Wednesday, October 31, 2012

Day III

Jon has stayed ahead of the nausea by requesting medication at the first sign of unease. Although his appetite is suppressed, he is eating well. He will come home tomorrow evening without a pic line (the chest catheter will be inserted next week). Pic lines are prone to infection--not having one to worry about while he is resting next week will be a blessing... Additionally, we have received many helpful nutritional tips from cancer survivors and caregivers. I am excited to pump Jon full of walnuts, pomegranates, kale, mushrooms, wheat grass, filtered water...and Spiz... On a side note, Josiah made a trip to the ER today. He has a few forehead stitches and an injured leg, but he is doing well. All things in perspective, we are grateful for this day...

Tuesday, October 30, 2012

Day II

Jon is feeling slightly fatigued and nauseous but overall, he is handling the initial dose well. He is committed to walking three times a day to avoid fluid accumulation. He reads every comment on this blog, facebook, and the youcaring site. All of your words, prayers, and support are an integral part of his strength and healing. Our gratitude overflows...

Monday, October 29, 2012

Day One

Jon was admitted to the hospital at 9am and spent most of the day preparing for neoadjuvant chemotherapy (chemotherapy used in patients with localized cancer in order to decrease the tumor prior to treatment by other modalities): cardio exam, pic line, IV, advanced directive paperwork....At 4:30, the meds were finally rolling. He is receiving Doxorubicin and Ifosfamide. Doxorubicin is given in a slow drip for 72 hours; and the Ifosfamide is given four hours a day for four days. His oncologist, Dr. P, has prescribed the maximum dosage allowed. Not only is chemo attacking the tumor, it is hunting for tumor "spies" throughout Jon's body. If interested in learning more about chemotherapy, the website www.chemocare.com is great. Jon is in good spirits--his pulse was lower today than any doctor visit over the last month. He is at peace with treatment and ready to fight with everything he has...His blood count will be lowest in 7-10 days...this is called "nadir"...From that low point, Jon's body will begin to rebuild/recover until Round 2 begins on November 19th.

Friday, October 26, 2012

Decision

Jon will be admitted to Baldwin Park Hospital at 9am Monday to begin chemotherapy. This decision arrived after many deliberations. All doctors agree there is no "right" answer, but we decided to go with chemotherapy before surgery for a number of reasons. 1) Dr. Sant P. Chawla, http://www.sarcomaoncology.com/s_cv_Chawla.html , strongly recommended chemotherapy before resection. His opinion arrived at our door through some glorious circumstances; and we felt we should listen. This decision will give Jon's team an opportunity to "observe" whether or not chemo is working on the tumor. If it doesn't work, there are a number of other "options" they can attempt. Without a biological marker, there is no way to know. Jon will stay in the hospital Monday-Thursday or Friday and then return home to rest for two weeks. He will receive chemotherapy treatment for seven hours each day. This is all we know...and we are walking forward in faith that this is a good decision. This is an overwhelming time; and we have not fully ingested the reality Jon has cancer. We are grateful for our village; and we move forward with open hands.

Conversation

Jon had a bone marrow biopsy yesterday with Dr. P at Baldwin Park. Ouch. His diagnostic testing is complete. Hopefully, the marrow will be clear of cancer cells. This is likely. Many have asked why there is such discussion about multimodal treatment. Here is the Cliff note version: Pleomorphic rhabdomyosarcoma is a rare tumor comprising 1-5% of adult soft tissue sarcomas; and sarcomas make up 1% of all cancers. This is EXTREMELY EXTREMELY RARE. We asked Dr, P (the Sarcoma chemotherapy specialist in southern California) how many PRMS cases she has treated. The answer was one. Adult-type rhabdomyosarcoma is defined as a soft tissue sarcoma showing pleomorphic or spindle cell pattern, with cells showing rhabdomyosarcomatous differentiation that is hyperchromatic and atypical nuclei, eosinophilic cytoplasm with scattered rhabdomyoblasts and positivity in immunohistochemistry for striated muscle markers: desmin and myogenin. Our tumor fits this definition. Dr. M recommends removing the tumor with wide margins (1-2 cm) and then beginning aggressive chemotherapy/radiation. His argument is to remove the "hot spot" and not allow the cells in the tumor to adapt to chemotherapy. The down side of doing the surgery first is that there is no biological marker to see if the chemotherapy is working. It is documented that Pleomorphic Rhabdos can be resistant to chemo. Also, a larger chunk of Jon's gluteus will have to be removed leaving a permanent hole. The plus side is you have removed a malignant mothership. The doctors at Kaiser wanted to start chemotherapy first for reasons listed above. Given Dr. Mascheranas does not work at Kaiser, his treatment protocol can only arrive as a "suggestion". The doctors at Kaiser must agree on a treatment plan before we can move forward. There is also discussion about the chemotherapy treatment. Jon is requesting that he be treated aggressively (per Dr. M's recommendation), which is not standard. We want to knock this sh** out....

