Adult Ependymoma: A Patient's Story

November 14, 2014 Update

This is Carol's sister Margaret, posting for Super Sister Carol. (I apologize about the formatting errors in this posting!!)

Carol was scheduled to be transferred to Woodbine Rehab on November 11, but there were a few complications, so she stayed two more days in UVa's ICU.  On November 13, she traveled by ambulance transport to Woodbine, and was greeted by sister Elizabeth and friend Senna and Senna’s son Nate.  Senna had an amazing "Onward" bag filled with items to help make Carol's stay a bit more comfortable.

Even though Woodbine expected Carol on the 11th, and UVa had faxed her medicine list to them, Woodbine hadn't filled the prescriptions.  As a result, that evening, Carol was in a lot of pain, and her medicine still hadn't been delivered from the pharmacy in Richmond (which is about two hours from Woodbine. (It is so hard to be nice and diplomatic when you just want to scream at someone who has gone home for the day without doing their job...)

Thankfully, yesterday was a new day.  Carol had assessments from speech/swallow therapy, physical therapy, occupational therapy, and nutrition.  All of the therapists agreed that Carol is super motivated.  The speech therapist gave Carol exercises to do "eeeeeeee/aiiiiiiiii/eeeeee" and is allowing ice chips (but not too many at a time.)  PT started today, and will happen six times a week.  In addition, a lot of the respiratory therapists from 2009 are still at Woodbine, including Reuben who we remember as just a very nice and kind therapist.  Carol lost some weight the last four weeks, so she will be getting 2,000 calories a day in liquid nutrition.  And most wonderfully, Eva, the nurse manager for the respiratory unit, arranged for Carol to move into a room with a bed by the window and for now, no roommate.  As you can imagine, this makes everything so much nicer.

Carol asked me to mention that she really feels demoralized by her latest surgeries and necessary rehab.  The incisions are healing nicely; but one is about 4 inches and the other is at least six inches and she is still in pain.  In addition, she had the bad news that the tumors' pathology came back as Grade III ependymomas. Tube feeding is not all that pleasant, and she is often hungry.  Having her trach revised and having to go on the ventilator at night is difficult for so many reasons. There is also the uncertainty about what the follow up spinal and brain MRI on December 22 will show and the whole unknown of what the future holds.

In the meantime though, Carol (and her mother and sisters!) are taking it a day at a time, and even an hour at a time when necessary. Thanks to all of you who would like to visit or send cards; our sister Lyn is scheduling visitors; please e-mail any of us (including Carol) if you need Lyn's e-mail.  Thank you for keeping Carol in your thoughts and prayers while she goes through this big transition; we want her to get better so she can go home soon! Our sister is UNBELIEVABLY amazing; we love her very much, and appreciate everyone's support.  Thanks to all of you for following Carol's journey.

Onward to great progress with swallow therapy and physical therapy!

Welcome to my website!  My name is Carol Welsh and I'm a fourteen year+ survivor of adult ependymoma, a rare brain tumor, which just this year spread to my spine.  On October 17, I was operated on for a tumor on my L1 and then on October 28, a tumor on my T6-9. The pathology on both has come back as Grade III Ependymomas. 

In addition, I've had three brain surgeries, one gamma knife radiosurgery, a placement of a shunt, a course of radiation and oral chemotherapy called Temodar which did not stop the growth of my tumor.  On March 22, 2005 I started a different "heavy duty" chemotherapy regimen, a combination of IV carboplatin and IV etoposide (VP-16).  I completed three more rounds of the chemo (April 12-14, May 7-9 and May 28-30) to buy some time while I investigated the possibility of a third surgery, which I eventually had on December 13, 2005.  With the recurrent tumor I had headaches, balance problems and severe double vision.  In fact, I had so many physical complaints that I was overwhelmed.  For over eleven months I took a daily dose of Decadron which has its pros and cons  - it is a vital steroid to control edema (swelling) around a brain tumor but it has horrible side effects.  Since my third surgery I have struggled to both accept and overcome my deficits. 

On December 19, 2008, I had sudden respiratory arrest.  Fortunately, I was in the ER already, because I had gained 10 pounds in about a month and had grown increasingly confused, exhausted and was hallucinating at night.  The CO2 level in my arterial blood was 75, climbed as high as 124 and it's a miracle that I wasn't in a coma.  I was intubated, ventilated, and ultimately ended up with a tracheostomy.  It appears that damage to my respiratory center in my brain stem from the 3rd surgery and/or the radiation caused complex sleep apnea and chronic hypoventilation, which is shallow breathing at night.  My right heart failed because of pulmonary hyptertension caused by repeatedly low oxygen levels at night.  I was, and still am, hypercapneic which means I have high levels of CO2 in my blood.  Here's a good article which explains both obstructive and central sleep apnea:  My throat is so collapsed during sleep (and during the day) that I can't blast through the tissue with a CPAP machine.  With the trach, you bypass this nerve-damaged tissue - but at a price (having a hole in your neck). My breathing crisis is a prime example of the sinister nature of brain tumors - and the key factor of tumor location and resulting damage to the most sensitive area of the brain, the brain stem. 

