Michele Nichols, 17+ year Thriver
By Michele Nichols 2/15/21
Las Vegas, Nevada
RLMS diagnosed in 2003
I remember the day I was in the doctor’s office for my routine annual exam, as she was ending the visit examining my abdomen. She asked if I had had lunch before coming to my appointment, and I told her I had not had anything to eat since early morning. It was after 12 noon. She said she could feel something, something “very big.” I was within days of my 56th birthday, and my life was about to change forever at that moment. She sent me to a diagnostic center for a CT scan, which revealed a 17-20 cm mass. Then, the next week I was sent to a surgeon, who read the CT Scan and thought I had liver cancer. He thought it was much too big to operate on and ultimately, I might need a liver transplant.
During the next month or so, I was sent to have a needle biopsy, without success. I had a core biopsy and was told I had adrenal cancer. However, when I saw my local surgeon, he said it was a sarcoma, leiomyosarcoma. I was referred to a UCLA sarcoma surgeon for consultation, who told me that no doubt I would need surgery, but I would want to be sure whether it was high grade or low grade. If high grade, chemotherapy would be recommended to try to shrink the tumor. The surgeon called me later, after I was back home, and said that his pathologist, who had viewed my blood slides, had told him all retroperitoneal sarcomas are high grade. (Not so.) I remembered what he had told me in his office and told the oncologist I had just been referred to that we needed a surgical biopsy to see what grade it was.
I think I was on automatic pilot as I returned to the hospital, where I was Chief Nurse Officer at the time. Work, for me back then, was an escape from thinking about what was happening to me. Was I scared, overwhelmed, stunned, in shock? Yes. I felt good and had never been sick, except for an occasional cold. Thankfully, I have been blessed with being an optimist. Also, I knew nothing of what sarcoma was or its modus operandi, how it progressed. All I remember learning in the beginning is how resistant it could be to chemotherapy and radiation, especially if it was low grade. The surgical biopsy pathology came back that it was low grade.
Being a registered nurse, I looked up everything about LMS and noted the percentage of long-term survival. The best I saw was around 62% for 5-year survival at that time, 2003. So, I focused on the higher percent, not the 48% that do not survive long term. With a strong faith in God, and a focus on being part of the 62% survivor tribe, I consented to the surgery, which came with possible negative outcomes, including lots of bleeding and possible death. In fact, during the surgery, the Nurse Director of the Surgical Services came back to tell my husband, who was waiting with my family, pastor, and friends, that they were going to have to close me up as they were not able to remove the tumor. My husband turned right around and told the group waiting that they needed to pray, and they did. The Surgical Services Director returned to tell my husband that the tumor was removed successfully and intact. There was no evidence of disease in any of the nodes removed. I was in the hospital 11 days, with complications, but recovered and then had radiation therapy, 28 treatments.
Then I had 8 years of NED (No Evidence of Disease). I was being managed by my local oncologist, who was not a sarcoma specialist. We do not have a sarcoma center in Las Vegas. During the 7th year (2010), my PCP and I became concerned about an injury I had to my left forearm. I had awakened in the middle of the night with extreme pain, like I had received a blow to my arm. It was sore, but there was no redness or bruising. Weird, right? An MRI said there was a mass but it could be a hematoma. Another MRI months later said it had increased slightly in size and was a schwannoma or nerve sheath tumor against my ulnar nerve. Ultimately, after it first was noticed, I had surgery (December 2011) and the surgeon, with my telling him I had a LMS history, said it always metastasizes first to the lungs. (Not so.) Just after the surgery he said it just lifted out and it did not look malignant.
While recovering, I received a call from my sister-in-law, that my brother who had been diagnosed with stage 4 lung cancer in October of 2011, had taken a turn for the worse, and all family was asked to come. I could not as it had only been two days from my surgery, and I could not travel. That was on Friday, two days before Christmas. My sister-in-law called me Christmas morning to tell me that my brother had passed away an hour before she called me. With hardly time to grieve, the next Friday was my post-surgical visit with my surgeon and he barely got into the door of the exam room when he told me that my mass was LMS. He was willing to do another surgery to resect more tissue, as the mass had some malignant cells on one corner, but never got the chance.
