She raised over $17,000 at the MS Walk at Jones Beach, NY, for team LIV LIFE last year. Her family was honored with the SPIRIT award from the NY Mets in September 2008. Olivia is currently doing a lollipop fundraiser in her school district and is speaking at an MS Society Spring Luncheon in April in New York.
Her mother, Rina, spoke at the MS Walk Kick Off Party for the MS Society in New York on Monday and was invited to a Research Programs Advisory Committee (RPAC) meeting at which doctors and scientists decide where the money goes from fundraising for the National Multiple Sclerosis Society.
So why is Olivia so involved with the cause of fundraising for MS research?
Olivia, age 15, has multiple sclerosis. She is a member of the growing Pediatric MS population.
In November 2006, Olivia had some numbness on her forehead and lip which didn’t last long. After developing a sinus infection from a cold, Olivia visited her pediatrician. Rina mentioned the numbness and the pediatrician said that it could be from the sinus infection.
A big day, January 31, 2007, Olivia had her braces taken off but later was very nauseous with a stomach ache. The next day she reported being dizzy and the pediatrician reassured them that a virus had been going around associated with dizziness. Only a few hours later, Olivia told her mom that she had two heads.
Would you be concerned? Certainly Rina was concerned for Olivia. Her eye doctor squeezed her in at the end of the day and diagnosed her with a 4th Cranial Nerve Palsy. Cranial nerve palsies can be caused by head injury or diabetes. They may also, but more rarely, result from aneurysm, meningitis, tumor, or multiple sclerosis.
Olivia was sent immediately to the ER for a CAT scan which showed inflammation and was admitted to the hospital to undergo an MRI (magnetic resonance image) on the following day, a Friday. A spinal tap was recommended by interns even before the results of the MRI came back. Olivia had not been seen by a neurologist, nor a pediatrician at this point, and her pediatricians didn’t have privileges at this hospital.
The first attempt at the lumbar puncture was unsuccessful and excruciatingly painful for Olivia. The intern apologized, “I’m sorry, we couldn’t get any fluid out.” Still thinking that Olivia just had a stomach virus, Rina demanded to see a neurologist or she would take Olivia home. By now, the double vision was gone and had lasted only two days.
When the neurologist did arrive, she conducted a complete neurological exam, then took Rina into another room to view the MRI images. Rina couldn’t believe what she saw. “That can’t be Olivia’s brain, there must be a mistake.” There were several obvious lesions and the neurologist said that it was multiple sclerosis.
In relaying this story, Rina describes how she broke down and collapsed to the floor. I can only imagine what devastating news it would be to hear the words that your adolescent child has an incurable, progressively disabling disease as MS. “How can you be sure?” The neurologist said it was 60/40 in favor of MS but that they needed another spinal tap to test for oligoclonal banding (O-bands). If the cerebrospinal fluid was positive for O-bands, then Olivia definitely had MS.
Over that weekend, the doctors tried two more times to complete a successful lumbar puncture, both times unsuccessfully. Finally Monday morning, Olivia was given sedation and underwent a spinal tap with the guidance of an x-ray machine. Cerebrospinal fluid was successfully obtained, but tested negative for O-bands.
The neurologist diagnosed ADEM (acute disseminated encephalomyelitis) and Olivia was released from the hospital. ADEM is often a one time event in children which mimics similar symptoms common in multiple sclerosis.
Clinical Features of Acute Demyelination in Children: MS in both children and adults is characterized by multiple episodes of neurological dysfunction secondary to inflammatory demyelination of the central nervous system (CNS). Just as in adults, however, not all children who experience an initial acute demyelinating syndrome (ADS) will develop MS. The term “clinically isolated syndrome or CIS” has also been applied to persons experiencing a first demyelinating event, although many authors restrict the term CIS to patients with an initial demyelinating event at high risk for future diagnosis of MS. As such, the term “CIS” is not universally applied across the entire spectrum of ADS events, particularly those considered to have a low risk of relapses.One week later after she was starting to feel better, Olivia developed restless leg syndrome, experienced numbness in her right leg which was accompanied by foot drop. Olivia was also experiencing intense headaches and a burn behind her right eye. Rina called the neurologist who ordered another MRI of the brain and spinal column which revealed more lesions. Olivia was admitted to Schneider Children’s Hospital on Long Island for 7 days and was treated with IV Solumedrol (high-dose steroids), but still didn’t receive a final diagnosis.
