Many parents have contributed to the "Parents Corner." They expressed the hope that what they went through might help other families. Many of these are clippings from emails. Last names have been removed to protect confidentiality. You will see that responses to treatment varied.

Click on desired links:

General Advice: What other parents should know
Response to ACTH
Tapering ACTH
Response to IVIG
Residual Neuroblastoma Despite Surgery
Relapse
Plasmapheresis
Speech Therapy Issues
Stem Cell Transplant
Reflections
Letter from OMS Parent

I. General Advice: What Other Parents Should Know

(by Kim and Dave, parents of 18-month-old Eric, who was diagnosed with opsoclonus-myoclonus 11/30/00)

I.

• Apply for a Department of Motor Vehicles Handicap Tag for your vehicle. The form is available from the state DMV office. A doctor would have to sign it and indicate whether it would be a temporary or permanent handicap. A state ID is required for the "handicapped" person, usually available at a DMV office. Our son could not walk, and having the restraints of the stroller belt around him made it almost impossible to put him in the stroller. We therefore had to carry him in to all the doctor offices', labs, stores, etc. Physically this is very draining to a parent. Out of all the people and agencies we saw no one ever suggested or mentioned this. The handicap tag has really helped us out A LOT! (my grandfather suggested this to us).
• Find the Early Intervention Program in your area. They do developmental testing, will give advice on who to see next and where to go (hearing tests, eye doctors, therapists). They will recommend therapies (speech, physical and occupational) based on their evaluations. If your child is under 3 years old and delayed more than 25% they qualify for "Part C" funding. This is a federal government program that is not based on income. Its purpose is to help children under 3 years of age get the therapies that they need. If you have major medical insurance, this funding will pay for therapies or co-payments that the insurance companies won't.
• Let people know about OMS. We have 2 older children in school and talked to the principal, teachers, counselors, and friends and parents of our other kids. We explain to every doctor, lab technician, hearing specialist, eye doctor, pharmacist, boss, co-worker and just about anybody our son comes in contact with what OMS is and can do to the child. People have been very interested and are much more willing to help out; they have been more lenient with appointment times and missed school and work.
• Keep in CLOSE contact with ANY medical people you and your child deal with. At times our family felt as if we where being a real pain in the ---. We soon learned however that communication about everything was very important and necessary to ensure the best care for our son.
• Apply for Social Security Disability and/or Medicaid. These both go by your income level. The process can be very daunting and tiring. I spent a lot of time on the phone, with appointments, and gathering paperwork.
• Prescription drugs may be free. This can be based on income. Contact the manufacturer of the drug; this may take a few phone calls and a lot of time to get to the right department or person. I found most people were not aware the company they worked for did this. You can also contact the National Conference of State Legislatures web site at www.ncsl.org to see what the requirements are for your state (income, age, disability, etc.).
• Keep a copy of all correspondence, receipts, articles and any other information you may receive. Our son has had OMS for approx. 9 months and lot of information we received in the beginning did not pertain or mean much to us. Since then we have referred back to copies of things so many times. Keep everything organized and in one spot. We have separate file folders for evaluations, therapies, drugs, medical receipts, to name a few. When we need to check anything it's easier to get the folder than to dig through a huge pile (because you will accumulate a huge pile of papers).
• ASK QUESTIONS. This may sound the simplest, but it is the most important thing you can do when you have a family member with "special needs". "Why is the lab taking blood? What are they checking for? What is in this medicine? What did my child do at therapy? Or anything that pertains to your child. By asking questions you and your family will learn more, have a better understanding of everything, not be as frustrated, can inform others about OMS, and most of all will be better able to care for your child and his family.
• When someone tells you it can't be done, remember there are always options! Thinking positive and knowing there is another option helped us to keep going and fight for things we needed for our son.
• Support of family, friends, and support groups. We thought we were the only ones who could give our son the best care. As a result we would not let family baby-sit so we could have much needed time for ourselves. If your family is well informed of OMS, they can handle your child almost as well as you can (at least for a few hours). Support groups on OMS are rare, but you can get involved in general groups on disabilities, neurological problems or even United Cerebral Palsy Assoc.
• Keep a journal of different behavior or sleep patterns, etc. This makes it easier to give accurate information and dates to doctors. It also makes it easier for you as a parent to recall specific things.
• Things that have a calming effect on most people may have the opposite effect on OMS people. We found this out the hard way with anesthesia, sleeping medication, Benadryl, and even over the counter medicine. Know what affects your child adversely and learn about all medication they will take.
• Don't take the behavior of your child with OMS personally. When our son is feeling good he loves being with Dad, but when he is not feeling good inside or having "night fits" as we call them, he only wants mom. We have also experienced hitting, punching and biting. These usually occur when he is not feeling well and can't communicate or control his feelings.

