Pediatric Multiple Sclerosis
What is multiple sclerosis?
Multiple sclerosis (MS) is thought to be an autoimmune disease that attacks the central nervous system (CNS), causing episodes of neurologic symptoms. These neurologic symptoms may include weakness, numbness, tingling, difficulty balancing, bowel or bladder problems, or changes to vision.
The central nervous system consists of the brain, spinal cord and optic nerves. MS primarily attacks the coating around nerve cells called myelin. The attacks leave spots (called plaques or lesions) that interfere with nerve conduction and produces the symptoms of MS. Symptoms of multiple sclerosis vary depending on the location of lesions.
Multiple sclerosis affects about 1 in 1,000 people. About 450,000 people in the United States and Canada are living with multiple sclerosis. Although the peak age of diagnosis is between 20 to 50 years old, approximately 2.7% to 5% of people are diagnosed before the age of 16, with the majority of these cases diagnosed after the age of 10.
Multiple sclerosis is more common in women than in men. It is more common in Caucasians than in Hispanics or African Americans, and more common in temperate areas of the world away from the Equator. It is rare in Asians and other groups.
Causes of multiple sclerosis
The cause of multiple sclerosis is still unknown. Researchers believe there is a genetic predisposition that is triggered by some environmental factor, such as a viral infection. That means that MS is not genetically passed down from one generation to the next, like hair color or eye color, but a combination of genes can make one person more susceptible to the disease than another person. Subsequently, the average risk of developing multiple sclerosis is 1 in 750, but the risk of a child whose parent has MS is 1 in 40.
Symptoms of multiple sclerosis
Signs and symptoms of multiple sclerosis include:
- Changes in vision
- Problems with bowel/bladder
- Emotional changes (including depression and anxiety)
- Speech difficulties
- Problems with thinking and memory
Evaluation and diagnosis of multiple sclerosis
Children who are diagnosed with MS have a type called relapsing-remitting MS. This form of MS is characterized by recurring attacks causing new or worsening neurologic symptoms, followed by periods without new symptoms, called remissions.
At the time of a first attack, it is not always clear whether the diagnosis of MS is appropriate since relapsing disease has not yet occurred. New relapses or attacks will confirm your child’s diagnosis and provide insight to help their doctors better manage their care.
There is no single diagnostic test for MS. In most cases, MS is diagnosed with a combination of methods. Your child’s healthcare provider will take a careful medical history and will order tests that may include:
- A neurological exam
- An MRI to check for lesions in the brain, spinal cord or optic nerves
- A lumbar puncture to check the cerebral spinal fluid for antibodies and proteins associated with MS
In some patients, the first MRI scan shows multiple lesions that are so consistent with MS that an MS diagnosis may be given after only one clinical attack has happened. While not as common, early diagnosis can offer an important opportunity for early treatment.
Treatment for multiple sclerosis
There is no cure for multiple sclerosis. Treatment is focused on managing symptoms during attacks, as well as slowing the disease progression to prevent relapses and limit formation of new lesions in the brain or spine.
Your child’s care team will determine the best treatment based on your child’s symptoms and condition.
Treatment of MS relapses
Steroids (corticosteroids, prednisone, or methylprednisolone), most commonly IV methylprednisolone, are used for the acute treatment of MS symptoms. IV methylprednisolone is given only for a few days. If needed, oral prednisone may be prescribed for a further one to two weeks.
Serious side effects are possible, but they are not common when treatment is short. Less serious side effects are common, however, and these include irritability and difficulty sleeping. Giving the steroids in the morning reduces the impact on sleep. Upset stomach is common and medications that reduce stomach acid are typically given to prevent this. High blood sugar levels may also be a side effect that resolves when the steroids are stopped.
Intravenous immunoglobulin (IVIG) is an IV medication that is made up of antibodies from healthy blood donors. These help decrease the unwanted immune response that occurs in MS. The most common side effect is headache, but other side effects include muscle or joint pain and low-grade fever. IVIG is not usually the first treatment used for an MS relapse, but may be used in certain situations.
