Leiomyosarcoma Direct Research Foundation

Persistence Prevails When All Else Fails:

“You’ve got Leiomyosarcoma and you probably only have six months to a year to live” “There isn’t much we can do to treat your disease and we are really sorry.”

– February, 2002.

What a thing to tell a person! Gheeze. Talk about being too brutally honest! Those two sentences are not what I wanted to hear! Talk about being hit by a truck! Stunned, scared, frozen, and unable to think. Forget about any meaningful comprehension. All those big words, lots of doctors, CT scans, needles, lots of needles, FEAR with a capital F. My world is crumbling!

Is this going to be how it really ends? Will I become a burden? Will I be in terrible pain? I AM MUCH TOO YOUNG TO DIE! I am only 64 and supposed to have a few more good years left. I still have so much to do, so many things to get done. I have children, grandchildren and a husband who needs me. Responsibilities and places I need to go, things I need to get done. Not me, not now, not yet!

My mother was diagnosed with Leiomyosarcoma after suffering a chronic bronchitis, upper respiratory infection for over 2 ½ years. We just couldn’t understand how she was constantly feeling sick, hoarse, and always coughing to clear her throat. Every six to eight weeks she was going back to see the doctor. She visited her primary care doctor 17 times during a three year period for the same problem! Each time, the same answer.....URI, upper respiratory infection, bronchitis, laryngitis... Well if something isn’t getting better, you have to re-evaluate the situation. I demanded that my mother get a chest x-ray. I couldn’t believe that she hadn’t had one sooner! Her doctor thought that it wouldn’t be a bad idea....NOT A BAD IDEA!? Why should a son have to recommend further study? I’m not even a doctor and could tell you it was a good idea about 12 treatments ago!

She got the Xray:
It showed multiple lesions in both lungs, a large 13 x 8 x 7 cm tumor on her right lung with a big necrotic (dead) center with a good bit of pulmonary edema. They referred her to a local pulmonologist, who promptly ordered a CT scan and a biopsy of the large lesion on the left lung. The cells came back abnormal and suggestive of sarcoma. That’s when the bad news hit.

The doctor that delivered the bad news was Dr. X, a partner of the original pulmonologist, who had never seen my mother before. He was filling in for the other doc who was out for a week on vacation. Here comes a perfect stranger who my mother has never met and he bluntly lays it all out on the table. “You have an advanced sarcoma and you only have six months to a year to live.” Here is the name of an oncologist you might want to see to discuss your options.

We went to see the local oncologist. He gave us more information. “Your mother has an unusual sarcoma. It looks like a smooth muscle tumor. Nothing works to cure it, but we can try some Doxil to see if we can slow the tumor’s growth a bit. It works about 20% of the time to slow growth for awhile. These things are very aggressive and it doesn’t have a very good prognosis.

Wait, you always want to get a second opinion, but where? A friend was treated for breast cancer at Fox Chase outside of Philadelphia. She said they are great there. We know a nurse who works w/ a thoracic surgeon there too. We make an appointment to see their sarcoma team the next week.

Again, more negative news. No cure. They offered to try some heavy duty chemo, which would require some hospitalization, but there is no way they would operate on this.

Options....we need some better options. To me, it makes sense to remove something with a dead center. Why can’t anyone just get the thing out? I read. I did tons of reading. I stayed up till the wee hours searching the net for “the best doc”, the specialist who is best at caring for this problem. Every free moment was spent looking for an answer. Who is doing the research on sarcomas? Where is the research coming from? What is the standard treatment? What is the right thing to do?

My friend suggested a cardiothoracic surgeon by the name of Larry Kaiser at University of Pennsylvania. He is supposed to be the best guy in Philadelphia, if not on the East coast.
I call for an appointment. Earliest available appointment is 5 weeks away! Holy Crap! We can’t wait that long! There has to be a better way. I called Dr. Kaiser’s office again and this time I asked to speak to his secretary. I got through to her and explained my mother’s predicament. She was able to get her on the office schedule for the next week. Awesome!

