Our daughter, Paisley Willow, was diagnosed with Chiari Malformation at the age of 10 months after an accidently fall where she suffered a skull fracture. For the next 8 months, we felt it was simply a diagnoses and hoped she would be one of the lucky ones that wouldn’t be impacted by it. Within months, symptoms began presenting themselves but we remained in denial. We blamed it on her chronic ear infections, teething or just becoming a picky eater. At 22 months, Paisley had a swallow study done which showed her 12th cranial nerve was being restricted. She was diagnosed with Hypoglossal Nerve Disorder. Within 2 weeks, Paisley had her 1st Brain Decompression surgery on June 13th, 2017. She spent 6 days in the hospital only to be readmitted on the 22nd with Chemical(Aseptic) Meningitis. Recovery was harder than we had ever imagined.
Paisley began feeding therapy shortly after her 1st surgery to regain skills and to work on many fears she had developed with feeding. Something still didn’t seem right even though her 3 month post op MRI looked good. We became concerned with her breathing therefore we had a sleep study done. Paisley was then diagnosed with Obstructive Sleep Apnea in January 2018. Over the next few months, she began getting worse again. Her behaviors before her 1st surgery were all coming back. She was holding her head, her neck, very irritable, withdrawing and starting to gag on her food. On April 16th she had a CTL Spine MRI which gave us the worst news… Paisley had developed 3 syrinxes in her spine. She now has Syringomyelia. On April 26th, 2018, Paisley had her 2nd Brain Decompression Surgery. It was discovered that scar tissue had formed a blockage. This recovery was even harder than the first. She developed Chemical Meningitis again and this time it took 3 months to fight that beast. Meanwhile, we were so worried about what the Meningitis was causing to her healing process.
Again, Paisley never seemed to get much relief. Her post op MRI in August showed the syrinxes were still present. We were becoming concerned with her left leg. It appeared to be weak when she would bear weight on it. Her right eye had developed a “wink” or squint before the 2nd surgery and it had only gotten worse. Paisley had another MRI in October but this time a CTL Spine. This showed, the syrinxes appeared to have slightly increased. There were many different discussions and approaches we attempted (We discussed a low level of hydrocephalus occurring and she began a medication to decrease her CSF production). We discussed a VP (Brain) Shunt or a Syrinx Shunt. We have taken the time to do as much research as we can. A major problem with Chiari is the lack of research available. The lack of funding towards Chiari limits the amount of research. We felt at a loss as parents and only wanted to help our daughter. We reached out to a Chiari specialist who consulted with Paisley’s Neurosurgeon and it was decided that Paisley needs her 3rd Brain Decompression surgery. We are heart broken but know Paisley needs some much needed relief so she can live life like a typical 3 year old. Her 3rd surgery will be on February 5th, 2019.
Paisley had her 3rd surgery on February 5th, 2019. The recovery began well but soon after we were worried as she just didn’t seem to be doing well. Her eye began acting up worse than ever. She told us that she couldn’t see. She then began telling us she was seeing 2 of things. We were told she was experiencing temporary blindness and diplopia. The Neurosurgery team felt at first it could be due to a medication so we switched medications. Then they felt it could be due to the steriod she was taking. She was also complaining about her right arm hurting and would not use it.
Her eye continued to worry us and they felt it could be due to swelling and should subside as she recovered. Once she weaned from the steriod, her eyes did not get any better. We noticed her left eye began drifting upwards and causing her a lot of discomfort.
Paisley ended up in the ER on March 5th, 2019 for a high fever and vomiting. We were really concerned she might have developed hydrocephalus or high pressure. Testing came back with the flu. We were sent home with medication.
We saw Paisley’s ophthalmologist in May and she was diagnosed with a 4th Nerve Palsy. Finally we had a diagnoses. Before having eye surgery to correct her eye, her doctor recommended we determine the cause of the sudden onset of the palsy. We had an appointment with Neurology and came up with a game plan.
Paisley had a lumbar puncture (her 1st ever) and both a MRA and MRI. We received a call from Neurology stating everything came back normal and there was no explanation for her 4th nerve palsy and to proceed with the eye surgery. In our hearts we knew something was wrong. Paisley was not playing like a 4 year old should be. Her legs have been bothering her and her energy levels are just not what they should be. Her tolerance for physical activity is minimal without some sort of head or neck pain. We also felt her opening pressure for her lumbar puncture was higher than we were comfortable with. We decided to reach out to a Chiari Specialist.
After much thought and watching Paisley only get worse, we decided to go see a Chiari Specialist. We traveled to him on August 12th. It was well worth the trip. The decision was made to do another Lumbar puncture as well as more imaging of her spine to check for instability. From previous imaging, we were informed she has slight retroflexed otonoid. We decided to go back to the specialist to have these procedures done. We traveled back on August 25th.
Before this trip, Paisley started having strange episodes with her legs. On a few occasions, she has told us her legs do not work and scoots on her bottom. She will tell us her legs don’t work and that they itch. She pretty much walks on her tip toes. She also gets the feeling of being cold all over her body and spends much of the day laying on the couch. We just want to finally get some answers for her and get her some relief.
Paisley had a Lumbar Puncture on August 26th and had a significant amount of CSF drained. We are hoping by lowering her pressures we will see a difference in her behaviors. She also had imaging of her neck and spine. She does have some instability that needs to be addressed. However, this is difficult due to her head tilt caused by her 4th nerve palsy. We also discovered the stent appears to be touching her 4th cranial nerve. We are questioning if the stent that was placed in her 4th ventricle during her 3rd decompression could be causing the palsy. If so, this most likely will need to be addressed. For now, we are observing her behaviors over the next week.
This journey has been beyond difficult and exhausting. It has taught us a lot about strength, resilience and life. It has taught us to never take anything for granted and to appreciate everything, no matter how simple it may seem. Paisley gives us our strength and is the strongest little girl we know!
After Paisley’s 1st surgery, we decided to remain positive and turn our journey into a mission; to help make change. So much more research is needed. More funding is needed. This is how Cans for Chiari was born.