Severe Dysautonomia In 21-Month-Old - Please Help

[sadiebean]

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

[sue1234]

Ann, wow! I can't imagine the multi-tasking this requires to take care of your precious baby!

First, with the nurse, I can't imagine 67F is cold enough to cause a "cold" in your nurse.

Second, I have learned that beets are a vasodilator! I used to eat them a couple of times a week, then was reading up on foods that react in the body, and that's when I found out about beets. So, you can see why he might reacting to them.

I feel for your son. Even though my issues seem to be nothing compared to his, I am also a very heat-sensitive POTS person. I have to spend my time in my a/c house, but am able to keep it at 76. Above 79 and I feel like fainting. Even at 75-76, if I get up to cook, within 10 minutes I'm breaking out in a vasodilating heat flush and feeling faint, so have to sit. I cannot wait for winter, so maybe I'll have an easier time with my few upright minutes.

What about Peter Rowe, who is an expert dysautonomia specialist in, I beleive, New York? He treats pediatric cases, but I would assume your son might be very young compared to his usual patients. At least he understands the concept behind his dysautonomia.

[corina]

i am very sorry to hear that things haven't gone better for your baby ann. i can't imagine how it must be to take care of your little one while he's having so many issues. i really hope that things will improve and that your son will have a better quality of life.

thinking of you and sending all my best wishes,

corina

[sadiebean]

Second, I have learned that beets are a vasodilator!

.....

What about Peter Rowe, who is an expert dysautonomia specialist in, I beleive, New York?

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!

[sadiebean]

i really hope that things will improve and that your son will have a better quality of life.

thinking of you and sending all my best wishes

Thank you so much! a

[arizona girl]

My heart goes out to you, this must be so scary and overwhelming. He was born with this malformation and it wasn't a brain injury? Have they given a prognosis or idea of what he will be able to do? Has he had any type of surgery or shunt.? Do they every refer to this as a chiari malformation? I did a quick google on "pediatric hindbrain malformation treatment" and this doctor at Seattle childrens hospital popped up. I know far away, but they might be a resource.

Daniel A. Doherty, MD, PhDDevelopmental Pediatrics

On staff since July 2005

Academic Title: Assistant Professor

Research Center(s): Center for Clinical and Translational Research Center for Integrative Brain Research

"Improving the lives of patients and understanding human brain development are the parallel goals that motivate my work. The excitement of identifying the genetic cause of a brain malformation is just the beginning. Understanding the disease mechanism and translating that knowledge into improved information and treatments for patients is the real payoff."

Closer to boston, there is also chiari institute in long island:

The Chiari Institute is the world's first comprehensive, multidisciplinary center for the management of patients suffering from Chiari malformation, a rare structural condition that affects the cerebellum; syringomyelia, a chronic disease of the spinal cord; and related disorders. Established in 2001 by the North Shore-Long Island Jewish Health System, the Chiari Institute is located in a state-of-the-art facility in Great Neck, N.Y. » Read more about who we are and what we treat.

I'm sure you are a good mom and doing your and doing your best under very difficult circumstances. The last thing you need is a home health nurse that doesn't want to do the job. Contact your insurance company who is providing the service and ask for a change in nurse assignments. You need the extra help. Hold on to your hope. Even in the darkest of times silver linings can emerge. xo

[jekecana]

I have no dr ideas but just wanted to send you [[[[hugs]]]] and support!

[sue1234]

Paul has high blood pressure - wouldn't a vasodilator be helpful...?

Sadie, speaking about my personal issue with hyperPOTS and in theory only, I believe I get high blood pressure standing because I have low blood volume(not confirmed). As I have been prescribed different vasodilators, I do really bad on them. My blood pressure drops to around 75/45. I believe I have high standing blood pressure due to low blood volume, so it "squeezes" to keep what volume I do have up in the upper body to get it to the brain. When I take(or eat) a vasodilator, it lets the blood volume "sink" more lower and my body cannot "fill up" those dilated vessels with the not-enough volume. This is all my personal theory on why I react to vasodilators with my hyperPOTS.

[Sallysblooms]

Paul has high blood pressure - wouldn't a vasodilator be helpful...?

Sadie, speaking about my personal issue with hyperPOTS and in theory only, I believe I get high blood pressure standing because I have low blood volume(not confirmed). As I have been prescribed different vasodilators, I do really bad on them. My blood pressure drops to around 75/45. I believe I have high standing blood pressure due to low blood volume, so it "squeezes" to keep what volume I do have up in the upper body to get it to the brain. When I take(or eat) a vasodilator, it lets the blood volume "sink" more lower and my body cannot "fill up" those dilated vessels with the not-enough volume. This is all my personal theory on why I react to vasodilators with my hyperPOTS.

I have high bp with my POTS. I take Benicar and it is normal with that.

I am so very sorry about your baby!

[Rachel]

Dear Ann,

My heart goes out to you. It is difficult for any of us to live with dysautonomia, but I imagine that it must be so much harder for you to have to watch your little baby suffer from this. I remember you posting last year, and just two weeks ago I was thinking about you and wondering how things were going. I am sorry that there hasn't been improvement.

Dr. Rowe at Johns Hopkins is one of the best specialists for dysautonomia, and he sees only pediactric patients. I don't know how young of patients he takes, but I do know that cares for some dysautonomia patients with very complicated cases. You can find him here: http://www.hopkinschildrens.org/staffDetail.aspx?id=3226

The other place that you might find helpful is the Akron Children’s Hospital, specifically their NeuroDevelopmental Science Center. Dr. Bruce Cohen is there now, and he has a great team of doctors. Dr. Cohen used to be my doctor when he was at the Cleveland Clinic. He is brilliant. My husband tells everyone that Dr. Cohen is the smartest man he knows.

"In his role at Akron Children’s, Dr. Cohen will partner with neurosurgeon Roger Hudgins, MD, and developmental-behavioral pediatrician John Duby, MD, in the clinical leadership of the Akron Children’s Hospital NeuroDevelopmental Science Center. This center brings together a wide range of pediatric specialists, including developmental-behavioral pediatricians, neurologists, neurosurgeons, neuro psychologist, psychologists and physiatrists, to care for children with ADHD, cerebral palsy, Down syndrome, epilepsy, Fragile X, head injuries, muscular dystrophy, neonatal follow-up, and spina bifida."

From: https://www.akronchildrens.org/cms/news/501ba6b5ce1ad963/

I wish there was more I could do to help. You will be in my thoughts and prayers.

Rachel