An ADS is classified as “monofocal” if the clinical features were referable to a single CNS lesion, such as optic neuritis, transverse myelitis, or brainstem, cerebellar, or hemispheric dysfunction; and as polyfocal if the clinical features are localized to more than one CNS location. This is based on the physician’s clinical examination, rather than MRI findings (which could show asymptomatic lesions). “Polyfocal” features refer to more than one CNS lesion and when accompanied by problems with thinking, is termed, acute disseminated encephalomyelitis (ADEM) (Krupp, Banwell, & Tenembaum, 2007).
Treatment for Acute MS Relapses: Corticosteroids. These agents are very effective at reducing the inflammation (swelling, brain irritation) associated with acute MS attack, and are associated with a more rapid recovery from an acute MS relapse. While it remains to be proven that therapy for acute relapses alters the long-term course of MS, hastening of recovery from an attack leads to reduced school absenteeism and enhanced quality of life. Acute MS attacks in our pediatric MS population are managed with intravenous methylprednisolone (Solumedrol) 20 to 30 mg/kg/day (maximum of 1 gram) as a single dose for 3 to 5 days. Children with complete resolution of symptoms receive no further corticosteroids. Children with incomplete clinical recovery following the intravenous treatment are prescribed oral prednisone tablets starting at 1 mg/kg/day, followed by a tapering schedule with reduction by 5 mg every 2 to 3 days.It took three months for Olivia to regain strength in her leg after this relapse. She played softball in school and was made team captain. Rina tells me that Olivia would fall a lot, though, and just laughed it off. Children have such resiliency to dust it off and keep going.
Differential Diagnosis of Pediatric MS: The diagnosis of MS requires that other possible diagnoses be excluded. Acute infections of the brain (i.e., viral, lyme, West Nile virus), deficiency of vitamin B12, inflammation of the brain with other autoimmune diseases (i.e., systemic lupus erythematosus, vasculitis, sarcoidosis), acute stroke or trauma, tumors, and metabolic diseases (i.e., mitochondrial or leukodystrophies) must be considered.They sought 2nd opinions, one of which was with an immunologist who explained ADEM in greater detail. Ultimately, Olivia was referred to Dr. Lauren Krupp, director of the National Pediatric MS Center at Stony Brook University Hospital & Medical Center. Dr. Krupp pioneered the very first Center in the United States to treat children and adolescents with multiple sclerosis. There is now a network of six centers nationwide committed “to sharing advancements, better practices and to collaborate so that every child with MS and their family can benefit from the collective knowledge of the entire network.”
Dr. Krupp ordered another MRI in May which showed no new lesions, only scars, Rina says. To meet the criteria for MS, three or more periventricular lesions were needed. Olivia had four or five and at this point the doctors were suspicious of MS.
