Hello, still waiting for a call (3 months) from Dr. Fargen for a diagnosis for my adult non-verbal autistic son, Ben. Today I requested a virtual call from Dr. Lee Pride radiologist in TX to try to get an official diagnosis. As you all know it’s difficult to get doctors to take this seriously especially with someone who cannot communicate. I can’t imagine the pain he and all of you are going through. Heartbreaking. Through body language and gestures along with lots of research we determined his symptoms long before we heard of Eagles. @churchyardyew was able to do a 3D video of his CT scan w/contrast and our nephew who is a new ENT in NC looked at the scans and agreed Ben has IJV. I have tried for hours to do a 3D picture of his right side showing the nerves etc. which seems to be his most symptomatic area but for some reason the 3D viewer does not have an active link. Below is the CT w/out contrast and I wonder why there is a gap on the right side where the arrows are ?? Is the styloid broken off or is it covered with nerves that can’t be seen on this type of CT scan? I was able to take some snapshots from the CT w/contrast video. Any help will be much appreciated. Some of his symptoms:
Elongated calcified styloids (right 4.8cm left 4.6cm) calcified hyoid
excessive salivation
hearing sensitivity (at times we can’t talk around him or he will scream and hit his head)
touching his right ear and throat area (definite ear and throat pain for years)
tilts his head to the left
rash on face mostly right side and forehead
right ear swells and turns purple/red randomly then hour later normal
hyperventilates and breathe holds
non-seizure episodes where his oxygen level sometimes goes below 85. pale and tired afterwards, dizzy, falls down, look like seizures but had 3 EEG’s w/no activity
low blood pressure
random choking when eats or drinks, sometimes screams when drinks water as if it goes down the wrong way (afraid to drink)
digestion/constipation issues. sometimes regurgitates. often diagnosed with acid reflux EDG showed no erosion. no h-pylori
no curve in neck, walks with head forward/toe walks (not from using any devices like phones) osteoarthritis spine
sometimes wants to eat continuous like he has low blood sugar or maybe trying to fix his throat (I know many say they have a lump in their throat), other times he refuses to eat
swelling on right side of neck muscle
clicking (a tech heard clicking during a trans cranial Doppler and thought it was TMJ however 2 dentists confirmed there is no erosion or signs that he has TMJ)
headaches (wants his head/neck rubbed) self abuse probably trying to re-direct pain.
extremely sensitive to medication side affects, has MTHFR gene
very thin, cannot gain weight regardless of how much he eats
had numerous trauma episodes including clavicle surgery, dental extractions/root canal
random low grade fevers
sinus - random runny nose - clear
rim of eyes red
allergies food/environmental
all of the other symptoms he can’t tell us that may or may not be related
@KarenG the best way to assess for IJV compression is by looking at the axial view at the level of C1. I’ve attached my own imaging to help you know what to look for. Look for the top vertebrae with a white circle in top middle:
Your poor son, his life must be so miserable
His styloids are certainly very long, and look quite needle-like, so not surprising that he has bad symptoms…you have done well researching this & advocating for him…
The left side styloid looks quite wide just at the top, I see what you mean about the gap on the right side- it could either be that he had very little styloid that side & it’s purely calcified ligament which you can see, or otherwise that it’s been fractured, which can occasionally happen, although it looks quite smooth at the top. Either way, it doesn’t really matter, as it all needs to come out! It wouldn’t be nerves covering it as nerves don’t show on a CT. I can’t see clearly enough on those images if there is IJV compression, it’s not that clear up near the skull base; IJV compression is common right at the skull base, between the styloids & the C1 processes. As @TML says it can be seen on axial views. As you say that your son’s neck is quite swollen one side, it could be that the SCM muscle is enlarged which can cause compression lower down as well. Clicking in the throat could be the calcified ligaments, and also the hyoid bone can sometimes cause clicking, but I can’t see the hyoid on those images so couldn’t say…
