Frequently Asked Questions
We have taken questions most frequently asked by parents about OMS from
email queries and responses. Here is a directory to the topics of conversation.
Click on the topic of interest.
· Behavior problems
· Giving Medications
and Educational testing
· Reactions to medications
· "Steroid sparers"
· "Travel Tips"
Q: "What is the difference between ACTH and steroids--concerning
the positive effects on the OMS--concerning the negative side effects
of such a treatment? Our doctors are very skeptical of ACTH treatment,
so they hesitate making a step in this direction."
Comparing the positive effects of ACTH and steroids, ACTH has a higher
likelihood of inducing a neurological remission. Both treatments have
similar side effects.
Q: "When a child responds relatively well to intermittent steroids
and IVIG, do you think it sufficient to go on with this kind of treatment,
or would you always advise to give a high dose treatment of ACTH?"
Corticotropin (ACTH) is the gold standard of care in OMS. In our experience,
using a high-dose protocol we developed, it is greatly superior to steroids,
and can usually induce a neurological remission. Available as ACTHAR
gel 80 IU/cc, it is given by intramuscular injection twice daily for
one week, then daily for two weeks, then on alternate days thereafter.
Intravenous immunoglobulins (IVIG) can be given during the course of
ACTH treatments, and many patients will derive synergistic benefit.
Q: "My son had one adrenal gland removed due to neuroblastoma
and he now takes ACTH. I read that the adrenal glands produces a steroid
that goes to the pituitary gland, then the pituitary gland produces ACTH,
which then cycles back to the adrenal gland and starts the cycle all over
the balance the body. Have you found any abnormalities of the adrenal
gland function in children like him?"
Children with one healthy adrenal gland can do quite well. The doses
of ACTH used to treat OMS exceed those produced by the body, so there
is plenty of hormone while on therapy. Once ACTH is discontinued, it
may be necessary to give a supplemental steroid called hydrocortisone
for a while, until the adrenal gland resumes making steroid on its own.
This requirement is usually temporary.
Q: "Sometimes my daughter will be seriously mean to her little
brother. Several times I've caught her hitting him in the head and laughing
as he's crying, running over his legs, feet, or hands with a big wheel
and almost enjoying it while he cries. It's unnerving because she'll be
smiling all the while with this terrible evil smile. I can understand
that she's jealous of him, but how she goes about expressing it is what
makes me uncomfortable. Have you experienced this type of behavior with
Yes. It is typical of children with OMS who have behavioral problems.
The aggression is directed at family members, particularly siblings.
It's important to treat it by modifying the behavior through setting
limits and expectations.
Q: "What is the effect of chemotherapy on the OMS? The papers
we found are very contradictory on this subject. Is it the removal of
the antigen, or is it mostly the immunosuppressive effect of the chemotherapy
The role of chemotherapy in the treatment of OMS has not been adequately
studied. I have taken care of children with paraneoplastic OMS who did
well or poorly despite cancer chemotherapy with cytoxan, adriamycin,
or other agents. I am aware of a child at another facility who was treated
so aggressively with chemotherapy as to require a bone marrow transplant.
So I think one has to be judicious.
Q: "Have you any experiences that the use of chemotherapy correlates
with improved neurological prognosis?"
The current controversy is whether a child such as yours with fully
resected neuroblastoma should also receive chemotherapy, not for tumor
control, but to destroy the lymphocytes that carry on the autoimmune
response. There is a protocol in this country to use cyclophosphamide
for that purpose for all children with neuroblastoma in combination
with IVIG plus steroids. I can't advise you yet about that without knowing
how well she can be made to respond to other measures.
Q: "My child doesn't take oral meds very well. He usually spits
them out, or if he is forced, his gag reflex kicks in and it gets ugly.
For many medicines, you can mix them in a small portion of applesauce-small
enough you know that the child will take it all. Most pharmacies now
offer flavors that can be used to cover the taste. Check with your pharmacy.
Q: "I am scared that my child will get Chickenpox. It was suggested
to us that she could have the vaccine after being off of IVIG for 8 months.
We were told that if we know she has been in contact with chicken pox
to bring her in to get the vaccine because it would be better than a full-blown
case. I know that many parents choose not to continue with immunizations
of any kind. What is your advice on this matter?"