II. Response to ACTH

Alex

"Good news!! Alex is steadily showing signs of improvement. As early as day 2 of the ACTH, he was on his tractor, moving forward and backward across the floor. Previously, I had to manipulate his legs to make him do that. Needless to say, I cried. It was a pinnacle for him. He is walking unassisted. His drooling stopped right away. He has slept for 4 nights without even waking up!!!!!! He even goes to bed willingly and doesn't even act like he needs his bottle anymore (sometimes we would go through 3 bottles a night getting him to sleep or soothing him when he awoke). He has not had a temper tantrum and is becoming less clingy towards Chuck and is more loving/cuddly towards me. He is starting to babble more and is really trying hard to say words...he even repeats them almost like he is trying to practice. He is becoming interested in toys that require "walking" to operate. Yesterday, day 5 was really a good day!!! I can hardly wait to see what each day will bring now. The child I am beginning to see again is the happy, talking, playful, inquisitive child that I knew 4 months ago. Chuck and I said the last four months feel like a year!!! To get to this point was a long journey, but we have a whole new appreciation for our faith, marriage, beautiful baby, medicine, science, and doctors!!!!"

Cori

"Cori is a little over 4 1/2. I brought her to the hospital on July 8th and she was
diagnosed with OMS a few days later. She had an MRI, chest x-rays, CAT scan, MIBG scan, spinal tap, a bunch of blood tests, the VMA & the urine test. Everything came out negative or inconclusive. Her neurologist is speculating that since there was a gradual onset of symptoms it was not parainfectious. There was no evidence of a neuroblastoma in either the CAT scan or the MIBG scan but the body may have flushed the tumor. She is on ACTH and has had as of today 21 injections. After 2 weeks there was an improvement in her gait but her hands are still shaky and her eye movements persist. She started a mild cold last Friday and her doctor feels that this is preventing the ACTH from working more completely."

Bailey

"Bailey had a brain CT and MRI during the initial diagnostic evaluation when she was first diagnosed with neuroblastoma. The resection was performed August 11 and the OMS did subside for about six weeks but then became severe again. She was hospitalized on November 14, to begin ACTH. Her tremors were so bad when she slept that the nurses would get quite upset because they could not check her blood pressure when they did a vitals check. The ACTH stopped the OMS symptoms within 48 hours, although we did have about a month of major irritability to contend with. We never pursued any other course of treatment because the ACTH worked so well for Bailey. I did request that we drop back to every other day dosing when Bailey became quite lethargic and had gained 15 pounds in fluid retention. The every other day dosage worked very well for her."

III. Tapering ACTH

Shannon

"Shannon is doing extremely well. She has lost five pounds so far and keeps losing inches. We are now able to give her the .07 ACTH with three days between shots. This is great for us because we just gave her the shot last night and don't need to do it again until Thursday night. I do need to point out that her speech is improving tremendously. She has begun to speak full sentences on her own without any prompting. For example, her little brother Sean was getting into trouble again (he's good at that) and I couldn't see him so I asked Shannon "Where is he?" and she said "Oh, Sean, what are you doing?" I almost fainted. So, we're very happy here."

IV. Response to IVIG

Cody

"I can recall the greatest fear was the lack of knowledge and information during the onset of the OMS. We searched long and hard for information regarding OMS with some success, but contacts I made with other parents who had experienced the same situation was invaluable.

Our son Cody was initially diagnosed with OMS in October 2001. My wife Kim and I first noticed Cody, our six-year-old son, stumble a few times while walking or running. Neither of us thought much of it but within a few days it developed into an equilibrium problem. Assuming it was an inner ear infection we took him to see our pediatrician. Fortunately our pediatrician recognized his problem and diagnosed it as an ataxia due to a post viral infection. The pediatrician said there was not much we could do and it should clear itself up in a week or two.