While we are headed into Philly, we need to find a sarcoma specialist. I searched the biographies of the oncologists at UPENN. I found one who said his interest was in Sarcomas and I found out that he was also a surviving cancer patient. His bio looks pretty good. I like the fact that he’s been down the tough road himself. I arranged for an appointment on the same day as the consult w/ Dr. Kaiser.

To prepare for the appointment w/ Dr. Algazy, we had to complete a medical history. Five years prior my mother had a hysterectomy for “benign” fibroids. Dr. Algazy asked for the slides from the surgery so that the lab at UPenn could review them.

My mother was growing weary of doctor’s visits. She was beginning to believe there wouldn’t be any better news. The trip to the city with it’s size and bustle were a bit unsettling for my mother who is use to the rural area of Lancaster County PA.

We went into see the oncologist at UPENN. The office was filled with cancer patients. Many were bald, pail, greenish looking, ill, cancer patients. This was very unsettling for my mother. Other than some left shoulder pain and a cough, she was feeling pretty good. She was at least pink and healthy looking. For the first time, the bold reality of her next six months was hitting her square in the face. Sickly and frail is not the way my mother envisioned living out the rest of her life.

We saw Dr. Algazy, and he noted that my mothers cancer probably started in her uterus and the lesions in her chest were metestatic lesions from the original tumor. He showed us the pathology report from the UPenn lab that stated that the “benign” fibroid contained abnormal leiomyosarcoma cells. Talk about shock. How could that be? How does a lab miss something like that? He noted that LMS was pretty rare and that he has cared for several LMS patients in the past. To our surprise, he offered us another option. There was a new drug called Gleevec that had just come out and it was showing some potential promise for LMS patients. The good part was that the side effects were relatively minimal when compared w/ chemo. He was very pleasant, positive thinking and very supportive of our idea to see Dr. Kaiser. He wanted to wait to hear what Dr. Kaiser had to say first before we mapped out a course. An option! We have an option!

Off to Dr. Kaiser’s office. We waited two hours to get in to the exam room and waited another 30 minutes to finally see the doctor. My mother was growing short on patience. He gave her the once over, took her films and came back in 20 minutes. “How would you like to be essentially cancer free? I’m going to open your chest and cut up to your neck and remove the big tumor. At the same time I’ll pluck out those other mets and for all intents and purposes, you’ll be cancer free!” We both sat there flabbergasted.

Who is this guy? He is cocky as hell. His bed side manner was very matter of fact. He was direct and to the point. I asked him directly, “why do you think you can do the surgery when all these others said there is no way?” His response was “I do the surgeries that others can’t.” He proceeded to detail the surgical risks and told us to think about it and let him know what you want to do. Like the wind he was gone.

Holy Mackerel! What just happened? What did he say again? Are you kidding me? Is he being too cavalier? My mother was a little upset with his bedside manner. Felt he was too quick, cocky and matter of fact. I liked his competitive style and swagger.

What a day! We see two new docs and get two new options. Who the heck do you believe? We laid out our options. We prayed a lot. We discussed it up and down. We wrote down the pro’s and con’s of each. We weighed the cost/benefits.

Big choice; do the surgery now, possibly die on the table, or become a vegetable and not get to live six months. You could do the heavy duty chemo, and live your six months frail and sickly with less then a 30% chance the stuff will work. You could try a new drug like Gleevec, but it’s unproven and who knows if it would work? You could do nothing and see how long you make it, knowing your prognosis wasn’t very good but that you might have the highest quality of life in the short term.

My mother had an intense fear of being a burden and being tied to an oxygen tank for the rest of her life. To her, that was a really big deal. It was a real possibility, but something that the surgeon “hoped” wouldn’t happen. It really scared her.

Wow, heavy stuff to think about. It was a very tough decision to make.

The decision: She opted to go for the surgery. It was her best chance to prolong her life. Everything I read about LMS I kept reading that surgery was the “best” option. I had seen what chemo does to patients and the odds of it working weren’t on our side. The best option was to get the tumors out. It had big risks, but the good outweighed the bad.