MRI Findings in MS: Magnetic resonance imaging (MRI) is a useful tool for supporting the diagnosis of MS in adults and children. The MRI appearance of pediatric MS, however, is not entirely similar to that of adult-onset MS, particularly in younger children. The increasing recognition and treatment with MS underscores the urgent need for pediatric-specific MRI diagnostic criteria. Mikaeloff and colleagues have demonstrated that the sole presence of well-defined lesions, as well as lesions perpendicular to the corpus callosum are highly specific for MS in children, although these criteria are only met by approximately 30% of pediatric MS patients (Mikaeloff et al., 2004). Using standardized scoring methods, Callen and colleagues have proposed pediatric-specific modifications to the currently accepted MRI criteria that are more sensitive and specific for the diagnosis of MS in children. (Callen et al, Neurology in press) These criteria required two of the following: (1) 5 or more lesions; (2) 2 or more periventricular lesions; and (3) 1 brainstem lesion. The validity of these proposed criteria predictive of MS outcome in children experiencing an initial demyelinating attack is currently being evaluated.On August 6, 2007, Olivia woke up with shoulder pain which was attributed to playing softball over the previous few days. Rina says that, as the day went on, Olivia couldn't open and close her right hand and began to slur her words. The next morning her right leg felt funny. Rina contacted Dr. Krupp immediately and was told to wait 24 hours before calling since it might not be MS-related. For new or worsening symptoms to be considered an exacerbation of MS, they must last for more than 24 hours and be separated from other relapses by 30 days.
Rina just knew that this was the episode that would finally get Olivia diagnosed with multiple sclerosis, just nine months from the initial numbness on her forehead and lip. Olivia was admitted to Stony Brook Hospital and started IV steroids.
“In a room full of interns, doctors and my family, I asked Dr Krupp if Olivia has MS. She said 'Yes!" I didn't get upset in front of Olivia. I left the room and broke down,” says Rina. “It's very hard for me to relive this moment. It was just devastating. I knew she was in good hands, however, and she was treated for a few days and sent home with home care. We had to decide on treatment at this point. Being so naive to all of this, it was a difficult decision. We chose REBIF [Interferon beta-1a]. It's a 3x a week injection. I was trained at home to give an IV for the remainder of the steroids and another nurse came to teach us about REBIF. Olivia lost use of her right hand and arm completely. She couldn't move in any way.”
Olivia’s leg was the same, no movement, and she needed physical therapy immediately. Finding an appropriate physical therapist was a challenge nightmare. Eventually, they found an occupational therapist for Olivia’s hand and arm and a different one for her leg.
“She was a real trooper. I don't think she cried at all. I can't even tell you how I was. I was numb,” says Rina.
How common is MS in children? Analysis suggests that 2% to 5% of all patients with MS are diagnosed before their 16th birthday (Ness et al., 2007). These estimates, however, are based on retrospective review of established adult MS populations and may underestimate the true prevalence of the disease in the pediatric population. The annual average incidence of a first demyelinating event in Canadian children is 0.9/100,000, but has been reported as lower in other parts of the world (Banwell et al., 2007; Pohl, 2008). The incidence of MS diagnosis following an acute demyelinating event is the subject of ongoing research.High school began for Olivia in September, and her mom called the school to explain that she had no use of her right hand and arm. Yet, Olivia wanted to go to school. She wore a brace to keep her wrist upright, but lasted only 2 days before she couldn't handle it anymore. Olivia found it too difficult to concentrate and she still couldn't write.
Olivia was home schooled for 2 months and it took her at least 4 months to start moving some fingers on that right hand. She started to regain strength in her leg, but continued to have bad headaches all along. Olivia has a tremor in her right hand and her right foot as residuals from her exacerbation which worsen every now and then since. Some days she felt pain in her legs but only for a few hours, maybe one full day, and then it would go away.
Paroxysmal symptoms: While an attack has been defined as a period of neurological dysfunction lasting for 24 hours, patients with MS can have brief episodes of numbness, tingling, visual loss, sensory, speech or balance problems , occur frequently (from 1–2 times per day to hundreds of times a day). These are called paroxysmal symptoms and were reviewed thoroughly in the last issue of MSQR.Olivia was extremely tired all the time, missed a lot of school, and couldn't get up in the morning. She had all the flu-like side effects that come with the interferon medication, Rebif, plus elevated liver enzymes. Olivia switched her disease-modifying treatment to Copaxone (glatiramer acetate) which is a non-interferon daily injection Olivia she gives herself every morning.