Little did we know how much our lives were about to change. This appointment was on a Friday, so we took him home to wait and watch over the weekend. Unfortunately, Cody's condition began to deteriorate by the hour. The following day we took Cody back to the pediatrician, who was alarmed at the change within 24 hours. An MRI was performed to look for the presence of brain tumors. None were found. We were again sent home, but our pediatrician began looking for a pediatric neurologist to go see the following Monday.

That weekend turned into the longest two days of my life. Hour by hour Cody's condition was becoming worse. By Monday mourning Cody could not walk without assistance, had involuntary muscle contractions and never slept more than ten minutes the entire weekend. When Monday morning arrived we were in the neurologist's office and almost immediately Cody was admitted to the hospital for additional testing.

The next four days were spent in the hospital enduring every possible test in a process of elimination to determine the cause of Cody's illness. As the days wore on Cody's condition continued to deteriorate but the doctors were ruling out a long list of other diseases day by day. Around the middle of this first hospital stay we started to notice a slight eye tremor that coincided with a decreased desire to watch TV or play video games. Then just like the myoclonus (muscle spasms and equilibrium problems) the opsoclonus (eye twitching) started out slow then increased in severity at a very rapid pace. This is when we finally received the diagnoses of OMS.

Once we got over the shock that our child in two weeks has gone from a perfectly health young boy to becoming very sick with an extremely rare disease, we simply accepted the facts then look to the future. Our first question: what is ultimate the prognosis? Will there be any permanent damage or will he recover completely? As a parent with a sick child we wanted very definite and affirmative answers to our questions. But we learned that OMS is so rare there is not much research as in other disorders, therefore the treatment options were somewhat limited and as a result the answers to our questions were rather vague. We were told his prognosis was anything from permanent disabilities to full recovery or anywhere in between. Not the answer we wanted to hear. With this in hand we set out to find the best doctors and treatment we could find.

At this point about two weeks had gone by, Cody condition had deteriorated terribly. He could not walk, had great difficulty communicating through speech, uncontrolled muscle spasms, severe eye twitching, never slept more than one hour at a time and had severe nausea due to the eye twitching. Cody was placed on large doses of ACTH to treat the OMS and various medications for the nausea. This treatment continued for approximately three months. The ACTH did not improve his overall condition but it did cause it to plateau. This was an improvement from the daily deterioration he had been experiencing.

As with most drugs there are side effects and the ACTH had some very pronounced ones. Cody's weight went from 55lbs. to 100lbs. in four months. He also started to experience some very severe mood swings. Part of the mood swings could probably be attributable to his frustration but I believe a major part was as a result of the high doses of ACTH.

Hours started turning into days, days turned into weeks and weeks turned into months. It became a horrible grind. Cody was not getting worse but was not getting better either. We had to feed, bathe him and cloth him. Cody could almost do nothing for himself. We were wearing down and it was very difficult on the overall family life. We could not see any light at the end of the tunnel and we were frustrated and completely exhausted.

I don't know how we could have made it if it were not for our families coming to our rescue. They allowed my wife and I to get away if even for only short periods. It seemed to rejuvenate our batteries. As care givers you must take care of yourself in addition to your child. If you run yourself down you will be of no help to your child, and above all things, be very patient.

This seemed to be our lowest point, the ACTH had stopped his declining condition but it was not improving either. I then began a search for a specialist in this field who started Cody on IVIG treatments.

IVIG became Cody's miracle drug. Within a week of his first treatment, Cody stood up and started walking by himself for the first time in three or four months. He now receives a treatment about every four weeks and each time we see improvement. I would estimate he is between 80 to 90% of a full recovery. The gross motor skills came back first, but the fine motor skills are recovering slower.

Overall Cody missed 6 months of school and was behind when he returned. The school and students were very patient with him and welcomed him back, but eventually there were some teasing problems that had a very pronounced effect on him. We were always very open with Cody and explained everything we could to him. He knew something was wrong, but did not understand why this led to frustration and insecurity that was manifested by teasing at school.

