Charlotte Jennie (CJ) had been complaining of headaches for about 2 weeks and vomiting inconsistently. We decided to take her to the pediatrician and they immediately sent us to St. Mary’s hospital for a CT Scan. The scan revealed a large (orange-to-grapefruit-sized) mass and Charlotte was admitted on 1/20/09 to MCV hospital in Richmond. She was diagnosed with a PNET tumor (Primitive Neuroectodermal Tumor) with its origin in the thalamus. PNETs are similar in structure (on the cellular level) to medullablastoma. Medullablastoma is the most common malignant brain tumor in children. On the other hand, PNETs are more rare and can sometimes behave differently. From what we understand from her doctors, her pathology revealed a very aggressive tumor that tends to regrow quickly without treatment.
She had 2 craniotomies in 2 weeks. Surgery was able to remove about 90% of the tumor and after an additional 3 week hospital stay, she came home for outpatient therapy. She emerged from the surgeries with some left side weakness but quickly regained the ability to walk and use her left hand with support.
Charlotte finished her first round of inpatient chemotherapy on March 13th and finished her second round on April 10th. We started a third round of induction chemotherapy on April 28th; however, a new MRI revealed that her tumor was growing in spite of the chemo.
At this point, we scrapped her current protocol and had a consultation with the clinic at MD Anderson in Houston, TX to get guidance on future treatment of her tumor.
Her third surgery occurred on May 27th and was very successful. Dr. Tye removed nearly all of the remaining tumor with just a few very small bits left over in places he wasn’t comfortable going, including some on her brainstem.
Her new chemo protocol developed by the medical team at MD Anderson began soon after her 3rd surgery. On July 11, 2009, two days after her fourth birthday, we packed up and headed back to Houston, TX for 30 days (7 weeks) of proton radiation therapy. She also received some chemotherapy during this time. Charlotte tolerated the radiation/chemo combo well and we returned from Texas on September 1, 2009.
Charlotte continued on a revised chemo protocol that included accutane, valproic acid, topotecan (all oral) and velcade (IV 2x/week every 2 weeks).
Charlotte’s last MRI (November 6, 2009) revealed that the tumor continued to grow despite radiation, chemo, and all other treatments. The tumor growth was once again placing pressure on the spinal fluid in her ventricles, causing enlargement of her brain cavity.
On November 8, 2009, Dr. Tye placed a shunt in her head. The shunt drained to her stomach and allowed pressure to be relieved.
Having exhausted all treatment options available to us, our time left with Charlotte was limited. We spent November and December traveling and experiencing life with Charlotte including a trip to Disney World sponsored by the Make-A-Wish Foundation.
CJ passed away peacefully at her home on January 7, 2010. She was almost 4 1/2 years old.