30 DAYS/30 STORIES® 2021
How in the world did we get here? Writing a story about how we came to be members of PCFLV still seems like some other reality. Our sweet daughter, Isabelle, has a bright disposition, incredible laugh, beautiful smile, and charming personality. At 4 years old she was, seemingly, healthy and her future was bright. On December 2, 2020 ,our life would change.
Throughout the summer and fall of 2020, at the height of the COVID-19 pandemic, Bellsie (our nickname for Isabelle) was having a few subtle behavioral issues that, at first, we were attributing to the fact that she was stuck at home with mom and dad around the clock. She was having tantrums, night terrors, severe separation anxiety as well as some mild gross motor regression. After the summer months came to a close, we noticed more things that Bellsie would not do, like run and jump, that other kids her age seemed to do with ease. As the fall progressed, her tantrums and night terrors had gotten worse. We still attributed these symptoms to the fact that she was stuck home all the time because of the pandemic and was simply acting out. The symptom that really began to be noticeable was her altered manner of walking and unusual gait. She was very stiff legged and unsteady on her feet. She also began to crawl up stairs and cry out of frustration. Just a few months prior she could hike over rocks with ease and now she could not go up 2 steps. As mom and dad, we were concerned and determined that we needed to make an appointment with our pediatrician to get a referral to see a pediatric orthopedic specialist. The final observation made was when mom watched Bellsie walk into a wall, as if she couldn’t even see it.
We made an appointment to see her pediatrician the next day, which was November 30th. As we described the walking issues, the behavioral issues, the night terrors and the separation anxiety, our doctor took many notes and began to appear very concerned. She performed a full assessment and immediately went to the other room to consult a neurologist friend at LVHN. We were directed to go to the emergency room as our pediatrician requested that a brain MRI be done immediately. This was very concerning to us as parents, because we had previously believed that she was experiencing some kind of musculoskeletal issue.
Due to the pandemic, only one parent was allowed to be with Bellsie in the room at the ER. Mom stayed with her while dad sat down in the lobby until they could get her into the MRI, which happened 2 days after we were admitted. The longer we waited, the more worried we became. December 2nd, Bellsie had her brain and spine MRI. Shortly after the doctors viewed the images, we were both called into the room to meet with their team. As we sat and the doctors spoke, they showed us an image, that will be forever imprinted in our heads, of an enormous fluid filled cyst that took up approximately 1/5 of our little girl's skull cavity. The doctors at LVHN explained that they would arrange for transport for Isabelle to be taken to Children’s Hospital of Philadelphia within the hour for their team of pediatric neurosurgeons to determine what it is and what needed to be done. Mom joined Bellsie in the ambulance ride while dad packed a bag.
When we arrived at CHOP, there were already teams of doctors and nurses in our room in the PICU. The team of neurosurgeons explained to us that Isabelle has a brain tumor called a craniopharyngioma that has a large cystic component, which is what we are seeing on the scan. They explained that this type of tumor sits right on top of the pituitary gland and between her optic nerves, making it inoperable. The symptoms that Bellsie was experiencing were caused by the massive cyst that was pushing on her brain and actually causing her cerebellum to be pushed down her spinal column. Spinal cysts were also found on the scan. The plan was to first address the brain cyst and, at some point, discuss how to address the tumor. Historically, craniopharyngiomas were immediately resected, leaving the patients with full pituitary failure as well as common issues such as diabetes insipidus and hypothalamic obesity, all of which we were told would be likely outcomes for our daughter.