We advise giving VZIG when there is a known exposure to chickenpox
because chickenpox can cause severe cerebellar ataxia in some childen.
Q: "My daughter is in pre-school until August and then starts
Kindergarten in September. Her school is requesting that she is immunized.
How do I make that decision or do you just mandate that OMS children shouldn't
It's always a tough call. We make an individual recommendation based
on assessment of the risk of relapse and the risk of serious illness.
Some of the questions for that assessment are:
1) How many times has the child relapsed previously?
2) How many serious infections has the child had?
3) Does he/she relapse consistently with illnesses?
4) Are the symptoms of OMS currently controlled?
5) Is he/she immunosuppressed?
No matter how scientific you are about it, it's still a roll of the
no live virus vaccines should ever be given and multiple vaccines should
not be given at once or in close proximity. Some parents choose to test
the water by giving one that's less likely to cause a problem, like
HIB, and see how it's tolerated.
Q: "We are going to a wedding and planning to stay with my in-laws.
My brother-in-law just called and said he has shingles that is being treated
with Valtrex. We are all concerned about our son. Is this something we
should keep him isolated from or is it of no concern?"
If your brother-in-law is otherwise a healthy individual (not immunosuppressed),
he will not shed virus from his mucosa (nose, throat, etc.). So if your
son does not have any physical contact with him, things should be ok.
If your brother-in-law's zoster lesions are in an area covered by clothing,
so much the better. Here's the important point. If your boy has never
had chicken pox before and is getting chemotherapy he is at risk. If
it is within 96 hours of exposure, he should get VZIG (a specific type
of IVIG). In any event, the incubation time is about 10 days. You should
check him for lesions at that time. If he contracts the illness, he
may need to be treated with Acyclovir. Again, this only applies if he
has NOT had chicken pox.
Q: "My child's case was said to be viral though no specific virus
was ever named. She had bronchiolitis 4 months prior to OMS. Do you think
there might be a connection?"
The virus that have so far been implicated in OMS are neurotropic
viruses, meaning that they can access the brain and cause encephalitis
or meningitis. Although almost every child in the U.S. gets bronchiolitis,
it is not neurotropic.
Q: "How much IVIG is enough? When we have tried to delay treatment
for 1-2 weeks (i.e., go 6-7 weeks between IVIG infusions instead of 4-6),
her balance deteriorates, her behavior goes to pot, and we see intermittent
Clinically it seems that she needs the IVIG. I don't know of any IVIG-dependency
states--I mean that the IVIG itself causes further need for IVIG. More
time may be required before weaning can be attempted.
Q: "Our daughter was started on IVIG treatment last week (she
is still taking oral steroids). She was given the treatment over three
days. However, over the weekend her symptoms appear to have got a little
worse to us - especially the ataxia. Our neurologist here said that the
symptoms should not get worse; the IVIG should either help or have no
impact. Can you give us any indication from your experiences as to how
long it generally takes for the IVIG to take affect (I understand that
it varies greatly with different cases)?"
Typically, benefit from IVIG is seen within the first month, even a
few weeks. However, 2-3 infusions (one a month) sometimes are required.
There may be some transient, mild worsening after the first infusion-usually
not. It isn't a reason to discontinue the IVIG.
Q: "We attribute much of my son's improvement to the use of the
IVIG at regular intervals, protecting him from potential viruses that
cause regression. All of a sudden, after more than six years of paying
for the treatment, his primary insurance company is denying payment stating
it is 'experimental' and not supported by 'peer medical literature.' What
can we do?"
IVIG is not experimental. It is supported by the literature, although
the number of articles out is admittedly small. Look on our website
under "insurance information" and you'll find a letter of
medical necessity for IVIG with references.
Q: "Is complete tumor removal one of the important tenets of
OMS treatment? Do you also advise to give chemotherapy if complete surgical
removal was not possible?"
Most neuroblastomas associated with OMS are not aggressive, probably
due to both host and tumor factors. I imagine that some day we will
probably not be removing them. However, the current recommendation is
to remove the tumor in the hope of reducing the antigen "burden"
on the immune system. Some children improve after surgery; others do
not. Tumor removal is seldom an indication to desist in efforts to redirect
the immune response. The reason for this is probably memory function
of certain T lymphocytes.