Some of his symptoms could well be ES; the choking, swallowing issues, neck and ear pain, hearing sensitivity, excess saliva, are all known ones. It could well be that his vagus nerve is compressed or irritated as it’s right next to the IJV, & this can cause BP issues, digestive system issues, and non seizure episodes possibly. These, headaches & being dizzy can also be vascular ES symptoms; it could be that the ligaments or hyoid bone are irritating his carotid arteries. Some members have had sinus issues, & we’ve had some discussions about medication sensitivity & allergies, although that’s not necessarily ES, maybe the inflammation that the styloids cause could contribute to general inflammation as auto immune conditions seem to be quite common, and also conditions like MCAS… The head forward position & lack of natural neck curve could possibly worsen his symptoms, there has been some discussions about these & exercises to try, but I guess that this would be difficult for him to understand and do…Do heat or ice help him at all? Some members have found the blood thinners help with the symptoms of IJV compression, but again this could be tricky as he’s sensitive to meds…
Hopefully you’ll hear from Dr Fargen soon, or Dr Pride. If you don’t hear soon, there are other doctors who might be able to help with a virtual consult. I’m sorry that you & your son are having to deal with this, will pray that you’re able to get answers, hugs for you as well
Hi @KarenG honestly your story brought me to tears. I just cannot imagine how you must be feeling having a non verbal son dealing with this condition. My heart goes out to both of you. I also have IJV compression and I can honestly say its miserable and has nearly brought me to my knees, but at least I can communicate this and discuss how I’m feeling. I have a lot of your sons symptoms, including the non epileptic seizures. I feel for you dealing with this, as my mum tells me this is very scary to witness. I am not sure exactly why these happen, if they are physical (seem to happen if I chin tuck for to long) or just the way my body is dealing with the stress, a type of PNES. If they are similar to mine, then I am conscious throughout, but cannot communicate, my muscles contract so much that they hurt and I do stop breathing for a bit. I really hope you manage to get someone to help your son soon.
Hi @Rosie I am so sorry you are going through this and appreciate your kind words. I hope you can find relief from the terrible pain you are experiencing. It is very hard to watch the non-seizure episodes. Seems like swallowing may be a cause because they seem to happen during times of excessive salivation. We have seen twice now where he ate a bite of food and went into an episode. I don’t know if the food/saliva goes down the wrong way or possibly how his chin is positioned like you mentioned. We will have to watch for that. He is definitely awake during them. Sometimes they do look like seizures with rapid eye movement. We are going to do a 72 hour EEG in Oct as we are trying to rule out everything we can. Something is causing them and I do believe it’s related to Eagles. I have often wondered if he had muscle pain during them because his legs sometimes stiffen like someone having a leg cramp. I can also tell he can’t control the movements. He is very thirsty after so that’s another reason we believe it is throat related. We have video taped them also to show doctors. Thanks for sharing because that gives me insight as to how he is feeling. We did talk to Dr. Osborne in CA on a virtual call twice. He said he has had 3 patients with these episodes and all went away after ES surgery. He is willing to do the surgery but the travel is complicated with Ben (24 hour drive) along with the astronomical out of pocket costs in CA. We have to get an Eagles diagnosis before we can approach insurance. Have you been able to talk with any of the doctors from the list about your symptoms? Praying for a speedy and full recovery.
@KarenG you’ve found it! Definitely compression between styloids and C1. So definitely will want to pursue surgery with a surgeon who is experienced with cutting the styloids above the level of C1! Compression appears more severe on his right side (left side of image where you circled)
Hi @Jules thank you for your concern and reply. Yea unfortunately he would not understand how to do exercises and he doesn’t cooperate well with other treatments like icing which makes us a little nervous about inflammation after surgery. That’s one of the reasons I like how Dr. Osborne uses the head wrap to reduce swelling. I think the vagus nerve may be affected. I have heard red light can help with pain and I wish he would allow us to use it on him. I appreciate having this group to learn and get support. It’s so overwhelming to have this condition and to be gaslighted by medical professionals is heartbreaking. I never would have known what to do if I hadn’t read what all of you have gone through to get help! We have always been gaslighted due to them blaming everything on autism but this is way beyond and borderline medical abuse. We had 2 renowned ENT surgeons from the physicians list (Ohio and TN) tell us Eagles was rare and Ben didn’t have it because he couldn’t tell them his symptoms. Like all of you I had to do all of the research and fight for the right to get a diagnosis and treatment. This is not a psychological condition. It’s real pain and I pray everyone who is battling this will get the correct treatment and help.
