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03/13/2012 04:32 PM

Hi! (new to the group)(page 2)

grandmabrk
Posts: 303
Member

Amy, be sure to follow up on the anesthesia report prior to appointments for any surgery. It isn't just the difficulty in inserting the tube. Table positioning of the patient can cause problems too. The anesthesiologist that did William's adenoid/tonsillectomy had a hands on appointment with him a couple of days before the surgery to go over X-rays of his neck and place him in different positions on a table with with foam positioners; so his neck would not become overextended while asleep if repositioning was needed.

Post edited by: grandmabrk, at: 03/13/2012 07:00 PM

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03/13/2012 04:44 PM
treblig66
treblig66Posts: 461
Member

Just a quick note, Amy. Make sure that whoever does the surgery knows about the KFS. They sometimes have to make alternate sedation arrangements for people with KFS, and intubation is often a definite no-no! I've joked about having "I have KFS" tattooed on my neck so if I'm ever in an accident they won't be messing with me and risking paralysis, but my wife said that to be on my neck, the words would have to be so small that the doctors wouldn't be able to read them.

--Gilbert

Oh, I see grandma just beat me to this post. At least you can see great minds think alike! But what's our excuse?

Post edited by: treblig66, at: 03/13/2012 04:46 PM


03/13/2012 07:03 PM
grandmabrk
Posts: 303
Member

LOL! Gilbert, I bought William a Medical alert ID bracelet when he was younger; but like you, I have thought about a tattoo.

03/14/2012 06:41 PM
amjolley
amjolley  
Posts: 73
Member

My Ped suggested not long after he was diagnosed to make a card listing his conditions and laminate it. So, if we were ever in an accident or such the doctors would know that his bones were already unusual before anything happened, and his eyes, etc. Scary as hell to think about but of course I want the best care for him no matter what.

The last time Thomas was put to sleep we went in before and discussed it and they had to get clearance from our Orthopedic Surgeon about how to incubate. When it came down to it though, the Dr said we had to do fiber optic and the anesthesiologists said that would be too difficult and that no matter what he would make sure Thomas was comfortable and the he was not endanger. Its awfully scary, but we all made it through. Hoping for no more surgeries for a while!


03/30/2012 07:18 PM
bajameson
Posts: 24
New Member

Lisa - just an update on Dylan's swallow study that was this afternoon. I shouldn't rag on our children's hospital anymore as he was able to get in so quickly for this test! He did really well during the entire study but they noticed when he was swallowing the thin liquids that some of it is actually going down the wrong tube so he's possibly aspirating those liquids every time he's gagging. They sent him home with a ton of thickener samples and instructions for us to thicken everything thin liquid he drinks. Also, they are sending notes onto the ENT we meet with in 3 weeks. Apparently, we'll be discussing everything that went on today in more detail with her when we go over his adenoids/tonsils. My husband had to take him today as I'm expecting baby #4 and couldn't be in the room with him due to the radiation. So I'm missing what I feel is like 100 more details/answers to questions but hopefully the ENT will shed some more light on the whole thing. Not totally looking forward to having to thicken all his liquids. . . at 2.5 yrs. old I'm not too sure how he'll take to it either. We shall see!

Take care,

Amy


03/30/2012 07:48 PM
bajameson
Posts: 24
New Member

I wanted to also thank you all for sharing your experiences on the anesthesia issue. I know it's something we will have to deal with in the future so it's good to have more information.

Dylan's had 2 surgeries (both by 4 mths. of age) and had no problems with the intubation. Well, at least that we know of. In between the 2 surgeries was his full-spinal MRI that required sedation but he had several vagal responses during the MRI and they ended up having to intubate him for the remainder of the MRI. He apparently was okay at that point with the tube then. I was mostly concerned about why he needed it but they didn't give a great explanation as to why he was having the vagal responses in the first place. I've never been at peace with how the children's hospital handles our questions however. There's never been a feeling like they've given us ALL the information.