Plasma exchange, also called plasmapheresis or PLEX, is used when neurologic symptoms are difficult to treat. It is a process where the blood is removed from the body and washed to remove disease-causing substances, such as antibodies from the plasma (the liquid component of blood). The blood cells are then returned to the body. Multiple treatments are required, given over a couple of weeks. Plasma exchange requires the surgical placement of a special line for IV access. Risks include changes in blood pressure and fluid balance as well as infection of the access site.
Medicines to prevent relapses and progression of MS
Treatments to prevent relapses and progression of MS may include the following categories of disease-modifying agents. Decisions about the best disease-modifying therapy can be complicated and are always made while considering other health conditions and what is best for each individual patient.
- Interferons (Avonex®, Rebif®, Betaseron®, Plegridy®): These medications mimic the effects of some proteins that your body can make to change how your immune system works. They are used to decrease the number of relapses and new lesions on MRI. Common side effects include flu-like symptoms shortly after the injection and injection site pain. Regular blood tests are required to monitor the medication. The type and frequency of injection is different with each of these medications.
- Glatiramer acetate (Copaxone®): This medicine is given by subcutaneous injection (just under the skin). This medication looks like one of the proteins that makes up myelin in the brain and spinal cord. The injection is given daily or three days a week. Common side effects include injection site reactions and, rarely, a reaction with chest pain. Regular blood tests are not required.
While there are several oral medications used in the treatment of MS, only fingolimod (Gilenya®) is currently under widespread use in the treatment of children with MS.
This medicine is taken daily and decreases relapses by trapping certain white blood cell in the lymph nodes to decrease immune system reactions. Blood testing is required before starting fingolimod and during treatment because it is associated with an increased risk of infection. Fingolimod also requires close monitoring with an eye doctor as it may cause an eye problem called macular edema.
The first dose of fingolimod is administered in a hospital or medical office setting because it can lower your heart rate. If you stop and then restart fingolimod, you will have to be monitored again when you restart it.
Infused medications such as Rituximab®, Tysabri® or Ocrevus® are given by IV infusion in a hospital setting. They are associated with increased risks of infection and require blood tests before starting as well as close monitoring and blood testing during treatment. These medications are most often used when injectable or oral medications have not been effective, but may be used in other settings as well.
Other treatments for MS
In addition to medication, certain lifestyle factors can help decrease relapses and manage MS symptoms.
- Taking vitamin D if needed. Having enough vitamin D decreases MS disease activity. We will check vitamin D levels and may recommend taking vitamin D3. Vitamin D may also decrease the risk of disease in other family members.
- Avoiding cigarette smoking. We know that exposure to cigarette smoke increases the risk of MS. Please don’t smoke, and avoid exposure to second-hand smoke. Family members who smoke should smoke outside and change clothes when they come inside.
- Eating a healthy diet and exercising. It is extra important for people with MS to be sure that they are making healthy choices. A healthy diet, regular exercise, and plenty of sleep are all essential. Maintaining a healthy weight is important.
Follow-up care for multiple sclerosis
Close follow-up for children with MS is vital to the long-term outcome, and this is provided in the Pediatric MS and Neuroinflammatory Disorders Clinic at CHOP. We follow children closely for evidence of relapse, and to monitor the efficacy of medications. Follow-up MRI studies will also be needed on a regular basis, and we will review those images in person during clinic visits.
Long-term outlook for children with multiple sclerosis
MS is a chronic condition that needs to be managed throughout life. The course of the disease is difficult to predict and varies from person to person. Some have long periods of remission while others have more frequent attacks.
Why choose us for multiple sclerosis care
The Pediatric Multiple Sclerosis Clinic at Children’s Hospital of Philadelphia provides a multidisciplinary approach to caring for children with MS. Your child will see a team of experts who specialize in demyelinating diseases like MS, and who are involved in cutting-edge research in MS and other neuro-inflammatory disorders. Your child’s care team will include a neurologist, nurse practitioner, nurse, occupational therapist, physical therapist and social worker. The clinic also partners with other specialties at CHOP such as ophthalmology, urology, pain management and neuropsychiatry to coordinate your child’s care as necessary.
Our team is actively involved in research studies to learn more about childhood demyelination and how it affects children and teenagers.