Eddie met a new audiologist and ENT today. He doesn’t dread the ENT, but he doesn’t look forward to those visits. E has had the same local ENT since he was an infant and the man is so kind. He has an amazing bedside manner and has even come into the office on a Saturday one time to see E. However, he does not adhere to a super conservative approach when it comes to surgery. And he doesn’t know a lot about Loeys-Dietz Syndrome…..but of course, that’s not unusual.
Over the years we’ve come to realize E’s experience with ear issues was not that uncommon in the Loeys-Dietz community…..tubes, recurring infections, cholesteatoma, typanoplasty, hearing loss, etc. And I have heard story after story of failed ear surgeries and procedures, so the idea of putting him through yet another one after the last one was so challenging (and ultimately unsuccessful) did not seem wise. I wanted him to see a doctor that understood LDS or at least was willing to learn enough about it to handle E’s issues uniquely.
We’ve been hoping for a number of years to coordinate his appointments at Hopkins so he could see a specialist there that is working with the Loeys-Dietz team and has a research interest in connective tissue disorders. However, the schedules have not been able to align. Last fall when E visited the NIH team, they wanted him seen by an ENT sooner rather than later because they discovered a nasal polyp they believed was causing him some complications.
All of these led us to Dr. Eleanor Kiell and Janet Black at Wake Forest/Brenners today. E’s hearing test indicated no real change from the last hearing test a few years ago. This is good. He has a mild to moderate hearing loss that typical affects low range sounds. The main issue is his perforated eardrum. There appears to be no issues with the bones of his ears. This is very good. Letters sounds such as vowels, m, and n are the more problematic, but it doesn’t seem to cause him a great problem in communication.
Now onto his nose. Bless his heart. Dr. Kiell performed a nasal endoscopy. Basically, she inserted a thin tube that had a camera and a light into his nose where the images were projected onto a screen and recorded. It is a simple procedure that I got to stay in the room for. No anesthesia. No relaxation methods. Just do it.
This was not comfortable for Eddie, to say the least. That tube was so far in his nose I could see the light shining into his throat. Seriously. No hyperbole here. But she was able to see the polyp. She said that it is not completely blocking his nasal passageway. However, the NIH’s measurements indicate it is causing him some complications including affecting the development of his face and craniofacial features and dental issues. Therefore, it will need to be treated. But first we need a CT scan.
Dr. Kiell is going to try to get a copy of the images taken of Eddie at the NIH in the fall to save us from another scan. However, if we are not able to get these, he will have a CT scan of his sinuses when we return to Dr. Kiell in a month. She said assuming he has sinus surgery to remove the polyp, it should be quick and easy, relatively speaking. Additionally, we can consider repairing his eardrum at that time with another tympanoplasty since he’ll already be under anesthesia.
With all that said, I don’t see another tympanoplasty in the future. That first one was brutal when he was 8 years old. He said it was worse than all the other surgeries he’s ever had because he was so sick and dizzy afterwards. And if it were to fail again (which is a real possibility because of LDS), we are just inviting issues with scar tissue in his ear. Eh…..doesn’t seem like the benefits would outweigh the risks to me. But the polyp is an issue that will need to be handled. It’s way back in his nose near the maxillary sinus. Not exactly thrilled about the idea of him having another procedure and being put under anesthesia again, but this seems quick and easy in comparison to some of the others he’s had. And we really liked Dr. Kiell so that’s half the battle. Will update when we return in a month. In the meantime, waiting on all the records to be sent so we can get Eddie an appointment with a doctor that will be his local cardiologist for between Hopkins visits. Hoping that won’t be too long because he’s due for another echocardiogram.