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Lorraine - British Columbia, Canada
The story of my experience with fibromatosis begins in the summer of 2002.
While I was getting dressed, I noticed that one side of my upper chest seemed 'different' than the other side. When
I investigated, I discovered a large, painless mass to the right of my breastbone, underneath my collarbone. I went
off to work, but I kept thinking about the mass. I went down to the walk-in clinic down the street from my workplace
and saw a doctor there. He examined the mass, and said he thought it was just that my ribs were differently shaped on
the one side. He sent me for x-rays though.
A few days later I had to see my family doctor to get my migraine prescription
refilled. While I was there, I mentioned the mass in my chest. She examined it and exclaimed 'Oh, my God!'
That was my first inkling that this could indeed be something serious. My doctor examined the x-rays that had been taken,
and told me that my ribs were fine, and the mass was not visible on the x-ray, so it was not made of bony material.
My doctor referred me to another physician specializing in breast cancer.
When I went to see her, she examined the mass and sent me to get an MRI scan of my upper chest. I had never had an MRI
before. The technicians instructed me to lie on a stretcher, which was then pushed deep into a large and noisy machine.
I could hear the scans even through the earplugs they gave me. The technicians were able to talk to me and explain what
was going on. I found the feeling of heat the scans generated in my chest somewhat odd. The whole procedure took
about 45 minutes. They seemed to run a lot of scans, and when the technician came to help me out of the machine, she
wouldn't meet my eyes as she told me that my doctor would be in touch with the results. I noticed that there were about
seven medical staff clustered around the screen where my scans were visible, although there were only two technicians when
I came in.
I was called back to my doctor within a few days. With compassion,
she told me that I had a large tumour in my chest, most likely a sarcoma. A sarcoma is a particularly nasty, malignant
form of cancer. The doctor referred me yet again to a thoracic surgeon to prepare to operate. She said she wasn't
going to wait for a biopsy because time could be of the essence, and biopsy results wouldn't change the course of treatment.
Whatever that tumour was, it had to come out.
The next few weeks were very tense. I had a bone scan that revealed
no cancer in my bones (good!), met the surgeon, and had a pre-operative info session at the hospital where the surgery was
scheduled. I spent time with my husband and family, and carefully chose my words to describe my situation to my 80-year
old mother. My co-workers brought me flowers on my last workday before surgery.
I went in for surgery early on a weekday morning. I remember being very
cold, and then waking up in really awful pain. Someone leaned over me and loudly told me that the surgery was over and the
tumour was benign. Then I faded out again.
I had a fairly uneventful recovery. The surgeon told me he removed
a large portion of my chest wall, including sections of my first two ribs. He'd placed a fabric patch where the chest
wall was missing, to keep my pectoral muscle from sticking to the lung underneath. I asked for a copy of the report
on the tumour from the lab. I'd never heard of fibromatosis but the doctor said it was a very rare benign tumour. My
doctors told me that I wouldn't need any follow-up.
After six weeks off, I was back at work. There was a significant amount
of pain in my chest and shoulder that did not go away no matter how many painkillers I took. I tried swimming and exercise
to see if I could strengthen my shoulder but it had no effect on the almost-constant pain. There were sharp, jabbing
needle-like darts in the back of my shoulder, a steady ache in my chest, and an explosion of pain that could knock me over
when I sneezed or coughed.
I use the internet a lot in the course of my job, and finding information
is part of my job description. I decided to see if I could find out more about the tumour. There wasn't a lot
of info, but there was some. As I got more information, I was taken aback to learn how frequently these types of tumour
recur. No one on my medical team had suggested any sort of follow up to see if the tumour was growing back.
I printed off a stack of medical reports highlighting the recurrence statistics
and brought those in to my family doctor. She could not feel any evidence of the tumour regrowing when she examined
the surgical site. However, she referred me to the local Cancer Centre. There I met a wonderful team of doctors
and nurses. One scheduled me for another MRI, while another one diagnosed my pain as nerve pain caused by the severing
of major nerves during the surgery. He prescribed two medications that relieved about 90% of my pain.
