Strychnine caused dogs seizures and painful death

When Frank and Carrie Corallino, of Sandpoint, returned home from work on Jan. 30, they instantly knew something was wrong with Scooby, their 2-year-old Labrador retriever-Rhodesian Ridgeback mix.

He greeted them at the door gasping and suffering seizures. They tried to rush him to the vet, but he died in Carrie Corallino's lap on the way. Returning home, they found their 6-year-old miniature Pomeranian, Chloe, dead under their bed.

Now the Humane Society of the United States is offering a $2,500 reward for information leading to the arrest of the person who used strychnine to kill the dogs.

"To use any poison to kill an animal, especially one as lethal as strychnine, is incredibly cruel," said Lisa Kauffman, the Humane Society's Idaho director, in a news release. "These were two beloved family dogs that went through hours of intense pain and fear before dying horrible deaths. Their owners are devastated. These dogs were like children to them, and we need to prosecute those responsible."

Necropsies and analysis revealed chunks of raw meat and high concentrations of strychnine, in pellet form, in the dogs' stomachs, the release said.

Strychnine causes severe, painful muscle spasms, difficulty breathing, uncontrollable arching of the neck and back and rigidity in the arms and legs, possibly leading to death, according to the Centers for Disease Control and Prevention. The poison is used primarily to kill rats. Symptoms usually appear within an hour.

"I've lost animals to sickness and old age, but just the thought somebody could do this to these precious animals just kills me," said Carrie Corallino. "They were good dogs. Nobody ever complained about them."

The dogs mostly stayed inside but were let out into a large fenced yard to relieve themselves. A friend staying with the Corallinos let the dogs out around 2:30 p.m. the day they died. Kauffman assumes someone placed the poisoned meat in the yard the night before or that morning, she said.

She said the neighborhood is "up in arms about it" and considering increasing the reward. "This is budding-serial-killer type of stuff," Kauffman said. "You don't do stuff like that."

Sandpoint Police Chief R. Mark Lockwood said animal cruelty can be a felony or a misdemeanor. A felony conviction can bring up to three years in jail and a $5,000 fine; a misdemeanor is punishable by up to a year in jail and a $100 fine.

"These are not a common occurrence," Lockwood said. "It's a vicious act. It's certainly going to cause some anxiety" among the public.

Multiple Sclerosis and seizures

Helen Yates, Chief Executive of MSRC, commenting on this paper said; "Whilst we are always saddened to hear of MS in the very young, it is very important that the medical profession is becoming increasingly aware that the condition DOES exist in children. MSRC is working to provide as many resources as possible for young people with MS and their families"

Introduction

Multiple sclerosis (MS) in children is being recognized with increasing frequency. The first descriptions of MS in children were published by Charcot between 1829 and 1849, though it was not for another 50 years that MS in children was again described in the literature (Hanefeld, 2007). There are now several national programs focused on the research and clinical management of children with MS. Recently, an International Pediatric Multiple Sclerosis Study group was constituted with the goal of fostering collaborative efforts (for more information, email: info@ipmssg.org).

Demographics and Epidemiology of Pediatric Multiple Sclerosis
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.

Genetics of MS

Genetic factors clearly influence the risk of developing MS, as MS can "run" in families. The risk of developing MS is approximately 30% if you have an identical twin with MS, 5% if you have a first degree relative (parent or non-twin) with MS, but only 0.1% if no one in the family has MS (Sadovnick, Dircks, & Ebers, 1999). Furthermore, carefully documented family histories reveal that approximately 20% of people with MS will have at least one first degree or distant relative with MS (Sadnovnick, Baird, & Ward, 1988). Family history data obtained from a large international study of MS demonstrated that 6% to 8% have a positive history of MS (Banwell et al., 2007). It is important to remember that the first degree relatives of pediatric patients with MS are still young, and may still be at risk to develop MS in the future.

A female preponderance in MS is well-established in the adult MS population. In children, however, the F:M ratio varies depending on age at first presentation. Males outnumber females when MS onset occurs prior to 10 years of age (F:M ratio, 0.7) (Simone et al., 2002; Ruggieri, Polizzi, Pavone, & Grimaldi, 1999). A female preponderance is pronounced in adolescence-onset MS (F:M ratio, 2.7 - 4.7) (Ghezzi et al., 2002). Hormonal contributions to pediatric MS risk in females after puberty require further study.

Immunological Studies

In order for the immune system to "attack", it must first recognize the "target." Scientists are very interested in learning what is initially targeted in MS. Complicating this search, however, is the fact that once the immune system is active, it will not only attack the initial target, but over time will also attack the injured tissue in the brain/spinal cord as well.

We found that children with MS harbor T-cell populations that proliferate when exposed to myelin proteins (Banwell et al., 2008). These T-cell findings may reflect the injured tissue response, rather than a primary immune aspect of MS and they represent one of several abnormalities in immune cell regulation in MS (Bar-Or, 2008).

Environmental Triggers

Infectious Triggers

Children with MS have been shown to have a significantly increased likelihood, relative to healthy age-matched peers, of being previously infected with Epstein-Barr virus (EBV) (Alotaibi et al., 2004). EBV infection leads to persistent B-cell infection, and B-cells are known to play a role in MS. Study of children enrolled from geographical regions has confirmed that the association of EBV with MS, suggesting that the association of EBV with MS is common among multiple world regions (Banwell, Krupp et al., 2007). Immunological studies specifically exploring T- and B-cell behavior in EBV-positive MS patients, may provide insights into how EBV infection influences the immune system in people with MS (Ascherio et al., 2007).

Sunlight Exposure, Vitamin D, and MS

The increased prevalence of MS in temperate regions has prompted consideration of the role of vitamin D and sunlight exposure as potential non-infectious environmental risk factors for MS. Exposure to sunlight is the primary source of vitamin D, and as such, in the winter months very little, if any, cutaneous vitamin D synthesis occurs. Individuals with MS have been shown to have lower vitamin D levels as compared to age-matched healthy controls (Nieves et al., 1994), although this finding is confounded by the potential limit in outdoor activity of patients with MS. An inverse relationship has also been demonstrated between serum concentrations of 25-hydroxyvitamin D obtained from young adults entering the military and their risk of MS diagnosis in mid-adulthood (Munger et al., 2006). The potential role for vitamin D supplementation in the primary prevention, or amelioration, of MS is an exciting area of ongoing research.