Treatment to Reduce Number of Attacks: Immunomodulatory therapy. Both glatiramer acetate (GA) and interferon beta (IFNB) are immunomodulators, and decrease the relapse rate and MRI accrual of new lesions in adults with MS (IFNB Multiple Sclerosis Study Group 1993; Jacobs et al., 1996; PRISMS 2000; Comi, Filippi, & Wolinsky, 2001). Overall, these medications reduce the frequency of clinical relapse by 29% to 34%.On May 8, 2008, Olivia came home from school dizzy and sick. She took a 4-hour nap and woke up with her eye bouncing up and down slightly. By the time Olivia saw Dr. Krupp, her eyes were “bouncing like basketballs” (ie. oscillopsia which is a type of nystagmus.) She went on IV steroids and tried oral steroids for a few days which didn't help. Olivia had to be blindfolded for days because of the dizziness. She had another MRI which revealed one small lesion on her brain stem. This time Olivia was very frustrated, and to make things worse, her skin became sensitive to touch.
Interferon beta – 1a (Rebif®). In a cohort of 46 patients with pediatric MS, 22 µg SC of IFNB – 1a treatment was initiated three times weekly (Pohl et al., 2005). In five additional patients with very active disease, treatment was started at 44 µg three times weekly. Side effects were similar to those described for adult patients: injection site reaction (71%); flu-like symptoms (65%); gastrointestinal symptoms (10%); and blood count (39%) and liver function abnormalities (35%).
Glatiramer acetate (Copaxone®). Glatiramer acetate appeared to be safe and well-tolerated in seven children with RRMS at the daily dose of 20 mg daily administered SQ for 24 months (Kornek et al., 2003). Reported adverse reactions included injection site pain or induration and a short lived whole body reactions such as facial flushing and fast heart rate. After a mean treatment duration of 14.7 months, there was a reduced relapse rate from a baseline of 2.5 to 0.1 on drug and stable EDSS were reported. Again, efficacy cannot truly be evaluated in retrospective reviews of small groups of children.
On July 9, 2008, Olivia complained that her tongue felt weird and hips were hurting. Rina rubbed her legs all night and prayed that it wasn't another relapse. The doctor recommended monthly pulse steroids monthly until the Copaxone reached full effect which can take 6-9 months, but the steroids were never scheduled.
On August, 6, 2008, Olivia had intense pain in her right eye and double vision when looking to the side. She was very nauseous, had pain in the neck and shoulders, and her skin was sore to the touch. Another week of IV Solumedrol.
Change of DMT should be considered in the presence of severe side effects, poor compliance, or in patients who appear to be poor responders. Again, while a standardized definition of treatment failure has yet to be adopted, most clinicians consider a patient to be failing a specific therapy if the child experiences more than two relapses in 12 months, or it the MRI demonstrates accrual of numerous lesions.When Olivia developed right leg weakness on September 1, 2008, Dr. Krupp suggested getting aggressive with treatment - too many relapses - and recommended Tysabri (natalizumab). However, Rina was worried about possible Progressive Multifocal Leukoencephalopathy (PML). Instead, Olivia began a chemotherapy treatment with Cytoxan (cyclophosphamide) for three months. The experience was very depressing, going to a cancer center for pediatrics, and lots of side effects. Olivia worried about losing her hair which thankfully did not happen.
After Cytoxan therapy, Olivia underwent another MRI scan. When the results came in on December 22, 2208, Olivia had 2 new lesions, one of which enhanced. Very disappointing news. Dr. Krupp still wants Olivia to go on Tysabri but the family has not decided what step to take next. The new lesions didn’t cause any physical symptoms, however Olivia does have some cognitive damage.
Olivia is a girl who has always received honors and had an A+ average in everything. Olivia had wanted to be a neurologist, but now can't remember her work. She’s had to drop out of AP (advanced placement) classes because of the stress.
“She is handling it well though. Like I said, she is one tough cookie and doesn't let anything get her down,” says Rina.
