Childhood Brain & Spinal Cord Tumors

A Guide for Families, Friends & Caregivers

Childhood Cancer Guides

Contents

Diagnosis

A journey of a thousand leagues begins with a single step.

— Lao-tzu

"We have the results of the scan back; I'm afraid it's bad news. Your child has a tumor." For every parent who has heard those words, it is a moment frozen in time. In one shattering instant, life forever changes. Families are forced into a strange new world that feels like an emotional roller coaster ride in the dark. Strong emotions will batter every member of the family. However, with time and the knowledge that many children survive childhood brain and spinal cord tumors, hope will grow.

Signs and symptoms

The brain and spinal cord make up the central nervous system (CNS). These organs coordinate all of the functions necessary for life, including breathing, regulating heart rate, thinking, and moving. Tumors of the brain and spinal cord begin with the transformation of a single cell. This renegade cell reproduces, creating more abnormal cells. Eventually, this collection of abnormal cells forms a tumor in the brain or spinal cord. The location of the tumor (also called a mass), its rate of growth, and the associated swelling determine the signs and symptoms that develop in a child. Chapter 3, Types of Tumors, provides in-depth descriptions of the various types of brain and spinal cord tumors.

Parents are usually the first to notice that something is wrong with their child. Occasionally, a pediatrician notices a problem during a well-baby visit, or the tumor is discovered by chance on a scan or other test. Unfortunately, some of the signs and symptoms of brain and spinal cord tumors mimic common childhood illnesses, which can make diagnosis difficult.

The following are some of the signs and symptoms that may indicate the presence of a childhood brain tumor:

• Headaches (often with early morning vomiting)

• Dizziness

• Seizures (convulsions)

• Staring spells

• Loss of peripheral vision

• Double vision

• Nystagmus (jiggling of an eyeball from side to side)

• Inability to look up

• One eye turns inward or outward

• Weakness in hands on one or both sides of the body

• Unsteady gait

• Change in speech

• Trouble swallowing

• Drowsiness

• Facial drooping or asymmetry

• Nausea relieved by vomiting

• Hormonal or growth problems

• Hearing loss

• Changes in appetite or thirst

• Behavior changes

• Change in school performance

The following are signs and symptoms that may indicate the presence of a spinal cord tumor in a child:

• Back or neck pain, which may awaken the child from sleep

• Scoliosis (curvature of the spine, resulting in leaning of shoulders to one side or a noticeable hump in the back)

• Torticollis (tilting of the head and upper spine to one side)

• Weakness or sensory changes in arms or legs

• Changes in bowel and bladder control

These symptoms can be present for a long or short period of time, depending on the location and growth rate of the tumor. A child with a brain or spinal cord tumor usually has more than one symptom.

Most parents react to their concerns by taking their child to a doctor. Sometimes the symptoms are attributed to a normal childhood illness, and parents bring their child in for more visits before a brain tumor is suspected. This is easier to understand when you consider that, in their entire careers, most pediatricians see only one or two children with brain or spinal cord tumors. Ultimately, the doctor orders a scan or refers the child to a specialist, such as a pediatric neurologist, for further tests (see Chapter 6, Coping with Procedures.)

Alannah was 4 years old when she was diagnosed with a brainstem glioblastoma. On December 23, my daughter's school called my wife to have her pick up Alannah because she had vomited, although she appeared fine afterward.

On Christmas Eve, Alannah woke up, and after playing for awhile, began complaining of a headache. We assumed she had picked up some sort of virus at her school. Later that day, we went to my parents' house for a traditional Christmas Eve gathering. Alannah began having trouble walking, and appeared to be looking at everything with her eyes shifted to the left. We laid her down in the guest room and a few minutes later, she threw up again. We still figured that we were dealing with a "bug," so we cleaned her up and went home. The following morning, she seemed fine except that her eyes were still fixed to the left. Later in the day, she started to have trouble with her balance and walking again. We took her to our local urgent care, still expecting to be told that she had a virus.

First, she was examined by a nurse practitioner. After checking Alannah's eyes, she quickly called in the doctor on duty. After a brief examination, he told me that he wanted to send her to the hospital by ambulance for a CT [computed tomography] scan. He said that while it might be a virus affecting her brain, he wanted the scan done to be sure that nothing else was wrong. Once we arrived at the hospital, several doctors examined Alannah. After waiting for about 2 hours, the CT scan was performed. Shortly thereafter, the physician called my wife and I out of the room and showed us the scan. She showed us what she called a "mass" on Alannah's brainstem, and told us that she would admit Alannah to the hospital and order an MRI. After another couple of hours in the ER we were transferred to a room on the fifth floor, in shock and disbelief, waiting for them to discover their mistake, and send us home.

