From January-March 2020:
First, Addie took another ABR test with Audiology. Previously, she'd flopped on two booth tests. In the booth, Addie listens to sounds as audiologists watch for her to react. Mostly, she just stared ahead. Sometimes, she seized or coughed. Since that was the only response, we marked those on her hearing chart. As you can imagine, the results of the two booth tests were widely far apart. One said she had mild hearing loss and the other suggested moderate hearing loss. Either way, those diagnoses were better than profound. However, questions remained as the disparity between the tests did not answer whether Addie could actually hear us. Thus, we decided to pursue another ABR. After 2-1/2 hours of testing, Addie gave us no response on any of the tones except for major seizing at the loudest tones. At those sounds, the earpiece vibrated and buzzed in her ear. Did she seize due to vibration or due to the loudness of the tone? Either way, the audiologist deemed that hearing aids would not be beneficial to her as the volume would need to be turned up so high (think motorcycle or jet plane) that it would send Addie into a seizure. And. . . no one hears during a seizure.
Additionally, the audiologist pulled up the full report of Addie's 2017 MRI and translated the findings for us. We learned that Addie has something called "EVA" (Enlarged Vestibular Aqueducts) in addition to cochlear damage. In EVA, the canals holding the fluid that helps in balance are enlarged. Instead of reabsorbing the fluid as it comes and goes, the fluid builds up. Then, if the person injures or bonks his or her head, or has a seizure, the canal breaks and the fluid rushed out eroding the hearing structures. This continual "bursting of the dam" eventually leads to complete hearing loss and it is irreversible. We don't know if it impacts balance as well. But how could it not? Maybe that's why Addie always seizes and vomits her food . . . nausea from the world spinning all around her? Chicken or egg first--chicken or egg?
So, while Addie's hearing loss remains profound, we were able to close the door on hearing aids. It felt so nice to finally have an answer! We can now focus on therapies that we know are working. With hearing aids, I could never tell if they were helping or hindering. Sometimes Addie seemed very stressed by the feel of them in her ears. I never saw any definite hearing response. It is hard to justify a $5000 purchase of something that may or may not be helping.
A big thank you to the Utah School of the Deaf and the audiologist there, Christine, who helped us navigate this world for the past two years by loaning us hearing aids to try and all the other hearing services provided. How we love and appreciate you! And thank you to Natalie, an audiologist for IHC, who helped us with the ABR and educated us on the MRI results relating to the ears.
Next, we visited Neurology. We met with a new doctor to get a different viewpoint. She explained that the western medicine view of the brain offers three things: 1) drugs 2) surgery or 3) ketogenic diet. We have had less than stellar success with drugs. Surgery isn't an option for Addie--as her seizures are multi-focal (all over her brain--you'd have to cut the whole thing out!), and the diet may be an option for Addie (if her Mom can get over feeding her baby chemical nutrition and risking Addie catching every cold and flu bug that her siblings bring home from school. Currently, Addie has not had a serious bout of a virus, though one or more of her family members have been ill all winter).
After this visit, we decided to pursue seizure control in this order 1) peripheral stimulation therapy as taught to us by Addie's chiropractic neurologist 2) CBD trial 3) ketogenic diet, and 4) drugs. During the visit, Chris asked the doctor, "Would the therapies that work for kids with cerebral palsy work for Addie?" The doctor responded, "She has cerebral palsy." My heart went, "Wait . . . what?"
The doctor said that often this diagnosis is delayed until the child is 2-3 years old to see if they outgrow the global developmental delay, her previous diagnosis. In Addie's case, after reviewing the MRI, she believes there is permanent brain damage that will impact Addie her entire life. Put another way, the immobility of her limbs and neck are not due to seizures but to the three spots of white matter in the center of her brain as seen on the MRI. Thus, she changed Addie's diagnosis from "global developmental delay" to cerebral palsy.
That was a shock! And--I had to bury the hope that if we could just get Addie's seizures controlled, she would eventually learn to hold up her head and move her arms and legs--learn to crawl and eventually stand and walk! Needless to say, I spent many nights this month crying myself to sleep over these closed doors.
Next we visited a new doctor with Pulmonology. Addie's lungs were especially crackly that day and the doctor felt highly concerned. She watched Addie "go blue" when we removed the oxygen from her nose. She also saw Addie's clubbed toes and fingers and said affirmatively, "This is not normal!" Since Addie had not had any x-rays of her lungs since leaving the hospital, her doctor suggested we get a CT scan of her lungs to get a good view of what Addie is dealing with. The doctor theorized that Addie has mucous pooling in her lungs. She gave us a prescription for a nebulizer, steroids, salt solution to break up the mucous, and ordered a cystic fibrosis vest to beat the mucous out of her lungs. When we started Addie on the nebulizer and steroid treatment, her heart raced to above 200 bpm. Her skin tone dropped to an ashen blue color. She became very lethargic and her limbs went cold. This reaction caused the doctor to pause the treatment until further analyses could be completed.
We went up for the CT scan, and the results showed no pooling mucous; the lungs looked surprisingly clear for Addie. The doctor felt so surprised and said that Addie must be misdiagnosed. It didn't make sense. She sent us to a cardiologist. We did a full work-up on her heart, with more ultrasounds, electrocardiograms, and the works. Addie wore a heart monitor for 2 weeks that observed her heart rate, especially during steroid treatments. In the end, the cardiologist could not find the cause behind Addie's excessive heart rate or her "blue" spells or her clubbed toes. No answers--surprise, surprise! He said, "I'm a plumber; you need to see an electrician." He did say, she has an enlarged aorta that will need to be monitored & measured annually. Lastly, he referred us to another cardiologist and then COVID shut down all appointments. So--the doors shut.
My mother heart told me that perhaps Addie's "blue" spells were due more to dehydration than anything else. So I tested my theory by adding 2 syringes of water to her diet every day, and we have not had any more blue spells. By small and simple things . . . .