Clinical Features of Acute Demyelination in Children

The First Attack

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.

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).

Specific ADS presentations include:

Transverse Myelitis: Transverse myelitis (TM), or attack of the immune cells on the spinal cord, leads to loss of strength and sensation of the limbs and difficulty with bowel and bladder control. TM was the presenting feature of MS in only 14% of children enrolled in a multinational pediatric MS Study (Banwell, Teller et al., 2005).

Optic Neuritis: Optic neuritis (ON), an attack of the immune system on the optic nerve from the eye, results in reduced vision, pain with eye movements, and difficulty seeing color. It has been thought that bilateral ON is more common in children and unilateral ON more common in adults. This may simply reflect, however, that young children may not notice or report loss of vision in one eye. In one study of childhood ON, in which some patients were followed for 40 years, 26% were ultimately diagnosed with MS (Lucchinetti et al. 1997). In a review of ON at SickKids (www.sickkidsfoundation.com), bilateral ON was more common than monocular ON, and was associated with a greater likelihood of MS diagnosis (Wilejto et al., 2006). Of the 36 children enrolled, 13 (36%) were diagnosed with MS within the two years of ON, an outcome that was highly correlated with MRI evidence of white matter lesions in the brain.

Acute Disseminated Encephalomyelitis (ADEM): For a diagnosis of ADEM, there must be a multiple neurological symptoms plus trouble thinking (encephalopathy). The demyelinating event in some children may be accompanied by fever, drowsiness or even coma, and neck stiffness.

What happens to children with ADS:

In a review of 296 children with acquired demyelination in France, 57% were diagnosed with MS, while the remaining 43% appeared to have a monophasic illness (Mikaeloff, Suissa et al., 2004). The children in this study were followed for a mean of 2.9 years (range 0.5- 14.9 years). Since patients can develop their second MS-defining attack years after their first attack, it is possible that the percentage of children in the French study ultimately diagnosed with MS will increase as the duration of follow-up lengthens.

Recurrent Attacks: Diagnosis of MS

Pediatric MS requires multiple episodes of CNS demyelination separated in time (by four weeks or more) and space (involving new areas of the CNS) just as is specified for adults. MRI evidence of new lesions in new CNS locations can be used to meet the requirement for disease dissemination in time (Polman et al., 2005).

Approximately 95% of pediatric patients with MS have recurrent attacks followed by periods of clinical recovery or stability (Banwell, Ghezzi et al., 2007; Boiko et al., 2002). This form of MS is known as relapsing-remitting MS (RRMS). Over time, children with RRMS may enter a phase of the disease in which they show increasing physical disability even in the absence of attacks (secondary progressive MS, SPMS). Primary progressive MS (PPMS), in which neurological disability worsens over time in the absence of clear attacks, appears to be exceptionally rare in children. Figure 1 illustrates the typical MRI features of MS in children.

How do children with MS do?

The time from the initial acute attack to the second, MS-defining event is highly variable. Younger children tend to have a longer interval from first to second attack (median 6 years), in contrast to most adolescent patients with MS who typically have their second attack within 12 months. The annual relapse rate reported in retrospective studies with long observation periods range from 0.38 per year to 1.0 per year (Simone et al., 2002).

In a multinational study of 137 children with MS, 13% of children with MS showed fixed neurological deficits that limited their ambulation (EDSS >4.0) after a mean disease duration of 5 years. Mikaeloff and colleagues, (Mikaeloff et al., 2006) documented EDSS scores of 4 or higher in 15% of children with MS enrolled in the French KIDSEP study after a median observation of 4.8 years (from second demyelinating event).

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.

MS Disease Course

In a study reviewing the disease course of 116 patients with MS onset under age 16 years, 53% of the 116 patients ultimately progressed to SPMS at 23 years post-MS diagnosis. In comparison to studies in adult MS, pediatric-onset MS patients progress more slowly and take a longer period of time to develop disability (Boiko et al., 2002). It is important to consider, however, that a 10 year-old child with MS will only be about 30 to 40 years of age when he/she is at risk for SPMS-and thus, actually younger than the typical age of onset of disability in adult-onset patients with MS. Children with more disability early in the disease are at greater risk of severe disability over time.

Symptoms of MS in Children

Many symptoms may accompany an MS relapse, which by definition, lasts at least 24 hours.

Sensory symptoms: The most common sensory symptoms are numbness and paresthesias (tingling) in one or more limbs. The sensory symptoms can be due to a myelopathy, which can produce a spinal sensory level. Sensory deficits that arise from lesions in the sensory cortex or the supraspinal pathways lead to numbness. Patients may also have radicular symptoms due to a lesion at the dorsal root entry zone of the spinal cord or the brainstem, although this is very rare. Patients with sensory deficits involving the dorsal column pathways subserving vibration and propioception, can experience a "useless hand syndrome" in which motor movement is preserved, but the ability to manipulate the arm in space is impaired (El-Moslimany & Lublin, 2008).

Motor symptoms: Weakness can occur in any extremity, singly or in combination. The most dramatic of the acute motor syndromes is an acute transverse myelitis. In most children with MS, TM manifests as a partial cord syndrome. Longitudinally extensive lesions that traverse the cross-sectional diameter of the cord are more typical of isolated TM or NMO (Pidcock et al., 2007).

Spasticity: Spasticity or stiffness of the limbs during attempted limb movement occurs in patients following severe relapses associated with residual damage to motor pathways, and occurs as a core component of the progressive disability seen in the secondary progressive phase of MS. As such, it is relatively rare as a major symptom in children with RRMS. When present, spasticity is disabling, causes disruption of sleep, and contributes to pain.

