sadiebean

Thanks very much for the responses. I am very grateful they treated him for sepsis and take no issue with it, especially as he had a fungal cellulitis at his g-tube site the week before which would give an entry point (though again no sign of bacteral infection, 36-hours of antibiotics, all cultures clear). So it could very, very well have been infection. And we'll be continuing the antibiotics at home for a full course. But we've had breaks in the skin and tracheitises and UTIs, and he has handled them better than most I know -- under his obvious congenital issues is a healthy boy, with a strong, proven immune system. And, I have nobody with whom I can discuss how likely shock from dysautonomia vs infection is - nobody. And I looked high and low for a long time. I raised my concerns in the ICU yesterday about not being able to distinguish between sepsis vs. autonomic, and not even a suggestion that there is someone I can speak to (And this is at a major #1 ranked, teaching children's hospital.) I need to understand what *could* happen JUST from autonomic dysfunction, or else everytime his symptoms get a *little* more pronounced, I'm going to be concerned I should take him to the ER (or even obligated as we have homecare nursing here.) Does distributive shock from sepsis and from autonomic failure look the same? Would you have low BP if the cause were autonomic?

Hi, My son, who is 4.5yrs and medically complicated, trach/vent, and has a serious hind brain malformation resulting autonomic dysfunction that is largely under control using the beta blocker atenolol. This past Wednesday night/Thursday morning we had to rush him to the ER, as he was apparently in shock - purple hands and feet, low blood pressure, dialated pupils, unresponsive to us tho responsive to noise and light, fever. He had been doing poorly for the last couple of days, but we'd thought he had a mild respiratory virus. But as Wednesday progress, he got worse and worse until finally his temp got very high and his affect was just not right. The ER got it under control with bolused fluids and started antibiotics, assuming massive sepsis. But so far all the cultures are still negative. But the docs are still convinced he's got an infection because of the severity of his symptoms. So my question is: *** Does anyone know if an autonomic crisis can look like or even be the same as shock? Including dangerously low blood pressure? (systolic of 60-70) *** I am very used to docs assuming infection when they see his autonomic symptoms. So I am trying to trust them that this was bacterial based. But since we have no sign of infection, and since he was much better 12 hours later, it seems to me that massive infection is very unlikely. He had several triggers in the day or two leading up to the event including being very, very tired; constipated; then treated with a prune juice/magnesium to help clear it which agitates his GI system; suspected respiratory virus; and slightly reduced fluid intake. We also gave him his beta blocker as usual on Wednesday, when now I know that he could have had a lower BP already, meaning the meds would have lowered it even more, possibly dangerously. Thanks for any input.

Thank you so much! a

Paul has high blood pressure - wouldn't a vasodilator be helpful...? I don't know much about this. Thank you for teh doctor recommendation - I will follow up with him!

Hi all, I posted over a year ago, and since then we've taken my baby home from ICU and have been trying to manage his cares here. Our son has severe autonomic dysregulation due to a major hindbrain malformation. He is trached and vented b/c of due to related, central apnea in his infancy, something we're working hard to wean him from. He is also g-tube dependent due to severe inability to swallow. His symptoms and triggers are numerous, and though we have an excellent team of doctors mostly at Children's Hospital Boston, we cannot find an effective treatment. He is on propranalol (beta blocker), and rescue meds of Tylenol, Motrin, Morphine and occasionally atavan. He's currently going through a 2-month-long bout of needing all of these meds every day to manage storms. He still requires fridig temperature in his room to avoid storming (fever, tachycardia, etc.) a) This is hard to maintain in the summer even in his own room with a new A/C set at 67 degrees and a fan circulating the air, b ) He has significant drainage of his sinuses (or it seems that way), possibly due to being in these cold temperatures all the time, and c) We can barely even take him out of his room, as in I can count on two hand the number of times in the past two month - I can't get the house cold enough. At all times he wears only a diaper, and often uses a cooling blanket as well. Don't know if there is another medicatino to try?? Clonodine was a disaster. d) Our night nurse wants to leave b/c he has sinus issues taht are aggravated by the cold in Paul's room - this is disasterous, we don't have enough nursing coverage as it is. He also has expiratory apnea when he cries related we believe to dysregulation of his CO2 levels. These are life threatening episodes. Wondering if SSRIs could help to treat these...? Besides heat, he has several suspected triggers for storms - definitely illness of any kind; we also believe skin irritation, full bladder, gastric distress of any kind, (we make his own, easily digestible, real blenderized food, but for example just introduced beets... big mistake...) We also use atrovent as of June, an anticholinergic nebulized through his vent tubing to reduce secretions... but it works by dampering the parasympathetic system, and may be making things worse... Please, please help - is there a specialist you can recommend? A list of meds we shoudl discuss w/our current doctors? Does anyone have experience wiht beta blockers? SSRIs? I don't need to tell you how overwhelming this is. I appreciate any insite at all. Ann

