When our daughter Summer was 19 months old, we started to think she might need glasses. We noticed that she seemed to be grabbing for things incorrectly - almost as if she wasn't really paying attention when trying to grab an object. We made an appointment with the pediatrician later in the week, but the evening before her scheduled appointment she seemed to have suddenly lost all sight completely. We immediately took her to Children's Hospital in Seattle.
After a night and day's worth of consultations and testing with various doctors, Summer was put under anesthesia for an MRI. The doctors came back out almost immediately to tell us Summer has a brain tumor. Based on its placement (off of her pituitary gland), and the fact that it had both a solid and cystic component, the neurosurgeon told us it was probably a Craniopharyngioma.
The diagnosis would be confirmed days later, but an hour and a half after that initial scan on April 29th, 2010, Summer went into emergency surgery to decompress the cyst and remove some of the pressure that had been building up inside her brain, pushing on the optic nerves and causing the vision problems. They acted quickly in the hopes that some of her vision could be restored, and that punching a bunch of holes in the cyst wall would keep it from re-filling.
We were lucky and could tell almost immediately that Summer had regained most of her vision. Later tests would show she suffers peripheral vision loss and might have some trouble seeing contrast, but that her visual acuity (how clearly she can see - i.e. 20/20 or 20/40 in her case) is within the normal range for her age.
Unfortunately, the cyst was not so easily controlled. Three months later, Summer's follow-up MRI showed significant growth in the cyst. At that point, it was significantly larger than it had been at her diagnosis in April. We couldn't believe the images, especially since she hadn't shown any symptoms. Or if she did, we didn't recognize them for what they were.
During a second surgery to decompress the cyst yet again, they placed a catheter into the cyst with a port that would allow them to draw fluid using a needle, rather than having to have additional surgeries to remove fluid. At that point we began to talk about other options since this cyst was proving to be a difficult one.
All along, our plan was to buy time. Most kids diagnosed with this tumor are older and go straight to radiation following surgery, which is a very effective course of action (80-95% success in controlling the tumor, depending on who you talk to). Summer's young age, however, made radiation an undesirable follow-up because of the damage it can cause on a brain that is still going through crucial developmental stages.
We tried a treatment called Intracystic Interferon treatment, a sort of chemical therapy where they inject a drug (using the port implanted during her second surgery) directly into the cyst to try to kill those cancerous cells. It's very rarely been used because of the success with the standard surgery/radiation combination (and there is rarely a need to wait for radiation since most kids are older at diagnosis), but had fairly good success with few side effects. After 12 treatments, Summer is now one of a handful of people to be treated with Interferon in this way in the US. Unfortunately, it didn't work for her and the cyst continued to fill with fluid.
Between the two surgeries and Interferon treatment, we managed to buy about six months before it became evident that we needed to start planning for radiation. Summer had two additional surgeries in the months heading up to radiation treatment - one to convert her port to a programmable shunt that drains the fluid from the cyst into her abdominal cavity, and a second to install a port-a-cath that would provide direct access to her venous system for the anesthesia she would receive daily during radiation.
In January 2011, Summer and I (Jessica) moved temporarily to Boston for proton radiation, a form of radiation that is considered to have reduced side effects but is only available in 6 or 7 places around the country. Summer underwent 28 treatments over the course of six weeks, and we returned home on March 1st.
She tolerated the treatments well, and experienced delayed fatigue and hair loss after we returned home, but seemed otherwise unaffected. We actually had a great time in Boston since Summer was feeling well, and we made some friends for life with families who were going through the same things we were.
After returning from Boston, Summer had her fifth surgery to remove the port-a-cath and we transitioned into monitoring mode. So far, things have been going well. After finishing radiation, we saw some initial shrinkage in the solid portion of the tumor, and its size has now remained stable for two years. The cyst also remains stable. Summer is on thyroid medication and receives daily injections of growth hormone.
Frequent trips to Children's Hospital had become our new normal. Now her doctors appointments are becoming less frequent - we are on a six-month followup schedule with most of her doctors. But we still worry about this thing on a daily basis, and are constantly watching her for signs of tumor growth or shunt failure.
Summer is definitely a spirited and spunky child, but we're grateful for it. I have no doubt that her spunkiness has helped her make it through five surgeries, chemo, radiation and countless tests and pokes with flying colors. I can't wait to see how she thrives now that this treatment period is (hopefully) over for good!