I had to wait about 5 months for the MRI. The doctor called shortly
after the scan and told me that yes, indeed, the tumour was growing back. This time it had grown into a less-accessible
location between my chest wall and shoulder. I went back to the thoracic surgeon and made arrangements for another surgery.
The second surgery went well, except for the fact that the surgeon was unable to get all of the tumour. Its indistinct
boundaries made it difficult to identify tumour tissue, and one part of the tumour was growing on the subclavian vein.
The subclavian vein is a major blood vessel that transports blood from the arm back to the heart. In order to surgically
remove the tumour from the vein, the part of the vein where the tumour was growing would have to be removed and the missing
section of vein replaced with a graft of a blood vessel taken from my leg. That's a pretty technical surgery and not
one that either I or my surgeon was prepared for at this point.
After surgery I had an appointment back at the Cancer Centre where my radiation oncologist set up a course of radiation
treatments for me. For 5 days a week, for 6 1/2 weeks, I would go to the Centre for treatment. The radiotherapy
did not affect me badly, and I was able to keep working close to my regular hours all of the way through it.
A couple of months after the radiation finished, I had another MRI to see if the radiotherapy worked and if the tumour
was shrinking. The MRI was clear - they couldn't find the tumour! That meant that the radiation had worked and
I had no visible evidence of the tumour anymore.
I will keep having follow-up MRIs to check for recurrance. If I go 5 years tumour-free I can consider myself cured. The
side of my chest where the surgery occurred is visibly swollen, and has been that way for months. No one is sure if
this is due to scar tissue, radiation reaction or the beginnings of tumour regrowth, but I suppose time will tell.
Catherine, Tennesee
I have FAP and I have desmoids..hehehe..kind of sounds like
an AA introduction ...
I was having severe pain attacks back in 1998. Usually it would wake me in the middle of the
night..the pain would have me doubled over, in tears and unable to breathe at times. This went on for 3 years. It was pancreatitis
brought on by a polyp growing at the pancreatic duct opening in the duodenum. Unfortunately I was pregnant through some of
this and had to wait until my baby was born to have surgery.
Three months after Emily was born, I had the lining of
my duodenum removed, the polyp removed and the ducts were resected. But then 3 months later the pancreatitis started again.
I was in and out of hospital for 2 more years. Put on TPN for a few months to rest the pancreas, and after many failed attempts
to keep my pancreatic duct open, I was transferred from my local hospital to Vanderbilt, TN for a Whipple procedure.
My
parents had come over to be with me during the surgery, and they heard the news before I did. I woke up in the recovery room
in absolute agony. Unfortunately because I had been on pain medicine for so long, I had developed a high tolerance to
it and it was taking a lot to try and control my pain from the surgery. They never did get it under control.
I had
to wait for 10 hours to get a room after my surgery. I was kept in the recovery area all that time, not allowed to have any
visitors. I thought that the surgery must have been a success as no one told me otherwise...I didn't see the surgeon or his
resident. About 3 hours after I came to, his medical student came in to check on me, and told me she was sorry to hear about
the tumors they had found...I asked her what she was talking about and that was when she told me that they cut me open to
do the Whipple, found masses of desmoid tumors on my pancreas, duodenum and in my mesentary and they were unable to perform
the Whipple, so closed me back up.
She apologised for being the one to tell me, as she thought the surgeon had already
come to see me. She left and a couple of hours later a nurse felt sorry for me as I had a rotten outcome and snuck my husband
in to see me so he could explain more to me. Around 10pm I was taken up to my room and more screaming occurred when they lifted
me to my bed..pure agony.
