These familes are sharing their stories. To read each family story, please click on their name.
Stories of patients with Neuroblastoma |
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| Cook Family | Detjen Family | Korenko Family |
| Moss Family | Robinson Family | Smith Family |
| Thomas Family | ||
Stories of patients without Neuroblastoma |
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| Buckle Family | Cedro Family | Diebold Family |
| Estes Family | Lambert Family | Matthews Family |
| Moats Family | White Family | Doctor Sheela Nampoothiri |
Buckle Family
Hi, my name is Marian Buckle.
My daughter has Opsoclonus Myoclonus. She is 2 years old. She had a tumor on her adrenal gland, which has been removed. She is not talking and sometimes she is unable to walk. She also tremors a lot, and her eyes are never steady. She is seeing an occupational therapist and a speech therapist.
I am very interested in your stories because my daughter is on ACTH and IVIG. I am wondering if I should be doing something else. She still is not progressing in anyway. I need help!!! I am literally going to go crazy; I just can not take it anymore. She cries most of the day, and I have to hold her on my shoulder all day long. When I put her down, she screams and is very angry. I don't know how long she is going to be this way. Does anyone know?
I just want to know if she will have to live the rest of her life this way. If anyone can help I would really appreciate it. Please help me!!! Thanks for reading my story. I do apologize, I know I am rambling, but I have so many feelings going through me right now. I have several questions! If you can please respond as soon as you are able, it would be greatly appreciated. Thanks for your time.
Does anyone know if there is a cure?
What other types of medication are out there?
Is this disease hereditary?
How long does a child have to live with this disease?
Are any of your children cured?
Marion Buckle
Please reply to:
Sandy.Wilson@avon.com
Cedro Family
Hi! My name is Jessie Cedro.
My daughter was diagnosed with OMS in August, 1997. She was 13 months old. Like most of you, we could write volumes about what she's been through, but I'll try to be brief. Casey just turned 4 years old, and we wanted everyone to know how she was doing.
Presently, Casey is a bright, active child. Her fine motor skills are at or above age appropriate. She will receive speech therapy at least once a week starting this Fall. She has trouble blending sounds like -gr, -bl. Socially, she is shy around people but warms up after some time. Around the house, she loves to do puzzles, play with play-do, and do crafts with paper, scissors, crayons and glue. She recognizes all letters, most of their sounds, and can write some letters also. She loves animals, especially her stuffed ones. Every animal and doll has a band-aid on it because they get boo-boos just like her (that part breaks my heart).
Casey still receives IVIG. This is the only medication that she has ever gotten for OMS. It seems to do the trick. She is being weaned off of it very slowly. She has always received 2 grams of immunoglobulin per kilogram of body weight. She started off getting this once a month. Now she is up to 16 weeks between treatments and trying for 18 weeks. We have not seen any symptoms of OMS since the onset in 1997. She has been checked for neuroblastoma 5 times, all negative.
Casey has received some immunizations since coming down with OMS (OPV, DT, and a flu shot) with no problems. We were told not to give her pertussis again. She is due to get MMR#1 and the chicken pox vaccine, but she has to be off the IVIG for 5 months to receive these. We know that many of you are choosing to discontinue immunizations.
We know that this is a devastating illness, and we were not given much hope 3 years ago. We still have many questions that continue to go unanswered. Everything seems to be "wait and see". But we're hanging in there and have seen so much progress that we are very optimistic about the future.
Thanks,
Jessie Cedro
jeff_cedro@oxy.com
Cook Family
Dear Opsoclonus Myoclonus Support Network,
Our son, Gavin, was a typical 21-month-old when one day, he woke up a little off-balance. I took him to the doctor and they ordered a CT of the brain. The test came back fine! Three days later, he wasn't able to walk, crawl, or sit up. He was admitted to the hospital, and they ran a bunch of tests to try to figure out what was going on. Finally a neurologist insisted that they do a CT of the abdomen. That is when they found the neuroblastoma.
We were so shocked because I had just finished up with my own treatment of breast cancer. Gavin had surgery to remove the mass. It was only about the size of a Life Saver candy, about 1 cm. It came back a stage 1. I know that this may sound strange to some of you, but it was a blessing from God that he got OM. If it weren't for the OM, we would have never had found the tumor until much, much later.
Gavin had four rounds of low dose cytoxin to help kill the tumor, IVIG treatments every month, and he is on 30 mg of Prednisone, every other day. He was on that every day but we've been able to wean him to every other day. I'm happy to say that he started to walk again in November of 2000. He was diagnosed in April, 2000. He still isn't really stable but he is doing great! We go to physical therapy every week, occupational therapy once a month (I work a lot with him at home), and speech twice a week. He is in an early intervention program and does great with that! I'm frustrated with how slow his speech is coming. I know that it is the last thing to come back but I am still impatient. He has about five words and is learning sign language too. We are just starting to work with communication devices so he will be ready for preschool next year. I have noticed that he is getting more frustrated with not being able to communicate. He will have rages at times.
Have any of you experienced this? Well I want to tell you that I appreciate this site. It is so nice to have other people who are going through or have been through this bad ordeal.
Sincerely,
Amy K. Cook
bcookfive@ascenture.net
Detjen Family
Our youngest child (born 4/30/00) has OMS due to Neuroblastoma, which was discovered in March, 2001. She was operated on, but a piece of tumor had to be left. The doctors will start a chemotherapy. Right now she gets IVIG and steroids and is much better.
We are looking for other concerned families, especially in Europe/Germany, as there is no support group existing here. We would be grateful for more information about development and treatment of OMS (e.g. has anybody made improvements with physiotherapy, acupuncture, other alternative therapies, etc.).
Marion and Stephan Detjen
Traunsteiner Str.
710781 Berlin
Germany
Tel: 030-21967207
Fax: 030-21967208
mdetjen@zedat.fu-berlin.de
Dejten Family Update - May 13, 2002
It has been exactly one year now since the outbreak of Anne's OMS. Soon (in April 2002) she will turn two years old. She has gone a long way, and we are quite confident that she will continue to make progress and will lead a normal life eventually.
By now, she is most times up on her feet trying to walk (looks like a drunkard, after a couple of steps she stumbles and falls). She understands everything we say and gives the impression of a bright and curious child; but she is still not able to pronounce words, not even "Mama". When we ask her to say "Ja" (Yes), she opens her mouth and out comes something like "Aaah".
Her sleeping is perfect, and her mood is good, except for the times when her OMS is very active. During some months, her eyes were completely quiet, then they started twitching again, but that doesn't seem to disturb her. After her surgery in April 2001, she received chemotherapy (cyclophosphamid, vincristin, adriamycin) until August, in order to fight a tiny bit of tumor which couldn't be resected, but also to fight her OMS. It helped her a lot, and the chemo was not half as bad as we had expected.
Since then, she has constantly received immuno-suppression with Methotrexate, four days of Prednison every two weeks, and IVIGs every 4-6 weeks. In August 2001 she started crawling and pulling herself up, in October we thought that she was just about to walk on her own, but then she relapsed, the symptoms got worse and better and worse, and it has been quite a rollercoaster. In spite of the symptoms, she was always able to make some progress. In case that she relapses again, we are thinking about plasmapheresis.