If you were to look at Cody now, you could not see any problem, but he is not completely recovered and the school needs to understand that. Reintroducing him into school, sports and social events has not been as easy as we expected. I suggest you take it slow and easy.

One of the most amazing things to me during this whole experience has been Cody's complete memory of all the events during his illness. Even during the worst times when he could not walk, talk or even look at you, he was still very aware of everything going on around him. To this day he will bring up different events and I will try to explain everything because it is good for both of us to talk it out.

The best advice I could give another parent is to never give up hope. There were times I was very worried that his condition would not improve, but with the correct treatment it does get better. Always think positive, never give up hope, praise your children on any sign of improvement, and most importantly of all, never let your child see you give up. You become your child's crutch during this time. They will react to your depression and in turn will react to your positive thinking."

Rebecca

"My daughter was stricken with OMS in October. She was eight years old. It's onset was extremely sudden and developed while she was home with the flu. She developed all symptoms in less than 48 hours: eye movements, shakes and inability to walk, dizziness and aphasia. Its cause was determined as viral as no neuroblastoma was found. Rebecca was in the Hospital for ten days and was diagnosed upon arrival in the emergency room. We started her on IVIG, 24 hours after she was diagnosed. After about a week she began to make improvements. When she went home she was seen on an outpatient basis for 3 more treatments of IVIG and then we stopped. We waited to see if the symptoms would return and they never did. It took her about three months to fully recover. She shows no sign now that she ever had anything wrong."

Casey

"My daughter, Casey, was diagnosed with OMS when she was 13 months old. Two weeks prior to diagnosis Casey was very irritable. One night I placed her on her back to change her diaper and her eyes rolled back in her head and she turned blue and was having trouble breathing. I took her to the hospital and was told that it was a breath-holding spell and something viral. (She had thrown up.) That was on a Friday
night.

Monday morning I was back to the hospital with her because every time she was placed on her back the same thing would happen in addition to some small jerking movements in her arms. Fortunately, this time she was able to see a pediatric neurologist. Two days and many tests later her eyes were now whirling around non-stop and the diagnosis of OMS was made. IVIG was the choice of treatment. We all crossed our fingers and prayed that it would work.

About a week later from the last day of the infusion her eyes slowed down and two days after that the eye movements and muscle jerking stopped completely. Casey was given IVIG monthly for a while, then once every 2 months. She has just gone 22 weeks (about 5 1/2 months) in between doses. We are now trying for a six-month interval. Her neurologist says that this may be the last treatment and we will see what happens.

Since diagnosis, Casey has never relapsed and the only drug ever used was IVIG. After reading much information about this illness, I know that this is very unusual. Casey was checked for neuroblastoma 5 times (octreatide scan) and fortunately one was never found. She is up to date on immunizations except for Pertussis (which she will never get again) and MMR because of IVIG. She did well with immunizations so far and has had a couple of colds with fever without relapsing. After being diagnosed, Casey received therapies (speech, OT, and PT) for about six months. She was released when she reached her age appropriate level.

At present, Casey is a bright and active 5 year old. She attends a regular public school and is keeping up with her peers mentally and cognitively. Her language skills are above average according to her last teacher. Physically, she is somewhat behind but seems to be able to do any task after some practice. Socially, she is shy, but it probably has more to do with me being so over protective than anything else. The school that she attends knows about her condition and they are keeping an eye on her motor, speech, and social skills."

V. Residual Neuroblastoma Despite Surgery

Anne

"To summarize our daughter's treatments and developments up to now: the surgery three weeks ago was able to remove more than 60% of the tumor. There is still a residual of about 2 x 2.5 cm located close to the pelvic nervous plexi. By the time of the surgery she received first dexamethasone, then prednisolone for six days. Four days after surgery she had another two days of IVIG.

At this time she showed a significant improvement of her OMS. Nights became better and the crying terror attacks subsided. She was able to keep her head up and straight again, her eye movements got much better. There are now long spans of time where she can fix her eyes with only a few interruptions of "flapping eyes." The cloni almost disappeared. She is now an attentive and friendly child and can concentrate well on performing a task.