Bladder and sexual function: Lesions of the distal spinal cord can impair both bladder and sexual function. While such deficits are rarely reported in children and adolescents with MS, recognition of these issues is critical. Impaired bladder emptying can lead to retention of urine, infection, and potential life-threatening sepsis. Impaired sexual function is a socially and psychologically devastating issue for sexually-active adolescents--and an issue that few are comfortable discussing unless a strong rapport and level of trust have been established between the pediatric MS care provider and the patient. Clinical interviews with parents out of the room are essential for these discussions.

Bladder impairment most commonly results from overactivity of the detrusor muscle of the bladder. This produces the sensation or urgency despite low bladder volume. Urge incontinence occurs if high intravesical pressure results in the loss of some urine.

Detrusor-sphincter dyssynergia is characterized by contraction of the internal urethral sphincter during an involuntary detrusor contraction. This is due to the loss of synchronization between the detrusor and internal urethral sphincter leading to incomplete bladder emptying and hesitancy (El-Moslimany et al., 2008).

Fatigue: Fatigue or a "sense of physical tiredness and lack of energy, distinct from sadness or weakness," is reported by approximately 40% of children and adolescents with MS. (Banwell, Ghezzi et al., 2007). Fatigue of sufficient severity to compromise participation normal activities, such as sports, social events, or completion of academic tasks is considered worthy of treatment.

Dysarthria: Children with MS can have different forms of dysarthria or impaired speech production. Dysarthria of the cerebellar type results in scanning speech which is characterized as monotonous speech interspersed with explosive consonants, resulting in irregular volume and indistinct articulation tremor of the voice. As cerebellar involvement occurs relatively commonly in pediatric-onset MS, speech impairment is also a notable feature of some children. Pseudobulbar dysarthria is caused by spastic vocal cords, which causes a high-pitched low-volume speech with slurred consonants- this is rarely seen in children. The precise frequency and severity of speech disorders in pediatric MS have not been described.

Tremors and other movement disorders: Tremors in MS are usually most notable when the child is reaching for an object or attempting to perform purposeful movements of the upper limbs. Tremor in MS is associated with greater impairment and functional disability due to impairments in hand-writing, self-care, and fine motor tasks. Transient tremor is a common feature of corticosteroid therapy, and patients and parents should be made aware of this in order to avoid concern over what they may perceive to be a new neurological deficit.

Pain: A significant number of adults with MS, and a lesser proportion of pediatric patients with MS, experience pain, which may be due to many factors. Patients can have musculoskeletal pain due to weakness, spasticity, imbalance, osteoporosis, compression fractures or osteoarthritis. All these processes are due to the disease or to immobility secondary to MS. Pain, and particularly back pain, reported by any child that has been exposed to prolonged or repeated corticosteroid therapy should prompt a careful evaluation for pathological fractures of the spine, ribs, or long bones associated with osteopenia.

Burning pain or " dysesthetic pain" is reported in some patients with MS. While the cause of this type of pain is not entirely clear, the mechanism could be spontaneous activity in the deafferented neurons, ephaptic transmission, or sympathetic activation (El-Moslimany & Lublin, 2008). Transmission of abnormal electrical discharges laterally across a demyelinated plaque might produce painful symptoms.

L'Hermitte's phenomeno: This is a specific sensory symptom seen in patients with spinal cord lesions. It is defined as a sensation of electric shock in the back and legs of patients brought on by neck flexion. The symptom usually remits quickly, but also can persist. Younger children describe this as "an elastic band feeling" or a "cell phone going off my spine." The presence of L'Hermitte's symptom should prompt imaging of the spinal cord.

Depression: Mood disturbances are a common feature of MS, and depression is a significant health issue that warrants recognition in pediatric patients with MS. Reactive depression, initiated often by diagnosis or by a severe relapse, is not surprising in children and adolescents facing an uncertain future with an unpredictable illness. Counseling sessions with a mental health care professional may be sufficient to address the issue.

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).

Seizures: Seizures occur in about 5% of children with MS (Boiko et al., 2002). Seizures and headache are particularly prominent features in children with tumefactive demyelination, a demyelinating phenotype characterized by one or more large areas of demyelination, perilesional edema with mass effect, and often ring-enhancement (McAdam, Blaser, & Banwell, 2002).

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.

Figure 2 depicts the varied appearance of MS in children, including the diffuse, ill-defined lesion appearance that may be seen early in the MS disease course of very young children. In children with ADEM, despite a rather dramatic MRI appearance, MRI resolution of initial lesions typically occurs. The capacity for lesion resolution suggests either that the neuroimaging features represent a greater contribution of swelling (edema) rather than demyelination or tissue injury, or that children have an enhanced capacity for rapid lesion repair. More advanced imaging techniques, such as magnetization transfer imaging, are required to explore this possibility. Longitudinal MRI studies are required to evaluate the rate of lesion accrual, and the progression of brain shrinkage or atrophy, and to determine whether these measures correlate with physical and cognitive outcomes of MS in children.

Laboratory Studies

More than 90% of children with MS will have oligoclonal bands (OCBs) in the cerebrospinal fluid (CSF) - obtained by performing a lumbar puncture or spinal tap- provided that sensitive techniques such as isoelectric focusing are used (Pohl et al., 2007). Although OCBs may be present transiently in children with monophasic ADEM, CNS lupus, and in patients with primary CNS infections, the presence of OCBs is strongly supportive of MS. Electrical tests called evoked potential testing can confirm the involvement of or detect clinically silent problems in the visual system, the auditory system, or in the sensory system.

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.

Treatment of Pediatric MS

The care of children with MS is optimized by a multidisciplinary team comprised of pediatric or adult neurologists with expertise in pediatric MS, nurses, physiotherapists, occupational therapists, social workers, psychologists, and psychiatrists. Compliance with medication, especially among adolescent patients, rests on a strong relationship between medical teams, patients, and parents.

Treatment of pediatric MS can be divided into: (1) treatment of acute attacks; (2) treatments to reduce the number of attacks and attack severity; (3) treatment of intermittent or persistent MS symptoms. Most care models for pediatric MS are based on protocols optimized in adults. Randomized control trials in pediatric MS are challenged by the relative rarity of MS in children, and by the fact that pivotal studies of MS therapies are restricted to patients over age 18 years of age.

