Blood tests are done at every chemo appointment to monitor a patient’s response to treatment. White blood cells fight infection and can be compromised when a patient is receiving chemo. The most important infection-fighting white blood cell is the neutrophil, so doctors look at the absolute neutrophil count (ANC). The ANC is found by multiplying the white blood cell count by the percent of neutrophils in the blood. For instance, if the WBC count is 8,000 and 50% of the WBCs are neutrophils, the ANC is 4,000 (8,000 × 0.50 = 4,000). A healthy person has an ANC between 2,500 and 6,000. When the ANC drops below 1,000 it is called neutropenia. The oncologist will watch the ANC closely because the risk of infection is much higher when the ANC is below 500. Monitoring blood counts allows the medical team to make changes before problems get serious. Keeping track of results lets the oncologist take action early to help prevent many cancer-related problems and cancer treatment side effects. I also keep a log in Kiara’s binder.
The world of pediatric neuro-oncology is very small and most doctors know each other from conferences. Do not be afraid to ask for a second opinion — you’re not betraying anyone and they appreciate the professional collaboration. There is also close work between neurosurgeons and oncologists, so it’s ok to loop in other doctors from other hospitals. Find your oncology nurse navigator. This is a nurse in your clinic with oncology-specific clinical knowledge that can offer individualized assistance to patients, families, and caregivers to help overcome healthcare system barriers. He or she will gather your child’s records and scans, send them to the other doctor/hospital for you.
Some hospitals submit tumor samples and thereby participate in clinical research; however, some hospitals have sophisticated labs and research grants to conduct research studies on site. Most doctors prefer to go with standard peer-reviewed, traditional treatments. The Dana–Farber Cancer Institute at Boston Children’s has research studies for ground-breaking discoveries. St Jude’s Children’s Research Hospital also offers clinical trials, if your child qualifies for the study.
Intracranial pressure (ICP) is the pressure inside the skull and in the brain tissue and cerebrospinal fluid (CSF). If there is a tumor, head injury, brain bleeding, or swelling, the brain can become pressurized like a water balloon, and CSF has nowhere to go. The pressure can crush brain tissue, shift brain structures, contribute to hydrocephalus, cause brain herniation, and restrict blood supply to the brain. In Kiara’s case, elevated ICP caused optic atrophy (blindness due to damage to the optic nerves). ICP is measured in millimeters of mercury (mmHg).
An EVD is a catheter that is inserted by a neurosurgeon into the brain to release cerebrospinal spinal fluid (CSF) and lower intracranial pressure (ICP). It’s basically a tube that drains fluid from inside the brain into a bag next to the bed that can be measured. Drainage can be continuous at a set level, fixed volume per desired time (i.e., every hour), or as needed according to ICP elevations. At the prescribed height, CSF (and/or blood) will drain whenever the interventricular pressure exceeds that set by the height of the collection system. Flow ceases once the pressure equalizes between the CSF compartments in the brain and collection system.
The EVD must be positioned so that that the pressure transducer is in line with the patient’s ear. (Technically, it must point to the Foramen of Monro, which falls at the level of the external auditory meatus of the ear in the supine position and at the mid sagittal line (between the eyebrows) in the lateral position.) Most nurses “eyeball” it, but some will get a carpenter’s level or laser level to ensure that it’s place accurately. Whenever the patient sits up or lays down, the EVD must be adjusted also, which is a slight — but necessary — hassle.
Hydrocephalus is an abnormal accumulation of cerebrospinal fluid (CSF) within cavities in the brain called ventricles. It typically causes increased pressure inside the skull. Some people may have headaches, double vision, poor balance, urinary incontinence, personality changes, or mental impairment. Other symptoms may include vomiting, sleepiness, seizures, and downward pointing of the eyes.
Hydrocephalus is typically treated by the surgical placement of a shunt system. Before getting a v/p shunt, Kiara had an alternate procedure called a Endoscopic Third Ventriculostomy (ETV) to try to combat the hydrocephalus. It worked well and doctors were fairly confident that there was no increased ICP in her brain, but because of increased vision loss, we opted for a v/p shunt to make sure.
Here is a great video that explains how it works:
Torticollis, also known as a crick in the neck, wry neck, or loxia is a dystonic condition defined by an abnormal, asymmetrical head or neck position. The child has a limited range of motion in the head and neck. The head tilts to one side while the chin tilts to the other. Usually there is a tightening of muscles and the patient is unable to have full range of motion.