Q: "Our doctor will not check for neuroblastoma again unless
my daughter relapses. She is 4 years out and has never relapsed. Would
this be your plan, too?"
I think that is very reasonable.
Psychological and Educational testing
Q: "I have a question about arranging the neuropsychological
recommended for children with OMS. I contacted a local psychologist, who
didn't quite know if this was the place I truly needed for testing. How
I find out who is the best person in my area?"
Families who are having trouble finding psychologists in their area
to do neuropsychological testing can check with the APA (American Psychological
Association). The APA may even denote who specializes in neuropsychology.
For children under three, the Early Intervention (Zero to Three) system
has developmental specialists who perform basic developmental testing.
This is nice because it is free of charge to the parents if the child
has disabilities, and OMS would certainly qualify.
Q: "Is there a danger that one can miss the right moment to prevent
long term damage?"
The exact window of opportunity for treating OMS is unknown. We do
know that early and adequate treatment affords the best chance. We aim
for full recovery within the first year.
Q: "I know that this is basically a "wait and see" game.
Given the information and the fact that my daughter hasn't relapsed since
diagnosis several years ago, what do you think the chances are that she
may never relapse?"
The chances are good. There are no guarantees. In the extreme, some
patients can relapse ten years later, but we don't think this is common.
Q: "In your experience, how quickly should we expect a complete
resolution of the OMS symptoms?"
Resolution of symptoms comes in stages. Response of myoclonus and opsoclonus
to high-dose ACTH takes place within days after starting. The improvement
builds until it plateaus. Response to IVIG may take a little longer-a
week or two-but follows the same general guidelines. Speech improvement
takes longer because there are brain developmental issues at play.
A common mistake is to wait months to see a substantial change. In
our experience, that indicates a non-responder.
Reactions to Medications
Q: "Our daughter was given Morphine, Benadryl, and Klonapin.
She reacted to both Morphine and Benadryl. She had a seizure that lasted
40 minutes after being taken off Klonapin. We want to know if this could
have triggered the seizure?"
Seizures are uncommon, but have been reported in children with OMS,
so we don't know if the threshold for seizures already was lowered in
your daughter due to the OMS. On the other hand, many children with
OMS have side effects from drugs like Morphine and Benadryl. We recommend
that they not be given these drugs. The small dose that some kids require
as a pretreatment for IVIG is probably okay."
Q: "My child is relapsing. He had a stage I neuroblastoma removed
and is still being treated with ACTH, IVIG, and cytoxan. On high dose
ACTH, he was 100% himself. After about 6 weeks, his doctor was so pleased
that he started the weaning process from ACTH. Then the symptoms came
back. Now he can't stand alone and is having the same eye jerking as he
had before. What can we do?"
The ACTH weaning was too rapid. The dose needs to be raised. If you
go back to the initial high dose, I would suggest using alternate day
dosing because you otherwise will see more steroid-like side effects.
It may take longer to see a response than before. Continue with the
other treatments as well.
Q: "We found a very remarkable statement on the Internet: 'Unfortunately
the majority of children are left with some degree of learning and/or
behavioral disability. There is no evidence that the dose or choice of
drugs influence this disappointing outcome in respect of learning or behavior
and the reason for this is the subject of significant research.' Do you
I disagree with the statement because I think it sends the wrong message.
It's based on old treatments and bad data. In severe cases, it's true
that learning and behavioral problems are frequent, but I think there
are some sad and changeable reasons why this is so.
Parents of children with OMS realize how difficult it is to get early
recognition and treatment for OMS. It's almost impossible to have it
instituted within hours or even days of the onset of the symptoms. So
no one has been able to collect data on what I would call early treatment.
As to the dose or choice of agents, there is very little published
data on multimodal therapy. Ours is in the works, and others are analyzing
their's also. So multimodal therapy (IVIG, ACTH or steroids, cancer
chemotherapy or anti-lymphocyte activation agents, plasmapheresis) was
not previously used. We now know that single agent therapy is insufficient
except in the mildest of cases. The old data is based principally on
children treated with single agents. One the whole, many physicians
are reluctant to use ACTH and when they do, they use it at low doses.
So I'm worried that that kind of pessimistic statement will cause a
relaxed view of treatment and many children with OMS will have a sub-optimal
outcome. I have no doubt that better treatments will come, but we will
need to institute them sooner, too.
