Bill, Andrea, Jake, Kylie and Ty

The Ippolito Family

Kylie Ippolito

 

           Kylie Ippolito is an eight year old girl from Point Pleasant who was diagnosed with Chiari Malformation in April 2008. Chiari Malformation includes a complex group of disorders characterized by herniation of the brain through the large opening in the base of the skull into the spinal canal. The herniated tissue blocks the circulation of cerebrospinal fluid in the brain.

 

     Kylie’s symptoms included debilitating headaches, leg pain, muscle weakness and tiredness. She had the beginning of a syrinx in her spine (which left untreated would cause paralysis) and scoliosis. Her brainstem was compressed.

 

    On July 30, 2008 she underwent surgery in which part of her skull was removed, along with the part of the brain which was herniated.  Sections of two of her vertebrae C1 and C2 were also removed.  The recovery has been long and hard.  The healing takes awhile and it could be months before knowing how successful the surgery was.  In her case, it has been 6 weeks since the surgery and her headaches have diminished.  She is smiling again, which was rare before. Her spine has straightened – no more scoliosis.  She still has a long road ahead of her which includes physical therapy and may consist of another surgery.

 

January 2009: (5 months Post-Op)

 

It has been awhile since I have had an update on Kylie. You all know that I usually am waiting for something positive to say, but things have been the same. Her Decompression surgery was 5 1/2 months ago. We just had her follow-up with Dr Kula, a neurologist from the Chiari Institute. The good news is that her headaches have been non-existent for the past few months. Great news! She has been having eye pain for the past month, but nothing like the headaches. She is swelling in the neck alot. Dr B calls it Lymphatic Stasis. As she swells, her jaw comes out and gives her an under bite appearance, along with her choking due to the constriction around her throat. Her voice is also altered and vocal cord still sounds paralyzed.  Hopefully this will subside when she is completely healed at about 1 year from surgery. This is crazy because from day to day she looks totally different!

The bad news is that because of her Ehlers Danlos Syndrome, which makes her ligaments very relaxed, she has little support of her head. It is so difficult for her to move it or even lift it. Physical therapy is working with her to strengthen the muscles in the back of her neck to hopefully pull her head up more. Her past MRI shows that her cervical spine is very curved at the top (it should come up straight) and her head is positioned wrong.  This has to be corrected and could lead to many problems down the road. At this time we are waiting until her one year mark to see where she stands. The solution to this problem would be a fusion of the cervical vertebrae to her skull. Dr Kula is checking her Vitamin D and Calcium levels to make sure she is sufficient. If lacking with osteopenia, the fusion will not be possible. She was prescribed an Aspen collar as well as an inversion table (to alleviate the pressure on her neck)

Last month our 10 year old son, Jake was diagnosed as well with Chiari Malformation ,asymmetrical cerebellar tonsils 5mm and 8mm and we already know that he has Ehlers Danlos Syndrome. He has been symptomatic since at least 2 years old with severe migraines and poor balance. We used to get mad at him being so clumsy, little did we know that he could not help it. As the years have progressed, his migraines have intensified. We had an MRI last year, due to the frequency of his migraines. His Chiari was missed by the radiologist. It wasn't until right before Kylie's surgery that I took out his MRI and saw it plain as day. When he had this MRI, we had no clue about Chiari. He just had a repeat MRI this year at another facility, they picked it up and verified my readings. Currently it seems that with any physical activity he develops a headache. We are in the process of further testing to evaluate how much blockage he has and will be making an appointment at the Chiari Institute. We were lucky enough for Dr Kula to read the MRI when we were there the other day. He definitely wants to follow up with him. The scary part is that he may end up with cranial instability if having the decompression like Kylie, because of the Ehlers Danlos.

I finally went to a doctor today. It has been a year since the fender bender that I was involved in and since then I have had the severe headaches, vision problems and brain fog. My entire life I have been very clumsy and off balance. I could never keep my neck hyper extended due to pain and immediate headache. Since Chiari is hereditary and at least 2 of my children have it, Dr B. indicated that I probably do as well. I am scheduled for an MRI next week. I have already been diagnosed with Ehlers Danlos. And to top it off, the doctor says I have a heart murmur and need to go to a Cardiologist. Here we go again, almost makes me regret going in the first place. Sometimes it is better not knowing.

This month marks the year anniversary the worst year of our lives. Last January on Martin Luther King Day, Kylie fell on her tailbone ice skating. This was the day that started all of her symptoms which has led us to this point. They say that everything happens for a reason. I am still trying to figure it out. Kylie is absolutely the strongest and most confident person I know.