Thursday, October 25, 2012

Quick Update

Jon is not having surgery tomorrow. The doctors are still discussing treatment protocol...it will likely be next week. We are exhausted...

Staging

Jon's PET results are in....there is no visible sign of metastasis. This is a huge sigh of relief. There was some uptake in the armpit nodes (2.5), but that is likely insignificant. The tumor had a SUV (standard uptake value) of 10.7. In lay terms, it lit up like a firefly...The average uptake for rhabdomyosarcomas is around 6--We need to get that malignant light bright out of there... Dr. M also sent us a confidential summary of his recommended treatment plan. The 6-page write-up was a thorough detailed plan outlining everything from chemotherapy timing/dosage to follow-up protocol. We were humbled....and so grateful. This plan has already been sent to Jon's treatment team. Dr. M's report described Jon as "well nourished"....I think part of that is due to his wife's amazing culinary skills.... Today we meet with Dr. P (chemotherapy) for assessment and a bone marrow biopsy. If the bone marrow is clear, Jon will likely be staged at Stage II, Group 1. He is Stage II given the histology (aggressiveness) of the cancer. We also received additional confirmation that Jon is in good hands. A generous and influential man in Jon's life arranged a phone call with the City of Hope CEO, Dr. Michael Friedman. Dr. Friedman assured Jon that Dr. M was the right expert to advise the case....We rest in this. Please pray for peace and mercy.

Tuesday, October 23, 2012

Gravity

Today we went to Children's Hospital and met with Dr. Mascheranas. So many people have recommended him; and he was "all he was promised to be". We spent nearly an hour discussing Pleomorphic Rhabdomyosarcoma and Jon's treatment. He humbly asserted he was an expert. He had already looked at the pathology himself and talked to Dr. Helmsteder (the oncological surgeon at Kaiser who will be removing the tumor). Here is a summary of what we learned: 1)Jon must have the tumor removed before chemotherapy/radiation.(There had been discussion that chemotherapy would begin first using the tumor as a biological marker.) Dr. M recommended immediate surgery (resection) with wide margins. This could happen as early as Friday October 26th. 2)There are two types of Pleomorphic Rhabdomyosarcoma. One type is strictly Pleomorphic. The other type is Aveolar/Embryonic with Pleomorphic "components". After the tumor is excised, the pathologists will determine the type. Both are "unfavorable", but the strictly Pleomorphic tumor can be "more resistant" to chemotherapy. 3)If Jon was 26, he could be treated at Children's Hospital. (He looks 26--that should count for something...) 4)Jon will start chemotherapy 1-2 weeks after surgery. Dr. M seemed to think one week would be sufficient. Dr. M recommended two different treatment protocols. One is standard for Rhabdo in kids (6 rounds every three weeks including radiation mid-treatment.) The other protocol has shown excellent prognosis in Ewing's sarcoma but is not "standard". It would mean chemo every two weeks for 6 months. If Jon could handle it, chemo every two weeks would help keep the cancer cells from recovering. As Caleb would say, it is like running a "constant Jedi blaster". 5)Getting it right the first time is paramount. If the cancer recurs, the prognosis goes "way way way way down". This was a memorable comment from a genius like Dr. M. 6) Dr. M recommended a chest catheter instead of a pic line. 7)Our family can stay together. Jon will receive chemo and then return home. If the kids are sick, he can wear a mask and washing hands will become an OCD tendency for all of us. Sick people will be warded off with Jedi blasters.... 8) As a third party, we asked Dr. M if he thought we would be better served at City of Hope (a local cancer center ranked top 25 in the nation). He objectively said "no". We have a great team collaborating to create an integrated treatment plan. Dr. M's voice at the table is a huge gift. Jon is an adult but he has a pediatric cancer. 9)Our kids will not inherit a genetic predisposition for Rhabdo. 10) Jon has many things in his favor. He caught the tumor early. If he were overweight and the tumor was hiding behind adipose tissue for months, this would be a different song. We are exhausted but so grateful for the love and support that surrounds us...We are on our knees in thanksgiving for our village. Jon met with a wise sage this morning and discussed the fear and feelings that are pressing in...Here is some wise concise wisdom worth sharing...very sobering...> *Say yes to pain medication *Say yes to light meds for anxiety during a procedure *Say yes to sleep medication when needed *Spend 10% of discussion time discussing mortality (but no more!) *Get all the rest you need *Work a flexible schedule throughout *Say yes when offered help for things you need *Find that person who knows most about your condition *Help Sarah get support