Marion Detjen
mdetjen@zedat.fu-berlin.de
Diebold Family
The following is a somewhat re-drafted description of my experience with Opsoclonus-myoclonus. Please feel free to share it with whomever you feel appropriate. If anyone, including any member of the OMS community, has any questions, they should feel free to call me, write me, or e-mail me. I have also provided my work number below. I am an attorney with a firm in Portland, Maine, so the phone will be answered as "Thompson & Bowie."
My full name and address are:
Thomas G. Diebold
8 Meadow Way
Cape Elizabeth, Maine 04107
(207) 799-9346 (H)
(207) 774-2500 (W)
tdiebold@yahoo.com
This letter is an attempt to set forth in small detail my experience as an adult with a post-viral onset of opsoclonus-myoclonus ("dancing eyes, dancing muscles"). The disorder can also be known as Kinsbourne's syndrome, or may have "ataxia" (unsteadiness) added. I have included dates below, to provide a sense of the time periods involved. In essence, I was admitted to the hospital about 2 weeks after initial onset of symptoms, remained in the hospital for 34 days, and will return to work not quite 3 months after onset. I had a full-blown set of symptoms, complete with complete, body-wide muscle spasms and tremors (the myoclonus) and eyes that constantly flashed back and forth rapidly (the opsoclonus). I did lose the ability to even sit up in bed, watch television or feed myself.
For a little background, I am 32 years old and am still recuperating as of early January, 1998. My symptoms began in mid-late October, 1997, as jumpy vision and shaking hands, after first suffering a nasty flu, or so I thought. In addition to first feeling generally lousy, my back hurt and I felt restless and itchy. Also noteworthy was that in early October, I fell down my stairs and struck the top of my head. I wasn't knocked unconscious, but I did see stars. My doctor does not believe it is related to the OMS, but I wanted to provide that history here.
The initial flu symptoms gradually transformed into more unusual symptoms that I had never experienced before. I felt like my body was wired in a fashion, like an electrical current was traveling through me. I was jumpy, unsteady, and became confused. My last day at work, October 22, 1997, I attended a hearing and was not able to take any notes at all or concentrate on what was being said. My eyes would periodically "flash" or very quickly erratically jerk from side to side. After my vision worsened, and I began to have shaking and weakness in my legs, I went to the emergency room on Sunday, October 26, 1997 where I was seen by a Physician's Assistant. He said there was nothing wrong with me, but sent me over to the big hospital in town for a CAT scan because of the head blow. The CAT scan revealed nothing. The resident in the E.R. correctly thought I had cerebellar damage based on the nystagmus problem (the erratic eye movements, or the opsoclonus) and the general trembling and unsteadiness in my hands and legs at that time (the myoclonus). Unfortunately for me, he was overridden by the physician on call, who, after conducting a brief neurological exam, diagnosed me with vestibular neuronitis (an inner ear disorder) - and told me it would get worse before it got better. I have read of this same misdiagnosis in the literature on this disorder.
Things definitely did get worse after that visit, and that week is now almost completely lost to me - I have essentially no memory of it. My wife and family tell me that I was irritable, but could speak coherently, although I did become increasingly disoriented and confused. My vision worsened as I lost the ability to read or even watch television. My family doctor prescribed valium, and despite taking it, I was not able to rest or sleep at all during this week. Interestingly, this was the first of many drug reactions for me that were not normal, especially with muscle relaxants and sleeping drugs, which typically caused the opposite of what they were meant to do. I expect this is true for many OMS patients, but would be interested in hearing more about unexpected drug reactions.
Beginning October 30, 1997, Thursday evening, things started a sharper acceleration downhill. I could barely walk at this point because, in addition unsteadiness caused by muscle shaking, I had lost my balance. Despite its clear visible symptoms, OMS also has more insidious efforts - at least it did in me. I gradually lost all balance, including the sensation of where I was physically located in relation to my surroundings. Part of my inability to walk was also because I lost the sensation of weight on my feet - I was later told by physical therapists that I was a very interesting patient for them because they could begin to predict what sensory perceptions I had lost and what supports or just simple physical contact of any form were necessary to help me. Thursday night I could not lay in bed because of incessant restless twitching and so I shambled and stumbled into the living room to sit in an easy chair. At some point in the middle of the night, I tried to get back into bed and my poor wife, who was laying awake this whole time, came out and had to help me walk into bed.
I was not already in the hospital at this point because I was scheduled for an appointment with a neurologist the next day, Friday, October 31, 1997. By Friday morning I had lost the ability to walk and was essentially not competent mentally. I can remember dragging myself on my elbows and knees to the bathroom to take a bath (I couldn't stand up for a shower) while my wife was downstairs for a moment rather than waiting for her - I was not a good patient and did not have a real awareness of what was going on with me. I do have a clear recollection of laying on the bed while my wife readied us for the trip to the neurologist and looking up at my brother while twitching spasmodically all over my body. I remember clearly his look of shock, but otherwise I could not really see around me because of the opsoclonus.
After looking at me for about 30 seconds and conducting a brief neurological exam, the neurologist had me admitted to Maine Medical Center, where I spent the next 34 days. I did have seizures, and brain swelling, and was totally incapacitated by full-body myoclonus (or body-wide tremors and shaking), but once I began to recover, recovery was quick. Essentially, in the space of a week to 10 days, I went from lying helpless in bed to walking slowly in the hallways. I was discharged on December 4, 1997, and have now been home for almost 2 weeks, and I feel a little stronger and less unstable each day. The eyes have improved and I can now read books for a time, and while the hands shake some, I can write this message. I hope to return to work sometime in January, 1998. (Post-script - I have been released for part-time work beginning January 12, 1998 and the symptoms continue to recede).
By Friday, October 31, 1997 and for the next several days, I was essentially in state of continual muscle spasm and body-wide tremor. I was given morphine in an attempt to relax the muscles movements and am told that did help. My eyes were flashing back and forth so much I couldn't even identify people coming into room. Sleep deprivation, which has been building since the week before I was admitted into the hospital, became a huge issue. The morphine was the only thing that provided any relief. The first week in the hospital, my condition actually worsened, and I still felt a burning tingling spreading in the areas where the tremors and muscle spasms were worse. Because my situation was somewhat acute, I was primed with essentially every treatment that is in the literature: steroids, IVIg, deprecote, muscle relexants, and other drugs I do not know of or recall. I did have seizures and delusions, and swelling of the brain, so things were very unpleasant for a period, more so for my family.
While I mentioned earlier drug reactions, I should elaborate more specifically on one particular episode. I was given an anti-seizure drug called deprecote, which initially helped. However, I became increasingly disoriented and delusional (with a severe paranoia thrown in), which culminated in several seizures over the course of a couple of days. I can still remember the particular delusions I was having, which essentially dealt with my imagining things falling on me or me falling onto the ceiling or something. Sensory and visual perceptions was very impaired at this point. Anyway, I had one especially unpleasant episode in which I, lost in a delusion about the blood-treatment machine falling on me and my wife, actually swung at her in an panicked attempt to get her out of range. Because of this and other concerns about my condition, I was given an EEG. This revealed toxic levels of deprecote in my brain. Apparently, my system, instead of eliminating the deprecote like most people, was retaining and storing it, so the deprecote level reached dangerously high levels. I do not know if this is my personal reaction, or if opsoclonus-myoclonus effects drug reactions.