Thank you for your reply @KarenG. I am UK based so our health system is very different to the US. I have been 15 months trying in vain to get to the bottom of my symptoms. Uploaded my CT here and all became much clearer. I have consulted Mr Axon privately and had a diagnosis of IJV compression. Fortunately I have also got an NHS appointment next month with Addenbrookes our top hospital where Mr Axon is also based. At the moment I am not sure where this will take me.
I hope you get a diagnosis soon, so it will allow you to sort out the insurance. A 24 hour drive doesn’t sound good, but I am sure somehow you will manage to get the best treatment for your son.
The surgeons who are the most experienced with vascular ES are Dr Costantino in NY, Dr Cognetti in PA, Dr Nakaji in AZ & Dr Hepworth in CO, so unfortunately none close to you. How they’d be with treating your son, I don’t know- this is often so hard for our members to get diagnosed & treated, so I can only imagine what it’s been like for you with your son, it must be very scary & exhausting… Dr Nakaji does require quite invasive testing (a dynamic venogram with pressure gradient manometry) which sounds like it would be stressful for your son, so might be a no…
If Dr Osborne is prepared to do the surgery and can remove the styloids at the skull base, then hopefully it should help. Some of the other vascular doctors do check for anything else compressing the IJV, like other muscles, to make sure the blood is flowing well through it, which I don’t know that Dr Osborne does. Regarding the cost & insurance, some members have been able to appeal and go out of network, there have been some discussions about that, @vcp02 posted some helpful info: Dr Osborne- Blue Cross Blue Shield Out of Network Waiver-2025 - Welcome / New User Help - Living with Eagle
Otherwise Dr Hackman in NC has done lots of successful surgeries, I think he takes the styloids off close to the skull base, & Dr Fargen also in NC.
Sending you a hug
@Jules thanks so much for all of the helpful info. It is stressful trying to manage all of the details with only a handful of experienced surgeons to choose. Unfortunately I put all my eggs in one basket with Fargen. All scans and reports were sent to his office and still no phone call so now I feel like I’m starting from scratch again. I called Dr. Pride because I saw he specializes in radiology so hoping we won’t have to wait long for him to give a diagnosis. UT Southwestern TX has a rare disease clinic which is a plus. We have to consider whether the surgery is done in a hospital which may be needed due to his special circumstances and we definitely want an experienced surgeon even if we have to travel. I was given a number for private Medical flights which will be helpful since it would be near impossible to take Ben on a commercial flight. I have to trust God to take care of all the details otherwise I would lose my mind. Thankful for all of you
@KarenG - We’ve just added Dr. Babu Welch to our list of doctors who do ES surgeries w/ IJV decompression. He also works out of UTS, TX. Here’s his contact info. I think he & Dr. Pride work together to some extent:
Dr. Fargen’s ofc is historically slow at getting back to out of state patient requests. Some time ago, one of our members told us he gives priority to patients who live in SC & it can be many months before patient requests from outside the state are considered & followed-up on. You may yet get a call from his office but when is the question?
I will pray that Dr. Pride will be able to diagnose your son & help quickly aid in forward progress for him to have surgery. I am so sorry for your son’s suffering & yours along with him.
Thank you @Isaiah_40_31 ! So appreciate it. I did see his name on the list so I hope we will get a call back from Dr. Pride. I feel good about it since they actually have a clinic for rare diseases. I understand Dr. Fargen as well as most surgeons are very busy but 3 months is a long time to wait to even get a phone call in order to get an appt. My son’s condition has intensified and even his doctor called Dr. Fargen’s office with an urgent request with no response. Hoping our experience will help others to decide if Dr. Fargen will be a fit for them. Thanks again for your kind words and taking the time to respond!
Hoping our experience will help others to decide if Dr. Fargen will be a fit for them. Thanks again for your kind words and taking the time to respond!