September 6 is our wedding anniversary. In 2017 we had planned to celebrate our 42nd wedding anniversary at one of Priscilla’s favorite restaurants Cooper’s Hawk. On the morning of September 6, my wife Priscilla had a scheduled CT scan at Highland Park hospital. She had a severe cough for several months. She coughed so hard I feared she would literally cough her head off. Her doctor Ivana Ruffolo had prescribed several drugs with limited effect. Priscilla met with Doctor Ruffolo’s counterpart who prescribed steroids. That finally stopped the coughing.. One of the symptoms that Priscilla described was that she occasionally had a pain in her right side that felt like she was being stabbed with a knife. The supposition was that she was coughing so hard that she strained a back muscle. During the September appointment, Doctor Ruffolo identified a mass located in Priscilla’s side and ordered a CT scan to determine what it might be. It should have been a simple trip to the hospital for the scan. I had a bad feeling that there was more going on than just the severe coughing. I accompanied her to the hospital. After the scan she was immediately admitted to the emergency room got an MRI. After the MRI was read, she was admitted to Highland Park hospital. We spent our 42nd wedding anniversary in her hospital room. The CT scan revealed that she had a perforated appendix. She received a high dose intravenous antibiotic. On day 2 she was taken to surgery to have a biopsy of the cyst on her side. Part of the plan was to drain the cyst. Once the mass was pierced the mucin was so thick that the doctor could barely draw a sample. The result of the biopsy was inconclusive but surgery was scheduled to remove the appendix. The appendix is a useless organ that has no function. Based on the scan the decision was made to schedule not only the appendectomy but also a full hysterectomy since an ovarian cyst was also identified. This required the Evanston hospital to coordinate the schedules of two surgeons. Doctor Haggerty conducted the appendix surgery and Doctor Moore conducted the hysterectomy with the aid of a surgical robot. It was difficult to schedule two surgeons with busy schedules snd the robot operating room. The timing worked out for us to spend two weeks at our condo in Stuart, Florida. We had to change our flights to return to Chicago a week earlier than planned. On the flight to Stuart we flew on United Airlines with a connection in Newark. We had a one hour connection and our flight departed from Chicago one hour late. The pilot whipped the horses and we made up just enough time to give us false hope. Then the arrival gate was moved to the farthest distance to our next flight. We made the OJ dash through the airport only to find that our connecting flight departed early. We are soooo happy for everyone on that plane. Our time in Florida was idyllic. No crowds. We could just show up at our favorite restaurants without a reservation and park in front. Swimming every day helped take our minds off her upcoming surgery. We flew home on a direct American Airlines flight “first class.” She deserved it.The surgery took four hours. As a result of the surgery biopsies revealed that her appendix was secreting mucin that penetrated her abdominal wall. This is what was creating the excruciating pain. Doctor Stephan Haggerty advised he scraped the area around the appendix but some mucin remained. The lab confirmed that Priscilla had low grade pseudomyxoma peritoneai, mucinous adrenocarcinoma of the appendix PMP. This is among the worlds most rare cancers with only 300 cases diagnosed per year. In addition since she had an abdominal penetration that occurs in only 5% of the cases. If you calculate the odds she is one in 0.0000001 which is seven sigma. This is the main reason it is so hard to diagnose. Only a handful of doctors ever come across this disease. At Highland Park hospital we met with the oncology doctor Matt Adess. He prescribed six rounds of Folfox chemotherapy followed by surgery and HIPEC.
Molecular testing also identified a rare DNA deficiency associated with Lynch Syndrome also known as HNPCC (Hereditary Nonpolyposis Colon Cancer). Further investigation is recommended to determine if she carries the germline mutation in genes effecting DNA mismatch repair.
The first step to prepare for chemotherapy is to have a port implanted under the skin near the right side collar bone with a tube inserted into the aorta. This is how the chemotherapy will be administered. Blood can also be drawn from the port. Chemotherapy can be administered one week after the port is installed. Priscilla’s first dose of Folfox was scheduled for November 20, 2017 in Highland Park. This is a four hour procedure which starts with blood draw and review of key blood components. The chemotherapy lasts for three hours. Fortunately the Folfox is a gentler dose than some others. It generally does not cause hair loss or nausea. After the three hour dose of Folfox a canister of Folfox is attached to the port to continue the chemotherapy for another 48 hours. The canister allows the patient to leave the hospital and live a more normal life. Prior to the innovation of the canister patients had to remain in the hospital for 48 hours.