While physical disability may occur relatively infrequently in the first decade in pediatric-onset MS, cognitive impairment may be a significant clinical concern (Banwell & Anderson, 2005). Formal neurocognitive assessments are required to fairly appreciate the breadth of cognitive impairments, as review of academic performance, however, many underestimate the deleterious effects of MS on cognitive capacity and academic potential. Cognitive impairments in attention and memory have been reported in approximately 60% of adults with MS (Rao, 1986), and emerging evidence suggests that impaired cognitive performance occurs in at least 30% to 40% of pediatric patients with MS. Deficits are most notable in attention, working memory, information processing, speed, and understanding of more complex sequential tasks.Rina reports that Olivia has been doing well now and trying out for Softball this week. She had some pain in her legs this past weekend but she is handling it. They don't think that it's related to MS since she had worked out with a trainer this week.
Olivia and Rina also just returned from a whirlwind trip to London which I will have to tell you about in a follow-up posting.
Rina says, “Olivia tells me everything. She tells me when she is dizzy, to when she thinks she is having a relapse. She knows how upset I get but I try not to show it. I always tell her, she will get through it. We do talk about it once in a while but we try to live without thinking about it. We live as if she was never diagnosed until something comes up.”
“I do watch her like a hawk. She texts me from school and I die. I always think she is telling me that she isn't feeling good. When she is not feeling good, my life stops and I concentrate on her. It has been tough since I do have two other daughters. They have been good with all of this but my 13 year old has had a hard time. My other daughter is 6 and is understanding.”
Some of Olivia’s friends have not known how to handle her diagnosis and changing symptoms. Some friends are good with it and some aren't. Olivia did have some counseling but didn't find great benefit from it. She needed to talk to other teens going through the same thing, but found that there really wasn’t anything for children or teens LIVING WITH MS, only for children of parents who have MS.
Olivia was supposed to start an MS support group through the National Multiple Sclerosis Society last year, specifically for children and teens living with multiple sclerosis, but she kept having relapses. And Rina wants to establish support groups for parents with children who have MS. Reaching out to make contact with a small number of other pediatric MS patients, Olivia has connected with one from Massachusetts, one from New Jersey, one in England (Patsy), and most recently one from Arizona.
Olivia wants to create wide-spread awareness of Pediatric Multiple Sclerosis. She dreams of a campaign similar in scale to Breast Cancer Awareness. Not necessarily a ribbon, but maybe a bow or pin to stand out. She wants celebrity participation, maybe conduct a fashion show, or some sort of huge party with well respected individuals. Olivia believes this to be the only way people will listen and I don’t disagree with her. Olivia is very creative and inspiring and has some very good ideas.
This is a tremendously important issue. Parents and pediatricians need to be aware of the early signs so that children can be treated quickly to prevent permanent disability. More research needs to be funded to determine the best practices in pediatric MS neurological care. Bottom line is that WE NEED A CURE!!!!!
Although recognition of pediatric MS is increasing, there remains a great deal to learn. Optimal care paradigms remain to be decided, and collaborative efforts are required to meaningfully develop such care plans. Research initiatives are also critical as understanding gained through the exploration of MS in the youngest patients may unveil clues involved in the beginning of the MS disease process.Olivia and Rina are passionate about Pediatric MS Awareness and are grateful to those who assist in their efforts. Maybe you - yes you - reading this now, know somebody who knows somebody who could help them in this endeavor.
Dream bigger than the National MS Society. Dream bigger than the MS Walk itself. What would you like to see happen to create awareness and raise funds for Pediatric Multiple Sclerosis?
Rina thanks you, Olivia thanks you, and I thank you!!!
For more information about Pediatric MS, and source for research quotes above, see - “Multiple Sclerosis in Children” by Jean M B. Ahorro, MD––The Hospital for Sick Children, Toronto, Ontario Canada; Brenda L. Banwell, MD––Director, Pediatric Multiple Sclerosis Clinic The Hospital for Sick Children, Toronto, Ontario Canada. Multiple Sclerosis Quarterly Report, United Spinal Association and the CMSC/North American Research Committee on MS (NARCOMS). Posted online on March 3, 2009.