Was the doctor being too cavalier? Perhaps. But I figured, a doctor of Dr. Kaiser’s status can pick and choose what cases he does. His waiting room was packed! There are plenty of cases for him to do. They can pick the ones that they know they can win. Bad results make for a bad reputation. Everyone we talked to that new Dr. Kaiser concurred. If he says he can do it, he can do it………. She set the date.

Three and a half hours of waiting and we got the call from the surgeon. He said that everything went well, but he couldn’t save any of the left lung. He had to remove the whole thing. He didn’t want to push things too much, so he didn’t touch the right side. “We will let her recover from this step and go back and get the right side at a later date”.

Dr. Kaiser threw us a slight curve ball but as long as she was alive and not a vegetable, we were happy. We got into see her several hours later. She could breath. She talked coherently and could move her extremities. We were anxiously optimistic. The right side still had active tumors and she was missing her left lung, but she was alive.

Not only was she alive, but at 24 hours she was sitting up in bed. Forty eight and she was sitting up in the chair and off the oxygen. At 72 hours she was up and walking the halls. Four days and she was home! The only thing she took for pain was some motrin for a couple of days. Simply amazing!

We followed up w/ Dr. Kaiser. Two weeks, two months, four months, six months, nine months, a year……. The nodules on the right side didn’t grow. They seemed stable. Dr. Kaiser said there was no need to go in and get them if they weren’t growing. He took the approach “Wait and watch”. He officially discharged her at 1 year. “Come back if you get growth”. When we left Kaiser’s office, my mother was beaming. She was feeling good. She wasn’t on oxygen and she was living a high quality of life! We made the right choice. She jumped in the air and clicked her heels. She outlived Dr. X’s prediction!

We could have done chemo, but the oncologists feeling was let’s wait till we really need it. Let’s wait and watch.

My mother was living a very high quality of life. If you didn’t see the huge scar on her chest, you wouldn’t know she had cancer. Her pulmonary function tests w/ only one lung were in the 58th percentile, age adjusted for two lungs! She didn’t miss a beat. She mowed her yard, and shoveled her driveway when it snowed. She entertained friends, sang in the church choir, enjoyed her grandchildren, was a mentor for a young girl, traveled w/ her husband and college friends to Europe and even hiked a volcano!

One year stable. We continued to follow up w/ Dr. Algazy. Two years still stable. Three years one scan looks like we have a little increased growth in a couple of nodules. The CT report (done at Heart of Lancaster Hospital) noted that she had a normal prostate and appendix and they couldn’t tell if her tumor had grown or not because they lost her films for comparison!

Wait…….normal prostate?, normal appendix?……lost films for comparison? Women don’t have a prostate. Do you think one was added when she had a hysterectomy? Her appendix was removed many decades ago when she was a teenager. Someone please explain how you lose a 10 pound jacket of CT scans in a small hospital? How does this happen? How is there such a disregard for detail at a full fledged hospital? This is no time to screw up a CT report!

After some communication with the COO of the hospital, we finally tracked down her films and had her report amended. She did have some growth. The latest scan showed two new tumors in her left chest, one was invading her anterior chest wall It may be time for more surgery.

We went back to Dr. Kaiser who agreed that it was time to get out the bigger nodules. He went in and removed two of the bigger nodules, but stopped when he palpated her lung. She had many smaller mets that felt like grains of sand in her right lung. He didn’t want to remove any more tissue as it would have been counter productive. His feeling was if you can’t get them all out, why continue?

She recovered from this procedure like it was nothing and was promptly discharged after a two day stay.

This is probably a good point to highlight the importance of being informed. Patients must stay vigilant and try to gain as much knowledge as possible. The internet offers many options to get good information. I have found the LMS listserve and it’s information/discussions invaluable in our journey.

It was definitely time to treat this disease before it becomes too late and begins to cut into quality of living.

In three years, there have been some advances in sarcoma treatment. There were several newer types of meds, different approaches w/ chemo, monoclonal antibodies, vaccines, angiogenesis inhibitors, mTOR and aromatase inhibitors. We had options.