Things began to improve for me about three weeks into my hospital stay once I started receiving pheresis treatments with what is called the Column A, or Protein A, or even Prosorba. I had 2 treatments with the general pheresis, and then 13 with the Column. Because I failed to respond to any other treatment, and because my spinal taps were still showing that the antibodies in question were still be produced the third week in the hospital, I was embarked on an aggressive and prolonged course of treatment with the pheresis. Initially, these treatments were extremely difficult to get through, and I went only 1/2 with the first Column treatment, but gradually I tolerated them better and better as I got stronger. I think this treatment should be tried more widely, as I had no response to steroids, deprecote, IVIgG and some others. Pheresis treatment is very much like dialysis, and essentially involves filtering the blood through a machine to replace plasma or filter out the antibodies caused the muscle shaking and eye movements.
While there were many extremely unpleasant aspects to this condition, families can help by being there at night if the OMS victim has the vertigo problem, which he or she probably will if the opsoclonus, or eye movements, is bad enough. If so, be there and touch them, hold them when they experience it. By using the term "vertigo" (which is the best term I could think of to describe this), I am attempting to describe a sensation to disorientation as to where one is in relation to objects around you - and falling toward them. After the incredible itchy restlessness subsided a little, night-time became extremely difficult because of this problem for me. I would lay awake all night clutching desperately to the railing of the hospital bed because my body completely and absolutely felt as though it was going fly into the ceiling, and that looked to be about 10 stories away. This went on while I was wide-awake. I couldn't close my eyes because of a strong spinning/twisting sensation would overwhelm me, and if I opened my eyes, I would have these perception problems. This was very unpleasant.
Family members may see the patient with opsoclonus-myoclonus suddenly accelerate their body movements at night as they go into the vertigo attack, and they may even start rolling off the bed or grabbing at things around them to stabilize themselves - that's what I did. I definitely noticed that if people touched me, it abated the symptoms, especially the vertigo, and enabled me to get at least a little sleep. I also noticed that things improved once padding was put around my bed, which served to cut down on my field of vision, so there could be less distortion to get lost in. One of the nurses administering the column A pheresis noticed that contact helped abate the muscle jerks, and held my hand during the 2.5 hours of treatment to stop the tremors from shaking the needles lose in my arms when my family wasn't around to do it.
I think families are also important in educating the nursing staff, and doctors, about the care a patient with opsoclonus-myoclonus needs. I believe it took a little while for the nursing staff to realize how incapacitated I was, including not even being able to feed myself. Further, because of the endless thrashing about, I was pulling the IV out of my arm, one time with the needle still stuck in. I was in a stupor at this point and didn't realize that I had blood all over me. Moreover, because of the stupor, and my inability to see and manipulate objects to my side (my field of vision and functioning capacity was limited to directly in front of me), I do not think I could have hit the button to summon the nurses myself. Fortunately my brother was there, as he was all-day, all-night for over three weeks, and he alerted the nursing staff. There were also a couple of episodes when, lost in a delusion, I tried to get up and roll out of bed. My brother would essentially tackle me and hold me until I became aware of myself again, or buzz for the nursing staff. Because of this problem, I was moved to an observation room in the hospital. Nights were the worst time for me, and mornings were difficult. When the symptoms were full-bore, I needed to be watched at night.
There is no particular pain associated with myoclonus-opsoclonus, but I did experience about four nights of excruciating pain because of what I understand to be a bladder spasm. My muscles were in a state of near constant contraction, and my bladder followed suite. I could not describe what was happening or what the pain was, so it took a while to figure out what was happening.
Family members should also pay attention to the calories being consumed by a patient with opsoclonus-myoclonus. Despite the fact the muscle tremoring is involuntary, many calories are being burned off as a result. I lost quite a bit of weight and in particular my legs really atrophied. I had nausea as a result of one of the drugs and just wasn't interested in eating. I was told that I was a couple of days aware from having a feeding tube inserted. My family constantly badgered me to eat and went out and got whatever I was interested in eating. I was given a green light to eat whatever I wanted, whenever I wanted. My family helped quite a bit in getting me to eat when I did not want to, and I think that made a difference. Make sure the opsoclonus-myoclonus patient eats well. Bowel movements, while an unpleasant topic, are also important and family members should be aware whether those processes are functioning properly.
Finally, I should mention that, as with most neurological disorders, there are emotional issues too. For me, I lapsed into depression and was very weepy. My doctors told me that emotional lability (or the inability to control emotional swings) was common and expected. Some may become violent or very irritable, others might be sad. I could almost feel that I did not have emotional control, but that came back as my symptoms improved.
As I write this, some two and half months after onset, I am almost well. If you saw me on the street, you might think I was completely normal. I am still weak, and my myoclonic symptoms (or the muscle jerks), manifest in a minor way if I do not sleep the night before, or I overexert myself, or I am startled, or if I get cold and start to shiver. I am reading a lot, but get a little dizzy or fuzzy if I focus too much. I have told that things should be normal in about six months. On the whole, I feel fortunate, and I am very much aware that I am lucky my condition was not related to a cancer, but was instead a post-viral attack.
Again, please feel free to contact me if there are any questions. Please also excuse grammatical or typographical errors. I think that other experiences and comments should be combined with this in one document and distributed.
Best,
Tom Diebold
tdiebold@yahoo.com
January 8, 1997
Estes Family
My daughter is 19 months old. She woke up on March 11th, 2000, throwing up, and her head was tilted to the left. I thought she had the flu. Three days later, she was still sick. I called my pediatrician and brought her in; they said she had the flu. She was still sick and didn't get any better for three weeks. We were in the doctor's office seven times and in the emergency room once in those three weeks.
Finally, she was admitted into the hospital for a spinal tap on March 27th. She was sent home the next day with an appointment to see a neurologist on March 29th. He had diagnosed her with acute cerebellar ataxia, and said see you in six weeks. She got extremely worse over the weekend. On Monday, I called the pediatrician, and he saw her that day. She was still throwing up and was very unsteady on her feet and her eyes were bouncing all over the place. I was very scared. I thought maybe it was an ear infection. But indeed it wasn't. The doctor said that with acute cerebellar ataxia, she would have her good days and her bad days. Take her home!!
I wasn't satisfied so I called the neurologist the next day and told him. He said he wanted to see her tomorrow morning. That was April 5th. When he saw her he admitted her into the hospital and did extensive testing. She had lost her ability walk, talk, and sit. Her body was tremoring from head to toe, and her eyes were bouncing. It was so awful. We spent two-and-a-half weeks in the hospital. We knew nothing!!
They changed her diagnoses to opsoclonus myoclonus ataxia syndrome with no neuroblastoma. They started her on IVIG and ACTH right off. She was very irritated already, and all she did was cry. Now with these medicines she was screaming; there was nothing I could do, nor anyone else. They put her on morphine and klonopin every time she opened her eyes. Then, they put her on oral prednisone.
I was very mad. She was no better, and on her 6th diagnoses. And I was scared. I asked, how would I have known that this is what she has if I had listened to the first doctor? She would have been home with the flu. How do I know now if this is what she has??
They sent her home anyways after two-and-a-half weeks, with a second opinion scheduled for six weeks later, in Massachusetts, on May seventh. Over the weekend she got worse. I called the neurologist. He said to admit her. So we went the next morning. She screamed the whole way there, and the whole time we were there. They didn't even know why she was there. They thought she was there for rehab. They were going to admit her, but they had nowhere to put her, so they sent us home. I called the neurologist, and he said to admit her back in Maine. She cried all the way back to Maine for three-and-a-half hours.
