Starting last summer J started showing signs of frequent headaches and migraines. We’ve seen this before and we typically make our rounds to the specialists, try some things that don’t work, and then one day things get better for a few months. So we made our rounds and the docs said to hold tight. We increased his med gabapentin and things got better... for about 2-3 months. Then his headaches and migraines came back with a vengeance in December. His head an ear pain was incapacitating. He couldn’t go to school, get through a session of therapy, or get through his day without crying in pain. My son has a ridiculously high pain tolerance, so we knew things were bad. He had one ear infection after another with tons of blood, pus and possibly a ruptured ear drum. We learned his tubes were clogged and not functioning so he went into surgery for new T-tubes to relieve the pressure and clear the infection. Slowly things got better, much better actually. We crossed our fingers and hoped that his headaches were brought on by ear infections. But it seems they weren’t.
I was thrilled J was doing so much better! He could go to school and function again! He enjoyed therapy and could get through his daily sessions again. He was mostly happy. But every day he still had moments of rubbing his head and eye, whining, grunting or crying. This is what he had done in the past and docs have said we don’t know if for certain it’s his head. It could be referred pain or behavioral. Except now, we do. Since Jayson started communicating on his iPad in September he has been able to tell us when he is in pain and where. It’s been eye opening and life changing, but also heartbreaking. Nearly daily Jayson is communication to us, “I hurt. Head. Eye. Medicine.” It’s usually combined with the head rubbing and whining so we believe he is telling the truth. His head and his eye hurt. A lot.
Doctors have surprised me at how much they care about Jayson and how he is feeling. Now that he has a voice, he is being heard. I was prepared for a long battle of taking pictures and videos over months to convince them my nonverbal son with a high pain tolerance is in fact in pain. But it wasn’t needed this time. In fact, their concern has thrown me for a loop. For perhaps the first time on this journey, they seem more concerned than I was. As I saw several of them over the last month and a half, I have described various symptoms and concerns, telling them it probably sounds crazy that he’s having so many things go on right now that seem unrelated. They have explained that they don’t think they are unrelated. They’ve explained the many functions of the brain stem and how essentially all of my concerns could be tied to brain stem compression, or chiari. No. Just no. I’ve convinced myself for my own sanity that J’s Chiari is stable, especially after cranial expansion surgery. I told myself this isn’t going to be an issue anymore. But what if it is?? It takes my breath away to think about.
Let me go back and explain our past few appointments and situations:
Back in December Jayson had ear infection after ear infection. He was miserable, constantly crying and blood and pus were continuously draining out of his ears. When this was happening, I repetitively told Mike, “Something is different this time.” We’ve dealt with dozens and dozens of ear infections. Nothing was like this time. None have ever seemed so painful. Usually we discover them when he’s acting like nothing is wrong! I wondered why things were so painful this time and planned to ask the doctor at our ENT appointment in February. As I prepared for the appointment, I remembered that my new friend, who is the mom of the other boy with J’s Syndrome, told me to ask about an ear condition. She said her son was diagnosed with it and it causes major balance issues as well as ear and head pain. I decided I better look it up to have some background info to ask my doc about it. Superior Canal Dehiscence. As I read through the description and symptoms, my eyes filled with tears. Everything fit. It would explain so much!! It could explain the ear and the head pain and symptoms. It would explain nearly all of the same things a brain stem compression would. It was a real possibility in my mind, so I prepared and collected medical records for hours so our ENT could see that this is a real possibility. The day of our appointment Jayson was sobbing in pain for over an hour, holding his ears and head. It was heartbreaking and so difficult to get him to the doctor in that state. But the doctor saw first hand what we were dealing with. We talked about ear tubes right away. I hesitantly mentioned Superior Canal Dehiscence wondering how I could reach my notes with an inconsolable, thrashing child in my lap. But I didn’t need them. The ENT was familiar with the condition and felt it was worthwhile to screen for it with a CT. I felt so relieved. With more answers, a diagnosis and more communication, things were becoming easier. Everything isn’t always a fight anymore. It’s so relieving.
So we had a plan to do a CT on each ear looking for Superior Canal Dehiscence the next time J was under anesthesia. That ended up being soon with his ear tubes, but we couldn’t fit the CT scans in then since J was an urgent add-on. I was worried about not having answers about his ears since our next procedures could be months or a year away!! But I tried to tell myself that it wasn’t urgent and we could just wait.
