Monday, March 31, 2014

Schwannoma Case Study # 3, Sean Anderson Tells us About his Fight With Schwannomatosis

Welcome to Schwannoma Case Study # 3, this one featuring a friend of the Schwannoma Survivors page Mr. Sean Anderson.  Sean has been kind enough to take a bit of his time to describe his fight with Schwannomatosis, the condition that results in multiple Schwannomas in a person's body.  Though the large majority of those who have Schwannomas have single tumors that are one off occurrences - people who are diagnosed with Schwannomatosis are pre-disposed to having multiple tumors at once in various nerve pathways almost anywhere in the body.  We want to thank Sean for taking his time to describe his condition.  He has had a hard struggle - but he has kindly offered to write a bit about it in the hopes that his story helps others who are living with Schwannomatosis. - Neil
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1)  How old were you at the time of your diagnosis?

It was March 2011, I was 36 years old. My diagnosis was given to me after a biopsy. I had a "walnut" size growth surgically removed from the nerve sheath of my spinal cord, in a lower lumbar vertebra. There was a fair amount of certainty of Schwannomatosis from MRIs, but the doctors, radiologist, and neurosurgeons would only speculate. It was only after getting in there to really see what was happening, and the biopsy, that I was officially diagnosed.

2)  Do you have any history of Schwannomas in your family?

Nothing official, however, it is worth mentioning that my mother was having weird pain and numbness in her finger from a lump growing in one of her knuckles. She was 50, and it was 1999. It was surgically removed. The surgeons, and doctors were baffled, It was biopsied inconclusive, and they told her it was some sort of weird nerve tumor. Due to other health issues, she's since had a number of scans that have not revealed or indicated anything further. It was mostly forgotten about, until my ordeal. She's not had any symptoms, or related issues since.

3)  Where was your Schwannoma located?  And what were the symptoms that lead to your diagnosis?

The short version:
The Schwannoma I had removed was directly on my spinal cord, in a lower lumbar vertebra. It was pinching off my sciatic nerve, and causing immense and growing pain in my lower back and down my left leg. It was slowly paralyzing me.
Here is the long version:
When I was a teenager, I had weird pains down my legs. I was always told they were growing pains. I accepted that answer, and was glad I was 6'1". Later, in my late twenties, I experienced sharp pains shooting through my legs. This wasn't very frequent. It was usually when trying to fall asleep, but not having my hips in my favored sleeping position. I accepted this as due to a physically demanding profession. There were a few lower back issues in my early thirties, but these were always thought to be work related. Then, one Saturday morning in January 2011, my dog jumped the neighbor’s fence, and was trapped in their back yard. I climbed the tall wooden fence, and was able to then climb down the other side. I pushed my dog over the fence, only for her to run off in another direction. In pursuit, I climbed back up the fence, and jumped to the ground. As I landed, my back buckled. I figured bed rest the remainder of the weekend would have me back to work Monday. No luck, Monday I was at my doctor. As common for a back injury, I was prescribed a few painkillers, and muscle relaxers. By Thursday it was clear something was very wrong. After some convincing, my doctor sent me to a small "open MRI", where they took images of my lower back. The results had my doctor sending me to a different MRI, for images to be done both, with and without, contrast dye. During this time, things were becoming much worse for me pain wise. Those results had me summoned into his office to be told I was now going to be sent back for a full spinal and cranial MRI. They'd found a "mass", and wanted to know what exactly is going on, this was nothing to do with an injury from jumping down from a fence.
Nobody likes to hear about a "mass". My general practitioner said I could call it whatever I want, a mass, legion, tumor, lump, or growth, it didn't matter. Without a biopsy, which I was assured was coming, they didn't like assuming anything. Like anyone else, I was thinking cancer.  The full spinal/cranial MRI revealed numerous "masses", including one on a certain nerve of my brain stem. I was promptly referred to a local neurosurgeon. The pain in my back, and leg increased daily. I was put on Dexamethasone to ease swelling until I could get into surgery, and was on moderately heavy narcotics for the pain. I was slowly loosing sensation, and strength in my left, and later my right leg. Without having this schwannoma removed, I would have been paralyzed pretty quickly, as it was growing very fast after the fence incident. The first MRIs indicated it was acorn size, but it was walnut size when the surgeon removed it, less than two months after my initial doctor visit. Keep in mind; this is inside my vertebrae, on my spinal cord.
The schwannomas, as they turned out to be, were many. My brain, cervical, thoracic, and lumbar portions of spinal cord all have schwannomas. The many in my very lower back are "too many to count".
Also, worth noting, there are none to be found anywhere else, only my spinal cord, from brain to bottom.
The third MRI had the neurosurgeon asking many long probing questions relating to family medical history, and any "other" weird symptoms of unknown cause. I was scared, because they seemed truly intrigued, and I was becoming paralyzed.
Sorry for the length here, but I've come to understand there is no short and simple way to come to a Schwannomatosis diagnosis. I hope this isn't more than people care to read. I also hope it helps someone else out there. The not knowing or understanding was brutal during those first few weeks. It seemed I could find nothing on the subject, just one description that website after website copy and pasted from somewhere.