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.

The most frequent side effects of high dose glucocorticoids are facial flushing, sleep difficulties, irritability, mild tremor, and increased appetite. In children, growth retardation is an additional concern, and is related to the cumulative dose. Every effort should be made to keep the total duration of corticosteroid exposure to a minimum. In our program, the total duration of tapering dosing is restricted to 21 days. Hypertension and hyperglycemia are rare, but important corticosteroid related toxicities, and thus all patients should be monitored closely with regular evaluation of blood pressure, glucose, and electrolytes. Many patients experience gastrointestinal irritation during corticosteroid therapy, and administration of gastric protection is suggested.

Intravenous Immunoglobulins. Some children do not experience sufficient clinical recovery with corticosteroids (steroid-resistant) or develop recurrent symptoms during the prednisone taper (steroid-dependent). Treatment with intravenous immunoglobulin (IVIg) can be helpful in these patients. Case-report level evidence supports efficacy for IVIg (in a dose of 2 gms/kg over 2-5 days) in children with acute demyelinating attacks (Nishikawa et al., 1999).

Plasma Exchange. Level 1 evidence exists for plasma exchange to treat severe relapses in adult patients with MS when they fail to recover after treatment with high-dose glucocorticoids (Keegan et al., 2002). Five exchanges over 8 to 10 days is generally recommended.

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%.

Interferon beta - 1b (Betaseron/Betaferon®). A retrospective review of safety and tolerability in a cohort of 43 children and adolescents with MS was reported by an international working group (Banwell et al., 2006). Given the variable size and weight of children, many pediatric MS specialists initiate therapy at one quarter of the adult dose, and increase monthly by quarter dose increments provided that tolerability is acceptable. In particular, it is critical to observe liver function, as some younger patients may demonstrate elevation in liver transaminases. Typically, the elevation in transaminases resolves if the interferon dose is reduced, and the escalation phase is performed over a longer period of months. Most common adverse effects (AE) included flu-like symptoms (35%), abnormal liver function test (LFT) (26%), and injection site reactions (21%) (Pohl et al., 2007).

Interferon beta - 1a IM (Avonex®). Data on the tolerability of weekly IM IFNB - 1a for treatment of RRMS in 9 children younger than 16 years of age was reported in a retrospective study (Pohl et al., 2007). Adverse effects included flu-like symptoms (44%), headaches (44%), fever (22%), and injection site soreness (11%). A reduction of annualized relapse rate from 3.1 (pre-treatment) to 0.3 and stable EDSS were reported. However, in the absence of a randomized double-blind control design, efficacy data must be considered with caution.

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.

Immunosuppressive therapy. Oral azathioprine has been used in MS to prevent exacerbations by some clinicians, although little has been reported regarding safety or efficacy. Azathioprine is not used commonly in adults with MS, as efficacy is considered limited. Side effects include cytopenia, gastrointestinal intolerance, liver toxicity, and skin rashes. The cost of azathioprine makes it an attractive therapy for patients or countries unable to afford the high cost of the immunomodulatory therapies. Efficacy, however, requires evaluation. Close monitoring of the complete blood count (CBC) and LFTs is recommended.

Disease-modifying Therapy

Initiation. The International Pediatric MS Study Group agreed that immunomodulatory treatment should be started in children and adolescents with active relapsing-remitting disease (defined clinically or by MRI scans) after MS diagnosis. In patients with a recent clinical exacerbation, any MRI change or enhancement on a follow-up brain MRI 3 to 6 months after the exacerbation would suggest disease activity.

Choice of medication. Treatment selection should occur after discussions with the child and parents focused on issues related to compliance, efficacy, and tolerability. The initial IFNB therapy is often initiated at 25% to 50% of the recommended full dose for adults with MS, followed by a stepwise escalation every 2 to 4 weeks up to full or highest tolerated dose. Use of acetaminophen or ibuprofen at the time of injections and, if necessary, 4 to 6 hours thereafter will lessen frequency and severity of flu-like symptoms during the first months of therapy. Glatiramer acetate regimen in children and adolescents is similar to adult regimen. No dose escalation is necessary.

Interferon therapy requires laboratory monitoring, monthly for six months and then three-to six-monthly thereafter.

One approach to evaluate treatment efficacy in an individual patient is to perform neurological examinations at treatment initiation and at 1, 3, and 6 months, and every 6 months thereafter. A repeat brain MRI scan with gadolinium should be obtained around the time of treatment initiation, and again after a period of therapy (typically at 6 or 12 months). These suggestions are based on the clinical model followed in our pediatric MS clinic - formal protocols have not been evaluated.

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.

Symptomatic Therapy

Spasticity. The goal of spasticity treatment is to improve mobility, reduce pain, and control painful muscle spasms. In severely affected patients, care involves positioning in order to prevent contractures and pressure sores. The treatments offered to reduce spasticity in children with MS are very similar to methods used in children with severe spastic cerebral palsy. Initial management utilizes daily stretching and physical therapy, with particular focus on range-of-motion exercises. If these are insufficient, baclofen, a GABA agonist, is the drug of choice for monotherapy. Tizanadine, a central alpha-adrenergic agonist, can be considered as monotherapy for patients who do not tolerate baclofen. Selective botolinum toxin type A injections can be considered if the above-mentioned therapies are not effective. It has successfully relieved severe leg adductor spasticity in some patients.

Fatigue. Many patients with MS complain of fatigue that is sufficiently severe to interfere with school performance or social activities. Amantidine is an NMDA receptor antagonist with antiviral, neuroprotective, and anti-parkinsonian effects. If amantidine is not effective, modafinil, should be considered. Modafinil (Provigil®) has been shown to be efficacious in adults with MS.

Tremor and ataxia. Occupational therapy and physical therapy can be helpful in providing adaptive equipment for safe walking and other daily activities. Clonazepam (Rivotril®) is one of the most effective treatments for MS intention tremor. Primidone can also be considered.