The diagnosis of a brain or spinal cord tumor is sometimes not as quick as Alannah's:

The first signs were so subtle: the slight but constant inward turn of our son's left eye became apparent to us within the first few months of life. By the time he was 7 months old, we were concerned enough to bring it to our pediatrician's attention. He felt it was just normal uncoordinated eye movement. Every well-baby visit we brought the same problem to his attention. When our son was a year old, we were finally referred to an eye specialist — a pediatric ophthalmologist. Another year of visits began: the specialist insisted his eye was fine. We were just as certain the eye wasn't right, and we thought we could now see the eyeball jiggling. Then the ophthalmologist referred us to a pediatric orthoptist at a local Lions' Club clinic. She listened to our concerns, examined our son's eyes, and wrote a letter for our pediatrician verifying the abnormalities. Based on her report, our pediatrician agreed to schedule an MRI. Our son was 2 years and 2 months old by the time he was diagnosed with a moderately large optic glioma.

Where should your child receive treatment?

After tentatively diagnosing a brain or spinal cord tumor, most physicians refer the child to the closest major medical center with expertise in treating children with brain and spinal cord tumors. It is very important that your child is treated at a facility that has a full complement of specialists who are experienced in treating children with cancer, who know the latest treatment and research advances, and who work together as a team on behalf of your child. An effective treatment team will include pediatric oncologists, neurosurgeons, radiation oncologists, pathologists, pediatric nurse practitioners, child life specialists, rehabilitation specialists, education specialists, psychologists, and social workers. Institutions that specialize in treating children with brain and spinal cord tumors will provide state-of-the-art treatment, offering your child the best chance for cure.

Just after surgery, a number of doctors came by and introduced themselves as members of our team. At first, I was startled to learn that a radiation oncologist had been assigned to us, because radiation was not part of our son's current treatment plan. But then she explained that each member of the team would see us whenever we came to the children's hospital pediatric brain tumor clinic. I feel better knowing our child isn't an unknown quantity to these specialists, in case we ever do need them.

Physical responses

Many parents become physically ill in the weeks following diagnosis. This is not surprising, given that most parents stop eating or grab only fast food, have trouble sleeping, and are exposed to all sorts of illness while staying in the hospital. Every waking moment is filled with excruciating emotional stress, which makes the physical stress much more potent.

Our daughter had many strong seizures while in the hospital, and my stomach would churn. I'd have to leave the bedside when the nurse would come to help. I had almost uncontrollable diarrhea. Every new stressful event just dissolved my gut; I could feel it happening.

To try to prevent illness, it is helpful to try to eat nutritious meals, get a break from your child's bedside to take a walk outdoors, and find time to sleep. Care needs to be taken not to overuse drugs or alcohol in an attempt to control anxiety or cope with grief. Although physical illnesses usually end or improve after a period of adjustment, emotional stress often continues throughout treatment.

Emotional responses

The shock of diagnosis results in an overwhelming number of intense emotions. Cultural background, individual coping styles, basic temperament, and family dynamics all affect an individual's emotional response to stress. There are no set stages of response, and parents frequently find themselves vacillating from one emotional extreme to another. Many of these emotions reappear at different times during the child's treatment. All of the emotions described below are normal responses to a diagnosis of cancer in a child.

Children's and teens' emotional responses to diagnosis are discussed in Chapter 17, Communication and Behavior.

Confusion and numbness

In their anguish, most parents remember only bits and pieces of the doctor's early explanations of their child's disease. This dreamlike state is an almost universal response to shock. The brain provides protective layers of numbness and confusion to prevent emotional overload. This allows parents to examine information in smaller, less-threatening pieces. Pediatric neurosurgeons and oncologists understand this phenomenon and are usually quite willing to repeat information as often as necessary. Many centers have nurse practitioners, physician assistants, and nurses who translate medical information into understandable language and answer questions. Do not be embarrassed to say you do not understand or that you forgot something you were told. It is sometimes helpful to write down instructions and explanations, record them on a small tape recorder or smartphone, or ask a friend or family member to help keep track of all the new and complex information.

When I left the doctor's office, I was a mass of hysteria. I couldn't breathe and felt as if I was suffocating. Tears were flowing nonstop. I had lost total control of myself and had no idea of how to stop my world from turning upside down.

* * *

For a brief moment I stared at the doctor's face and felt totally confused by what he was explaining to me. In an instant that internal chaos was joined with a scream of terror that came from some place inside me that, up until that point, I never knew existed.

Denial

Denial is when parents cannot acknowledge what is happening to their child. Psychologically, they are unable to accept it. Parents simply cannot believe that their child has a life-threatening illness. Denial helps parents survive the first few days after diagnosis, but gradual acceptance must occur so the family can begin to make the necessary adjustments to treatment. Life has dramatically changed. When parents accept what has happened, understand their fears, and begin to hope, they will be better able to advocate for their child and their family.