At age 10 mths. and just this past Dec. he had tubes put in his ears with the Dec. surgery being the time he was supposed to have his adenoids out as well. These took place at a different local hospital not specific to children. This past Dec. was when he had the intubation issues. The anesthiologist at that time took an immense amount of time explaining to us what happened and why he thought it happened. . . saying something to the effect that when really young children have had multiple intubations that their airway can react by not growing as they should. Almost as if it just shuts down from all the irritation to the airway. That didn't sit "right" with me mainly because I had never heard of that before (but I'm no expert so what do I know?!) but also because I do know that Dylan's neck is created in a much different way than the typical neck. So it's bound to need to be taken care of differently. BUT at least he was willing to sit and talk with us in depth about his opinion on what happened.

With each situation where he's been sedated (including other MRIs and a CT scan) we discussed everything with anesthesia prior to the surgery/procedure but no one has ever expressed too great of concern for his fusions, etc. Perhaps I should show greater concern myself from here on out? Actually, I know I will with what happened to him in December. I think it all really just boils down to the same recurring theme. . . no one truly knowing or understanding the effects of KFS. I'm just so thankful that we know the Great Physician and place Dylan in His hands daily. He created him and knows him better than we or any doctor could ever begin to, so He is where we place our trust.

We're going back to the children's hospital to see a new ENT with hopes that she'll have a better explanation of everything going on with his whole lower head/neck region. Hoping too to get more information out of them about intubation versus alternative sedations for when he does get his adenoids out. If we get any ground-breaking news (ha!) I'll be sure to pass it along!

Thanks again,

Amy


03/30/2012 08:19 PM
lisaraman
 
Posts: 795
Member

Amy- thanks for the update. Wow- aspiration, huh? Other than gagging, does Dylan have any symptoms of an underlying asthma that might indicate chronic aspiration, like chronic cough? Does he get pneumonia or bronchitis frequently? so glad you found that out. The thickener will hopefully only be a temporary thing while he's little. Maybe he'll outgrow or the ENT will sort out why this is happening. I hope it's not neurological. Does he have a cervical scoliosis? Sorry for so many questions. Just concerned....it's the nurse and the mother in me trying to help sort it out. Smile So glad that Children's came through for you-at a time when you really need them to be responsive. a point for them

BTW- the vagal response......very interesting. My daughter as you know, is an adult, but she has had 2 episodes like that. The first was when she was at Cleveland Clinic getting the ano-rectal balloon to rule out Hirschprung's- and to have uro-dynamicsd one. When the nurse blew up the balloon , Andi went pale, BP crashed and she almost did too. Was very scary. Her physiatrist made light of it, but it happened again when she had her ears irrigated. Never let a doctor or nurse shove a syringe full of saline into Dylan's ear or rectum unless they know he has high risk for this, because andi passed out on both occasions. I'm not sure what the connection is, but I think it;s problems with the inner ear that develop with KFS. One more mystery of the deep for us to ponder .....

And finally, congratulations on the pregnancy!! So wonderful. YOu have your hands full. Andi was my second of 4, and like Dylan, she was sick from day one. Makes for a busy mom ....I remember Smile

Best, Lisa


04/01/2012 12:29 PM
bajameson
Posts: 24
New Member

Thanks for the questions, Lisa! I appreciate any help in figuring it all out so ask away! Smile

He actually does have underlying asthma/bronchial issues. Although he has never had pneumonia. It's more that when a colds hits him it seems to go directly to his lungs (versus sinuses like his twin sister - our oldest daughter is similar to Dylan but is not near as bad). He really didn't have a problem with it though until Feb. of last year when he tested positive for RSV (with bronchiolitis). After that, every little cold he came down with turned into major breathing issues. Since he was just over a year old when diagnosed with the RSV no one wanted to officially diagnosis him with asthma/RAD but every time we had to take him in (which we did only when it was he was really having troubles) the nurses/doctors all said, "Oh, he'll probably end up with at least the RAD diagnosis." Our pediatrician has always been on top of it, watching him carefully. And we have a good plan in place to help try to keep any major problems from coming on. He's been A LOT better this winter but he also didn't come down with as many colds, etc. either. The SLP and nurse who performed the swallow study asked about his chronic breathing/coughing too. So I do feel we're on the right track. There is always a next piece of the puzzle to figure out for sure.