4)   Could you describe, in whatever detail possible, what kind of surgical treatment was performed on your Schwannoma, and if you would consider it successful? (Or describe what you may know about the surgery that may yet be performed, if you haven't been operated on yet)

Let me start by stating it was successful, because today I can walk. It's been a little over three years since the surgeries.
After weeks waiting to get into the operating room, I underwent the surgery to have this mass biopsied, (and removed if the surgeon felt it would be possible while he was in there). Prior to the operation I was educated at length on the risks of bringing a scalpel to my spinal cord, as well as the probabilities of waiting, and the certainties of doing nothing.
85% was removed. The biopsy confirmed all schwannoma suspicions. The initial surgery went off without a hitch. There were monitors hooked up to my head, hands, and feet, to determine whether motor control nerves were being affected. All cutting on this mass stopped when nothing more could be removed without sacrificing motor control, and thus, my mobility. After all, this was primarily to get some of this mass for a biopsy. I recovered in the hospital for a few days, and was sent home to return a week later to have my stitches taken out. The day before I was to have my stitches removed, my spinal cord tore open at the site of the schwannoma. I was unknowingly leaking "cerebral spinal fluid" internally. The pain grew fast, and tremendously. Calls to the doctor were fielded by an assistant believing I was trying to hustle more pain pills. I was told to go to an emergency room, if it was in fact, as severe as described. "You have an appointment tomorrow morning" I was told. Feeling stupid, I simply ate more pills in an attempt to combat the insane pain. Soon, cerebral spinal fluid (CSF) began gathering all around the entry site, and later began to seep through the stitches, and lots of it. A friend found me in a terrible state, and got me to the emergency room as the headache began. This headache (very much more than an "ache") was "intracranial hypotension" from my brain settling within my head, no longer enveloped in the very necessary CSF that protects it from contact with the skull.
The next morning there was an emergency surgery to go back in, through the same incision, and repair the rupture. I was informed that a bit more of my vertebra was removed, and my spinal cord was now patched even higher. I was made to keep flat and lie still in the hospital for days, having MRIs to monitor the progress. The day before I was going to return home, again, my spinal cord tore open. MRIs, and PhDs suggested, my body was regenerating spinal fluid at too high a rate, and the pressure was blowing out the patch.
Just like you may give blood and your body manufactures more, so goes CSF. As long as you're not losing it at a rate greater than your body can reproduce it, apparently, it's not a big deal. However, since my body was going overboard in its production, it was a big deal.
The final surgery was done. More of the vertebrae removed. This time, a "shunt" was placed into my spinal cord, a sort of “bleed off valve”. I was told I would spend two weeks lying perfectly flat, not moving, and having a nurse come drain off 10ml of CSF every hour. Being still was highly emphasized, I was given whatever needed. It was explained that if this didn't work, it wasn't known what more could be done, and I should prepare for a life of altered mobility. It was said that they really didn't want to induce a coma, so lay still I did.
I won't get into all the issues involved in a nurse coming to bleed off 10ml of spinal fluid every hour, around the clock. I will mention that it was very slow, and could sometimes take ten minutes, and they insist on waking you each time, if they can. There were a number of incidents, and issues. One particular time, after a nurse came and opened the valve, she left to briefly attend to something, but forgot to return. It was bad; I'll leave it at that.
In conclusion, it worked. I had three surgeries to my spinal cord within 18 days. Finally, I was able to go home from the hospital without it tearing open again. Today, I walk around, and can drive a stick shift. I'm calling it a success. Had I elected not to have any of this done, I would surely have suffered incredible pain, for a long time, only to be left completely paralyzed in my left leg at the very least.

5)   Having gone through the experience, what do you think are the most critical questions for someone to ask their doctor about surgery and treatment of this kind of schwannoma?