Monday, October 22, 2012

Doors Open

Today Jon went to Kaiser Sunset and gathered his pathology report and other diagnostic results and delivered them to pediatric oncology at LA's Children's Hospital (on foot)! He scheduled an appointment with Dr. Muscheranas tomorrow. This consult/second opinion is an out of pocket expense but well worth it....Jon brought home a copy of his pathology report which states at the end of the report, "THANK YOU FOR SENDING THIS INTERESTING CASE IN CONSULTATION". Gotta love being a lab rat! The biggest blessing today came from Dr. Ruby Kalra. Dr. Ruby used to work with Dr. M (mentioned above) and happens to be good friends with our nearest and dearest, Rachel McIntyre. Dr. Ruby sat on our couch for an hour while Rachel entertained the kids in the yard and discussed the treatment protocol. We learned a ton and got a glimpse of what this "Ironman" is going to look like. Here are a few details: 1. Jon is being staged. He will have a PET scan and a bone marrow biopsy Wednesday. As my sister has always said (TNM)-tumor, nodes, metastasis....These tests will determine if/where the Rhabdo cells are hanging out..... 2. He will meet with Dr. P (Chemotherapy) on Wednesday or Thursday to discuss the treatment options. 3. It looks like surgery (resection) will be first. Chemo will follow...Again, this depends on the consult with Dr. M and the other oncological surgeons. 4. Dr. Ruby has worked with Rhabdo. She presumes Jon will have roughly one year of chemo in three week cycles. Chemo lasts a week....he hits bottom at ten days...and then gradually rebuilds bone marrow; and just when he is feeling good, bam--the cycle begins again. I was reminded of the Ironman Jon and I "fought" in 2008. Standing on the beach at 5am getting ready for the 2.4 mile swim/112 mile bike/26.2 mile run...one can't think beyond the first mile of the ocean swim...it is TOO much. All that said, it was helpful to get a glimpse of the this mountain's height. We learned that even after the tumor is resected, Rhabdo cells may be hiding out anywhere in Jon's body--this is why they use chemo to "mop up the mess". Radiation might also play a role.... Our dear friends, the Ateeks, also set up a fundraising site to help us manage the extra financial expenses this journey will demand. We are so grateful. Caleb and Josiah are doing well. We explained to Caleb that there are cells (cancer) in daddy's body that want to take over. Daddy will need strong medicine to stop these cells from spreading. The medicine "may" make daddy sick, but it will eventually make daddy stronger.