After a CT scan, the team of CHOP neurosurgeons and ENTs from The University of Pennsylvania determined that an endonasal approach was the best treatment option and scheduled her first brain surgery for Friday, December 4. The surgery was performed by Chief of Neurosurgery, Dr. Storm as well as attending physician, Dr. Chen who became her primary surgeon. Doctors Storm and Chen, with the help of their team and the ENTs from Penn, were able to drain most of the cyst through Isabelle’s nose using a stent. The plan was to keep the stent in for the cyst to constantly and naturally be able to drain into her digestive system. Following surgery, Bellsie had a slow, steady drip of clear fluid coming from her nose. Dad was up all night wiping her nose every minute or two, not knowing that this was a cerebral spinal fluid (CSF) leak from the stent placement. The next day, Saturday, December 5th, Isabelle went in for her second brain surgery to repair the leak. In order to do this, the doctors had to install an EVD tube out the top of her skull to divert the CSF and alleviate pressure from the patch near her stent for the next 3 weeks. She had another surgery on December 11, to repair the patch that was still leaking. We remained in the PICU while her EVD tube drained CSF and were finally discharged from the hospital on Christmas Eve.
Upon returning home, Isabelle had slowly started to recover from her stay at the hospital. She began to get an appetite back and her incision was healing well. Her walking began to improve, and she was no longer having tantrums or night terrors. Her nose continued to slowly run, which we were told would happen as the cyst continued to drain. Over the next several months, Bellsie would go through several more brain surgeries due to stent migration and stent blockages, which eventually would total 9 surgeries in 6 months to try to drain the cyst. In March, after noticing a heavier than normal flow of fluid out of her nose we spent a week at CHOP and they determined a CSF leak needed to be repaired and they, ultimately, abandoned the stent as a treatment option. Using a fascia tissue patch from her leg, they sealed up the stent hole permanently. As they let that heal, the doctors let some time pass to see how the cyst would grow without draining. Bellsie was starting to develop headaches and we had to determine what was next. Our neurosurgeon, Dr. Chen, explained that the next approach would be to install an Ommaya reservoir as a way to drain the cyst without invasive surgery each time. The reservoir would simply be implanted in her skull with a catheter going into the cyst that they could draw fluid from.
The Ommaya placement surgery was successfully performed on May 3. Shortly after, Isabelle was reporting headaches which began to increase in frequency. An MRI was done in early June to determine if her headaches were due to cyst growth. Following the MRI, the neurosurgeons said that the cyst growth is now normal, and we would be referred to a neurologist to address her increasing frequency of headaches. The neurology team believes that after 9 brain surgeries, Bellsie is now prone to migraines. We have been trying different medications to treat the headaches to bring our little girl some relief.
The road ahead is uncertain. We are now in a maintenance phase of quarterly MRIs and cyst draining through the Ommaya, when necessary. The tumor is still in there and it cannot be surgically removed. Our endocrinologist told us that the risk of pituitary damage is greater during surgical interventions. For now, it is unlikely that we will experience any of the common irreversible pituitary issues that some people with this tumor have to live with. The only current treatment for the tumor itself is through radiation treatment. We are told this would most certainly have great impact on Isabelle’s hormone function as well as intelligence and personality. Nobody on the neuro-oncology team want to go this route unless absolutely necessary. For now, we are buying time managing cyst growth as medical science continues to work on a new solution for her treatment. There is a possibility of a clinical trial coming to CHOP that would shrink the tumor through medication and not through radiation or surgery; that would be the miracle we have been praying for.
Fortunately, you would not know by looking at her or interacting with her that she had been through all of this. The ophthalmology exam revealed that Isabelle did not sustain any optic nerve damage and her vision was, in fact, better than most kids her age. Good Shepherd OT and PT did great work with her over the span of 4 months to get her movement back on track. She has regained the ability to run and jump and her focus and dexterity are exceptional. The Ommaya reservoir does not seem to bother her at all. We have received tremendous support from family, friends and through our new friends at PCFLV. This foundation can really be leaned on and makes us feel we are not alone. The team of doctors at CHOP literally saved our little girl’s life and have given great care with managing her quality of life after diagnosis. Amidst all of this chaos this past year, we remain grateful that our little girl is mostly happy and acting like a normal 5-year-old. We continue to pray that she can experience all that life has to offer. This past year taught us to not take a minute with her for granted and just take each phase of her treatment as it comes.
Written by Bellsie’s parents, Rob and Lynn
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