We discussed options w/ the Oncologist. He recommended a monoclonal antibody clinical trial and thought it might be a good option for my mother. We went in for the consult, but unfortunately, she didn’t qualify for that trial because of a pre-existing medical condition.

Dr. Algazy was great, but he just didn’t have access to much of the newer medication. Though we were very happy with Dr. Algazy, we also realized that it was time for us to work with an oncologist who actually specialized in sarcomas and could offer us more of the latest treatments. We are greatly indebted to Dr. Algazy and appreciate the high quality of care that he provided. He is a great physician.

We switched to Dr. Staddon’s care at Pennsylvania Hospital because we wanted to get into his newest mTOR inhibitor clinical trial. Unfortunately, it was full, but we did discuss other options. There is Yondellis, which is a drug made from sea squirts. There is always Gemzar and Taxotere which is a chemo combo that has been pretty successful on LMS. There was rumor of a newer targeted therapy that he was supposed to get on clinical trial in the next couple of months. Another option is to sit and wait. It is growing very slowly. It wouldn’t be wrong to sit and wait. My mother was living at such a high quality, that any treatment (particularly chemo) could interrupt that quality of living. The fact remained, her disease was growing. We waited another 3 months. .

Back to the list: Back to the net: More reading and research; What is the best current treatment? What is showing the most promise? How can we target our therapy and minimize the side effects and unnecessary trial and error of treatment? What can we learn about my mother’s tumor? The more we know about her tumor, the more effective we can be when we attempt to treat. We tested her tumor for a mutated p53 gene. If it was mutated, she might respond more favorably to Yondellis. (ET-743). She did have a mutated p53 but she was unable to get Yondellis because she had not failed another chemotherapy yet. Are you kidding me? If there is a chance that she could really benefit from this drug, why can’t she get it? Dr. Staddon’s appeals to J&J were not successful. She couldn’t get the drug. Just doesn’t make much sense. My search continued.

In the mean time, the tumors continued to grow. In fact the most recent CT scan showed increase growth of both tumors on the left side of her chest. They were putting pressure on her heart. It was time to head back to Dr. Kaiser for another opinion. My thinking was to get rid of this darn tumor in the left anterior chest that was pressuring her heart before it was too late.

Dr. Kaiser reviewed her scans and even ordered an MRI to determine if the tumor had actually invaded her heart. The results showed some evidence of invasion into her right atrium and right ventricle and therefore, she was not a candidate for further surgery.

I’m getting anxious…..It is time to treat!

I come across some information on DiaTech and the process of MiCK Assay. I notice there is a phase II clinical trial being offered on sarcomas. MiCK Assay tests a fresh tumor sample for chemo sensitivity, but bases it on to apoptosis (how well the chemo causes cell death). It is a new technique that seemed promising to me. It only makes sense that if you have to do chemo, you might as well use one that has been found to be sensitive. This might minimize side effects and the whole stress of the trial and error method that is utilized by far too many oncologists. All we needed was a small bit of tissue or fluid. She had that met in her anterior chest wall that should be pretty easy to get to. Let’s give it a try!

I contacted DiaTech and told them we wanted to get our tumor tested. They told us we couldn’t get into the trial, but they would be happy to test my mother’s tissue sample for free. They sent the collection materials to Dr. Staddon’s office.

Staddon wasn’t in favor of the test. He was a skeptic to say the least, but he did make the arrangements to have an interventional radiologist get a chunk of the tumor for the MiCK Assay. Because it was so anterior, the radiologist was able to utilize ultrasound and aspirate a good portion of fluid from the tumor that we could send off for testing.

If only someone would have read the packing instructions for the shipment of the sample. Seems the sample got packed wrong and it exploded in the box before it ever got to DiaTech. Someone’s got some explaining to do! After some phone calls to the COO, the head of radiology and anyone else who would listen, I did some barking and tried to get some explanation of how this could happen! Alright, another medical screw up.

Of course my mother wanted to try again. A week later, we gave it another try. This time they only were able to get 1/10th of the fluid they got the first time. Just our luck! They sent it anyway and the result was insufficient cells. You’ve got to be kidding me!