I will answer strictly speaking only about the kinds of schwannomas I'm familiar with, located on the spinal cord, as well as what has been transmitted to me by the professionals who've worked directly with me, on my case.
My understanding is that at this time, there is no such a thing as "treatment" for schwannomas, and I mean nothing. There is only removal, by surgery, and ONLY when the risks of inaction outweigh the risks of surgery. The risks of surgery are very high, as you may expect with the spinal cord. We're talking about cutting directly around, and on your spinal cord after all. It was expressed to me that, obviously, the closer to your head, the more valuable the real estate. It's the difference between losing the use of your legs and bowels, or the function of vital organs. Oddly, the one in my brain, which terrified the crap out of me, was of the least concern to the surgeon. He explained the brain is soft; it has plenty of room to allow growth. The ones that scared him, we're the few within my cervical, or neck, area. There is far less room to grow, and they can affect any number of things below, in any number of disastrous ways. They are also definitely NOT somewhere to go operating, unless to simply preserve life. Location, location, location.
Observation with MRIs, and alertness to any odd symptoms, seems the only thing to be done.
- Know ALL the risks. I've lost all sensation in much of my left leg. There is a "dead patch" that goes from my anus to my pinky toe. It wraps around my leg in places. I feel neither pain, nor temperature. This wasn't emphasized as a possibility in pre-surgery discussions, as they were clearly much more concerned about motor control, and the possibility of me being in a wheelchair, or incontinent. I mention this only to urge someone to know ALL the risks of the operation, however slight, and try to see past what are immediate concerns of the doctors. Believe me, I have no regrets, it's a very small price to pay, to be relieved of the suffering prior to the operation, and maintain full control of my legs. It just wasn't something I was expecting, assuming I had any idea what to expect post-surgery. We sure knew what to expect without surgery, I was already in the middle of it.
- Ask to know about your doctor's experience in any operations he’s performed relating to the spinal cord. I didn't, I trusted that he'd done many, and often. This isn't somewhere to trust. Get verified confirmation. The risks are simply far too high. If anything goes south, live the remainder of your life knowing you had a surgeon who was very experienced, and you weren't just part of someone's learning curve. In this respect, I was fortunate to have someone qualified, though I hadn't confirmed anything on my own.
- Ask about details of what recovery may be like, assuming everything goes smooth, as well as, if it doesn't. I was only concerned about getting this thing removed as soon as possible, recovery was an afterthought. Nobody seemed focused on it much.

6)   Can you describe what the recovery process was like for you – if you feel you have made a positive recovery…and what things you have done that have been of most help to you?  (Also, you are free to describe what doctors tell you to expect from your recovery if you haven't been in surgery yet)

I'm going to describe recovery, beginning with my return home, and assuming the reader is someone who's not experienced any long term, post-surgery hospital stay. There were a few surprises for me. I suppose I was supposed to "know" a few things, or, actually read through the volumes of paperwork you go through at the hospital. I doubt I'm the only one who didn't.
- Be aware that you may experience somewhat serious episodes of constipation. This is a side effect of the opiate pain relievers. I was given stool softeners that were little help after weeks of all sorts of pain medications.
- I didn't have any kind of physical therapy rehab. I remember everybody I came across later asking, then being in disbelief about it. I don't even remember it being discussed, or offered. I speculate that it was assumed that I was in good overall shape before, so, I’d return to good shape if I could still use my legs. This is what I assumed. I would advise getting answers from your doctor before, and have a plan. I wish I had.
- One of the toughest things for me was being unable to bathe, or shower for a week after I returned home. Having three surgeries through the same site in rapid succession made for an ugly scar, and tough stitches, stitches they did not want to get wet. This sounds insignificant, but it troubled me, maybe more than it will others.
- Worth noting about the lead up to the surgery, and the recovery after, was weight gain. This was due to the steroids (Dexamethasone) I was taking to keep the swelling down, preventing paralysis, and easing my pain. My experience was very bad. My primary care doctor was getting frantic that I was going diabetic, and forming cataracts from the high doses of Dexamethasone. The eating, and weight gain was crazy. I gained forty-five pounds, in about thirty days. I was covered in a rash of acne, had terrible gastric & indigestion problems, and emotional mood swings, swollen feet, and ankles.  In the hospital, post-surgery, they had to give me daily insulin injections.
- I've made a positive recovery. Today I have a few limitation, they are minor, if not insignificant, bearing in mind the magnitude of the whole thing.