She spent five more days in the hospital with a UTI (urinary tract infection) that triggered her OMS to be worse. They weaned her off the prednisone, and she was admitted into the hospital in July for more testing. We spent ten days in the hospital for more tests looking for the tumor and more. She still couldn't walk, talk or sit. It was so awful to see my little girl like this. They put her on depakote, cytoxan, and clonazepam.
Now, she can stand some days, and others she can't. Her eyes still bounce; she still can't talk. She gets very frustrated. She will bite, pinch, and hit her head.
She has speech, physical and occupational therapy twice a week. She still isn't any better. I feel very helpless now. They think she might be having seizures. She is going for an EGG on November 14th. She has had six treatments of the cytoxan. I don't know if she is getting any better. Maine just is not up to date with the technology.
I don't know what to do anymore. We are still looking for someone to help our cute little girl. It you want to write, you can at christine-2@excite.com .
Thank you,
mom...
christine-2@excite.com
Korenko Family
My 3 year old daughter has OMS and has had it since she was 19 months. To begin with our daughter was misdiagnosed. We were told that she had acute cerebellar ataxia, and she was given prednisone. Meanwhile, we had a friend do research over the internet and found out about opsoclonus-myoclonus syndrome but, her doctor didn't think it was true. She ended up going psychotic on the prednisone, the doctor reluctantly put her on ACTH. We did see some improvement. From not being able to do anything, she was able to crawl and her eyes were not as crazy, and she didn't scream 24 hours (only 12).
We finally got approved for a second opinion from another neurologist. She was correctly diagnosed with OMS relating to neuroblastoma (tumor in her pancreas). She was put on a correct dose of ACTH and was started on IVIG. The IVIG was a miracle drug. Three weeks later she was walking!!! She was on 48 units of ACTH every other day and got the IVIG every 2 weeks. This lasted about 5 months. We were able to stretch the IVIGs to every 3 weeks but, when we tried to go to oral steroids, she crashed hard. Couldn't walk, stand, crawl and her eyes were bouncing. She was put back on the ACTH but, she was not responding as well. She was able to walk again but, she had that real ataxic drunk looking walk. Poor thing looked beat up and bruised all over.
The IVIGs were back up to every two weeks. Every day was getting worse. Her stability and eye movements was constantly changing. One hour she looked "okay" and the next it took everything she had to stand still for 30 seconds. Mornings were worse, it took a good hour to get organized enough to walk where she wanted to walk.
Because of the neuroblastoma, she has been followed by a pediatric oncologist. The oncologist oversaw my daughter's IVIG infusions, and she was (is) an absolute angel. She decided it was time to get more aggressive. Her doctor took the approach it's an autoimmune reaction (disease), and it should be treated like one. Her neurologist was reluctant to do anything different because it would be considered experimental. Her oncologist and I did a lot of research. She made a lot of phone calls, and I spent time on the computer. In March we began a treatment of cytoxan (a chemotherapy drug that is used to kill tumors, but is also used for immune suppression) and plasmapheresis (dialysis of the blood that cleanses out all circulating antibodies). My daughter gets this once a month in conjunction with her IVIG and steroids (2 weeks ago we were able to get her off the ACTH and go to a lower dose of prelone (prednisone).
My daughter is doing absolutely wonderful. She still has tremors, and she is not as agile as other 3 year olds, but I can't complain. Mornings are no longer a problem!!! She's able to climb, jump and run. Her speech has also improved and her overall disposition has greatly improved. I can tell that she "FEELS" better and therefore, her overall performance is greatly improved. Seeing her run around doesn't send every adult into a frenzy of being afraid that she's gonna tumble.
The major side-effects that we have had to deal with is her lower ability to fight infection and hair loss. The cytoxan has made most of her hair fall out. This greatly disturbed me at first because she had the most beautiful head of ringlets, but seeing her do so well is WELL WORTH IT!! As with any drug, there are side-effects, so our first goal is to get her off the steroids- those have enough of their own side-effects. She'll get 4 more doses of cytoxan. (There have been cases of kids that have the OMS and had been treated with chemo for the neuroblastoma and the OMS disappeared.) As far as other treatment- she gets physical, occupational and speech therapy. She attends a small preschool that is centered on kids that have disabilities. They focus on the areas that need help and also focus on the attributes of the child.
The biggest challenge we now face is her behavior. The temper tantrums and night terrors have subsided, but we are facing behavior that is aggressive and hyper. One challenge at a time!!!! Take care and remember that YOU are not alone!
-lisa korenko
Lambert Family
On April 9, 1970, right on her "due date", our youngest daughter was born. She was the prettiest baby! Our third little girl, and her birth was quick, easy, with no complications. She looked very different from her big sisters, who were 4 and 8 years old. They had been blondish bald babies, and she had a head full of dark brown hair. We named her Frances Anne and the other girls dubbed her "Francie".
For the first four years of her life, she was very healthy and happy. Her temperment was so easy going and sweet that I never had to raise my voice to her. She was fun loving and well coordinated, so we were quite concerned when one day, about 2 weeks after her 4th birthday, she began acting "strange". She was lethargic and rather intentional in her actions. For instance, when asking for a drink, she would first look at the glass and then reach for it with care as if she had to think through the process. Also she began to walk unsteadily and need help on the swing. My mother-in-law and I both felt there was something wrong with her but couldn't define it enough to call the pediatrician.
Then about 4 days into this, first thing in the morning, my oldest daughter called to me. "Come help Francie! She can't get up off the floor, and she's shaking all over!" The poor child was in full body tremor and had lost control of her bladder.
I called the Dr's office to be told to bathe her fever down and see how she was. When I told them she had no elevation in temperature and was cold to the touch, they said to bring her in right away. At first the Dr. thought it was chorea or a brain tumor, and we immediately took her to the hospital. There we met our first neurologist (unfortunately, not a pediatric one), and he did a lumbar puncture which was supposed to rule out a basal skull tumor. It showed nothing. Then began a round of testing and isolation, and she slipped into a comatose (non-responsive) state where she stayed for over 3 weeks. During this time, testing for lead poisoning, meningitis, rare bacterium, etc., all proved nothing identifiable. She was fading away from 42 lbs. at admission to 24 lbs., and our pediatrician was contacting facilities all over the world looking for an answer.
We decided that as she had come out of the coma after 3+ weeks, and I could take care of her at home as well as I was doing in the hospital, we should go home and await help from the medical world. This was 1974, before computers and the internet.
She was very like a newborn in her physical abilities, but her mind was right-on for a 4 year old. This led to much mental anguish for her and for us all. She would ask me to stop her shaking (the tremors of her body), and I would feel so impotent! Also, she threw temper tantrums when she couldn't do anything for herself or control her bowels and bladder. She had been a very fastidious little girl and was mortified at being treated like a baby. She really hated being fed as she threw up so much of what she tried to eat due to spasms in her tummy.
I would not put up with the tantrums and would confine her to a double bed until she would apologize and behave better. This made me very unpopular with the rest of the family, but I decided that we didn't know how long she'd be handicapped, and she'd have to get along in the world on proper social terms.
The following March (a long year later), we were scheduled to see a Dr. Frederick Samaha at Pittsburgh Children's Hospital for a consult. He examined Francie, and then told us that he'd seen one other child like her. He had read a paper and consulted Dr. Marcel Kinsbourne of Toronto Children's, who had tried using high dose ACTH given IM to control the tremors and disabilities. Also, he told us it was only a "hunch" he had and used the term "nystagmus" (dancing eyes) for the first time we'd heard it. We agreed to try the ACTH.