The next morning the oncologists came down and explained a bit about the tumors, that there
wasn't much known about them and they wanted to put me on Tamoxifen & Sulindac. I couldn't take the Sulindac because I
had tried that before with horrible results, so it was decided on Vioxx and Tamoxifen. They didn't wait until my iron levels
had increased to normal before starting me on the Tamoxifen and that led to a very bad and severe experience. Many ER
visits as I was in pain still, couldn't stand up without getting dizzy and feeling faint, and nauseas, tingling in my limbs,
climbing out of my skin..and the hot flushes caused by the Tamoxifen.
I saw my oncologist after I had been sent home
for the 5th time by the ER doctors, and was admitted on sight. White as a ghost, black circles under my eyes, unable to stop
vomiting, and enzyme levels astronomical. He gave me an iron transfusion and I returned to normal within hours of it being
administered to me.
I was on the Tamoxifen for only 4 months, but it didn't appear to be working. I went to MD Anderson
for a second opinion on the tumors and to see if I could qualify for the Gleevec trial they were doing..I didn't qualify.
They told me to get off everything and if they grew they would then try me on Adriamycin, if they didn't grow they were satisfied
to call it scar tissue. I went home to Tennessee, and went off everything. I tried to use a Chinese herbal formula to shrink
the tumors, but stopped that after 4 weeks because I don't know if it was coincidence or not, but a recent CT scan showed
that my tumors had doubled in size in a 3 month period...not good.
Surgery was scheduled for January 8th 2004. I saw
the surgeon before he put me under and told him that a lump on my chest on the sternum was causing severe pain, and he said
"okay, it's out of here, we'll remove it whilst we have you under"...
I woke up from surgery..pain was controlled much
better, mostly because I had been cured of my pancreatitis 7 months beforehand and had been off all pain meds for that time.
My doctor came in to see me and my husband in my room and told me that I had lost about 7 inches of rectus muscle, and clear
margins. He then told me that the small lump on my chest wall was actually the size of a baseball growing under my sternum
towards my lungs, and it was lucky that I noticed it. He said it didn't look like a desmoid, but pathology proved that it
was.
I've been pretty good since then...except my skin remaining was so thin as the tumor was close to the outer layers,
that my incision split open when I got home from having staples removed and I had to pack it for 2 months.
It is May,
and I am having pressure on my bladder again..some pain around the area where a small desmoid was in a previous scar but didn't
appear to be growing, and my chest hurts if I touch the area. I went for a checkup because of all this pain. The surgeon said
he could feel scar tissue around the parts, but if there is bad pressure on my bladder it could mean something is growing
deeper than what he can feel. Neither of us want to find anything at the moment because I'm due to go visit my family in Australia
in 3 weeks time. I have an appointment for when I get back in August, and will have a CT scan then.
Oh and the tumors
in my abdominal cavity????...on a CT scan after my surgery in February, showed that they aren't there anymore. They have just
vanished...I haven't been on any medications so that can't be the reason for their eradication...my mother believes I was
given a miracle..so do I...especially when my doctor told me that when he read my report from the surgeon at Vanderbilt, he
believed I would have only had 3 years to live. That knocked me for six because no one had told me they were as serious as
that. I knew they were serious, but not enough to end my life so soon....
Lets hope they stay away.
Stephanie - Pennsylvania
My story started when I was diagnosed in 97 with FAP. I had my colon removed in 98 to avoid colon cancer. I initially
had an ostomy that was reversed to a Jpouch. I lived with this for about 2 years. I slowly started feeling a hard
"area" in my right lower abdomen. I was trying to get to my surgeon for a jpouch scan, however with bad weather, and
difficulty finding someone to take me to the appt, since they had to put me out, the scan got pushed off month after month.
Finally, when I got to the surgeon, his eyes got sorta big. He first asked if I was pregnant. It wasn't a nodule, it
was as if I was pregnant on one side of my body. I said, no I already checked that. Well, he did the jpouch scan and
then sent me for a CT scan.