7)  Are you able to do all or most the things you did before? (Also feel free to describe your abilities even if you are still living with the tumor or tumors)

I am able to do nearly everything I was able to do before.
In addition to this condition, I also have degenerative discs in my spine.  For the most part, I get around like someone with a weak, painful back. There are occasional flare-ups, but there are also days, or weeks that go by with little more than sharp pain when I bend over to pick stuff up, or when putting on shoes and socks. I have learned to always recline when sitting, and avoid walking, or standing for long periods. Running, jumping, lifting, or carrying heavy loads are no longer options for me.
I don’t want to go into the long list of issues I have, because I feel they are insignificant compared to what the many Schwannomatosis sufferers live with, also, they can easily be imagined as what anyone recovering from lower back issues such as a slipped or herniated disc would experience. This is not to say that this is all I will ever experience. There are doctors waiting, and watching to see what’s going to happen next.
I’ve been told to expect, and be alert to, or expect any combination of the following: Incontinence, seizures, pain, numbness, and burning, itching, loss of strength or movement.


Sunday, March 30, 2014

Schwannoma Case Study # 2, Kris Sres, with Posterior Mediastinal Schwannoma, Lower Esophagus

Hello everyone!  Welcome to case study number 2 in our series "Schwannoma Case Studies."  This one features a brief Schwannoma case study from a lady by the name of Kris Sres in Australia.  She is experiencing a Schwannoma in the lower esophagus, and as this was one of the more unusual Schwannoma stories that I have heard - I asked her to answer our Q and A for a case study.  We thank Kris for her time, and wish her the best in the future with her treatment - Neil
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1)  How old were you at the time of your diagnosis?

43

2)  Do you have any history of Schwannomas in your family?

 Nothing that I know of.

3)  Where was your Schwannoma located?  And what were the symptoms that lead to your diagnosis?

 Located in posterior mediastinal, grew out from out layer of the lower esophagus, right behind the heart.  Only symptom is swallowing difficulty. 


4)   Could you describe, in whatever detail possible, what kind of surgical treatment was performed on your Schwannoma, and if you would consider it successful? (Or describe what you may know about the surgery that may yet be performed, if you haven't been operated on yet)

Based on two surgeons I visited, "keyhole surgery is less likely due to the odd location", open chest surgery is most likely. Might be possible to perform the surgery from my left side even though the tumor is on right side of the esophagus. 


5)   Having gone through the experience, what do you think are the most critical questions for someone to ask their doctor about surgery and treatment of this kind of Schwannoma?

Always ask what the options are for the treatment, what is the percentage of the complication after/during the surgery, what are the possible complications, what is the percentage of the tumor regrowth, if it's possible to treat it without surgery. 



6)   Can you describe what the recovery process was like for you – if you feel you  have made a positive recovery…and what things you have done that have been of most help to you?  (Also, you are free to describe what doctors tell you to expect from your recovery if you haven't been in surgery yet)

What I could expect from recovery: leakage when I swallow at the point where stomach is connect with my throat as there is a chance they have to cut my esophagus during the surgery then pull my stomach up to connect to throat; possible lung complication as they will need to deflate one lung as least...Those are only what I remember ... didn't really try memorize the rest -:)  



7)  Are you able to do all or most the things you did before? (Also feel free to describe your abilities even if you are still living with the tumor or tumors)


Yes, I'm able to do everything I can before I learned I had a tumor -:) only thing I do little different is I have to eat slow...

Schwannoma Case Study # 1, Lumbar Schwannoma at L 5 S 1 , with Regrowth, Requiring Three Surgeries

Welcome to the first of our new series titled "Schwannoma Case Studies."  These are personal question and answer case studies where Schwannoma Survivors have agree to share their stories in hopes of helping others.  The subject of case study number 1 is a person who chose to remain anonymous so that the entire story could be told.  We commend this person greatly for their courage, and the great heart shown in surviving this ordeal.  And we hope that this information will be of help to others battling Schwannomas and their aftereffects. - Neil
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Schwannoma Case Study #1, Lumbar Schwannoma at L 5, S 1, with Regrowth, Requiring Three Surgeries


1)   How old were you at the time of your diagnosis?
      
I started feeling the pain of the schwannoma at the age of 15, but went misdiagnosed until the tumor was discovered, when I was 18.

  
2)   Do you have any history of Schwannomas in your family?

There have been no others in my family to have a schwannoma...Thank God.


3)   Where was your Schwannoma located?  And what were the symptoms that lead to your diagnosis?

