Seanna & Bennett’s Story

Bennett is the youngest of our four boys in the Eisel Family. When Bennett was 2 years old he started complaining of neck pain. At first, I thought that it most likely was a stiff neck or he was playing too rough with his older brothers, but as the weeks went on I just knew that something was not right. I took Bennett to our pediatricians office where he was examined by the NP. We left with a referral to see neurology and an order for the first of many brain MRI’s. Later that week Bennett was diagnosed with Chiari Malformation type 1 and after more imaging our family learned that he also had a syrinx on his spinal cord, which unfortunately was very close to his brain stem. We met with our neurosurgeon, Dr. Sean McEvoy, at St. Louis Children’s Hospital. A few weeks later Bennett had his first decompression surgery which was a bony decompression. This surgery would work on making more room at the base of his skull where the spinal cord and skull meet. The surgeon would make the opening at the base of the skull bigger to give his cerebellar tonsils more room to hopefully not compress against his spinal cord which was blocking CSF flow resulting in a syrinx. The surgeon would also perform a laminectomy of his C1 and C2 vertebrae with the goal to also give that opening more room for his brain.

Recovery for Bennett was not easy, he struggled with pain control and required a few extra days inpatient to help get his pain under control. Bennett progressed and did well over the next few months, but shortly after that his neck pain returned. Over the next few months I started to see the symptoms that I knew prior to surgery return. After another round of imaging we were told the Chiari was unchanged and that the syrinx had returned and once again was close to his brain stem. On Bennett’s 3rd birthday he had his second decompression surgery, this time the surgery would involve the dura (the membrane around the brain). The surgery went well but once again Bennett struggled with his pain control, this time they involved the pain management team and they came up with a plan that worked. Bennett came home 8 days after this surgery. Everyone was hopeful that this would be the last surgery. Once again, a few months later, his symptoms came back, including some new symptoms.

Bennett had another round of imaging, which once again revealed that his Chiari remained in place, and once again was pressing against his spinal cord resulting in a syrinx, this time the syrinx was much bigger. Dr. McEvoy felt it was best at this point to admit Bennett and do several different tests to make sure that we were not missing something else that could be causing his cerebellar tonsils to continue to drop below the skull. Bennett was admitted to the PICU for 5 days, where a bolt was placed in his skull to monitor for increased intracranial pressures (ICP). Dr. McEvoy also wanted to look closer at his spine to make sure that there were no tumors that could be causing pressure and pulling the cerebellar tonsils down.. Once the bolt was removed they did several different imaging scans and it was determined that his ICP was ok and that there were no tumors on his spine. Bennett was scheduled for his third decompression surgery, and after a lengthy discussion with Dr. McEvoy, this time they felt it was best to resect the part of his brain that continues to slump below the base of the skull (cerebellar tonsils). There were many risks associated with this surgery, due to how vascular that area of the brain is, the biggest risk being a stroke along with there being a large risk for CSF leak. The risk of not doing the surgery was causing spinal cord damage due to the size of the syrinx and since we were seeing issues with Bennett walking already, we needed to proceed with the third surgery.

Bennett was inpatient for a longer stay after this surgery. By this point in his journey we had become friends with several of the nurses on the floor that Bennett would be admitted on. We saw how they loved our boy, they had gotten to know him and knew what worked best for him for pain control. The nurses, techs and doctors knew his sassy personality, they could read his little face as well as we could at this point. One thing to note, my cousin who is like a sister to me, happened to work on that floor at the time. I just keep thinking about how crazy life works. What are the chances that she would work on the exact floor at Children’s that we would spend so much time on? Molly just being there each time brought us so much comfort and peace. The bond Molly and Bennett have and even had prior to any of this is so special.

After being home for about a week Bennett developed a CSF leak and was readmitted and taken back to the OR again to patch the leak. We came home and returned less than 24 hours later with a fever and once again, he went back to the OR for spinal fluid cultures and he began treatment for meningitis. During this stay, Bennett was diagnosed with an Immune Deficiency and began receiving infusions to help boost his immune system called IVIG. After several scary days, Bennett was able to come home and we continued his IV antibiotics for a few more weeks at home. Bennett also continued with the IVIG infusions monthly outpatient. After his last surgery Bennett continued to get imaging frequently. His chiari has remained stable. Bennett works with PT, OT and Speech therapy weekly. He has struggled with school but is making huge improvements since moving to a new environment with more support in place for him. Bennett continued the IVIG infusions for a few years, and was just able to wean off of them this past year.

Currently Bennett is on the list to see genetics and neuromuscular, to make sure that there is not a link to his Chiari and some of the other things that have come up for him. Bennett has taken us on a journey we never ever thought we would be on. We are beyond lucky to have the team of doctors and nurses that we have following him. I would truly be lost without our nurse navigator Jenn at Children’s. Our family was very fortunate in the outpouring of love and support from our family, friends and community. Dr. McEvoy once said to us “if you have seen one Chiari you have seen one Chiari” meaning every Chiari is different and unique. Bennett certainly drove that point home in his journey.

Bennett is perfectly imperfect and while some days are harder, I am so glad that he is ours. Being in the Rare Disease community is a roller coaster. Navigating this world is HARD. Advocating is HARD. There is every book under the sun for every single disease, but I need the “Book for Bennett”. For the longest time I would just say “I’m fine. We are fine” and push forward. Some days I say “I wouldn’t change a thing” and other days I am screaming with how unfair this can all be at times. Finding other Rare Disease parents and talking with them, and helping through some of the hard over the past few years has lit a fire in me to help bring this community together.

Bennett is the bravest person I know. He is our Brave Bennett.