During my 2nd and last week of recovery, I had experienced light headedness and a dull pressure in my chest off and on. I called my PCP and she sent me to the ER on suspicion of pulmonary embolism (PE) and I was admitted, continuing testing for cardiac problems when PE was ruled out. It turned out I had a myxoma (a non-cancerous heart tumor) in my left atrium along with a patent foreman ovale (PFO) (an opening in the septum between the two atria of the heart). After being discharged, a CT scan was ordered of my chest, abdomen and pelvis, and a 5 mm node was noted in my right lung. The myxoma and PFO was prioritized and two months to the day from the surgery on my forearm, and after a boatload of tests and a cardiac catheterization, I had open heart surgery to remove the myxoma and repair the opening in the septum (February 2012).
After recovering from that surgery, I was sent to various physicians to see if I needed further surgery, radiation, and/or chemotherapy on my arm. It was when I was sent to my radiation oncologist that he felt radiation would be appropriate just to make sure all cancer cells were killed. This was 7 months after my forearm surgery. He asked if I would want a second opinion. I opted for that and he referred me to the UCLA Sarcoma Center, and specifically to Fritz Eilber, MD, Surgical Oncologist and Director of the UCLA-JCCC Sarcoma Program. Dr. Eilber said it had been way too long for anything to be done for the left arm, so observation with PET scans every 3-4 months was recommended. This all surprised me as I had thought I would be seeing a radiologist for the second opinion regarding radiation, not a surgeon. I had also been told by my local oncologist that PET scans were not done for sarcoma, when I had asked him years before. I am very thankful that I was sent to Dr. Eilber and that now I have been going on 9 years under his care. I also have a new medical oncologist locally who stays in touch with Dr. Eilber to coordinate my care.
I saw Dr. Eilber in July 2012, and had my first PET scan in January 2013, which showed a mass in my left gluteal area. It was LMS. It was resected intact and I went home that same day in February 2013. This became the first of six more LMS surgeries I would have at UCLA (5) and Las Vegas (1). In fact, my next surgery would only be 4 months later when the PET scan showed a mass in my left deltoid. In June 2013, this LMS was removed intact. Dr. Eilber had asked me whether I would consider chemotherapy, but my tumors had been low grade and I had been informed that low grade was very resistant to chemotherapy and radiation, so I declined. Surgery was always presented as the “100 % cure.” Of course, I always knew that cure was only for one mass surgically removed at a time.
For a little over three years, I only had the right lung node which was remaining stable or slightly increased in size and SUV (standardized uptake value, which indicates how active a tumor is). Then, during late 2015 - 2016, a questionable finding in my right hip area was seen on my scans. It was identified as a mass on my right iliacus muscle and back to UCLA I went, for surgery and a three day stay as an inpatient. Again, it came out intact and low grade. No other treatment was suggested. At Dr. Eilber’s suggestion, I did see a UCLA sarcoma medical oncologist, who reviewed my history and said his recommendation was for continued observation through scans.
My scans continued every 5-6 months. In 2016, my PET scan picked up multiple left lung nodules, most under 4 mm, with one over 5 mm. Also, the right lung nodule continued slowly increasing in size and SUV. In late 2018, a mass was seen in my right thigh, and my right lung nodule had increased to almost 2 cm, with the left lung nodule still slowly growing, but not significantly. A decision was made to biopsy the right lung nodule and do an MRI on my right thigh. The MRI revealed a mass in the right vastus lateralis muscle. The lung biopsy was done and after three attempts, the interventional radiologist told me the pathologist could not find anything but blood and debris, nothing to evaluate. I took CDs and written reports of my MRI, PET scan and lung biopsy, to Dr. Eilber, who told me he could not do surgery at that time, because of the possible disease in my lung and in my leg. I learned that if you have probable malignant lung nodules and have probable disease elsewhere, surgery is not an option. He said he could refer me to a cardiovascular surgeon there. We also spoke about Cyberknife or SBRT (stereotactic radiosurgery) for the lung nodule.