Urologic and bowel disorders. A urinary tract infection should be excluded in all patients with bladder dysfunction and treated accordingly with appropriate antibiotics. Detrusor-sphincter dyssynergia responds to combination of anticholinergic agents with intermittent straight catheterization. Formal urological assessment is highly recommended.

Coping With the Diagnosis of MS

The diagnosis of a chronic illness such as MS leads to significant impact not only on the child, but on the entire family. Some children/teenagers will require psychiatric support, and we recommend that all children/teenagers be offered a psychiatry or social work referral. Many pediatric MS patients adopt an "invincible" attitude, a common coping mechanism for this age (Boyd et al., 2005). Psychiatric support for these patients may be delayed until such time as they are willing to participate in these discussions. Parents of pediatric MS patients, on the other hand, typically seek support immediately. The Canadian and US National MS Societies provide several resources for children with MS and their families, including a parent handbook.

Conclusion

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.

Jean Marie 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

MSRC

View drug information on Avonex; Clonazepam; Copaxone.

A feast for the eyes but a health concern for dogs

In no time at all spring will have sprung. With the change in seasons come some pretty, but potentially hazardous, plants. Keep your hounds safe this spring and summer. Admire the following poisonous plants and flowers (provided by the ASPCA www.aspca.org) from afar.

Lilies
Members of the Lilium spp. are considered to be highly toxic to cats. While the poisonous component has not yet been identified, it is clear that with even ingestions of very small amounts of the plant, severe kidney damage could result.

Tulip/Narcissus bulbs
The bulb portions of Tulipa/Narcissus spp. contain toxins that can cause intense gastrointestinal irritation, drooling, loss of appetite, depression of the central nervous system, convulsions and cardiac abnormalities.

Azalea/Rhododendron
Members of the Rhododenron spp. contain substances known as grayantoxins, which can produce vomiting, drooling, diarrhea, weakness and depression of the central nervous system in animals. Severe azalea poisoning could ultimately lead to coma and death from cardiovascular collapse.

Oleander
All parts of Nerium oleander are considered to be toxic, as they contain cardiac glycosides that have the potential to cause serious effects—including gastrointestinal tract irritation, abnormal heart function, hypothermia and even death.

Cyclamen
Cylamen species contain cyclamine, but the highest concentration of this toxic component is typically located in the root portion of the plant. If consumed, Cylamen can produce significant gastrointestinal irritation, including intense vomiting. Fatalities have also been reported in some cases.

Kalanchoe
This plant contains components that can produce gastrointestinal irritation, as well as those that are toxic to the heart, and can seriously affect cardiac rhythm and rate.

Amaryllis
Common garden plants popular around Easter, Amaryllis species contain toxins that can cause vomiting, depression, diarrhea, abdominal pain, hypersalivation, anorexia and tremors.

Autumn Crocus
Ingestion of Colchicum autumnale by pets can result in oral irritation, bloody vomiting, diarrhea, shock, multi-organ damage and bone marrow suppression.

Chrysanthemum
These popular blooms are part of the Compositae family, which contain pyrethrins that may produce gastrointestinal upset, including drooling, vomiting and diarrhea, if eaten. In certain cases depression and loss of coordination may also develop if enough of any part of the plant is consumed.

English Ivy
Also called branching ivy, glacier ivy, needlepoint ivy, sweetheart ivy and California ivy, Hedera helix contains triterpenoid saponins that, should pets ingest, can result in vomiting, abdominal pain, hypersalivation and diarrhea.

Peace Lily (AKA Mauna Loa Peace Lily)
Spathiphyllum contains calcium oxalate crystals that can cause oral irritation, excessive drooling, vomiting, difficulty in swallowing and intense burning and irritation of the mouth, lips and tongue in pets who ingest.

Epilepsy Awareness Month

When families discover Epilepsy Durham Region (EDR) for the first time, the reaction is always the same -- relief.

"You can see the weight lifted when they realize they're not alone," said the agency's executive director, Dianne McKenzie.

Unlike cancer or heart disease, which have high profiles in the community, epilepsy is still largely misunderstood and as a result, newly-diagnosed people and their loved ones often feel isolated and have dozens of questions ranging from "will I lose my driver's license?" to "can my boss fire me for having a seizure?"

Fittingly, this year's theme for Epilepsy Awareness Month in March is "You're Not Alone."

In addition to raising awareness about epilepsy and reminding Durham residents that there is support available, EDR staff will be using the month of March to raise much-needed funds.

It costs about $180,000 a year to run the EDR office, with no government funding or cash flow from sources like the United Way. The agency has just one full-time staff person and relies on contract employees and volunteers to keep things going.

Every year, the number of people who need counselling, referrals or education programs increases -- last year the need for EDR services jumped a staggering 97 per cent.

To help keep up, EDR is challenging the 36,000 people in Durham who have epilepsy or are touched by the disorder to each donate $5. If everyone did, it would be enough to keep things running for another year.

"I am optimistic about the support and commitment in our community, but for some reason, it's just harder to get help for epilepsy than it is for other causes," Ms. McKenzie said. "This is something that anyone, at any time could develop and we need to make sure there are services here in our community."

An estimated one in 100 people have epilepsy and one in 10 will have at least one seizure at some point in their lifetime. It is not a disease, but rather a general term for more than 2,000 types of seizure disorders, that occur as the result of brief changes in brain cell activity.

EDR offers an array of services including a parents' forum, resource library, Changing the Face of Epilepsy free lecture series and coaching for children, parents and families. For more information, visit www.epilepsydurham.com

Man suffers seizures following attack

A 30-year-old man was arrested after he allegedly punched a friend in the head on Thursday, which caused the friend to have seizures.

Cory Boylan was charged with assault in the incident, which happened at about 9:45 p.m. at Johnny Mac’s Bar & Grill, 2279 Hubbell Ave., according to a police report.

Boylan was at the bar with his friend Dale Gifford, 23, and two women. No one was drinking, the report said. Boylan apparent made unwanted sexual advances toward one of the women, and then called Gifford’s mother a derogatory name.