I had the CT scan about a week later, April 28, 2000, to be exact. I was suppose to have my bridal shower the
following Saturday. Well, immediately, the radiologist that read my scan called my surgeon. He said, "you have to get
her admitted, this huge mass is compressing her ureters and her right kidney is backing up" I happened to be in the
same facility that day seeing the gynecologist. When I told him that I just had a CT scan for this "mass" , he looked
panicked. He called down to get results and came dashing back into the room telling me what the radiologist had said
to my surgeon. He said they want to admit you and put a nephrostomy in your back to drain your kidney, so
it doesn't do more damage. Well, when I got dressed and got to my car, I noticed my beeper (from work ) had like 4 pages
on it. It was my surgeon's office down at Johns Hopkins Hospital in Baltimore. They wanted me down immediately to drain
my kidney. I asked if it could wait until Sunday ( it was a Friday) because my bridal shower was "tomorrow".
They said, no the ureters are very delicate and we shouldn't really wait. I guess I didn't comprehend the situation
at the time.
Well, that afternoon, we drove 2 1/2 hours to the hospital to be admitted. I was expecting my kidney to be drained
immediately, but for about 3-4 days, I just had doctor after doctor coming in to see me. It was diagnosed as a desmoid. Every
discipline possible was in to see me. I was a real learning experience for these med students and residents. It was
the size of a football. Not quite sure how I didn't notice this big thing, because, I only weighed 110 pounds. But they said,
it was filling in the space where my colon was. Nobody knew quite what to do. They really never dealt with this before.
They did MRIs, MRAs, blood tests, all kinds of kidney tests and the works. They were consulting all over the country with
specialists. Finally they put the nephrostomy in to drain the kidney. Boy, did that hurt like "#$@#" They
gave me a shot of morphine and that knocked me right out.
Well finally on Wed, may 3, the surgeon felt they could go in and "pull" it right out. All the tests showed that it
was an independent structure and didn't look like it was sharing any blood supply and blah blah blah. They scheduled
the surgery for Friday, may 5, he had an opening available. Well, Thurs, may 4, they had me sign all the consents, for
the urologist to fix the ureter, the GI doc and I think there was a vascular guy, just in case. Reality hit when they
asked me what I wanted to do with my kidney if the ureter couldn't be saved. They didn't want to lose the kidney, in
case down the road I would ever need chemo (although at this time they ruled out trying radiation and chemo, because
it doesn't work on desmoids) So they wanted to auto transplant it to the other side and hook it up to the working ureter
if needed. How was I to make that decision in 5 minutes.
Well, I told my surgeon, going in to the surgery, I didn't want to live with an ostomy and to not take my Jpouch out.
I said take out as much of the tumour as you need, but please save the pouch. My husband (fiancé at the time) and I
said everything would work out, because everything always works out for us. they gave us best and worst case scenario.
What I didn't know until obviously months later was the degree of risk. My sister spoke with the surgeon and he told
her how risky the whole surgery was. He just agreed with her when she asked if it didn't look good. She was online reading
the mortality rate of these big abdominal tumours. Just what everyone needed to hear!
Well, to cut this short, the surgery was 15 hours long. When they opened me up, things were not as they looked on the
scans. My intestines were stretched over this tumour so tight, he didn't know how I was even getting food
to pass through They had to take my jpouch, all but 3 feet of small intestine, and they removed the gall bladder. I
have a jejunostomy. ( The surgeon came out of the surgery and told my mom that the jpouch had to go and she said that Steph
didnt want that. He made her tell him to let him remove it. He said if he couldn't remove it, his option would be
to close me up and my systems would probably shut down in 1 -2 weeks) They had to do a vein graft to my right
leg as the tumour was compressing a major vessel. I had nerve damage in my left leg from positioning during the surgery
(95% back to normal). The ureter WAS able to be repaired. They put stents in them to make sure scarring wouldn't shut
them down. So both kidneys stayed. I now live on TPN. I have a central line in my chest to give me nutrition.