Two days later she was admitted, and we began the injections of 40 units each morning. I learned to give her the shots, and we went home to return 2 weeks later with Francie in full control of herself...a miracle!! The miracle was short-lived in that the ACTH caused so many other problems. In three months Francie went from 30 to 60 lbs and became "cushingoid". The side effects of the steroid had caught up with us! We dealt with severe hypertension (Lasix and Potassium Supplements, Aldactone, etc.), depression, mood swings, fear of exposure to Chicken Pox which would be fatal (ZIP transfusions), hirsutism (inappropriate body hair). Every time we'd try to reduce the dosage, she'd return to symptomolgy.
Her condition at that time, 1975, was referred to as Polymyoclonus-Kinsbourne's Syndrome. It was so rare (we'd been told 13 recorded cases worldwide) that nothing much was known about it. It seemed that each time we'd get to about .10 units every other day, she'd revert and she hated it because we'd have to go to 40 units a day to control her again. She also had migraine headaches to deal with as well as all I mentioned before.
Even still, we tried to keep her living as normal a life as possible. She was able to manage school with understanding teachers and administration, and even took dancing and piano lessons from wonderful teachers who would accommodate her needs. We never told her she could not do whatever she wanted to do, and we were able to find people who would work with her. She rode horses and was a Brownie Scout. I had to make all of her clothes, as she was as wide as she was tall. Only occasionally would she be the butt of joking from her peers, and we talked her through those situations. Her sisters were very good with her and even had the usual spats that sisters have about her getting out of some chore or other
When Francie was 9, we were transferred, due to my husband's work to Brookhaven, MS, a difficult time for all and especially Fran. She hated her new school and would feign sickness to stay home. We found a wonderful pediatric neurologist, Dr. Gwen Hogan-now at LA State U. Hospital in Shreveport, Jackson (70 miles north), who was familiar with the condition. After 3 tries, he was able to withdraw the ACTH totally, so in March of 1980, Francie was finally free of the steroids...5 years of them!! Most of her symptoms faded quickly, and all we saw was a beautiful 10-year-old slimming down and having only a mild hand tremor and slight small motor skill difficulty.
These still remain at age 31. Also, for about 6 months, she had trouble with nausea at times, and a mild dose of Mebaral or Depakane took care of that. When she was 13, we moved back to Ohio and at that time she was still on a very low dose of Mebaral daily even though no seizure activity showed on an EEG. It seemed to help her from feeling "queasy" in times of stress.
She began menstruating early for our family, at age 11, and that could have been due to the steroid use. At 14, she was seen by Dr. S.J. Horowitz at Rainbow Babie's and Children's Hospital in Cleveland, Ohio. He pronounced her the most completely recovered child with OMS on record with nearly no residual effects of the disease. I need to state here that early on in the course of this affliction, Fran had several tests for neuroblastoma, which were all negative. We thanked God and all the doctors who worked with us, and Fran went on to graduate high school against the predictions of some teachers. With some tutoring along the way, she received her BA degree from Kent State University in 1988.
She is now 31 and lives alone and works for a Community Action Council as a Head Start teacher. She is not married and seems at peace with that. She is very self-sufficient and a lovely, caring young woman. She volunteers each week at a homeless shelter and has many friends to socialize with. She did lose about two years of social growth in her teen years due to her illness, but it's not any longer a consideration. The only reminders of her OMS are weakness in her joints and mild hand tremor. Also, she cannot cursive write and has difficulty with fine motor skills
A few years back, she spoke to a group of parents of handicapped children of her experiences growing up. She gave us the highest praise as parents by saying that she never realized she was not "normal".
I hope this saga gives parents of OMS children hope and courage to hang in there. I know that today the ACTH and IVIG is often used. Plasmapherisis and anti-viral drugs also offer promise of control of OMS, as it is now known. The etiology of Fran's OMS was never known, but thought to be viral in nature. I have found it interesting that I and the nurse who primarily cared for Fran at the onset of her illness have both been diagnosed with late-onset Multiple Sclerosis. Some schools of thought have MS as viral in origin??? Life is always a challenge is it not?
May all of you do the best to treat your children as the wonderful gifts they are and expect them to be all that they can possibly be as they mature.
Feel free to contact me any time, and Fran would be glad to write to you, also. I can give her address and phone number upon request. She does not have e-mail at this time.
God bless,
Judy Lambert
Jujolam@aol.com
Matthews Family
The Our child was 22 months old when she first developed severe signs of OMS. Previous to this, she was behaving differently-more clingy and insecure. She began having bad temper tantrums. She started to walk only if she could hold your hand. We thought it was the terrible twos. Things did not feel right; her behavior was so inconsistent. One day, the babysitter said she would walk only if she could hold onto furniture. She also trembled when we changed her diaper. When she was lowered to a 45degree angle from the surface, her tantrums would become so severe she could wiggle herself out of a car seat in a rage of anger. She woke up from an afternoon nap and crawled out of her room shaking and crying. It was so frightening. We both cried. What was happening to my baby? She then calmed down and appeared normal again.
I took her to the emergency room that day, in March of 1994. I tried to explain my daughter's symptoms to the resident neurologist. My daughter was playing happily and seemed so healthy. I thought maybe I was imagining it all, even though her gait at this time was just slightly tremulous. The resident examined her eyes and noticed that her eye darted one time. From that we admitted her and began looking for brain tumors.
Within 3 days from admission she could not walk, stand, sit, or crawl. Her speech was really shaky, too. For approximately 6 weeks we did tests. In this time we did not treat her for fear of not being able to find a neuroblastoma. The diagnostic tests she had done included MRI of head and body, CT scan of the head and the body, MIBG nuclear scan, ultrasound, chest x-ray, VMA 24 hour urine sample, lumbar puncture, and numerous blood work ups. Everything was normal, except that she had one slightly elevated VMA level in her urine. I believe this to be a by-product of a neuroblastoma. It never appeared again, however, on other urine collections.
Our daughters history was unremarkable. Normal birth, breastfed, high on all her milestones, talking in four word sentences, starting to potty train, social and bright for her age. Family history is unremarkable as well. We couldn't figure out a cause for her disorder. Weeks previous to these symptoms she did have a flu but seemed to get over it without difficulty. We established her diagnosis as Opsoclonis Myoclonis Syndrome, based upon her symptoms. Her tremors were intentional-meaning only when she moved (not to be confused at all with myoclonic seizures). When she was held, she could be still. Her eyes would dart around continuously, even when she was sleeping. Initially her imbalance was so severe she couldn't even sit without being propped up with pillows.
Our daughter was treated initially with high dose steroids. ACTH initially and then was weaned over four months. Her symptoms came back as she was weaned. We then started her on Imuran. We also did a course of IVIG. She would get flu like symptoms after the treatment. We went through a week of hospitalization with IVIG, and a week of flu symptoms which would wear her down. It seemed we would just get her a little better, and we would be in the hospital again for our monthly IVIG. In November, 1994, we started triple therapy of IVIG, ACTH and Imuran. We were devastated to have to start all three all over again. We started after Christmas. The symptoms were as bad as before with the steroids and IVIG. We stopped the IVIG first because we felt it just made her sick and defeated the purpose. We continued with Imuran and ACTH until the ACTH was weaned. Her symptoms came back again--very severe ataxia, darting eyes, severe mood swings, fine tremors--although she was slightly better than her initial symptoms at onset.