On November 21 while still attached to the canister we traveled to the University of Chicago Hospital on the south side of the city. It was a 9:00 appointment and we had to go through the city traffic at rush hour. We allowed ourself three hours to make the commute.
Fortunately there is a PMP specialist at the University of Chicago hospital. We met Doctor Kiran Turaga. He advised that he changed his medical practice in Milwaukee to Chicago to have the opportunity to treat more patients with PMP. He challenged us to become experts in the disease to most effectively cure the disease. Doctor Turaga agreed with Doctor Adess’s treatment plan. This included the six rounds of Folfox chemotherapy, Cytoreductive CRS surgery to remove all visible cancer and Heated Intraperitoneal Chemoperfusion HIPEC which is high temperature chemotherapy. The HIPEC is 90 minutes of heated sterile chemotherapy injected and circulated in the abdomen at 108°f. This kills the cancer cells in the mucin and any remnants of tumors that were not completely surgically removed. There are theories that the cancerous mucin MUC2 is created by an interaction between goblet cells and certain bacterias such as H.pylori and salmonella. After the surgery and HIPEC there will be ongoing tests to insure the cancer is in complete remission and no tumors develop.
We met with several RN’s to discuss pre surgical screening, nutrition and physical theropy. Priscilla needs to be very healthy and strong to be able to have the best results from the CRS and HIPEC. Before we left the hospital Priscilla went to the lab to give blood to establish a base line for her tumor markers. We do not know if she has any tumors at this time. We made an appointment for pre-surgical tests on March 6, 2018. We were told to contact one of the RN’s Laura Tattersall in mid-February to schedule the surgery. They only plan surgery one month in advance.
The timing of the chemotherapy and surgery allows us to still keep our holiday plans for Thanksgiving with JP, Rachel and Eleanor in Lake Villa, IL and Christmas with Alison, Oscar and Jackson in Framingham,MA. Then we will travel to our condo in Stuart, FL for January and February and return to Chicago in March. If necessary, we would have completely changed our schedule but it was not required.
On December 4 we arrived at the Highland Park hospital for Priscilla’s second round of Folfox chemotherapy. After the blood work was reviewed it was determined that her white blood cell count was too low to proceed with the chemotherapy. Apparently the Folfox impacts the bone marrow which effects the white blood cells. The decision was made to postpone the chemotherapy for two weeks to allow her white blood cell count to improve. Doctor Adess was surprised by the low white blood cell count resulting from only one chemotherapy treatment. He advised that Neulasta could be administered after the chemotherapy to improve the white blood cell count. The cost is $15,000 per dose. This was a disappointing day.
On December 10 and 11 we drove about 1,000 miles from Lake Bluff to Framingham. The highlight, if you can call it that, was a lake effect blizzard south of Buffalo, NY. Cars and trucks slid off the road but we just kept on truck’n until we reached the LaQuinta Inn in Batavia, NY. Batavia is east of Buffalo and west of Rochester. We enjoyed fine dining at the Bob Evans restaurant.
It was a joyous reunion with our daughter Alison. Her husband Oscar and two year old son Jackson were visiting Oscar’s relatives in Brazil for three weeks. They returned on December 17 which is one day before Jackson’s 2nd birthday.
On December 12 we got back to work regarding Priscilla’s cure. At 11:15 am we arrived at the Framingham Union Hospital to arrange her pre-surgery physical therapy. Doctor Turaga advised that surgery was dependent on Priscilla’s physical ability to recover from the surgery. Elaine was our therapist. She completely understood the requirements and started teaching Priscilla several core strengthening exercises. She was concerned that the PT prescription provided by U of C was not detailed enough to satisfy a Medicare claim. She argued on the phone with Lauren from U of C to provide a prescription that included not only a diagnosis but the recommended PT such as core strengthening. You would have thought Elaine had asked Lauren to sacrifice her first born child. They argued for several minutes until old age and treachery finally triumphed and Lauren agreed to amend the PT prescription.