Hi all, I posted some weeks ago re: my 7-month-old infant with auto dysreg. One of his biggest triggers is being overheated. Paul gets a fever VERY easily. Right now, he's still in the hospital. We keep his room to 65-69 degrees during the day, and usually he's naked. He might wear a little outfit, but if he's held with an outfit on, he's almost sure to climb to 100 degrees and need meds (morphine, atavan) to come back down. The other day we went for a hearing screen to a different room, also cold and air conditioned. But not as cold as Paul's room. I held him for a short while, then put him in the bed. He overheated to 100. (And incidentally, he had morphine and atavan in his system before overheating, since we were trying to help him sleep for his test. Didn't do anything to prevent the fever.) Overnight he can tolerate clothes and a warmer room, when his temp dips and no one is holding him. And the hospital is a fairly controlled environment... At home, obviously we'll have a/c and a cold house, but at this point I don't think I could ever bring him out of the house unless the temp is JUST right. Car rides to the doctors? Don't know how that will go. I'm looking in to getting a custom-ordered cooling vest from Cool Sport. He's on labetalol, and that's helping. But are there other meds that any of you take for temp dysregulation? Anyone else have such severe temp instability? Thanks! Ann

Oh, one more thought I had re: auto dysreflexia - Paul has an extensive spinal syrinx, and I wonder if this is coming into play, since dysreflexia is really mostly seen with trauma to the upper vertebrae. So this is one lead we will be following up in with Boston.

Paul is doing well, thanks! He gets overheated on pretty much a daily basis, but it and the other associated symptoms are more manageable since labetalol. He's medically complex, trached and on the vent due to central apnea and lung disease of prematurity, and he has a g-tube. He was intubated at birth, and tho his breathing has improved he still desats off the vent. All related to his severe Chiari. It's been so, so scary, to the point of despair before the labetalol. His HR has gone as high as 245bpm, etc etc. Somehow I have much more faith that we'll be able to manage at home, though I don't think too much on the details. We'll save a lot in heat I guess! We also have a 5 year old with spina bifida, though with none of these lifethreatening and rare complications. Paul is very, very rare. We've never heard of another baby with these symptoms, have yet to find another family dealing with this. Even his neurosurgeon in Boston, which is ranked #1 in both pedi neurosurgery and pedi neurology, had never before treated a child who was born not breathing and who experienced these symptoms. Truthfully, I don't know who will follow Paul once we're discharged, since no one now is even officially treating this. I've followed up with Dr. Blair's office again, and will be getting back in touch with Boston (again, we transfered to CT to be closer to home when Paul was 2.5 months old) to see what they recommend. As for coming home, Paul needs to be 8kilos to try the at-home vent, and is 6.6 kilos and gaining very steadily (as in, 1/2 kilo a week these days!) so hopefully sooner than later. But it's been a long haul. I'm so glad I found this message board though. It's been our first lifeline on this subject after much searching. Ann

Wow, thank you all for your support and information. @Mack's Mom - Thank you for this fact sheet! I've printed it from the Nat'l Assoc site and it's now in Paul's bedside chart. In fact, Paul was storming yesterday and I just had a hunch it was somehow related to diaper rash (concurrent w/onset of symptoms) and lo and behold, skin irritation ranks w/constipation and bladder issues! Paul has spina bifida so he DOES have a spinal injury, it just seems to relatively mild (he moves his toes and feet) but with everything else, now I'm sure it's involved. Also, he has a large syrinx. I'm wondering if we need to treat that aggressively. His chiari was decompressed in January, a risky surgery in hopes of relieving the apnea. It helped, but not completely. (my computer's battery will run out soon, may not be able to respond to all posts before then...) @firewatcher - the Familial Ctr at NYU flat out said no, because Paul doesn't have familial dysauto. I'll go knocking thre again if I need to though. @mighty mouse - yes yes please I would love to finally be in touch wiht another parent going through this. Thank you! @dsdmom and others - will check out Mayo, Cleveland, JHopkins & UPenn - thank you!! @spaceorca- Paul was at Boston Childrens from birth till 2.5 months before coming to Hartford. They were preoccupied with 7 surgeries, prematurity and major apnea, so I hope that if we turn to them they'll delve further into the dysauto/dysreflexia than they did. We may well transfer Paul back there, but it's 1.5 hrs from home and we have 2 and 5 year old boys. must go before comp crashes. THANK YOU!!!!!!!!!!! Ann

Hi all, The short version of our story is that our 6 month old baby has significant autonomic dysregulation, secondary to a severe Arnold Chiari II defect of the hind brain. He's been on labetalol for a month or so now, and it's helped a bunch. He's been in intensive care since he was born, as he has central apnea and is on a vent. (We are waiting for him to be big enough to come home on the at-home vent.) We have not been able to rely on the team of doctors at the hospital we're at for much of anything; the labetalol came after many many questions and much prodding on our part. So we're pretty much on our own. His symptoms have improved, but he still storms. And we really just want to know the right thing to do next. I've emailed Dr. Blair Grubb but haven't heard back. Any suggestions? Btw - the baby's symptoms while storming are: tachycardia, bronchospasms, fevers, tremors, inability to track, listlessness, hypertension. He seems to be triggered by a full bladder, constipation, overheating (his room is often freezing cold, never above 68 degrees, and he's usually naked but for his diaper). But this has been all our own legwork - surely we could be missing something? Thank you for any input!! Ann