I've hesitated on posting on this for about a week now because 1) every time I try to talk about it, I end up in tears and 2) because there are so many what ifs that it can become quite confusing. But, I would like to keep everyone up to date on what's going on with Aiden and for anyone that likes to pray specifically you will know what to be praying for.
We've been talking about Decompression for Aiden's Arnold Chiari ll Malformtaion for months now. But every time we get close to the "final" MRI, his shunt malfunctions and delays everything (no complaining on our part though). This all started back in October when Aiden was hospitalized for a cold. His apnea was increasing and he was in rough shape. After doctors realized the problem was most likely neuro related they decided to do a detailed MRI (where they sedate Aiden to get the most detailed pictures of brain and spine possible). Those MRI results came back BAD BAD BAD indicating that his Chiari Malformation was to blame for all of this. But since decompression in infants (Aiden was only 3 months old at the time) is so risky, they decided to do the fenestration(burrow hole they made in the top of his head that's now affectionately known as Bumble) surgery instead in hopes it would relieve some of the pressure on Aiden's brain. It worked for about a week and in the middle of November the apnea came back and we began the ping pong game of shunt revisions every few weeks since then. The apnea has come and gone since birth really but since it was officially diagnosed as Central Apnea in October, it's been an on and off thing. He will have 3-4 nights of apnea and bradycardia spells and then nothing for a few nights and so on and so forth. All of this coming and going as he has shunt malfunctions and revisions. So we knew that decompression was coming at some point but the surgeons had hoped we could wait until Aiden was a little older to operate.
What is Arnold Chiari ll Malformation? It is where the brain tissue protrudes into the spinal canal. It occurs when the lower part of the skull is abnormally small which pushes on the brain and forces it downward. This structural defect is in the cerebellum. This resulting pressure on the cerebellum can block the flow of cerebrospinal fluid and cause a wide range of symptoms. Almost all children born with spina bifida have chiari malformation, but most never shown symptoms or have problems with theirs. It is rare for a chiari ll to be symptomatic and more rare for it to happen in infants. Symptomatic Chiari Malformations are the leading cause of death in children under 2 with spina bifida due to respiratory failure. It's been recorded that more than 30% of infants with symptomatic chiari malformation die before age two. Again, there are a wide range of symptoms indicating that there is alot of tightness in the base of the skull or a significant amount of brain tissue has "slipped" into the spinal column. Aiden has many of these symptoms-central apnea, gagging/choking on his saliva, retracting breathing, no/poor head control, arching of the neck, weak/low cry. The only major symptom Aiden is still not officially diagnosed with yet is stridor. Stridor is usually the most serious cause for problems with the chiari due to paralyzed vocal cords and many(but not all) of these children end up needing a trach for a while until their vocal cords heal. He definitely squeaks and wheezes when he breaths and gets excited but it's still not very loud and is not happening consistently. An MRI is used to show how far down the brain tissue extends and if cysts are present on the spine. Aiden currently has a very large cyst on his spine in his C vertebraes (part of your spine closest to the skull) .MRI along with how serious his symptoms are will determine if decompression is warranted or not.
What is Decompression? When Chiari Malformation symptoms are serious enough then decompression is necessary, it is the most common treatment plan for Chiari. Depending on the severity, how "tight" it is and if a syrinx(cyst) is present or not will determine how much they need to remove. The surgeon usually doesn't have a set plan until they get in there and see what needs to be done. It's designed to make more room for the herniated cerebellum and to relieve pressure on the brain as well as improve flow of cerebrospinal fluid through the area. Again, there are multiple ways to perform the surgery but one example is...the surgeon will make a Y shaped incision at the base of the skull (the back of the head and neck). A small part of the skull is removed to enlarge the opening giving more room to the structures within. Sometimes the first, second, or even third cervical vertebraes are also removed to relieve pressure and widen the space. In most cases the dura mater (the covering of brain and spinal cord) is also opened and expanded to create space within and a dural graft(either synthetic or a piece of covering of the bone from the skull) is usually applied to cover the expanded area.
Recovery. Recovery varies greatly after decompression surgery, especially in infants. There are many factors-age of infant having decompression, severity of symptoms and damage caused before decompression, and what was removed and done during surgery. Most infants are in the hospital 4 or 5 days after surgery and it's usually a few more weeks recovery at home with physical therapy involved to get them moving their necks. Unfortunately it's not uncommon if the vocal cords are severely damaged prior to surgery for the infant to require a tracheostomy and need a feeding tube after surgery. That's a whole other story and a path we aren't even going to think about right now. Ideally Aiden would come out of this surgery with showing improvement of his symptoms over the course of a few months. It usually doesn't happen immediately, everything takes time to heal. The downfall of having the decompression as an infant would be bone regrowth. Because infants are still growing so rapidly, there is always the risk of the bone regrowing and symptoms reappearing or symptoms becoming worse because of compression/tightness in that area.
So our greatest prayer is that Aiden doesn't even need the decompression as an infant and that we don't even have to think about it ever again. But with his current symptoms and prior MRI's, we are preparing that Aiden will need decompression. The neurosurgeon has tentatively scheduled Aiden's decompression for around the 2nd week in March. He will have his detailed MRI on February 24th. We will meet with his neurosurgeon that day to discuss the results and where we go from there. If the MRI shows improvement since the MRI in Oct/Nov then we may be able to postpone the surgery a little bit longer but everything depends on the severity of Aiden's symptoms. We would appreciate as many prayers and thoughts as possible on Friday that the best decisions for Aiden are made by the neurosurgeon and my husband and I. It's been a source of great stress and worry for us lately as we know the risks both ways involving a symptomatic Chiari Malformation. We will be wearing our Team Iron Aiden gear that day to show our sweet boy that he can do this and we are all rallying around him to feel better soon!!! Thank you everyone for your love and support in this very difficult season in our lives. We appreciate it more than you could ever know!
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