And he does have cervical scoliosis too. A 24-degree curve causing a headtilt to the left at his c-2/c-3 fusion/malformation.

Andi's experiences with the vagal responses are good to know too. So glad she was okay after her episodes. Thanks for sharing!

And thanks for the congrats on the new baby. We're excited! Know we'll be busy but are truly blessed. We're just glad to know it's another boy so Dylan can have someone besides his sisters to tackle/wrestle with. . . I think they're getting tired of it! Smile

Thanks again,

Amy


04/01/2012 12:54 PM
lisaraman
 
Posts: 795
Member

Hi Amy- good to know that you are on top of the aspiration issues. You understand the signs and you are correct in assuming that the viruses settling in his chest is not a good long term sign, so it's good that you are on top of it. The cervical scoliosis as you can imagine has eVERYTHING to do with it...although doctors won't agree. Andi has that, and everything has always settled into her chest. She also has cervical dysphagia, which increases the mild, but chronic aspiration. Like you , we didn't know it was happening til she had her swallow study and it was revealed that she had a pill sitting in her cervical esophagus rt neuromuscular weakness and cervical scoliosis. Dylan will probably be diagnosed with torticollis (cervical dystonia) and neck spasms constrict the swallowing ability too. Of course, that means other things get caught there besides pills, and miniscule particles of food, pills and fluid end up in her airway, esp when she is sleeping. This is esp a risk for babies. Andi's head tilt is also to the left. Watch his left ear for increased fluid and infections too. Sorry to dump all this on you, but it is the way of gravity. So happy that Dylan will have a brother to play with. Good luck with all. Lisa

04/24/2012 08:10 PM
bajameson
Posts: 24
New Member

Hi Lisa,

Thanks again for all the information. We had a good appt. yesterday with the ENT. She is mostly concerned with his airway issues when being intubated. She scoped him with a little camera to see his adenoids and vocal cords. Said adenoids didn't look to be a problem but that his tonsils were bigger than she'd like to see. However, nothing warranted them being taken out at this point especially with all of his airway issues. She does want a sleep study though because his sleep is so disrupted and I have found him in the middle of not breathing while sleeping (having to jar him to start back up). If that study suggests to have the adenoids/tonsils out then she'll do it. But the promising thing is that she's realized his airway/intubation issues and will only proceed with a surgery if we've had a full-scale anesthesia meeting prior to surgery.

Dylan's been on the thickener for a little while now and he's doing okay. Coughing less, but not drinking as healthy as he should since the only thing he'll drink with it is flavored water or juice. He will not touch plain water or milk. . . go fiure! To get him to take the juice with the thickener we've had to thin with a couple more ounces than they originally wanted us to do. He'll just need monthly OT follow-up at this point. Some of his issue is motility so I'm thinking he'll be on the thickener for longer than we had hoped. We'll see. Although the ENT was concerned about Chiari Malf. as well. I know his full spinal MRI at age 3 mths. was supposed to look for it but we never heard either way for sure which is why she's concerned. I assumed (which once again I'm learning I shouldn't) that since they never said he did have it that he was fine. But the ENT is going to make radiology look at it again to get an affirmation one way or the other if they have enough iamges to tell. She said that may be the problem. . .if they don't have enough images to tell her now we all know that means another MRI to check for it.

Read about Andi's car accident. . . we'll be praying. That could not have been fun in any way, shape or form for anyone.

Amy

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