Gifford, who suffers from cerebral palsy and has a metal plate in his head from more than two dozen surgeries to allow him to walk, repeated the insult back at Boylan’s mother.

Boylan walked over to Gifford and punched him in the left side of his face, despite knowing about Gifford’s medical condition, the report said. Gifford “was knocked to the floor where he had two to three seizures,” it said.

Boylan left the bar and went home, where he was arrested. He told police he was sorry for punching his friend. Boylan faces up to a year in jail if convicted.

What is the link between a mouse and a form of Epilepsy?

Mice bred to produce too much of a protein associated with a particular form of calcium channel gene experience a "pure" form of absence seizures, identified by brief lapses in consciousness, said researchers at Baylor College of Medicine in a report that appears in the current issue of the Journal of Neuroscience.

"This shows that an extra copy of a gene in a mouse can cause a pure form of absence epilepsy, without any other neurological problems " said Dr Jeffrey L. Noebels, professor of neurology, neuroscience and molecular and human genetics. He is director of the Blue Bird Circle Developmental Neurogenetics Laboratory, where this research took place. Noebels said much of the work was done by Dr Wayne Ernst while a graduate student in his laboratory.

The model is already proving useful in screening drugs that work against calcium channel defects, he said.

Calcium channels are special pores in cell membranes that open and close to let in charged calcium molecules at appropriate times in the life of a cell. Too much or too little calcium can affect the cell’s ability to carry out its normal function. Such defects in neurons are associated with epilepsy and other neurological disorders.

Childhood absence epilepsy accounts for 2 to 8 percent of all epilepsy in children. Most such seizures are spells in which the child is not aware of his or her surroundings and is not responsive. Such spells last only seconds, in most cases.

Previous studies had produced mice that had absence epilepsy as well as ataxia, a disorder associated with problems walking and standing.

When the new "T-type" calcium channel gene is over expressed in the neurons by inserting a second copy of the gene, it causes the pure form of absence epilepsy similar to that seen in children. In a twist of the old song, the mouse now has "two for T", said Noebels.

(Source: Baylor College of Medicine: Journal of Neuroscience: March 2009)

According to Epilepsy Canada, approximately 1 per cent of the population, about 300,000 Canadians have epilepsy.

Sadly, people with epilepsy deal with discrimination every day due to the many misunderstandings and stigmas surrounding the disorder.

They are often refused employment although they have the same abilities and intelligence as everyone else. Children are often taunted and laughed at by their schoolmates. And teachers often do not realize when a student is having an absence seizure and accuse them of not paying attention or daydreaming.

Epilepsy not a Disease but a Symptom of a Neurological Disorder

Epilepsy is not contagious and it is not a disease. Epilepsy Canada explains epilepsy is a symptom of a neurological disorder -- a physical condition which causes a malfunction of the electrical signals which control the operation of the brain. It is characterized by sudden brief seizures whose nature and intensity varies from person to person.

Anyone Can Develop Epilepsy

Anyone can develop epilepsy at any time. In fact, according to Epilepsy Canada, each day an average of 38 Canadians learn that they have epilepsy. This year, an average of 14,000 people will learn that they have epilepsy, 44 per cent are diagnosed before the age of five, 55 per cent before age 10, and 1.3 per cent are over the age of 60. About 60 per cent of new patients are young children and senior citizens.

The cause of epilepsy in 75 per cent of children and 50 per cent of adults with the disorder is unknown. In the remainder the following causes are often identified: brain tumor and stroke, head trauma, injury, infection or systemic illness during pregnancy; aftermath of meningitis, or viral encephalitis.

What Does a Seizure Appear Like?

A seizure may appear as a brief stare, an unusual movement of the body, a change of awareness, or a convulsion. A seizure may last a few seconds or a few minutes. There are more than 40 different types of seizures.

Although treatments are available to reduce the frequency and severity of seizures, there is no known cure for epilepsy.

Advances in Epileptic Medications

In recent years, advances have been made in medications. Side effects, which may have limited the usefulness of anti-convulsant drugs in the past, are now less problematic. A number of add-on drugs have been developed to control specific seizure types. More than 60 per cent of people with epilepsy are able to control their seizures using medications.

Brain surgery is possible when medication does not help and when the site of the malfunction is clearly defined and accessible without affecting the personality or brain function.

The following is what you should do if you see someone having a seizure:

• Remain calm. You cannot stop a seizure once it has started. Experts suggest that it is best that you let the seizure run its course.
• Do not try to revive the person.
• Prevent injury to the person having the seizure. If he is in a chair ease him to the floor. Protect him from injury by removing objects that are likely to cause injury -- anything that is sharp, hot or breakables.
• Do not put anything in the person's mouth while he is having a seizure.
• Do not restrain him.
• Loosen his clothing.
• After the seizure the person should be allowed to rest or sleep.
• If the person undergoes a series of convulsions, seek medical attention immediately.

For more information contact Epilepsy Canada Toll free: 1-877-SEIZURE (734-0873).

The copyright of the article Epilepsy a Neurological Disorder in Epilepsy is owned by Cheryl La Rocque. Permission to republish Epilepsy a Neurological Disorder in print or online must be granted by the author in writing.

Parental nightmare: Rare disorder has seizures as one of the symptoms

Five-year-old Hattie Read wants to know why she can't go to heaven. Her big brother Sidney is already up there, and her little brother Freddie is going soon, so why can't she? Can't she go up to play for a bit, then come back in time for school?

Her mother Vicki explains that heaven doesn't really work like that, and that Hattie won't be going there for a long, long time because she is not poorly like her brothers.

Hattie was just 11 months old when her brother Sidney died of a rare degenerative condition. He was three. Now, four years later, Hattie is watching her two-year-old brother Freddie die of the same condition.

Vicky Read with Hattie and Freddie. Her son Sidney died aged three

But how do you explain to a five-year-old why she has already lost one brother and is about to lose another? Sometimes her mother is at a loss over what to say.