Back home, I went to see my radiation oncologist to get his opinion regarding Cyberknife, for which his group was the lead in Las Vegas. Since my lung nodule was next to my heart, he told me there would be the possibility of an inflammation of the heart tissue. I decided to see a cardiovascular surgeon I knew, to get his opinion on surgery. I opted for the surgery, and that was done in January 2019. Interestingly, the lung nodule “popped out” (it was written that way in the operative and pathology report). Therefore, no resection was performed. I still had the incision and recovery from lung surgery to go through.
A PET scan in May 2019 showed the right thigh mass to be increasing, and this time, Dr. Eilber contacted me and said he could resect the mass. That same month, he removed the LMS mass, about 4 cm, intact. I thought, now I just have the left lung nodule to be watched. However, my very next PET scan, in the summer of 2019, showed 2 liver masses, both just below 1 cm.
On that same scan an interesting thing happened with the left lung nodule SUV. While the size of the nodule had increased from 10 x 9 mm to 12 x 9 mm, the SUV had actually dropped from 2.0 to 1.3. I was not on either chemotherapy or radiation therapy. This was in September 2019. I also had an MRI of the abdomen to evaluate the liver nodules which had increased in size to 15 x 14 mm and 18 x 17 mm, respectively. For all my scans, I copy Dr. Eilber on them, so that he can see what is going on with me. This prevents my having to travel out of town for check-ups, as my local medical oncologist does that.
Interestingly, for whatever reason, I did not hear from my medical oncologist for next steps. I thought I would wait until early 2020 and call to make an appointment with him around March. In late February 2020, I saw my PCP for a regular check-up and at the end of the visit, I asked if she could order my PET scan, and then I would follow-up with my oncologist to schedule my appointment. I did this since normally at my previous appointment, the oncologist would have ordered the PET scan and scheduled my appointment a week later. I had the scan on March 13th and the very next week, we were on COVID lockdown. The results of my PET scan were that the liver nodules were larger, but there was no change to the left lung nodules, except for the SUV increasing slightly to 1.4.
Although the liver nodules had increased, I basically waited until the first part of May to see my oncologist and to contact Dr. Eilber, as none of the hospitals were doing elective surgeries because of the COVID patient overflow in them. In May, my oncologist sent me for a CT scan of my abdomen with and without contrast, followed by an ultrasound at the request of the interventional radiologist he referred me to, and I had a telehealth visit with the IR a week later. The CT scan and ultrasound picked up a third nodule under segment 2 of the liver. This was the largest of the nodules and because of its location, it was decided I needed to have that one surgically removed. I went back to UCLA in late June when the hospitals opened back up for elective surgery, and Dr. Brian Kadera, a partner with Dr. Eilber, coordinated with interventional radiology to do the microwave ablations of the two liver nodules and resect the third nodule along with a portion of my liver. All was done by laparoscopy and after seven little incisions, I stayed the night for observation and went home the next day.
Now, I was thinking again, I only have the left lung nodule to be concerned about. About one month from my liver surgery, in late July, 2020, I got up to walk and a searing pain occurred in my left calf. This did not go away, except when I was sitting with my legs up, or walked the pain out. I made an appointment with my PCP, who sent me to get an ultrasound of my legs. While waiting for this appointment, I noticed a bump on my right forearm near the elbow. I told my PCP about that and told her I would make an appointment with my oncologist after taking care of the leg issue.
The ultrasound showed occlusion in my left calf in superficial veins and possible DVT (deep vein thrombosis) in my right upper thigh. My PCP called my oncologist, who suggested anticoagulant therapy and for me to call and make an appointment with him as soon as possible. I saw him within a few days and he ordered a PET scan to evaluate the mass in my forearm. The scan indicated that there was an ill-defined mass in my left forearm measuring 18 mm. It also indicated that the lung nodule had decreased in size from 10 x 12 to 9 x 10 mm, SUV 1.4. This was interesting as I was still not receiving any chemotherapy or radiation treatments. Back to UCLA, and Dr. Eilber resected the right forearm mass, intact; it was LMS.