Q: "Our daughter has really had a bad go with her OMS, all this
brought on by an influenza virus that struck the whole family about 2
weeks ago. Her cold symptoms are gone but now her eyes are dancing in
her head, she has trembling, not wanting to walk sometimes, and more frequent
meltdowns with increased severity. She had IVIG 3 weeks ago. Do you have
Take her for an IVIG infusion now. I know this is earlier than the usual
once monthly dosing, but you can do this as long as it's been at least
2 weeks after the last one. We don't do this for just any infection,
but ones associated with relapse.
Q: "A question regarding relapses. My son is doing great, but
if he should relapse, what would we see? Would some of his symptoms start
slowly coming back, would he crash so to say and look like he did at his
worst point, would the eyes just start shaking...what exactly is a telltale
sign of a possible relapse?"
Relapse is the return of any symptoms of OMS. How quickly it happens
and how severe it gets is very individual matter. The worst relapses
are usually seen in kids with serious infections, especially with fever.
However, children prone to relapse may do so with almost every viral
Q: "Do you have any advice on what we should do about school for
our son? His tutor is concerned that he is going to need a little extra
attention and wanted us to look into schools that have 12 to 15 students.
She says he is doing great, but she can tell when it is time for a treatment.
He has a hard time staying focused unless it is something he is interested
in. She is concerned about a traditional classroom setting working off
a blackboard. She is also concerned about his fine motor skills. First
grade will be a lot of writing and she is concerned about his self-esteem
because he told her the kids were mean to him at his old school. In addition
he needs more time to complete tasks and his writing is not always legible.
I am concerned about his stamina and going to school all day. He still
gets tired if he does too much each day. Do you have any suggestions?"
The specifics from neuropsychological testing will be your best guide.
But in general, I think your tutor's recommendations are sound. The
guidelines are similar to those for attention deficit disorder, with
which the schools are more familiar, and you might make that analogy.
In addition, your son should be allowed to use the computer when possible,
rather than having evaluations dependent on handwriting. Regarding the
fatigue, he may need a shortened school day with home tutoring to cover
the rest of the day's material. I recommend counseling for children
old enough to perceive their deficits to avoid frustration, anger, and
Q: "If we opt to continue with the steroids, would you say that
Dexamethasone, Prednisone, or Prednisolone is a better choice?"
If steroids are to be used, we recommend intermittent pulse doses rather
than daily dosing. Dexamethasone given for a week at the beginning of
each month may give more benefit than other steroids. What you want
to avoid is using steroids every day, as that greatly increases side
Q: "Regarding the 'steroid sparers,' I am under the impression
that they are different than ACTH and that help in the weaning process.
How do the sparers help and what are they?"
It is also important to begin a "steroid saver" about one
month after starting ACTH to maintain the neurological remission obtained
by the ACTH. Despite a slow 20-week taper of ACTH or steroids, some
patients will relapse unless they are also taking a steroid sparer.
Azathioprine can be used at 3-5 mg/kg/day. Other steroid sparers are
available, too. The use of these agents does not cause tumor regrowth.
Q: "If you keep trying to wean from ACTH and you have problems,
does that mean the harmful cells are cloning over and over again and don't
ever die off?"
During the first year after the appearance of OMS, we assume that's
the case, and give additional immunotherapy. The studies on lymphocytes
we do help look into that problem. I wouldn't say that they will never
die off, especially as our treatments improve.
Q: "We're planning to go on vacation, but my child is several
medications for OMS. What special precautions should we take?"
There are no absolute restrictions. We do have a few suggestions to
consider while traveling:
Print off our website and tuck away in a purse the "List of Medications
To Avoid," should your child become ill and need to be seen in
If your child does come down with a fever, take along some children's
Tylenol, give dose as directed for child's age to keep the fever down,
easy diet, and push the fluids.
Best to keep away from people who are actively ill with a fever, head
cold, or flu symptoms.
At family get-togethers, avoid passing around your OMS child too much
the required hugs/kisses! ACTH and chemotherapy suppress
the immune system and leave the child more vulnerable to infection.
know who is harboring a summer virus.
Bottom line: just use common sense. Have a great vacation and enjoy
yourselves; you all deserve it! :-)