancers, given her father's diagnosis of colon cancer at age 57 which brought about his death at age 59 1/2 (his had mets to liver and lung).  Will advise more on that once the results are in.

I held a fundraiser at work and we raised $2,500 for The Cholangiocarcinoma Foundation, which was great for bringing awareness to bile duct cancer.  The mother of one of my colleagues was also recently diagnosed with this very disease, which certainly seems to suggest that incidences are rising.

My mother's attitude remains resolute, happy, positive, upbeat, grateful, thankful, calm, and hopeful.  If attitude is everything, she's nailing it!

Will post again once I have more to say; this about brings everything current with my family's situation.

Hang in there, everyone!

FOLFOX regimen is going ok. She's tolerating it MUCH better than Gem/Cis. Have been told FOLFOX is the new standard of treatment for CC. Far fewer side effects. Radiation is going ok too - just some esophageal soreness from the pass-thru on the sternum treatment. Continued abdominal distension which looks odd pretty much continuously. Food cravings are limited just to comfort foods, soup mostly. There was a chance this radiation round could be reduced from 15 treatments to 10, but new X-rays taken last week prompted the full 15 to be taken after all.

The USC doctor asked for blood tests showing tumor markers, which hadn't been ordered since original diagnosis in late March. At that time, tumor markers were 248. Bloodwork last week showed reduced tumor markers down to 134 so clearly something has been working, though its unclear if that's a result of the neurosurgery or an indicator of the chemo efficacy.

Quality of life is pretty much as normal right now; days are just peppered with dr. and treatment appointments. No real prompt to stop aggressively pursuing treatment at this point. She still has strength, hope, optimism and a great attitude. As do we.

Hi all ~ haven't posted since my original, and there are some updates to share about my Mom.

After recovery from neurosurgery to remove the diseased C4 vertebrae (bone/spine has been her primary metastasis from CC), 10 rounds of daily radiation targeted on the C4 region was completed. Side effects were minimal, just some moderate nausea that was easily controlled with Zofran, and a mild sore throat (frontal region of the C4 treatment site).

Then chemo began (Gem/Cis) - suggested regimen was 6 cycles initially, and Mom made it through 5 before electing not to complete the 6th. Side effects included daily, constant nausea which Zofran didn't help with a bit; inability to keep food down resulting in weight loss; no appetite; GI distress; abdominal tenderness; body aches and flu-like symptoms; sleeping difficulties; vein pain which prompted the installation of a collarbone port; numbness in left toes/ball of foot and calf; etc. Nonetheless, her attitude has been steadfastly positive and upbeat -- and remains so.

On Monday, 7/8 she had the first round of follow-up tests (xrays, PET scan, etc. etc.) since diagnosis 3/21, spinal surgery 4/5, radiation 4/25-5/7, and first cycle of Gem/Cis chemo (5/13-6/28). We all had high hopes for improvement, but results conveyed in appointment 7/9 with primary biliary oncologist showed a "mixed bag". Generally, new lesions or an increase in size. Several have decreased in size or stayed the same. Continued mets to spine showed several that need immediate attention (L7, L10, L11, Sacrum, Sternum, Thoracic areas, etc.). One is pressing on the spinal chord. New symptoms include upper back skeletal pain, difficulty rising/getting out of bed, pain when turning/twisting from the waist. Gem/Cis was doing something, but barely.  Suggested switch to either [1] palliative care, or [2] different chemo regimen (FOLFOX). She's a fighter and is not in the mindset of palliative care at this time, so we're switching to FOLFOX and setting up a new chemo regimen. Appointment with radiation oncologist 7/10 included new tattoos/setup to begin new (second) round of radiation today (7/11).  Will include 15 cycles, with simultaneous chemo.

Radiation oncologist is at St. John’s (Santa Monica).  Primary oncologist is at UCLA, who referred her to the Norris Cancer Center at USC since he’s limited only to UCLA programs.  Met with a doctor there this morning to look at her case and see what he thinks.  Will report back.

Thanks for ongoing interest and concern.

Thanks, Lainy. 

As to pain, her pain level immediately after neurosurgery yesterday was a 10+ on the 10 scale. (She had all 5 of her children without anesthesia and has an incredibly high threshold...had been managing it for the bone mets during the prior 2 weeks with just 3 Advil a day, if that's any indication). She was given hydromorphone (Dilaudid) yesterday after surgery, having received some morphine just prior. Then started to feel nauseous so was given something else for nausea. When I left the hospital last night after 2am her pain was down to a level 5, though she didn't think she could sleep with it that high (and didn't, largely due to the oxygen machine beeping continuously throughout the night). Spoke with her this morning and her pain is managed now with no nausea, so that's clearly good. Expect to go back there to visit in a couple hours; she won't be released until Tuesday or Wednesday (5 days inpatient at St. John's...thank heavens for insurance). 