We feared the long-term effects of steroids only masked her symptoms when she was medicated, and did not fix her problems. We requested plasma exchange initially, but the doctors felt she was too young. We chose to work with what she had left and make her function as well as possible. She ended up not being treated medically for four years. When our daughter was 6 years old, we started hearing about plasma exchange as a possible therapy. Our neurologist did not support it at all. He felt it was not scientifically proven as a therapy. I was previously a pheresis nurse for 5 years, however, and I had some knowledge about it. I spoke to a metabologist and a hematologist about this treatment option. They felt it was very reasonable to try plasma pheresis. They got me in touch with a pediatric nephrologist who was in charge of plasma pheresis. The support from then on was wonderful. They said it was worth trying and they would indeed try what ever it takes. I couldn't believe what I was hearing. We felt for the first time we weren't alone.
On June 22, 1998, our daughter went to the O.R. and had a tunneled central line placed. The surgeon did have some difficulty. Our daughter lost some blood with the procedure, however, she was fine afterwards. She was very energetic and well enough to start her first treatment with plasma exchange. The doctors felt strongly that she should start on immunosuppressive therapy. We hesitated but decided to go ahead. She started on 40 mg prednisone and 25 mg Imuran daily within a week. She was showing signs of improvement-talking clearer and her thought processes were better. I think she was feeling better and was more communicative.
She had a total of 15 treatments. They weren't without problems. She was loosing blood with each procedure, and she lost her pressure at one point and passed out. It didn't last long, but it scared me, and we ended up giving her blood the next day. That was the worst episode. It was worth it, however, as she improved over a month to the point where she could walk well unassisted. It's been five moths now since her pheresis started. She only had 2 weeks of pheresis, and the drugs are keeping her symptoms down. She is on 15 mg prednisone every second day and 25 mg Imuran everyday. She hasn't used her wheelchair since before her treatment. She walks well on her own, she climbs up stairs walking, unassisted, and she is less shaky with her fine motor movement. She continues to show improvement all the time. The change is unbelievable after four years of no therapy. Our next thought is to try pheresis again with the column A to see if we can get more of an improvement.
She continues to challenge us with behavior, and her comprehension of some things is delayed. Its hard to pin point her learning disabilities; she is smart in terms of manipulation and things like memorizing songs and poems. If anyone has more insight on this we would appreciate hearing from you. If there are any further questions or we can assist each other in any way, or just to hear your thoughts and discussion, please contact us. It would be great. We can be reached by telephone, conventional mail, or e-mail.
Thank-you,
Karen Matthew
21 Wimbleton Cres. St
Albert A.B.
CANADA T8N-3T81
(780) 419-6080
pipedown@telusplanet.net
Moats Family
My experience with Opsoclonus Myoclonus began with nausea on May 15, 1989. I was 44 years old with no medical problems. My GP doctor gave me medication for nausea, and I continued to work. Then, on July 15th, I went to my ophthalmologist because I had double vision. I worked for two weeks with my hand over one eye. He referred me to a neuro-opthamologist. I had an MRI and scores of other tests. Myathemia Gravis was ruled out, but no diagnosis of my problem. Then I was referred to a wonderful neurologist.
Again, more tests. Lupus and MS were ruled out. He put me in the hospital for 9 days and I was given cortisone by IV. I improved slightly-was also taking 60 mg a day of prednisone when I came home. He finally conferenced with another doctor who was a professor of Neurology and Oncology at UAB Hospital in Birmingham, Alabama.
I was diagnosed with OMS. I had an inflammation at the stem of my brain, and this could have caused the symptoms. He had come from Cornell University Hosp. in NY and had seen 5 other patients that had OMS. He told me that I was the 6th patient in medical history to be diagnosed with this. I was also treated with Cytoxin, a chemotherapy drug, which was really an experiment since tumors are usually present with OMS.
I had continuous tests to be sure no tumors were found-mammogram every 3 months, also chest x-ray, blood work every week for months, etc. I had to quit work August 18th. By this time I could barely walk and was so jumpy the doctor put me on Ativan. Thru it all, I recovered completely and went back to work June 1991 and am still working. Thanks to answered prayers and a wonderful doctor who cared so much and had so much interest to help me.
If I can help anyone with my experience I would be glad to. At the time, the doctors told my husband they didn't know if I would survive. I did and am glad to finally find a place to tell my story. Thank you for all you do.
Louise Moats
Crmoats@aol.com
Moss Family
Our family was effected by OMS when our daughter was 14 months old. Her OMS was the result of neuroblastoma. Her tumor was located on her left adrenal gland. The tumor was resected, and no chemotherapy was necessary.
When our daughter was 13 1/2 months old, she began to vomit with no known reason or explanation. Within two weeks of this, she started to become very unsteady and was unable to walk or crawl without frequent rest periods. Within a matter of days, she was unable to sit, stand or crawl. After our last, almost daily doctor's appointment, she was barely able to support her own head, and her eyes would roll back. Her pediatrician rushed us to the hospital with the anticipation of a brain tumor. Though it only took two days for the actual diagnosis, it seemed like an eternity.
Well, since we knew what we were facing, or so we thought, we soon became overwhelmed with surgery and medications. Seven days after her surgery, she showed no signs of improvement from the OMS, so we began IVIG. She continued on this course of treatment for some time and showed slow and steady progress. We reconsidered prednisone only after several colds put us back to what seemed like square one.
So the steroid trail began. She responded well to the prednisone with no apparent side effects. Her progress was continuing again until the round of colds, at which point we combined IVIG with prednisone and she did beautifully. I feel that at this point is when her recovery began.
To date, she remains on prednisone, though the dosage has varied, and IVIG-this, too, varies in duration between treatments. She is currently able to do just about everything a child her age could do. She is now three years old, and on standardized testing, she rates at or above age appropriate skills for speech and cognition, and at about 24 months for fine and gross motor skills. She still cannot run without jerky movements and cannot jump off the ground, but she has come a very, very long way from the baby that was once written off by some as the most unlikely to succeed.
Thank you for listening to our story. The short condensed version of the worst time of our lives.
The Moss Family
michaeleelain@yahoo.com
http://www.gabriellemoss.com
Robinson Family
Hello. My name is Rhonda Robinson and my daughter was diagnosed on May 23, 2000 with neuroblastoma associated with OMS.
My daughter was born a very vibrant and healthy child. Just like my other three girls who are older, she did everything above average. She was walking by 10 months and just a joy to have around. We are in the military and were stationed at Misawa, Japan. On April 13th, she started having problems walking. It was not alarming; we just thought she might have had an ear infection. By that evening we decided to take her to the emergency room to have her checked out. They weren't overly concerned. They observed her and decided we would just watch her over the next few days. By the next day, she was starting to shake and we took her back in. They scheduled an MRI that came back fine. We were sent home being assured there was nothing to worry about.
The next day that was proved wrong. She was shaking more, and her eyes were darting back and forth. She could not pick up her juice cup without dropping it. Over the course of a month, we were finally given the diagnosis of Cerebellar Ataxia. We were once again assured it would be okay after a few weeks.