Nevertheless, there is still a light, fine tremor, mostly of her head, and an ataxia that makes it difficult for her to hold her balance. Even being held, she stands on her legs very shakily and sometimes loses her balance when sitting. She is robbing, but it costs her a lot of strength. The development of her language is far behind. The few words she already knew are all lost. But all together we have the impression that she did show significant improvement to the treatments with IVIGs (over two days) and dexamethasone (4 mg per day over five days), which she got three times up to now, always interrupted by three weeks. It is noteworthy that everytime the symptoms started to get worse after about 3 weeks of the end of the treatment.

The strategy of our doctors is now to remove the rest of the tumor, so they started chemotherapy five days ago. Anne receives a light therapy that is normally given for INSS stage IV-S, she gets Adriamycin, Vincristine and cyclophosphamide. The OMS shall be treated with one dose of IVIG and dexamethasone over five days in a rhythm of two to three weeks."

VI. Relapse

Shannon

"Shannon is not doing well. Her cold symptoms seem to have diminished and now I'm seeing a lot of ataxia, maybe? I don't know how to explain it. For example, today she was lying in her carriage. She sat up and looked down at the floor for a moment and her entire body was like jello. She rolled right off the side of the carriage and slammed into the floor without having any control over her limbs? She couldn't break the fall. She ended up splitting her lips open, scaring herself pretty badly. I fixed her up and she's fine again. She's happy and playing with her brother. What is this, now? Is this the OMS ataxia occurring or something else? I can't take my eyes off her now because if she's sitting or standing and shifts her body weight one way, the rest of her body follows."

VII. Plasmapheresis

Karly

"Our daughter had moderate to severe OMS. At the age of 2 years, she was treated with ACTH, azathioprine, and regular IVIG infusions. The symptoms of gait and speech disturbance improved initially within a few weeks of initiation of treatment. However, when the ACTH dose was tapered, she deteriorated. One year from the time ofdiagnosis, all therapies were discontinued. Due to the debilitating nature of her handicaps and progressive deterioration with each viral infection, it was deemed necessary that further treatment was necessary to improve her quality of life.

To make a long story, we performed pheresis on her when she was 6 years old, after being drug and treatment free for 3-4 years. At this point, she was using a wheelchair for long distances and could only walk with assistance as her balance was poor.

We did a total of 15 exchanges and started her on prednisone. She showed improvements in the first few treatments. It was subtle at first, but within a week, her balance had returned. I would say she was about 70% better. Although it is difficult to put a number on it.

I know you are thinking, Ah! It was the prednisone; her improvement was better when she was on high dose ACTH. We weaned the prednisone to 10 mg every second day. Not once did she show signs of backsliding.

Since then, we have shared information with another physician, and they have had 100% success on a small child under the age of 2.5 years with plasmapheresis. Just this summer, a child who was 8 years old had 10 treatments and the symptoms have totally disappeared with no steroids. This is amazing!

My point is that it's time to look at pheresis as a first line of treatment! We asked a numerous neurologists to try this treatment and none of them supported it. Other parents were given the same non-supportive response to treatment with pheresis."

Christina

"My daughter Christina got OMS at age 4 1/2 years. No tumor was ever found and she wasn't sick at the time of onset. Her mainstay of treatment was ACTH, which was the only thing that worked. We tried IVIG, prednisone, and Imuran, but none of them worked. The ACTH seemed to be not working as good as normal when we decided to try plasmapheresis. Six months ago we started pheresis, and within two treatments, we saw a 75 % improvement. At the time of pheresis, she was on Imuran 25 mg, as we took her off the ACTH before starting pheresis. Her last treatment was 4 months ago and she is OMS free, with no steriods in her body at all.. I would say that this is a cure for this OMS. It has helped drastically."

VIII. Speech Therapy Issues

When Cori was in her 2nd and last year of preschool I had requested that she be
placed in a speech program administered through the school in kindergarten. I
wrongfully assumed that it would start up along with school in the fall. It was
not until January that she was finally evaluated and she started therapy with a
school supplied speech therapist in February. The improvements that I heard
during this time period were of less slurring. Other articulation errors persisted. They said that she did not require speech over the summer so I assumed that it would continue in the fall of her 1st grade year.

This was not to be the case, even though her IEP was in place and her eligibility of speech therapy was noted. The problem was that the speech therapist had some complications in her pregnancy and would not be able to return to work at the school until January. I found an outside therapist, Dr. Karen Czarnik, Ph.D., and after evaluating Cori in October who began therapy with her.