Give it another try? Heck, if it was worth trying the first time, why not give it a third try? Only this time, we are getting a thoracic surgeon to get a nice chunk of the tumor for testing. This would require general anesthesia and has risks associated with it. Are we sure about this? My mother believed in it, so we proceeded. It was an hour long procedure. They got a nice chunk. It was shipped correctly this time. My mother did have a little bleeding problem, but she survived the adventure without much incident.

It was now six weeks since we began this tumor testing quest. We hit a few snags, but we finally had a chemo match for my mother’s tumor. The bad note was that it was Taxotere which is one of the rougher chemo’s out there. At least we know it was sensitive and hopefully could be effective. It was an option for her.

In the mean time, a new targeted therapy, ARQ-501, which showed some real promise in phase I clinical trials, became available to my mother. Dr. Staddon was going to be one of two sites that had the new drug and it had very minimal side effects.

We had to make another choice….What will the next step be? She opted to give the ARQ trial a shot since it had the least side effects . If it failed, we would get going with the chemo.

The first scan after eight weeks of ARQ showed some shrinkage of one nodule, a decrease in pulmonary edema and slight growth in another met. The jury is still out, but we are continuing w/ the ARQ clinical trial. Time will tell if this drug will work for my mother.

My mother’s battle isn’t over yet. She’s still doing fairly well. Last night she had a group of people over for dinner. She tires a little quicker, but she is still alive and kicking! She is very much alive to enjoy her children, grandchildren, friends and living. She still has a high quality of life. She is not dependent on oxygen and enjoys getting out and about. In fact, the other night she was visiting the neighbors and it started to rain quite hard. She told me tonight that she actually ran home from the neighbors to beat getting soaked! (I’m sure running was relative and didn’t resemble anything close to a sprint).

The point of this lengthy story is that my mother has prevailed long after she was given the diagnosis of LMS. It is now 9/06 and she is still alive and kickin! She was diagnosed in 2/02 and was given 6 months to a year to live. In retrospect, if her original pathology report had been read correctly, she would have been diagnosed in 1997. Not only has she outlived their prediction, but she is still going strong on only one lung that is loaded w/ small mets!

There is hope. I am amazed how each week/month there is a new treatment option; another way to attack the cancer. The national genome project, the LMS tumor bank with Dr. Brooks, and the microarray being done at Stanford are just some of the things that will help to find the genetic answers and target better treatments. It truly is a battle against time. There is hope.

There were numerous times in my mother’s disease progression that the doctor’s and medical staff just screwed up! There are a lot of truly great physicians and support staff whose goal is to provide the best of care for their patients. There is also a good number of doctors/staff who do not pay enough attention to detail. Negligence and bad medicine happens all the time in the real world. You need to stay on top of your care and become an active participant in what happens with your life. Ask questions and expect understandable explanations. Don’t be afraid to seek a second or third opinion. If you don’t understand medical terminology get a good medical dictionary and take a tape recorder into your consultations with your doctor. Keep a notebook with all your reports and records. Be persistent and attentive.

Part of my mother’s success is due to her faith in God. I think that a belief in a holy power provides strength in times of trouble and weakness.

Part of her success is having an advocate to research and learn about the disease, disseminate information, and help with decision making. It is important to have help fighting the battle.

Part of her success is a will to keep going and not let a terminal disease get you down and keep you from living. There will be good days and bad days. You will experience a spectrum of emotions. Try to find a way to live each and every day.

We are still battling this dragon and our journey if far from over. We don’t give up hope and pray for a breakthrough soon!

For us, Persistence prevails when all else fails.

Good luck with your journey!

Note: This story was written by Eric (nussatcjb@aol.com) about his mother Fritzi (donferd@netscape.com). You can reach either of them at their email addresses.

For more information on possible alternatives for LMS patients,
visit Patient Resources on the lefthand menu of this website.

Back to Main Page - Our Stories

Last Edited - 27 September 2006 01:02 am