All in all, my recovery from that desmoid and surgery was uneventful. A 3 week hospital stay to get PT, to make
sure no internal bleeding, to make sure the kidney was ok and to wean me on to TPN appropriately. At first no one was allowed
to tell me how serious everything was because I had the control/ the will to live or die. But I knew I had an ostomy
in ICU. I woke up with all my friends around ( they snuck them all in) and looked at my fiancé and said, well at least camping
will be easier with an ostomy. I always felt camping with the jpouch was a pain in the butt, since you had to go to the bathroom
more often in the woods. They all just laughed. I always dealt with every thing pretty good. I guess I felt that if
I didn't, I let this disease win.
We did get married 2 months from the day I got out of the hospital. There was not a dry eye at my wedding. It was a
wonderful day.
Unfortunately, yes there is more that I will make short. In 2001, a nodule was found in my left rectus muscle,
right next to my ostomy. I want to say it was about 6 cm big. They were scared to leave it in, so chose to remove
it before it got too big to where it invaded my small intestine. I met with the plastic surgeon. They were prepared for
grafting from a muscle in my leg if the tumour invaded too much muscle. However, they did not need to do that. The plastic
surgeon was able to "stretch" the muscle around the stoma. I did feel as if it was too tight at first. He said I
may need to come back in to have it loosened, but it should loosen on its own. For awhile, it hurt when food came out of my
stoma, but then loosened up like they said.
In October of 2002, I was getting sharp pains in my right groin. I had been getting MRI's every 3 months, but
no one told me there were any tumours. Finally I cried to my surgeon and said is there anything going on in my groin, because
I am in ALOT of pain. He said well there are two small (like 2 cm big) tumours but felt they were so small, they weren't
causing any trouble, so he was just monitoring them. When I explained the pain I was having, he said that the
location of them could definitely be causing the pain. He was leaving it up to me to decide to have them
removed or not. I was in so much pain, I chose to do so. So he did the surgery in January 03, there were 3 tumours
all connected together. He said after seeing them, he doesn't doubt I was in pain. A third one was found once he got
in there. They had to call in a plastic surgeon to reconstruct my groin muscles. The muscles had to be removed
along with the 3 tumours. They used alloderm, which is tissue from a cadaver. It takes the place
of whatever was in there. Pretty neat stuff. I had PT for about 3 months and am again about 95% back to normal.
I still get stiffness, but nothing a little stretch doesn't fix.
Finally we get to where I am today. Again, in June 2003 I found a nodule in my left rectus muscle. Same spot
as the first one. My surgeon was hesitant to do surgery as my surgeries were getting closer and closer. He said
if we keep cutting me open, there wouldn't be any Stephanie left. He consulted with some desmoid gurus. I was
first put on high doses of tamoxifen for about 6-8 weeks with the tumour still increasing. Then we tried gleevec which
is supposed to be less toxic then traditional chemo. I hurled my guts out. Which at least meant I was absorbing it.
That was an issue, because I only have 3 feet of intestine and don't absorb everything. Depends on where it is suppose to
be absorbed ( stomach or intestines) Anyway, the tumour grew significantly after being on gleevec for 6 weeks. I am
now on Adriamycin, which is the traditional chemo. I am finally getting shrinking results and am very pleased. Yes,
there are side effects, but if we can shrink it and maybe get rid of it without cutting, maybe we can stop this
cycle of these tumours growing.
Every desmoid I have had has been where there has been surgery or repair or some sort of cut. So we are hoping this
chemo can wipe this horrible cycle out of my system for a little while anyway and move on with my "normal" life for awhile.
If anyone ever has any questions, please feel free to email me at stephot1@hotmail.com
David,
Great Britain (submitted by his brother, Chris)
Hi
Lorraine,
I
came across your website a few weeks ago when my younger brother David (25) whom was suffering with aggressive fibromatosis
moved into a critical condition where we decided to look for help ourselves outside of his consultants non-advice. If I may I will give you some history of David &
our family.