It is hard to appreciate fully just how many tiers of support are needed for the 20,000 British families who, like the Reads, are caring for a dying child.

It's not just a case of looking after the sick child, there's the emotional and practical support needed by the parents, siblings, grandparents and friends whose lives are falling apart. This help might be needed for weeks, months or even years.

When Vicki, 35, and her husband John, 43, were told that their seven-month-old son had just weeks to live they contacted the Ellenor Lions Hospice, which provides at-home care for sick children in Kent, through its Shining Lights team of nurses.

When the team started in 1994, it was one single nurse visiting dying children in their homes.

Now the charity offers a bank of specialist nurses available day and night to the 60 families on their books, as well as doctors, counsellors, social workers, play therapists and music therapists. These resources are all available, free of charge whenever the families need them.

'To lose a child is every parent's nightmare, but the prospect of losing both my sons is almost unbearable,' says Vicki, from Crayford in Kent.

'I simply couldn't have managed without Shining Lights. They were there the day I brought Sidney home to die, they held me the morning I found him dead in his bed, they've been by my side as I've watched my second son deteriorate. They are my sanity, my backbone, my saviours.'

But despite their unimaginable importance to families such as the Reads, the recession is putting such services at risk. Children's hospices rely on charitable donations, and the economic crisis means these have dropped dramatically.

The Shining Lights nurses have been an invaluable help to Vicky and John Read and their children

Only yesterday it was reported that a third of hospices will not be able to raise the money they need to fund their services this year.

'I've been told it costs £13,000 to keep the Ellenor hospice going for a single day and almost all of that comes from donations,' says Vicki. 'It's a huge amount of money, but to me and all the other families they look after, it's priceless.'

Vicki and John's first-born son was rushed to hospital when he was just 12 weeks old, after he started to have a fit in the middle of the night.

For four months he stayed at Guy's Hospital, in London, while doctors desperately tried to bring his fits under control.

Finally, when Sidney was seven months old the Reads were given the devastating news; their son had a terminal condition and had weeks to live.

'For four months we spent every day at the hospital watching the seizures removing the light from our tiny, beautiful, helpless boy,' remembers Vicki.

'He lost the ability to do simple things - like smile, sit and eat. He became deaf and partially blind, but no one could tell us why. It was hell.

'Then in November 2001 we were told that Sid had a very rare condition called Alpers' Disease, which is a progressive degenerative disease of the central nervous system. It causes seizures and severely affects the brain and the liver.

'We were told we'd be lucky if we had weeks. They said we should take him home, to try to enjoy some life at home with him. We were in shock. What had we done to deserve this?

'The hospital told us then about a team of nurses who would help us in our son's final days. One of the Shining Lights team came into the hospital to meet us and talk to the doctors about Sid's condition. I couldn't tell you what she said. It was more than I could handle.'

In the middle of November 2001 the Reads brought Sidney home to die.

Vicky says: 'A Shining Lights nurse called Ellie helped the family.

'She had organised for all the machines to feed him and monitor his breathing to be delivered and set up in his room. It was such a shock to see the house looking like a hospital. In the early days Ellie was there every day, acting like a coordinator, putting us in touch with the medical team, and talking to us about what would happen.

'At first, I resisted their help, but there was so much to take in. Not only were we trying to come to terms with losing our son, but we were trying to manage the monstrous machines that were keeping him alive. Then there were medicines to give him morning, noon and night - anti-convulsants, secretion driers, reflux medication, a whole concoction.

'Every day we had up to three visitors. The physio would work on his joints because seizures make them stiff.

Hattie with her brother Freddie who sits in a specially-made chair. The Shining Lights nurses organised all the machines and equipment needed to take care of him and his late brother Sidney

'The occupational therapist would do exercises with his hands to keep them open and help him touch things. The dietician would try to control Sid's constant vomiting by fine-tuning his feeds.

'It was overwhelming. I felt frustated and resentful at first. Sometimes I'd look around the house and think: "This isn't my life." You lose your privacy, but these people are keeping your son alive so you are grateful and try to accept it. But we were living with a timebomb. Every day, we woke up wondering: "Is this going to be the day?" We were just waiting for his last breath.

'I tried to be superwoman. It was my way of keeping out emotions. Then one day I broke down. I was alone in the house and there was no milk for my tea. I sat in the kitchen thinking: "Is this it for the rest of my life? I can't even leave the house to get milk."

I called the hospice and within the hour someone had popped in with a pint and from that moment I accepted them.

'From then on, I would ask them all the questions flying around my head,' says Vicki.

'Like what actually happens when he dies? How do I organise a funeral? All the questions I couldn't ask my friends, who found it hard to know what to say.'

'It can be hard for some families to let us in,' says Debbie Carroll, one of five Shining Lights nurses who has cared for both Sidney and Freddie.

'We are turning up at the worst time of their lives. We try to approach every family differently, and be there as and when we're needed - be it on the phone or in the house. Every case is different.

'Although we can't make a difference to a child's future, we can make a huge difference to their journey. It's about being positive, keeping the family as a unit and focusing on the good times instead of the bad.'

And this is exactly how Vicki and John learned to cope.

'Slowly, we got used to this life. We decided to stop sitting around and waiting for Sidney to die. We started taking Sid out, visiting friends and going out to the park - normal things.

'We contacted Milwall football club and told them about Sid. They used him as a mascot at one of their games and gave John and Sid a tour of the changing rooms and let them stand on the pitch.'

Weeks turned into months, turned into years and Sidney defied doctors' expectations by surviving. The hospice team were still coming every day and became part of the family. They even joined the family at Sid's christening.

The Reads decided to try for another child, despite being told that because they were both carriers of the defective gene that had caused Sidney's condition, there was a one-in-four chance this would happen again.

Hattie was born in January 2004.

'It was a big gamble because there are no tests for this condition,' says Vicki.

'We just had to go home and wait to see if she would be affected. We got past that 12-week point, and she was healthy. It was a miracle.'

But 11 months after this miracle, Sidney died on the morning of December 14, 2004.