Within a month I began to hear a difference. Cori began to make a very conscious
effort to improve. I also learned some important facts from this particular speech pathologist. It was pointed out to me that while Cori’s articulation problems were very similar to children who have "delayed" type problems, since the cause of Cori’s problems were neurologically based, the appropriate remedies were vastly different from those employed with the very typically seen "delayed" speech articulation errors in children. By this time, Cori had also been diagnosed with ADHD. Consequently, it was the recommendation of Dr. Czarnik that Cori be seen by a speech pathologist with the credentials at the masters level or above, that she be given one on one
sessions, and that she be seen more frequently than was dictated by her IEP.

With this new information, I requested a review of her current IEP. At this meeting I found that while there was agreement in the specific articulation problems, it was new information to them regarding the structural challenges which Cori was facing. Translation here is that they wanted to work with her in a small group, with a bachelor level speech assistant, and approach the the remedial speech in the same manner as if she were "delayed". They were surprised that I was requesting someone with more appropriate credentials. Another IEP meeting was scheduled for the following week with the administrator of the speech program.

This meeting felt more like the bargaining we have all gone through at a garage
sale. The administrator told me that a bachelors level person was very appropriate and that Dr. Czarnik was not familiar with the requirements by the state of Illinois. At this point I repeated Dr. Czarnik’s statement that while the minimum requirement was a bachelors level, the minimum RECOMMENDED requirement was at the masters level. Since this administrator seemed so righteous, I decided not to waste my time arguing with her. Afterall, I do not have a background in speech pathology. I simply
requested that another IEP meeting be scheduled when Dr. Czarnik could be present.
All of a sudden, this administrator said that she could provide someone at the masters level. She started talking details, and I asked that this be continued in a phone
conversation with Dr. Czarnik and this was agreed upon. I think I won this one.

IX. Stem Cell Transplant

Justina

"My daughter was one of the first children to do plasmapheresis and she used ACTH, cytoxan, cyclosporine, IVIG, and prednisone. I am sharing this with you because her course was extremely experimental. Despite all the trials of medication, she'd only gain a complete response with high dose Cytoxan, which she received for about a year. As a last resort to get her off medication, we went forward with an autologous stem cell transplant. We used Busulfan, Cytoxan, and ATG. Since then, she has been completely medication free and has absolutely no motor dysfunction. This was a kid who had a very aggressive and hard-to-treat case of OMS. She is now 6 years old and can ride a scooter, walk a balance beam, and summersault across the lawn. She does suffer from the residual brain damage. Her speech and cognition is affected.

While in no way do I advocate that parents immediately jump into a stem cell transplant, I do find it amazing that we have virtually put her OMS into complete and full remission. Our doctor was not in agreement that a transplant was appropriate, but after seeing Justina's response, she too feels that my daughter is done with active OMS."

IX. Reflections

I've always wanted to put my thoughts about our experience on paper.
This serves as a summary of the syndrome for others who think something
is wrong with their child, but don't know what it is exactly.

We Will Endure

At eighteen months, he was doing great,
Then OMS changed all of our fate.

This syndrome took our child away,
Piece by piece, day by day.

Spinning, tripping, falling down,
Frustration and anger seemed to mount.

Eyes listlessly just seem to stare,
They roll around…."God we're scared!"

He seems confused by simple words,
His speech is now a drunken slur.

Add drooling to this growing list,
"This isn't normal!" I insist.

He starts to choke on certain foods,
It's started to affect his moods.

Sweat coats his brow, his face turns red,
In rage attacks he hits his head.

Nights are dreaded by daddy and me,
He wakes in terror, hallucinating.

Nothing calms him, especially me,
He only seems to want daddy.

Shaking hands can't hold still,
This needs to end, we've had our fill.

Bruises mar that angelic face,
Bumps and cuts make our hearts ache.

Test after test say nothing's wrong,
Waiting for answers seems too long.

Doctors baffled say, "Wait and see,"
Misdiagnosed our poor baby.

Stop the madness, let it end,
We want our son back again.

My eyes have cried too many tears,
These past few months have seemed like years.

We'll beat this yet, there'll be a cure,
Those affected will endure.

--Kerri Drabek