We were referred by ENT to audiology to check J’s ear tubes. Sure enough, they were both clogged. The audiologist confirmed what I had read— there is little more painful than having Superior Canal Dehiscence and no way to relieve pressure through open canals. She said that could explain why these rounds of ear infections were so much worse. His ear tubes were completely clogged. Maybe this was leading us to answers. She said he also failed his clinical hearing tests, which he often does. His behavior concerns and his inability to verbally respond and follow directions make it difficult to test him. So the audiologist ordered a sedated ABR hearing test to get a better look at his hearing.
Jayson had a terrible fall at school that no one witnessed. The teachers said he likely lost his balance while trying to stand (as he often does) and hit his face on the chair or table. He split his lip open and lost a tooth. We had to quickly take him to the hospital where we discovered he needed stitches and some teeth pulled since they were misplaced. Fortunately, we had ear tube surgery scheduled the next morning. They coordinated things so the ENT could stitch up his lip and a dentist could come in and assess his mouth and pull 3 of his teeth while we were doing the ear tube surgery. It was quite the traumatic episode, but Jayson was brave as usual. After J's ear tube surgery things got much better like I mentioned and he healed quickly from his accident.
A couple of weeks later I prepared myself for our neuro appointment. He had healed from his ear tube surgery and things were better, but he was still in daily pain. I had videos and pictures cued and a list of concerns ready. I wanted to discuss another brain MRI, not knowing whether or not it would be necessary. After just beginning our conversation, he told me he and two other of J’s doctors feel an MRI is necessary. I didn’t have to ask. I didn’t need evidence. His symptoms were concerning enough they spoke for themselves and it scared me. He said he wanted the MRI sooner rather than later. That scared me even more.
Another one of J’s doctors candidly explained J fits the mold for a child with a recurring Chiari and brain stem compression. He said he’s glad our neurologist ordered and MRI. If he hadn’t, he would be asking about one. He said if the results are questionable and doctors are fearful of risks for a second decompression, he has seen so much benefit from decompression. It should be something J’s docs should really consider. How are we having a conversation about brain surgery??? How did we get here?! I left that appointment so grateful for the candid comments of a doctor we love, but with so much fear in my heart.
So here we are today at Primary Childrens getting a brain MRI, 2 ear CT scans and an ABR hearing test. We've had so many of these tests. Too many to count, honestly. This is his 11th or 12th MRI, his 6th or 7th CT scan and his 4th or 5th ABR. So why does it feel so different this time? Why do I feel like an elephant is sitting on my chest? Why can I not feel hope no matter how hard I try? I've thought about it, and I think I understand why things are so different this time, why my anxiety is so high and why I can't find something to hope for.
There is no ideal situation here.
There is nothing specific to pray for and nothing I really want to hope for.
Every possible outcome to these procedures feels so very heavy, sad and hopeless.
In the past I have hoped and prayed for clear imaging, that nothing concerning will be found. But it's different this time. If Jayson's MRI and CT scans show that his Chiari is fine, that there is no evidence of pressure and no change since his last MRI, and that his ears are typical then WE HAVE ABSOLUTELY NO EXPLANATION FOR HIS DAILY HEADACHES AND SUFFERING. I personally know decompressed Chiari patients who are on disability and live a painful, miserable life with daily headaches. I don't want that for my son. I want his headaches to be fixable. I want there to be an explanation. I want to be able to tell him the pain won't last forever. But if absolutely nothing shows up on his scans, I can't tell him that. I fear and believe that if everything checks out okay, his doctors will just say he is prone to chronic headaches and migraines and nothing will change in his life. I can't feel good about that. I can't celebrate clean scans knowing it means there is nothing we can do to ever relieve his pain since we don't know the source. That feels heavy, discouraging and hard.