These years I can barely withstand all the physical problems I face every minute:  from double vision, headaches, balance trouble, legs that feel and respond like cooked pasta, trach pain, abdominal pains, swallowing difficulties to sheer exhaustion and most troubling, a sense that I am removed from the world around me and overwhelmed. 

I've created this site to help other patients and caregivers learn about adult ependymoma and to give my story as one example.  Besides the details about an ependymoma itself, I hope my website describes "the complete change in my lifestyle" -- a sentiment first articulated to me by a fellow ependymoma survivor, Kathy (Claremore, OK).

According to the American Brain Tumor Association and the National Brain Tumor Society, ependymomas are glial tumors which arise from ependymal cells which line the ventricles (spinal fluid spaces) of the brain and the center of the spinal cord. Ependymomas make up 3 - 6% of the estimated 52,236 primary brain tumors diagnosed in the United States in 2008.  Ependymomas occur at the peak ages of 5 and again at 34.  While they are rare in adults, ependymomas are the third most common brain tumor in children. Most occur in the posterior fossa (the lower back portion of the brain) and of these, nearly all occur in the fourth ventricle.  As primary tumors, they originate in the brain.  A 2002 eMedicine report states 5-year survival rates of 76% for adults and a dismal 14% for children.  According to the Chicago Institute of Neurosurgery, about 10% of brain ependymomas will spread to the spinal cord through the cerebro-spinal fluid. 

Ependymomas are classified in four divisions:
     1.  ependymoma (the general term for the tumor)
     2.  anaplastic ependymoma (more aggressively growing cells)
     3.  myxopapillary ependymoma (occurs more often in the spinal cord)
     4.  subependymoma (grows slower than a typical ependymoma)

Ependymomas are graded using the World Health Organization (WHO) standard - grades I and II are considered benign and grades III and IV are considered malignant or "anaplastic."  However, benign ependymomas can be anything but benign.  "Low grade" is a more descriptive term than "benign."  As space-occupying lesions in an extremely limited space, often they are malignant by location, and sometimes they can recur, perhaps not as fast as might be the case with anaplastic ependymoma, but they can recur nonetheless.  Mine recurred the first time after three years.  The location of a brain ependymoma can be devastating.  Think real estate as in, "location, location, location."  Where the tumor is and the skill of the neurosurgeon in attempting to remove it are most important.  Some people are wrecked from the surgery to try to remove an ependymoma that might be attached to one or more cranial nerves on the brainstem.  The cranial nerves are twelve pairs of nerves that are the critical sources of a person's ability to breathe, smell, see, chew, taste, move and hear.  My surgeries resulted in several deficits because of the "insult" to some of these nerves.  Fortunately, I have regained these abilities at least partially.  Some patients, though, never regain some vital functions, such as their swallowing, breathing, walking or speaking ability. 

Is a Grade I or II ependymoma the same as brain cancer?  It's not a simple answer.  Ependymomas are tumors and they can recur either locally in the brain or into the spinal cord, so in that sense they are cancerous.  However, sometimes they are slow-growing and do not spread to other parts of the body and in that sense they do not behave like cancer.  Most importantly, and frighteningly, ependymomas, even if benign on the WHO scale, can be deadly simply by their location, either if they grow and cause death or if they are removed and cause death from the surgery.  Ependymomas are treated like many cancers with surgery, radiation, and/or chemotherapy.  New growth of a slow-growing tumor might not show for years.  The scary truth is that any form of ependymoma that is either inoperable surgically or unresponsive to radiation and/or drug therapies eventually will kill the patient, on a timeline that is specific to the individual case. 

Like many patients, I've always wondered how long my ependymoma was in my body before it caused any problems.  I asked Dr. Fine this and he wrote, "It's impossible to say how long you had the tumor.  The fact of the matter is that this is a very slow growing tumor so it's conceivable that you had the tumor for years or even decades.  Just impossible to know."

[In July 2013 a biopsy of a lump I found in my right breast revealed DCIS, known as "Stage Zero Breast Cancer."  I had it removed over two operations, including plastic surgery on my left breast, and thankfully there was no sign of invasive cancer.  Happily, radiation was not indicated. I decided against taking an "insurance" regimen of Tamoxifen.  I highly recommend my network of breast cancer specialists, including genetics counselors, in the DC/VA area if anyone ever would like my contact list!]

Everything I have included on this site is based on my personal experience.  

I'm not a physician - Check with your doctors on everything!


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