Second Opinions and Fast Treatment

We are collecting input from a variety of physicians in the Los Angeles area. Given Pleomorphic Rhabdomyosarcoma (PRMS) is a rare sarcoma, it is essential that we gather input from a variety of players. There is a pediatric oncologist at Children's Hospital, Dr. Mascarenhas, who will meet with Jon this week after he is formally staged. Dr. M has seen more Rhabdomyosarcoma than most. Given Jon's great health, he can possibly be treated as a young adult. Dr Girvigian has also ordered a gene translocation test (on the advice of another pediatric oncologist named Dr. Cooper). Our friend Cathy Shin, a pediatric surgeon at Children's hospital, is facilitating a second opinion with Children's pathologist. We don't doubt the diagnosis, but we will both have more confidence going forward with a second opinion. A variety of treatment options have been put on the table: this has yet to be etched in ink. It will likely be a combination of surgery, chemotherapy, and radiation. The order is still being discussed. Today Jon will have a PET scan...His CT of the pelvis/chest abdomen showed no sign of metastasis...we are hoping the PET scan shows the same good news... We have also looked into City of Hope, but unless we can prove that Kaiser can not offer adequate treatment, we will not be released to City of Hope. It is looking like Jon can receive great care at Kaiser in collaboration with Rhabdo specialists at Children's.

Diagnostic Timeline

Jon found a small marble sized mass on his inner thigh (above his right knee) on September 21st, 2012. This mass would later prove to be inconsequential. After the advice of a family friend/physician, Jon decided to have this potential "lipoma" removed at an outpatient surgery center at Kaiser Sunset. On Friday September 28th, Jon noticed his lymph nodes were swollen in his neck; and he complained of chest tightness and myalgia in his legs. He also had a small angioma on his chest. We were betting on the fact that none of these symptoms were related, but we went into Kaiser that evening to see Dr. Garis, a primary care physician. Jon checked out fine.... The next morning after my cycle class at the Annex, I drove home and Jon showed me the "other mass" he had just noticed on his left glute. I was concerned, but Jon was convinced it was a muscle knot. Monday morning, October 1st, Jon went into the surgical outpatient center at Kaiser Sunset to have the mass on his leg removed. The surgeon looked at Jon's leg mass and advised that we wait to remove it. He said there was inflammation and it was located near a major vein. It wasn't a lipoma, but the surgeon wasn't concerned. Jon then showed the surgeon his gluteal mass. Jon was immediately advised to have diagnostic imaging done on his glute. The following few days were chaos. We were both concerned given Jon's other symptoms. On Tuesday October 2nd, we enlisted the help of a radiation oncologist from church. Our primary doctor agreed to refer us to him so he could open Jon's case...He ordered a stat CT scan which was done on Thursday October 4th. Within 30 minutes of having the CT scan, surgical oncology called and said the first available appointment with Dr. Difronzo was Monday October 8th. Both of our hearts sank because Dr. Difronzo was the chief oncological surgeon at Kaiser Sunset. We weren't sure whether all masses (benign and malignant) went through him.... On Friday October 5th, we were called in to meet with Dr. Girvigian, our Radiation Oncologist friend from church. He told us Jon had a clearly differentiated 4x3x2 cm mass in his left gluteus (coming from the muscle). A biopsy would be needed to assess the mass. He ordered blood tests, which were completed that day. On Monday October 8th, Dr. Difronzo offered an "on the spot" core needle biopsy. Jon jumped at this opportunity to find out the pathology of this mass. The core biopsy was completed within the hour; and we both went home to wait. The next day, Tuesday October 9th, Jon went to Baldwin Park to have an MRI with contrast. Jon's case was mentioned in the sarcoma meeting at Baldwin Park that afternoon, but there wasn't enough diagnostic information to discuss it. The results of the MRI were hopeful--the mass lit up on the boundary but the inside was dark. This could have been an indicator that the mass was benign. The pathology process began, and many words came across our lap (ganglion cysts, Myxoma, etc.) Most of the pathology hypothesis were benign. By Friday October 12th, Jon and I were resting in the fact that the mass was likely benign. On Monday October 15th, Dr. Girvigian called to say that all the hypothesis that were stained came back negative. This put us back at "square one". Jon's slides were then sent to a soft tissue specialist at Baldwin Park named Dr. Syed. On Wednesday October 17th, we met with Dr. Helmsteder, an oncological surgeon at Baldwin Park. He told us that the pathologist was leaning towards Pleomorphic Rhabdomyosarcoma. We held on to this preliminary diagnosis loosely...hoping for a miracle or another "oops...not it".... Jon walked into my office at 12:11 pm on October 19th and told me it was confirmed. We both were turned upside down...