When she was 7 months old, Kiara gave us our first scare. She developed a fever and was unable to lift her head off her shoulder. We took her to the pediatrician, who rushed us to the ER fearing it was meningitis. Fortunately, tests results came back negative and a CT-scan showed no blockage in her neck. With pain meds, she regain full range of motion. Doctors reassured us that “tumors don’t go away with Advil.” After two days in the hospital, we were discharged with a diagnosis of torticollis. Looking back I believe her brain tumor was there in its early stages, and this was our first missed sign.
When Kiara was diagnosed, other cancer moms told me to start a binder for her documents. It was about a year later when she began chemo treatments and she was a new school with a number of specialists that I finally put a binder together.
In Kiara’s binder I have sections for:
- General Medical. This is where I put info from her pediatrician and dentist, as well as a medical history, immunizations, and labs/scans log.
- Neurosurgery. This section has the contact info of her neurosurgeons, information about her v/p shunt, and other handouts.
- Oncology. This is where I keep the contact info of her oncologist, her chemo roadmap, a log of her blood counts (ANCs) at each chemo treatment, and other notes about how her chemo treatments are going.
- Vision. This section contains contact info for her ophthalmologist and optometrist, and handouts/reports from them.
- Therapy. This section includes contact info and handouts for PT and OT specialists.
- Education. This section has contact info for her principal, assistant principal, SpEd teacher, O&M, TVI, etc. It contains ARD and IEP info and other school-related documents.
- Disability. This section has the contact info for her state HHS case worker and any other government-related disability documents.
- Other. This is a catch-all section that has documents, such as FMLA papers, donations and gifts, etc.
I wanted her binder to be bright and colorful, and I found this great Medical Printables set on Etsy that makes it adorable. Fortunately the seller also gave me editable files so I was able to make some additional forms that I needed, such as Chemo Counts and School Conversations.
When I first met Kiara’s oncologist, I was panicked and confused. Kiara was being rushed into emergency surgery to place an EVD to keep her brain from further herniating. We had just learned there was a tumor, but nurses, assistants, and practitioners didn’t want us to be “alarmed” or “worried” about the tumor — wait to worry, they said, until after the resection when the pathology report comes back. Yeah, right. Fortunately those days seem to race and crawl all at the same time, and Kiara’s oncologist was patient, reassuring, and informative.
An oncologist is a doctor who treats cancer. Usually, an oncologist manages a person’s care and treatment once he or she is diagnosed with cancer. The field of oncology has three major areas: medical (chemo), surgical, and radiation. Pediatric neuro-oncology is a very small and specialized field, so most of these oncologists know each other and are happy to collaborate for the best treatment possible.
If you start seeing an oncologist, you’re probably at the point where you’ll be going to a cancer center. With the oncologist comes a Nurse Practitioner, Nurses, Social Worker, Child Life Specialists, Nurse Navigator, and others that you’ll work with going forward.
A neurosurgeon is a doctor who specializes in the diagnosis and surgical treatment of disorders of the central and peripheral nervous system including congenital anomalies, trauma, tumors, vascular disorders, infections of the brain or spine, stroke, or degenerative diseases of the spine. Less technically speaking, they’re the ones that “go inside” and scoop out what’s not supposed to be there.
They also have nurse practitioners that do many tasks pre-op and post-op. In Kiara’s case, all of the nurse practitioners are brilliant and beautiful women (seriously, could they be more perfect?). Kiara’s neurosurgeons themselves are unbelievably smart and capable.
Neurosurgeon vs. Neurologist
When a patient has neurological symptoms, often their initial evaluation is with a neurologist, who might obtain imaging to get to the source of the problem. If they discover a structural problem, such as a tumor, they’ll refer the patient to a neurosurgeon. Both doctors treat the same organ, but neurosurgeons operate and neurologists don’t.
Neurologists treat disorders, such as stroke, Alzheimer’s disease, headache, epilepsy, Parkinson’s disease, sleep disorders, multiple sclerosis, pain, tremors, brain, and spinal cord injuries, brain tumors, peripheral nervous disorders and amyotrophic lateral sclerosis. Neurosurgeons treat head and spine trauma and injuries, brain or neck aneurysms or clogged arteries, brain and spinal tumors, and anatomical and structural defects.
Pro Tip: Try to see a neurosurgeon. If you have a clean MRI, then they will refer you to a neurologist or other specialist that can manage your treatment. If their first available appointment isn’t for months, go to the ER and see the neurosurgery nurse practitioner on call. It’s worth it.
Many of the other parents of cancer kids that I’ve met described one of the primary symptoms of their brain tumor as visual impairment. Kiara often said that she saw “flashing” or was “seeing stars.” We thought it was just from standing up too quickly, but it was actually her optic nerves dying.
If your young child says, “I can’t see,” and you’ve already seen an ophthalmologist to know his or her eyes are healthy, make an appointment with a neurosurgeon and get an MRI.