Regarding pain prior to all of this, she'd been walking around the house with a heating pad on her abdomen, and had chronic neck/shoulder pain (which we can now attribute to the liver tumors, and her diseased C4 vertebrae which the neurosurgeon said was so soft, it was like the inside of a bagel (his actual quote, however unmedical - lol). 

She wants to be aggressive with her treatment, and has the most amazing upbeat, positive, optimistic, no fear attitude.  She has an incredibly strong faith, and I know this is all helping her carry through (...mind/body connection).  Her optimism is humbling.  I don't have a fraction of her strength; I've cried myself to sleep every night with all this.

I ordered 25 CC bracelets from the Foundation and now everyone in my family is sporting them in solidarity for her.

Hoping, praying, caring, trying....we'll do anything we can for her.  Thanks, all.

Hi all ~

Two weeks ago, my healthy and beautiful 58-year old mother of 5 children (ages 22-33) was diagnosed with Stage IV cholangiocarcinoma with mets to her liver, bone, lung, and lymph. We've been told that neither resection nor liver surgery are possible due to tumors on both lobes, though I wonder if this is really true given the liver's ability to regenerate, and given her strong underlying fitness and health baseline (discussed below). Also clearly not a transplant candidate given the Stage IV. When a scan early this week revealed more bone disease than originally thought, she was rushed into an emergency night-time MRI, and the next day scheduled for neurosurgery to remove her completely diseased (and fractured) C4 vertebrae and surrounding discs, replacing them with a titanium cage to which the C3 and C5 will ultimately fuse. This procedure was successful yesterday, though during it her esophagus incurred a tiny puncture which received 2 stitches so she can't eat or drink for 5 days (currently 115 lbs., 5'4"). Once she recovers from this (2+ weeks?) she'll start a traditional front-line chemo course for CC. (Likely the 2-drug combination of Gemcitabine + Cisplatin (Gem/CIS) though discussions are also being had over FOLFOX, and 5-FU).  One oncologist suggested only palliative care for containment and pain-management, and the other suggests an aggressive "all out" approach to try and kill all the cancer, though all have been careful not to use the word 'cure'.  One of her several UCLA oncologists (this one specializing in hepatology and biliary issues) is consulting with Dr. Javle at HDAnderson in Houston, and they're discussing treatment options. She may simultaneously have radiation to treat some additional bone spots in her thoracic and lumbar regions. Pathology reports, MRI's, PET, CT, upper endoscopies, barium imaging, x-rays, etc. have ruled out the usual primaries (breast, brain, lung, liver, colon). Belief at this time is that the primary is either CC, or it may fall into the range of "3-4% with undiagnosed primaries".

We've recently learned that she was accepted into an anti-PD1 trial, which is not chemo but leads the immune system to fight the cancer. Tissue samples from 2 separate liver biopsies and the C4 vertebral tumor are also being sent to GenenTech and the Caris Foundation for evaluation.

Interesting family history background -- Her father was diagnosed with Stage IV colon cancer at the age of 58 (same age of her current diagnosis), with mets to his liver and lung. He died from these complications at age 59 1/2. His cancer was treated with 5-FU, one of the same chemo's they're considering for my mom's treatment today, 23 years later.  (Is this really the best course? Had medicine not advanced beyond this in 23 years?).  It has been suggested to us children that we be genetically screened for Lynch Syndrome, and/or request routine PET scans in our mid-thirties onward for early identification, should anything exist.  So far, the apparent trend with my mom and her father suggests a genetic predisposition, though his parents lived into their 80's on both sides (both dying of heart issues), her mother is still alive, and her 2 brothers (53 and 55) have no known issues of this nature though are pursuing testing at this time.  Any known links with Lynch Syndrome and CC diagnosis?  So far, doctors are mixed on this.

She has maintained a gluten and dairy free diet, takes many herbal and other supplements (including recancostat currently), avidly exercises, has utilized acupuncture and chiropractic throughout her life, and has always been proactive about her health and wellness (colonoscopies beginning at age 35 - always clear, etc.).  No routine preventative exams revealed any of this; there were no liver enzyme spikes, or other abnormalities. She discovered a lump in her stomach during a Pilates class 3 weeks ago, and her PCP referred her to a specialist which revealed the lump to be liver tumors.  Here we are today.

I guess what I'm looking for from this board are tips, suggestions, advice, help, support, cutting-edge treatments, research, or any other things people who have loved ones with this rare cancer typically seek.  I've never done this before and it's heart-breaking, scary, and sad.  I'll continue to post updates and her treatments, procedures, etc. in case it's of interest or help to others out there.  Thanks and best wishes for health to all.