Our
Nan recently passed away, from other causes but had desmoid fibromatosis in her earlier life within her stomach, but following
successful operations had all evidence of the tumour removed. She did have FAP
& the faulty gene had been identified in our family which subsequently can now be tested for with just a blood test. David tested positive for the faulty FAP gene, our aunt (mums sister) & Mel (our
sister) tested negative, and I have never had the test but am well.
Our
Mother (Nan’s Daughter) passed away in her early 30's due to a decade of battling with aggressive fibromatosis tumour
within her stomach, she had numerous operations over the years but in fact finally passed away from septicaemia (blood poisoning)
from the years of drug treatments, tumour toxins etc. David was just 9, Me (Chris) 12, our sister Mel 16.
However
when David turned 15 a lump which was initially believed to be fatty tissue appeared at the top of his spine. Sometime after a small biopsy of the lump which also reported as fatty tissue led to the lump being removed
in major surgery. Its size
was that of a small football, following further tests the tissue lump removed was found to be desmoid fibromatosis. Unfortunately the operation could not fully remove the tumour as a small bit was left because it was too
close to the spinal cord. He
was treated with Radiotherapy, given the all clear and led a somewhat normal but scarred life for the next 2yrs.
He
then had a ground-breaking skin-transplant at the Royal Marsden Chelsea-London, which really made us wonder whether they should
touch the benign area of the dormant tumour?
You
see in our mum’s life, when ever they cut into the tumour, it then almost seeded & grew more aggressively, but they
said that this is not the case.
Sometime
after this operation (about a year) the tumour had started grow again, this was seen both in scans etc & could be seen
to start to protrude from the scar tissue. They then treated the tumour with various different assaults over the next few years from Radiotherapy, Chemotherapy
& numerous other drug trials (around 7(stage 1)).
The
tumour wasn’t necessarily growing fast but it was growing steadily & started to come up around his neck.
As
it got larger, the drug trials & strength of various Chemotherapy treatments was increased & to be honest we believe
may had possibly encouraged growth, however you have to try just in case.
The
last six months have been so hard, the tumour moved so fast into David’s neck area & up towards his head, physically
it turned into making him resemble hunchback as his small nephew commented & David laughed away. The pain where it was
stretching his nervous system & the drugs to mask the pain collectively started to cripple David physically. The Marsden, although we feel it wrong to be bitter, seemed to abandon David with no contact as they don’t
do any sort of home visits when David got to the point where he could no longer taxi to the Surrey Marsden. They still had him under one of their leading consultants
so no-one else could touch him or medically decide for him other than a pain doctor in prescribing him morphine, netenox etc.
We
became desperate, David had not seen his main consultant for 9 months & his growth from then to when we found your website
was absolutely astounding. David
wanted surgery, we all wanted it but were naturally scared of the risks & with no communication we took to the net to
see what we could find. Naturally there is little to be found about aggressive fibromatosis, and what we could didn’t
resemble David’s condition. On reading the fear of understanding (lots coming from your site and links) led to a realisation
of surgery being an impossible turn. Hind sight is a great thing, but with it
years ago David would have insisted on further surgery, there is no treatment & if there was for someone else it doesn’t
make it so for everyone. What
my mum's tumour responded to, David’s didn’t.
David
never stopped fighting, he never complained nor seeked pity, he was a young man who battled from childhood with aggressive
fibromatosis, he was a hero. David passed away 2 weeks ago, he read your website a week or so before, he
still held hope for surgery because he was a born fighter. I know he has won
his battle & is at peace with him mum now, reunited.
Thank-you for your site, it is informative and helpful, we were led to believe only 1 or
2 people in the world suffered with this condition when our mum died years ago but from information on your site we now know
this isn’t the case. Find out what you can, don’t believe your consultant
knows all & stay strong, so much needs to be studied.
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