This was the most horrible thing to ever happen to our family. During the next few weeks, our child could not lift her head, roll over, or even move toward us. The only thing that calmed her was to be held close to you. We didn't get any sleep and were about at the end of our ropes. We finally said we wanted to be sent to a specialist, which was at Tripler Army Facility in Hawaii. We arrived on May 23rd, and after observing her, the specialist told us what he thought it was, which is a parent's worst nightmare. CANCER!!! They ran a full body imaging on her and found the tumor and introduced us to the words Opsoclonus Myoclonus.
Our daughter received two rounds of chemotherapy, and her first dose of IVIG which initially had awesome results. The shaking and the eye movements came almost to a screeching halt!! After a year, she is now taking a few steps at a time, but still cannot walk without assistance. She is on ACTH and has been taking this for a couple of months. They are starting to wean her off slowly. She is still going to receive a couple of more treatments of IVIG which she takes about every four weeks. She has ongoing therapy for her physical and speech needs.
Her special needs preschool is absolutely awesome!!! She is improving greatly from the treatments. We still struggle with screaming because she is so frustrated at times trying to communicate with us. We are hanging in there by the grace of God!! We know first hand like other families how difficult this is. I am praying for every child and person in this support group. It was amazing hearing the stories that so much mirrored ours. Thank God we found this support group. Thanks for listening to our story!!
May God bless all your children and strengthen each of your lives!
The Robinsons
Smith Family
Dear Judy,
I just recently discovered your OMS support group, and I cannot begin to tell you how overwhelmed I am-- after all these years of feeling so alone with this very rare condition. My daughter is 20 years of age, and I understand that you also have a daughter that age.
My daughter was diagnosed at 13 months of age, April 30, 1980. She presented initially with irritability and tremors, and that was the beginning of a very long journey.
I was 33 when I gave birth to a very healthy and beautiful baby girl by C-section-- my first child. My second child, Lauren, was born C-section Feb. 1983 with Down Syndrome and multiple birth defects; she ultimately passed away of leukemia a week before her first birthday.
As I know you all can understand-- how frightening it was initially not knowing what was wrong with our daughter. We were fortunate that we lived near Boston Children's Hospital. Once they ruled out a brain tumor, the neurologist on duty that night assured me that he was suspicious that our daughter had a neuroblastoma. I remember thanking him.
The tumor was in her chest, Stage 2, and malignant. She had radiation therapy as well as chemo (cytoxin), and immediately was treated with ACTH. We were told at the time that the OMS would most likely disappear after the tumor was removed and treated. Her OMS had gotten increasingly worse prior to the surgery, and they had to pad her crib completely. All she did was cry.
After the surgery she was very weak, and the OMS did subside with the ACTH therapy. The OMS persisted for years after-- they were suspicious of other tumors. Each time we attempted to wean her, she would relapse terribly. The worst was her mood swings and irritability, and no one seemed to understand what we were going through. The neurologist would send us for family therapy- we were always made to feel that she was spoiled and that our parenting skills were in question. That was the worst for us, and it put a terrible strain on our marriage.
I can relate to all that I have read from other parents, and even now it is comforting to me after all these years. What was unclear to us and her doctors was that she never had these outbursts while in school. She was always very sweet, shy and withdrawn. Upon picking her up from pre-school, one day of many, she would have a tantrum and pull her self out of her car seat and attack me as I was driving-- can you relate? I often wondered if my daughter were possessed-- I would relate this to the doctors and therapist and they looked at me like I was crazy!! After all, she was good at school.
This went on until she was 15 yrs. Old, and I took her to a neurophysiopharmacologist at Mass General, Dr. Tom Spencer, and he was not at all familiar with OMS. However, he did feel that she had a severe case of ADD, and he agreed to treat. After many tries, she ended up with Wellbutrin and Chlonodine, along with the maintenance dose of ACTH.
Prior to this treatment, her conditioned had worsened, and she would cry all day long with extreme bouts of violence to herself and towards us. I remember her waking up in the morning screaming and crying, pulling at her self and at her hair. We literally had to lock her in her room or restrain her to prevent her from hurting herself on the advice of the therapist. Once the meds took effect our lives were transformed. I could not believe how the quality of her life improved.
From then on all of our efforts were spent trying to get the appropriate programs for her in school-- I won't get into that because you know how frustrating all that can be. Socialization was another big issue with her- she had no friends; that, too, was very sad for all of us. If you have any further questions about that, let me know.
When our daughter was 17, we discovered the most wonderful program on this Earth for kids like ours. It is called the Riverview School and is located in Sandwich, Mass., in Cape Cod. It is a residential program and the director is Rick Lavoie. The students come from all over the world, and it took me all that time to find it, and it is only less than 2 hours from where we live. The kids like Traci all are a little slow with significant learning disabilities. She has been there 3 years, and we have seen such growth.
This past summer, we finally weaned her off the ACTH, as well as the anti-depressants. Ironically, she no longer has any tremors. She had significant tremors while on her medication all these years.
I am a little worn down now from sharing this info, but at the same time I know, too, that this has been therapeutic for me. I want very much to hear from you, and I will keep in touch.
Sincerely,
Joanne Smith
JSmith0923@aol.com
JSmith0923@PeoplePC.com
Thomas Family
On June 13 2008 my son was a normal healthy bad guy fighting little boy, when he woke up on June 14th he was tripping and falling, by June 15th my son no longer walked, talked, or sat up without help. We had gone to the hospital 2 different times staying there 4 days at a time. We had seen 2 different child neurologist and no one could explain to me was happening to my son. They had said that it was post viral cerebellentis but that did not make sense to me. After our first hospital stay we researched day and night what this could be, from that point on we asked EVERY doctor we saw or had contact with in the hospital and out patient work if this could be "OMS" but they would scuff it off or one doctor told us that his eyes are not moving enough (I believe he meant constantly). Another doctor told us that this "OMS" was so rare that it cannot possibly be what was happening to our son. My husband and I never gave up on looking for an answer. On the second hospital stay we asked for them to do a scan of the abdomen and complete torso area, but they proceeded to only do the lower neck area and head. None of the doctors could tell us why it was not "OMS". After 3 months of symptoms and NO treatment of any kind our doctor stated he was all out of answers on what it could be and referred us to UNC Chapel Hill for 2 months away, when we tried to get the appointment for a closer date we were denied by Chapel Hill and our local neurologist would not make the call to try to get it pushed closer, so at that point as frustrated parents we took matters into our own hands. My husband and I could not sleep or eat, we researched day and night and finally found a doctor in Illinois that specialized in OMS we sent them and email with video, and they contacted us the next day telling us we needed to be there in 3 days. Three days later we were in Illinois verifying that our son was truly one in ten million who had OMS. We returned to Asheville, NC to begin treatments. We contacted Wake Forest Children's Hospital, they brought us in quickly, and we began IVIG infusions. We began to see improvement on speech a few days later. A week after our IVIG infusion we began ACTH injections twice a day. Improvement was drastic, within 4 days our son was walking again!!! We also began Rituxan (this is a chemo) soon thereafter. Our son is currently walking and he regains more and more speech each day. The extent of cognitive loss cannot be fully determined until complete recovery has come. Our son is extremely immune suppressed due to all the medications he is having, so he cannot be in general public. He will continue to receive IVIG infusions and ACTH for 2 more years. It is a constant battle with our insurance over coverage due to ACTH costing so much per vial. I don't understand how a human can put a money value on a child being able to walk, talk, and mentally function on a normal level. It is a everyday battle living with OMS but it is a battle we are willing to fight!!