As of my last PET scan in December 2020, the left lung nodule remains stable and no other disease is evidenced. Once again, I am thinking I just need to watch the left lung nodule. I had switched my oncologist to another within the same group, different location, at the strong suggestion of a dear friend. She was seeing an oncologist who was current on sarcoma and regularly communicated with Dr. Eilber, which my original oncologist did not do. I am very thankful I have changed to Dr. Rupesh Parikh, as he caught things in the computer records that were not noted, like the fact I had a right kidney removed with my primary surgery. He also ran genetic tests to see if I had any genetic mutations that would indicate the affinity for cancer. The tests revealed I had a TP53 mutation. If working properly, this gene is the policeman for the genome, keeping defective cells from growing, therefore lowering the risk of cancer. My mutation indicates I have a hereditary pre-disposition syndrome, which has multiple syndromes listed under it. Mine best fits Li-Fraumeni Syndrome, which is inherited from one or both parents having the TP53 gene mutation. It increases the chance of sarcoma, breast, leukemia and other cancers, and many people with Li-Fraumeni have three or more of these cancers in their lifetime.
In this new community for me, I have learned that those with Li-Fraumeni Syndrome are children of affected parents. Often childhood cancers are seen and in most affected adults, cancer is seen at an early age. Some in this community refer to themselves and each other affectionately as “mutants.” People with Li-Fraumeni are discouraged from having radiation, as sarcomas can result. However, the benefits must be weighed when treatment decisions are being made, and most oncologists will opt for the radiation treatments if the benefit to reduce or eliminate a mass is optimal, and surgery is not possible or does not have clear margins. I am just getting into a clinical study, with the Dana Farber Cancer Institute, called “LIFT-UP,” or Li-Fraumeni & TP53 Understanding & Progress, with Dr. Judy Garber, principal investigator.
It has been an adventure through the years, with much anticipation with scans and surgeries, and radiation. There are things I would do the same, given my progression through the years, but maybe earlier, and other things I would not have done at all. I hope by sharing them with others, it may help them make their decisions and enjoy life.
In retrospect, knowing what I know now, things I would have done differently would be:
· I would have contacted the UCLA Sarcoma Center to oversee my care specific to my cancer, from the beginning. And I would have also made sure my local oncologist interacted with my sarcoma oncologist from the beginning.
· I would not have had the radiation therapy following a surgery where all nodes were clear of cancer. Radiation is not recommended for people with Li-Fraumeni Syndrome unless the benefits outweigh the risks.
· Ask your oncologist to do genetic testing to see if you have any gene mutations that affect you and your family. I had always been told LMS was not a cancer that was inherited and that it was unknown why people get it. Genetics may answer that question.
Things I did, that I am glad I did, and I would do again:
· Stay grounded in my faith in God, knowing He has a plan for me.
· Take advantage of educational offerings on LMS. Connect with a reputable LMS organization, like LMSDR, to keep you informed about all the current research, treatments, educational conferences and to provide support.
· Always ask for copies of your lab work, scans, pathology reports and keep them together for your records.
· Stay moving, active and eat healthy, to keep your health at its best, so that your recovery time from surgery, radiation, chemo, etc. is smooth and quick.
· Always stay positive and when you get down, talk, cry, or scream it out and then get right back up and move forward.
· Stick to your gut feelings. If you have doubts or run into obstacles in your care plan that do not seem right to you, question it and seek a second opinion.
· Be persistent about tests. Become familiar with your scans and know what contrasts, oral or IV are done. If those things do not happen, question it and do not go forward until you have the answer and you have checked with your doctor.
When you first hear those fateful words that you have a rare incurable cancer, it can seem like you are down before you even begin. Take it one day, sometimes, an hour at a time. Read the long-term thriver stories. Being stage 4 is not the imminent death sentence for this disease. Be encouraged that you can live an active, otherwise healthy life in between the scans and treatments. New therapies and treatments are being discovered every year. And, for some, they remain NED indefinitely. There IS hope and I continue to hold onto it! Won’t you join me?