I walked into the empty hospital playroom and saw my wife clutching Matthew's teddy bear. Her eyes were red and swollen from crying. I had no idea what had happened. A minute later the doctor came into the room with several residents (doctors who are receiving specialized training after they completed medical school). He told me that Matthew had a tumor and that he was very sick. I remember thinking that there had to have been a mistake. Maybe he was reading the wrong chart? My initial reaction was that it was physically impossible for one of my children to have a tumor. Tumors only grow in the elderly. Kids don't get tumors!

Guilt

Guilt is a common and normal reaction to a diagnosis of a brain or spinal cord tumor. Parents sometimes feel they have failed to protect their child, and they blame themselves. It is especially difficult because the cause of their child's tumor, in most instances, cannot be explained. There are questions: How could we have prevented this? What did we do wrong? How did we miss the signs? Why didn't we bring her to the doctor sooner? Why didn't we insist that the doctor do a scan? Did he inherit this from me? Why didn't we live in a safer place? Was it because of the fumes from painting the house? Why? Why? Why? Nancy Roach describes some of these feelings in her booklet The Last Day of April:

Almost as soon as Erin's illness was diagnosed, our self-recrimination began. What had we done to cause this illness? Was I careful enough during pregnancy? We knew radiation was a possible contributor; where had we taken Erin that she might have been exposed? I wondered about the toxic glue used in my advertising work or the silk screen ink used in my artwork. Bob questioned the fumes from some wood preservatives used in a project. We analyzed everything — food, fumes, and TV. Fortunately, most of the guilt feelings were relieved by knowledge and by meeting other parents whose children had been exposed to an entirely different environment.

It may be difficult to accept, but parents need to understand that they did nothing to cause their child's illness. Years of research have revealed little about what causes childhood brain and spinal cord tumors or what can be done to prevent them.

Fear and helplessness

Fear and helplessness are two faces of the same coin. Nearly everything about this new situation is unknown, and the only thing parents really do know — that their child has a life-threatening illness — is too terrifying to contemplate. Each new revelation about the situation raises new questions and fears: Can I really flush a catheter or administer all these drugs? What if I mess something up? Will my boss fire me if I miss too much work? Who will take care of my other children? How do I tell my child not to be afraid when he can see I am scared to death? How will we pay for this? The demands on parents' time, talents, energy, courage, and strength are daunting.

I stood at the elevator bank in the basement of Children's Hospital waiting for the elevator, saying to all those around me: "I can't even say those words out loud! Come on everybody say it with me: My daughter has a brain tumor! A brain tumor! A b-r-a-i-n t-u-m-o-r! Now that we know how to spell it, let's say it over and over ... braintumorbraintumorbraintumorbraintumorbraintumor!" Needless to say, I'm sure all the docs, nurses, and patients who were standing there with me just chalked it up to my temporary insanity, shock, denial, and complete flip-out that I was going through.

A child's diagnosis instantaneously strips parents of control over many aspects of their lives and can change their entire world view. All the predictable and comforting routines are gone, and the family is thrust into a new world that is populated by an ever-changing cast of characters (interns, residents, fellows, pediatric oncologists, IV teams, nurses, and social workers); a new language (medical terminology); and seemingly endless hospitalizations, procedures, and drugs. This transition is especially hard on parents who are used to a measure of power and authority in their home or workplace.

My husband had a difficult time after our son was diagnosed. We have a traditional marriage, and he was used to his role as provider and protector for the family. It was hard for him to deal with the fact that he couldn't fix everything.

Until adjustment begins, parents sometimes feel utterly helpless. Physicians they have never met are presenting treatment options for their child. Even if parents are comfortable in a hospital environment, feelings of helplessness may develop because there is simply not enough time in the day to care for a very sick child, deal with their own changing emotions, educate themselves about the disease, notify friends and family, make job decisions, and restructure the family schedule to deal with the crisis. The sense of helplessness often diminishes as parents gain a better understanding of the new environment and accept it as their new reality.

It's not a nice way to have to live. What's waiting around the next corner? That's a scary question. One of my biggest fears is the uncertainty of the future. All that we can do is the best we can and hope that it's enough.

* * *

Sometimes I would feel incredible waves of absolute terror wash over me. The kind of fear that causes your breathing to become difficult and your heart to beat faster. While I would be consciously aware of what was happening, there was nothing I could do to stop it. It's happening sometimes very late at night, when I'm lying in bed, staring off into the darkness. It's so intense that for a brief moment, I try to comfort myself by thinking that it can't be real, because it's just too horrible. During those moments, these thoughts only offer a second or two of comfort. Then I become aware of just how wide my eyes are opened in the darkness.