At 12:45 pm we drove 35 minutes to the Dana Farber Cancer Clinic in Milford. This is the location where Priscilla will have two courses of Folfox chemotherapy while we are visiting Framingham for the holidays. We are happy to utilize Dana Farber because they are a well known cancer clinic and we can get a second opinion. The session with Doctor Tahir went well. He did say that Dana Farber did not utilize HIPEC very often and when they did they transferred the patients to another hospital. He reviewed the data from Priscilla’s last attempted chemotherapy session where Doctor Adess decided to postpone. Doctor Tahir commented the white blood count was so close to the lower limit that he would have conducted the chemotherapy treatment and administered the Neulasta. We scheduled the appointments for her next two chemotherapy sessions on December 18 and January 2.
We took the back roads from Milord to Framingham just to see the sights. We met Alison at Jack’s Abby microbrew in Framingham for dinner. It was a fun early evening.
A highlight of our first week in Framingham was participating in an open microphone night at the Nobscot Cafe. I play the electric ukulele and try not to suck. It was a very fun evening. Priscilla’s older sister Alison and her husband Fred and daughter Jennifer joined us. Priscilla and Jennifer joined me on stage to sing back up on Bob Dylan’s song “Don’t Think Twice.”
We have been paying close attention to Priscilla’s nutrition. The recommendation is five small meals a day with lots of protein from nuts, cheese and meat as well as lots of fresh fruits and vegetables. Our daughter Alison is a vegetarian so the meals for the three weeks while we visited Framingham were perfect.
We have also reached out to the PMP Appendix Cancer Support Group. The moderator Denise Kirby Wesley contacted us to verify our situation before connecting with us on Facebook. This will be an insightful view into our journey.
On December 18 Priscilla had her Folfox chemotherapy at Dana Farber. They drew blood and then hooked her up to an IV for three hours. A volunteer social worker stopped by and asked if we wanted lunch. We had chicken noodle soup and a small turkey sandwich.
When the IV chemotherapy was completed Priscilla was connected to a portable pump that continues to deliver the Folfox for another 48 hours. The pump used ay Highland Park hospital was a small cylinder. The Dana Farber pump is a rectangular box. Both of those pumps provide the same result. The Folfox chemotherapy does not appear to have had any major residual side effects. Priscilla can continue to do everything as she has in the past without nausea. She does experience tingling in her fingers, especially when exposed to cold, fatigue, and some anxiety. There are drugs available for nausea and anxiety and to help with the related sleeplessness.
On December 20 the pump was disconnected. The nurse provided an injection of Neulasta to help improve Priscilla’s white blood cell count. The next round of chemotherapy will be on January 2. That will be our final round at Dana Farber. After the pump is disconnected on January 4 we will drive to our condo in Florida. The chemotherapy will continue in Florida at the Hematology Oncology of the Treasure Coast clinic in Stuart. We are working with our contact Orchid Foster to get all paperwork sent to her and appointments scheduled. It is now December 27 and I still do not know if Northshore sent the records to Orchid. I spoke with Michelle and she advised the records were emailed to Orchid two weeks ago. I explained that I had spoken with Orchid only a few minutes ago and she has not received anything. Michele assured me she would fax the documents immediately. What is it about doctors and hospitals that they are are trapped in the 1980’s and still use fax machines as their primary means of communication? On November 28 Orchid confirmed she received the faxed copy of Priscilla’s medical records and scheduled her to meet Dr. Wardhiem on January 11 for a consult prior to scheduling the chemotherapy on January 18. We need to provide the medical records from Dana Farber to Orchid as well. The location of the Hematology Oncology of the Treasure Coast clinic in Stuart is about one mile from our condo.
Priscilla had her first physical therapy session with Elaine on December 21. As it turns out Lauren’s youth and exuberance overcame Elaine’s old age and treachery. Lauren did not send the updated PT prescription. Now we shall see if I have to battle Medicare for payment.
Christmas Day was the turning point. Priscilla had shown no effects of the Folfox chemotherapy until today. She experienced severe nausea for the first time. We wondered if it might be the flu or food poisoning. A call to Dr Tahir did not confirm that the nausea was the result of the second round of chemotherapy. We had made a mistake by not having Priscilla take her anti nausea medicine. The learning was that even tho there was no effect for over a month she should have been taking her anti nausea medicine. She has eaten only a few bites of toast and peanut butter and a few bites of cornbread in three days.