My tumor was located at L5-S1 on two occasions, but on the third recurrence, a piece of it was also located in the sciatic nerve to my right leg. My pain started when I was 15. I shouldn't even call it pain at that point, because it just felt like my tailbone was a little bruised. By the age of 16, that "slight uncomfortableness" had turned to pain. With every step I took, I could feel the pain radiate down my right leg. It was no longer a pain that I could tolerate, so I had many trips to different doctors to figure out what was wrong. Unfortunately, I was misdiagnosed for 3 years. I heard everything from, "there's nothing wrong with you", you bruised your tailbone", "you must be depressed, are you having trouble with a boyfriend", "you must be anorexic", to my favorite "you are just doing this for attention". I had also started experiences changes with my bladder and bowels. It was becoming increasingly difficult to urinate and I suffered from constipation. At times my pain became so severe that I ended up on many occasions in the ER, with some doctor shoving his finger where the sun don't shine, trying to see if he could feel why I was in so much pain. By my senior year of high school, the pain was unlike anything I had ever felt in my life. Walking was unbearable and sleep was nearly impossible. I could no longer lay flat, so I had to sit up in bed and wait until I passed out from exhaustion to get any rest. This lasted until my first year of college when I went to see an Orthopedic doctor and ask for a MRI. Upon that visit, he first asked me how I heard about MRI's and second, proceeded to tell me "it's not going to show anything, but I will do it just to shut you up". Well, the rest is history. I was called right after the MRI, by (a very panicked) him, to inform me that all the pain I had been suffering from, the pain that made me contemplate taking my own life, was due to a tumor in my lower lumbar. I was shortly contacted by a neurosurgeon to schedule a meeting to discuss surgery.


4)   Could you describe, in whatever detail possible, what kind of surgical treatment was performed on your Schwannoma, and if you would consider it successful?  

I had my first surgery in January of 1995. The surgery took a little over 8 hours. I had a laminectomy and removal of what was thought to be the whole tumor. I spent a week in the hospital on a morphine drip, which hardly did anything for the pain. I had paralysis down the front of my right leg (foot drop) and my bladder and bowels were also paralyzed (considered dead). So, at the age of 18, I had to learn how to self cath, which I picked up before I even left the hospital. I also became laxative dependent due to the paralysis of my bowels. It took a good 6 months to fully gain my strength back and I even built up the strength in my right leg, so I no longer had foot drop. Given everything I had been left to deal with, I considered the surgery to be a success because the horrific pain I felt for so many years was finally gone. However, 4 years later, my pain returned and the tumor had regrown from cells that were left over. I had my second surgery in 1999 during my Masters program in college. I was very eager to graduate on time because I was in an accelerated 10 month Masters Program. I had the surgery during Christmas break, and was back to school and my internship 2 weeks later (yes, that was pushing it, but I did what I had to do). A short 3 years after that surgery, the tumor regrew again and was also growing in the sciatic nerve to my right leg. I had my third surgery in 2002, and my neurosurgeon was extra aggressive at trying to remove all the cells, but without the cost of paralyzing me. My recovery time was about 4 months and I now suffered from chronic pain due to Adhesive Arachnoiditis, caused from the surgeries. It has been 11 years since my last surgery, so I would consider it a success, even though I due suffer from chronic pain. Also, I have trained my muscles to release my bladder, since my nerves are paralyzed, and I no longer have to straight cath. My Urologist said he has never seen anyone do that before, but since it has been working for me, to continue doing it. I am aware that maybe one day, I may have to go back to straight cathing, if I can no longer have the same results with my muscles due to age or other factors. I'll cross that bridge when I come to it...


5)   Having gone through the experience, what do you think are the most critical questions for someone to ask their doctor about surgery and treatment of this kind of schwannoma?

As far as questions to ask your doctor about this type of surgery, I really don't know. He can tell you what could happen, but until the surgery is performed, no one knows what the outcome will be until you wake up. That is just the nature of this beast.


6)   Can you describe what the recovery process was like for you – if you feel you  have made a positive recovery…and what things you have done that have been of most help to you?

With each surgery, the recovery process was pretty much the same. Lots of rest in the beginning, and then slowly pushing yourself to do more every day.


7)   Are you able to do all or most the things you did before?

Yes, I am able to do everything I did before, but, sometimes with consequences. I realize that if I push myself too hard, my pain is going to flare up (sometimes severely), especially down my right leg. Also due to the paralysis in my bladder, if I do any type of jumping, especially on a trampoline, I know to wear some kind of protection. With all this said and everything I've been through, I'm just happy to be alive...     
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