White Family
My daughter is a healthy, normal 10-year-old, who has come down with OMS. In the past my daughter rarely got sick (Some winters she never even caught a cold!), but on Oct 10th, 1998, she complained of dizziness. She was admitted to Scottish Rite Children's Hospital here in Atlanta. Along with the dizziness she was very sleepy and could not walk unassisted. Her eyes started to shake a little at first and progressed to "dancing eyes". She underwent MRIs, EEGs, and spinal taps. She underwent every blood test any doctor could think of, and they all came up very normal (except Epstien Barr virus was evident). The Infectious Disease doctor felt it was viral encephalitis only because of an elevated white cell count. My daughter never had a fever or complained of feeling bad (prior to the dizzy spells). They took cultures for the virus, and they all came back negative.
They started her on steroids for the virus and she began to improve. It was only after she was moved to the rehab floor and her steroids were cut out--they were killing her stomach-- that her shaking started. A doctor from neurology that had never seen my daughter recognized her symptoms as OMS. This was 3 1/2 weeks after she was first admitted to the hospital. They did 24-hour urine tests, test for neuroblastomas, an additional MRI, and a spinal tap. All came back normal, yet she could no longer eat on her own, nor talk; she had lost her motor skills and could walk only if fully supported. Her shaking got so bad that my daughter could not sleep without medication (the jerking would wake her up). She was very light sensitive and could not be placed on her back.
My daughter was started on 50 units of ACTH, and we saw immediate improvement. She left the hospital the day before Thanksgiving, and she was placed in a day rehab program. From the time she left the hospital, her ACTH dosage was dropped by 5 units per week, and she had gone to every other day shots.
By the end of December my daughter had improved enough to go back to regular school. She regained all she had lost and was, except for a little unsteadiness, back to normal. Our family felt my daughter had beat this thing, and we were almost out of the woods.
Now the bad news. On January 20th my daughter called from school complaining of dizziness and shaking eyes. We had just dropped her ACTH dose to 10 units that Sunday, Jan 17th, so her doctor had us raise it back to 20 units. Over the week her dizziness stopped but her eyes still jerked some, especially if she was concentrating (reading). She was given an infusion of gammaglobulans on Jan 29th. Although we were told it would take a week or two to see results, she is now complaining of dizziness again.
If anyone has had experiences with OMS that are similar to mine (older healthy child with no neuroblastomas) I would love to hear from them.
-thanks
Bianca and Ric
White
993 Bailey Rd
Woodstock, GA 30188
ricbiaeva@mindspring.com
White Family Update - May 6, 2001
I would like to give you an update on Eva's progress. We've had a good outcome; I pray it gives those that read this some hope. Thank you for having this site- I have communicated with many families over the years.
2 years, 4 months later
By the summer of 1999, with the help of the gammaglobulan infussions, Eva was finally weaned off of ACTH. Her eyes still twitched periodically, especially when she was stressed or tired. She was doing great in school (As and Bs) except for Math, where she had processing problems. It was as if her brain couldn't follow the flow of the problem solving all the way through. Homework also took her a long time to do. Eva's school was wonderful getting her one-on-one help, which really helped. Eva was also taking Hebrew, which was challenging for her as well.
We were able to stop the infusions in the fall of 1999- we found as she got better that they were counter-productive. There were also emotional issues to deal with as well. She was always afraid that she would get sick again and, if, we saw a movie about a daughter growing up and leaving home, she would get very sad. Don't underestimate, especially for an older child, how emotionally hard this is.
We are also very concerned each winter, that Eva will get a cold, kick- starting her immune system again. This has not happened. In fact, for the last 2 years, she really hasn't gotten sick at all. I am still afraid and will probably not allow any immunizations.
By year 2001 Eva has recovered almost completely. Her coordination is back to normal; in fact, she made "company" in ballet this year. Her eyes no longer twitch, and her school work is easier for her to do. I have noticed that she has trouble finishing a long book and does better with short stories. I am not sure if this is related to her illness, but I am an avid reader and have always read to her, so I feel it is.
I hope the fact that Eva got better gives the parents still going through this some faith. There is not a day that goes by I don't think about Eva's illness, and the miracle of her recovery. May God be with all of you.
Bianca
ricbiaeva@mindspring.com
Doctor Sheela Nampoothiri
I am a paediatrician who recently had the rarest opportunity to treat an 18 month-old male baby with opso-myoclonus without a neuroblastoma. He most probably developed OMS following an attack of viral fever. For treating this child, I searched through internet and happened to contact with various doctors who are experts in this field.
Initially, the child had various treatments, but when he approached me I started him on ACTH. He showed good improvement following 6weeks of ACTH, following which the abnormal eye movements and irritability got under control, but the shaky movements of the body persisted. The next option was IVIG but as the parents were very poor, they could not afford IVIG. Hence, I thought of trying plasmapheresis.
In my detailed internet and medline search, and from various medical journals, I came to understand that nobody has ever tried plasmapheresis under the age of 2 years. I got motivated by the dramatic improvement of symptoms in Diebold and Matthew's child after pheresis. I had personally contacted these 2 families via e-mail. I contacted the nephrologist who had given plamapheresis to Matthew's child.
With the consent of the parents and with full optimism, I started him on pheresis under the guidance of one of my colleagues who is a nephrologist. We gave 7 cycles of pheresis over a period of 22 days. I wanted to continue a few more cycles--again the financial burden was the big problem. After 7 cycles, I stopped pheresis and started the baby on prednisolone 10mg and azathioprine (Imuran) 12.5mg once daily (10kg child). Two weeks after phresis the child started to walk without any support. His dramatic improvements were the most unforgettable moments I had ever experienced in my career till now. Now I am trying to slowly taper steroids. He is now on prednisolone 10mg, alternate days.
I got as much thrilled as his parents, and now he is able to climb stairs, walk, run and has started to say a few words, also. I am only happy to share my experiences with a parent of a baby with Opsoclonus Myoclonus Syndrome, as I know how terrible is it for a parent to go through these sufferings.
The parents of the child whom I had treated cannot communicate in English. Hence, on their behalf, I would like to share my experiences of treating the baby with OMS, as I very well know that every little bit of information in the management of OMS would be so valuable for a parent of a child with OMS. I had gone through the home page of Buckle Family, and I could feel the terrible stress the mother was undergoing.
I hope that my experience as a paediatrician would be useful for many parents.
Yours sincerely,
Dr. Sheela Nampoothiri
Consultant Paediatrician
G-7
"UJJAYINI"
PANAMPILLY NAGAR
COCHIN 682036
KERALA
INDIA
sheelaknpn@hotmail.com
Update - March 2, 2002
Now the child is 4 years old, and I have stopped the Imuran which he received for 6 months following plasmapheresis. He is now 16 kg and is getting only 7.5 mg of prednisolone on alternate days and is totally asymptomatic. Now I am planning to taper off and stop steroids within 4 months. He never had any relapse of OMS after doing plasmapheresis inspite of multiple attacks of viral fever which he had contracted during this period. He has started to talk sentences and is able to count and recognise English alphabets.
I have specifically avoided immunisations following OMS for him as it is ideal to avoid immunisations in a progressive neurological problem and still there are few grey zones in the etiology of OMS regarding the probable association of OMS with immunisations.
DR.SHEELA NAMPOOTHIRI
CONSULTANT PEDIATRICIAN
G-7
"UJJAYINI"
PANAMPILLY NAGAR
COCHIN 682036
KERALA
INDIA
sheelaknpn@hotmail.com