There have been times I've prayed for something to be found on imaging, so we would have an answer and treatment plan. But that was before I knew how horrific and traumatic the answers, treatments and surgeries are. Knowing how risky and painful Chiari decompression surgery is, I absolutely do not ever want to repeat it. I've known kids and adults who have died during surgery. I've known others whose surgeries resulted in brain damage or even more pain. I don't want any of that for Jayson!! We got lucky that his first surgery went as well as it did. I now know and understand the risks. I now know how terrifying the surgery is and how painful and hard recovery are. I really don't want to live that with Jayson again... ever. But I also know it's likely. The research shows the likelihood of a child needing a second decompression surgery greatly increases if they had their first surgery early in life, if they have a craniofacial syndrome with craniosynostosis and if a stent was never put in the brain. Unfortunately, all three factors apply for Jayson. We know this is a real possibility but I want to avoid it as long as humanly possible. We also know how traumatic Jayson's cranial expansion surgery was. None of us will ever be the same because of that surgery. Just thinking about the possibility of Jayson needing that surgery again is enough to make me start vomiting. I can't think about it without having a panic attack. Yet, we know it's likely he will need another one. His doctor told us his brain will be full size between ages 8 and 10, and many children need at least one more surgery at or around that time. I don't want this. I never want this. I don't want his scans to be clear and have no answers, but I definitely don't want anything like these answers. I don't know how we would all get through this again. My boy shouldn't ever have to endure any of that again.
So then, logically, I should be able to hope that he tests positive for the ear condition Superior Canal Dehiscence, right? It could explain his pain and symptoms, but it isn't Chiari or intracranial pressure. That should be what I pray for. But no. As I've learned more about this condition, it would be like receiving a life sentence of complete and utter misery. Let me copy and paste some descriptions of symptoms with SSCD:
Vestibular symptoms and signs: The vestibular symptoms in SSCD can be debilitating and often provoke patients to seek medical attention. Patients may note that loud noises cause them to see things moving or that they experience a similar sensation when they cough, sneeze, or strain to lift something heavy. They may perceive that objects are moving in time with their pulse (pulsatile oscillopsia). Some individuals can bring on the sensation of motion—and cause their eyes to move—by pressing on their tragus (the area of skin and cartilage located just outside the ear canal). Patients may experience a feeling of constant disequilibrium and imbalance.
Auditory symptoms and signs: Patients with SSCD may also complain of symptoms such as hearing their eye movements, hearing their own voice too loudly in the affected ear (autophony), or having a distorted sensation of sound in the affected ear during activities such as running.
Jayson has both vestibular and auditory symptoms that lead us to believe this is a possible diagnosis. His recent fall has finally helped his doctors realize his balance issues are in fact a big deal. And we know his ears and hearing have been affected and sensitive. As I read about this condition and watched videos of patients describing their lives, it broke my heart. It is painful and very disabling. Although this condition isn't life threatening, I really don't want this for my boy either!!! There is a surgical treatment but it is highly invasive and much more extensive and risky than Jayson's previous surgeries so I don't think we'd ever consider it. You can manage symptoms by avoiding triggers. We would drastically have to change our lifestyle which we'd be more than willing to do. Jayson would likely never be able to fly in an airplane and we may look into relocating if it would help symptoms. This would be a very difficult condition to deal with and we really don't want this to be the explanation of his symptoms.So really, it feels like there are not any ideal outcomes. There is nothing that I am really hoping or praying for and any outcome brings with it an immense amount of anxiety and heartache.
What we really want is our boy to be happy and pain free. We want him to enjoy life again, ALL of the time. We want him to be able to participate in his favorite activities without rubbing his head or having to lie down. We want him to be able to stay safe and school and not have to worry about him losing his balance and hurting himself. We want him to be able to sleep without moaning and rubbing his head. It's a lot to ask and not a lot at the same time.
I think many people take their health and comfort for granted. That's all we want for our sweet son.
So now we wait... I should have access to the MRI and CT reports sometime this weekend and I will hear from the audiologist about his ABR right away. I really didn't want today to come. I don't want answers and I don't want to hear that we don't have any. I just want peace and I can't think of a way that peace, comfort or hope can come from any of today's results. I guess what I am hoping for is a miracle. Something we haven't thought of. A way for everything to be okay without a lot of suffering. God is good and we know that. He has helped and guided us all along Little J's Journey. It is possible He has this under control and we are about to learn of His great plan for Jayson.
And my faith is about the only thing that brings me hope on these hard days.
So for now, I'm not praying for answers and I'm not praying for clean scans. Today I'm praying for peace and I'm confident that is something my loving Father can give me.
Thanks for your love and prayers.