'We had started taking him to a special needs nursery and he'd been cast as an angel in the school's nativity. I had spent all night sewing blue tinsel around the edges of his angel wings, so they wouldn't be girlie.

'That night I went up to show him, but he must have taken one look at them and thought: "Sod that, I'm going to get my real wings.''

'I woke up at 8.30 the next morning and there was absolute silence in the house. I just knew he'd gone. I went into his room and saw his face. It looked really peaceful, but I was so devastated I could hardly breathe, I screamed.

'The first people I called - even before my husband, who was at work - were the Shining Lights team. By this stage, they felt like family. I knew they would know what to do and could be with me straight away. I was in a state of panic.'

Although such a reaction may seem unusual, the nurses are able to offer practical help in a way some family members might not.

'Families don't know what to do the minute someone dies,' says Debbie, who has worked as a Shining Lights nurse for six years and was in adult palliative care previously.

'We can be there to help with the practical arrangements, to call the GP or organise a death certificate, we encourage parents to hold their child and really take their time to say goodbye. We arrange everything that's needed.

'Yes it's sad, and some people think it must be terrible doing my job, but it's the most rewarding job anyone could do. If we can make those final days comfortable and less frightening for the parents, then I consider that a huge privilege.'

Two hundred people attended Sidney's funeral, including all his nurses. They wrote messages on white balloons that were released into the heavens, and watched the white casket take Sidney away.

'It was a beautiful day, and I couldn't have done it without the hospice nurses,' says Vicki.

'Several came to the funeral, some on their day off. I will be eternally grateful to them.

'For the next year, my heart crumbled to pieces. Even though Sidney wasn't around any more, the nurses rang every day to see how we were doing and organised bereavement counselling.

'You spend a lot of time crying, but eventually you realise you can't shut yourself out, that you still have one child who needs you.

'We were desperate to have more children. Because Hattie was fine, doctors couldn't say whether Sidney's case was a one-off or whether there was still a one-in-four chance of passing it on to another child, as we'd been warned previously. It was a huge risk to take, but we were both prepared to take it.

'In December 2006 I gave birth to another beautiful blonde boy, who was the image of his big brother. Fourteen weeks after his birth, on Mother's Day, we were sitting in the kitchen when I saw a funny twitch in his eye - just like Sidney.

'At first I couldn't say anything, I just watched him. Then I picked him up and squeezed him really tight. I thought if I hugged him, and he felt all my love, it would stop. But it didn't. It all started again.'

Freddie was taken to the Evalina Children's Hospital, part of Guy's and St Thomas' Hospital in London, where he stayed for three months having tests before being sent home, just as his brother was, to die.

'The same nurses who cared for Sid are now looking after Freddie. When they arrived at the house again, I could feel their emotion, their sadness - but they're so professional about it. We started making arrangements for physiotherapy, occupational therapy, the nutritionist - it's all strangely familiar.

'They have also been involved in helping Hattie to cope. We have a nursery nurse who plays with her and takes her out. Hattie was only 11 months when her big brother died, but she is five now and aware of everything. She says Sid comes to visit her, that he comes into her room and gives her a hug. I'd like to believe that.

'She knows Freddie is poorly which is why he can't walk, talk or play like other children can. It's very hard to know how much to say and it helps to have someone else involved.

'Freddie is much more alert than Sid was and we try to live a normal life - last month we went to the 30th birthday party of one of the nurses. Like Sid, Freddie can't smile, but he can cry and even though his eyes roll like a jackpot machine sometimes he'll lock his eyes at you for just a second. It's magic.

'My greatest sadness is that we won't have any more children - you think lightning doesn't strike twice, but it does. But despite everything, I am so happy the boys have come to me, they are a blessing. They needed someone to love and look after them and that's what they got - from their family, and the hospice team.

'The idea that these hospices might have to cut services because of lack of funding breaks my heart. I cannot imagine how anyone could cope without them. Unfortunately, it's just not something you know about until you are in this situation.

'This winter has been very hard; we thought we were going to lose Freddie. His breathing difficulties are getting worse, but he is sticking in there - he is our little lion cub.

'We tell him that he can go - whenever he wants - that he doesn't have to stay alive for us, but he'll go when the time is right. Until then I know I have all the help I could ask for. I just hope other families will be able to say the same in the future.'

• To make a donation to Ellenor Lions Hospice, tel: 01322 422831 or visit www.justgiving.com/ellenorlionshospices

Internet Hoax leads to scare!

When Andrea Hodges' 3-year-old nephew Zander got sick, she gave him over the counter medicine.

But a few days later, she said she received an e-mail that would terrify any loved one.

"As I was scrolling down here, I saw that there was bleeding in the brain," said Hodges.

According to the message, Triaminic brand cough syrup, the brand Hodges gave Zander, contained an ingredient that could cause seizures and strokes in kids under the age of four. Hodges said she called the 1-800 number on the back of the bottle, and the person on the phone told her to stop giving Zander the medicine.

This is where the story takes a complicated twist: the e-mail had nothing to do with the reason the Triaminic representative wanted Hodges to stop giving Zander the syrup. The e-mail is an urban legend that's been going around the Internet for nearly 10 years. And the ingredient that supposedly causes seizures is not even listed in the ingredients on the Triaminic label.

Here's why the company told her to stop medicating Zander. Late last year, children's cold and cough drug companies decided to voluntarily re-label their products to say the medications shouldn't be given to children under the age of four.

Andrea Hodges said she's done with over-the-counter remedies and said from now on she's sticking with tender loving care, the cure she knows will always get her busy boy back on his feet.

Here are some tips for medicating your kids:

• Never give adult medicine to kids.
  
• Never give two or more medicines with the same ingredients at the same time and never give a child antihistamine to make them sleepy.

If you have any questions, talk to your doctor. Most say a few days of rest and lots of fluids will usually take care of most colds.

According to the Triaminic website the re-labeling process takes time. So it's likely there are still bottles left on store shelves, like Andrea Hodges found, that include a dosage for kids under four. But the company says kids under four should not use their products.