On December 27 Priscilla had scheduled her second physical therapy session. She rallied as best she could but in the end did not have the strength. She cancelled.
As of December 30 Priscilla continues to improve. She is eating regularly and has enough energy to play with Jackson for a while each day. She went to the Natick Mall today to get some exercise by mall walking. Chasing her grandson Jackson around the mall gave her the workout she wanted. Taking her medication on a timely basis has been the key. We will not make that mistake again. Her next chemotherapy treatment is scheduled for January 2.
I attended the open mic night at the Nobscot Cafe for the second time on December 28. Steve and Carrie who organize the event were already playing when I arrived. There were two comedians that performed before me. I had practiced three new songs to add to my set. One new song is usually a good number but what the heck. I took the stage and found that I was having technical difficulties with my electric ukulele. I would strum a cord and there was only a muted off key sound. Then it was fine, then the problem persisted. It took me two songs to realize the the microphone was set up improperly on the stand and I was so close that I was pressing the neck of my ukulele into the microphone pole. For my second set I made sure the microphone was properly positioned. I did not suck on my second set. I learned something that night.
On January 2 Priscilla had her third round of Folfox chemotherapy. Due to the New Years holiday the clinic was very busy with two days worth of patients. We arrived at 9:00 am and she did not get connected to the IV until 1:00 pm. The staff were very apologetic regarding the delay. It was our intention to depart for Florida on January 4 after the chemo pump was removed. The news was full of weather predictions of the bomb cyclone. On January 4 we drove to the clinic in near white out blizzard conditions. Our RN Katherine had called and suggested we arrive early to have the pump disconnected. When we arrived at the clinic there was a skeleton staff and only a handful of patients. Given the deteriorating road conditions we were happy to get in and out earlier. We spent one more night in Framingham and helped dig out from 18 inches of snow. The deep freeze was coming in right after the blizzard. The temperature was forecast to be a low of -6°f before the windchill.
We departed from Framingham on January 5 and drove to Richmond, Virginia. Priscilla traveled very well. She had no symptoms of her chemotherapy except fatigue. We woke up on January 6 to a temperature of 12°f. We will keep driving south. We need to be in Stuart by January 11 for Priscilla’s appointment at the Hematology Oncology clinic with Dr Wardhiem to arrange her chemotherapy program while we are in Stuart. Her next chemotherapy session will be on January 16. The clinic is only one mile from our condo.
We drove 15 hours from Richmond, Virginia to Stuart, Florida and arrived at our condo at 11:00 pm. We unloaded the car and slept well. The temperature was 65°f. We were happy for that compared to 12°f the day before. By Wednesday the temperature was 80°f and the water temperature in the condo swimming pool was 88°f. Yes, it was worth the drive.
On Tuesday I received a call from Orchid Foster at the Hematology and Oncology of the Treasure Coast cancer clinic in Stuart. Orchid advised that Dana Farber had not sent Priscilla’s medical records. I wonder if I should attach a $20 bill to each request to get medical records sent. I maintain a complete copy of all Priscilla’s medical records so I drove over to the clinic and provided them with a copy of her medical records. It was a good test run. I know where to park and find the clinic when I take Priscilla there the next day.
On January 11 we met with Dr Michael Werthheim. He reviewed Priscilla’s records and approved the next rounds of Folfox chemotherapy to be administered in Stuart. He commented that he was surprised that Priscilla’s chemotherapy did not include Avastin which cuts off the blood flow to existing tumors. His supposition was that the original surgery removed all known tumors and the Avastin was not necessary. I emailed Dr Turaga at U of C to get a clarification.
The conditions at the clinic are much different from our previous two hospitals. At Highland Park and Dana Farber Priscilla had a private room for her chemotherapy. In Stuart they only provide chemotherapy services on Thursdays. The patients are stacked like cord wood side by side in reclining chairs along the walls. I am not sure there will be a chair for me to visit Priscilla during her treatment. We shall see.
While we were at the clinic I requested a referral for a hospital associated physical therapy department. I have contacted them to arrange Priscilla’s pre surgical PT while we are in Florida.
JP, Rachel and Eleanor arrived in Stuart on January 13. It is always a little risky to vacation in Florida in January. Last year they had great weather during their January vacation. This year not so much. It is in the 60’s and very windy out of the north. We swam on Saturday before the temperature dropped. Priscilla has been going to the pool everyday to work out for an hour. Hydrotherapy is very good for her. She spends most of her time in the pool stretching and power walking.
I arranged a half day fishing charter for Tuesday. The marine forecast shows offshore conditions to be very rolly. I asked Captain Mike of Mattanza charters if he fishes the intercostal. He said yes and there are numerous fish species to catch. JP, Dan Carlson and I will meet Captain Mike at Pirate’s Cove marina in Port Salerno at 8:00 am. I need to find a full day fishing charter for when Jeff and Stacy visit. We shall see if Captain Mike can put us on some fish.
We had a great day of fishing. We loaded the boat with Sheepshead and Spotted pin fish. Mate Alex was attentive and cleaned all of our fish. We enjoyed a nice fish fry that night.
Priscilla had her first chemotherapy at the Hematology and Oncology clinic. Their processes are more prone to error than Highland Park or Dana Farber. There are no wristbands or double checking birthdays or double checking medications before they are administered. She was lined up in a chair along the wall with the all the other patients. It is an efficient use of space. After her chemotherapy was completed she had her pump attached. I picked her up and we met JP and Eleanor for lunch at Alice’s Restaurant. The pump disconnect is on Saturday at a location that is 30 minutes away. She has three scheduled appointments to get a white blood cell booster. For some reason Florida does not allow chemotherapy patients to have Neulasta. The Neulasta provides 10 days of time release medication. She also has an appointment on Monday to meet with her physical therapist and start her PT program in Florida.
We received sad news that Priscilla’s mother Jean Mary Smith passed away at the age of 92 on January 18th. She had been in a nursing home for many years and recently entered a hospice when she was unable to eat on her own. Priscilla’s youngest sister Sarah was with her when she passed. There will be a celebration of life in April.
On Saturday we (JP, Rachel, Eleanor) all drove to the clinic in Jensen Beach so Priscilla could be disconnected from her chemotherapy pump. We arrived at 9:15 am and she was last person on the list to be disconnected. The Cracker Barrel restaurant was nearby so stopped for breakfast. One of the many cute things Eleanor says is “don’t forget Eleanor.” She says this everything we park the car get out. She has never been forgotten. Maybe that is because she always reminds us.
JP, Rachel and Eleanor flew back to Chicago on Sunday. The weather in Florida did not cooperate for their visit. The day they left the temperatures climbed back into the 80°f’s.
On Monday Priscilla met with her new physical therapist. She was given numerous exercises to strengthen her abdomen prior to surgery. We walked several blocks to one of Priscilla’s favorite restaurants , the Blue Door. We had a nice lunch on their patio and walked back to the clinic so Priscilla could have her first of three injections this week of the white blood cell booster. We resolved the Medicare billing issue with the accountant at the clinic. She had previously told us she needed a copy of Priscilla’s Medicare card for her file before she would bill Medicare. All the other clinics and hospitals just ask for the Medicare number and do not ask to see the card. We spent an hour on the phone on two separate occasions to get a replacement card. Medicare will send the replacement card to our Lake Bluff address but it cannot be forwarded. In three weeks I will have to stop my mail forwarding and have the person watching my house collect my mail and forward the card to us. However, the accountant said she is billing Medicare already so this all seems to be much ado about nothing.
Monday night was open mic night at the Vine and Barley bar in Stuart. Priscilla came with me and I rocked the bar with my electric ukulele.
Tuesday brought good news that Eleanor will soon have a baby brother. John Parker Simons Jr will come into this world on June 5, 2018.
This is a full week of beating the disease. Priscilla has appointments for white blood cell booster injections on Monday, Wednesday and Friday and physical therapy on Thursday.
I have been reading the daily posts from the PMP Pals and the vast majority of.posts confirm the CRS/HIPEC is the best possible treatment. They refer to it as MOAS which is Mother Of All Surgeries.
It was a fun night out at Gettin’ Crabby with Dan and Iris on Thursday night. As we walked in I was approached by a gentleman that yelled out “you are the electric ukulele player from the Vine and Barley.” I had no idea I had fans wherever I go.