Pediatric Treatments

Taking medications for any condition is always challenging. Taking medications for a chronic disease is even more challenging due to the long duration of treatment and the number of medications you may need on a daily basis. It is therefore important to understand the reason for the different treatments you are receiving. It is also important to understand what happens if you would not take the medications as prescribed by your medical team.

The major reason you receive treatment for your vasculitis is because “systemic” vasculitis can be a severe disease which can lead to organ damage or failure, and can potentially be life-threatening. Luckily, in the last two decades, better treatments have become available for vasculitis and these medications are more effective and cause fewer side-effects.  However, virtually all medications available today have the potential to cause side-effects.  For example, medications which suppress the immune system can increase the risk of infection. Corticosteroids (i.e. prednisone, prednisolone, IV methylprednisolone) can have a negative effect on bone health.  Certain medications may cause stomach upset.  You might need additional medications to prevent or treat the symptoms of these potential side-effects. Your health care team will watch out for side-effects.  It is also important that you quickly communicate any symptoms which you think may be related to your medications or your disease. 

Always ask your doctor if you do not understand the reasons why you are taking certain medications. Understanding what the medications are for – i.e. controlling the vasculitis, the prevention of organ damage, or prevention of medication side effects – is really important as it will help you to know the risk and benefits of the medications you are prescribed. It is also very helpful for you to learn the names of your medications and to know the doses which you are on. If you cannot remember all the names, you can keep a written a list of medications, take pictures of the bottles or bring them with you to appointments. Try to keep track of when you need refills so that you do not run out of medications. 

Successful treatment of vasculitis usually includes induction and maintenance phases. “Induction therapy” is the initial treatment and takes place during the first 3-6 months after you are diagnosed with vasculitis.  After the vasculitis is quieted down during the induction phase, maintenance therapy is started to help keep the disease quiet.  Sometimes induction and maintenance phases can include some of the same medications, for example, rituximab can be used for induction and maintenance.  However, often during the maintenance phase, the medication dose is lower.

In vasculitis, there can be symptoms of systemic inflammation that affects the whole body (fevers, weight loss, feeling fatigued or tired), as well as local inflammation that can affect certain organs (such as kidneys or lungs).  The goal of vasculitis treatment is to have your vasculitis enter “remission”, which means that the signs and symptoms of the disease, both systemic and local, are totally controlled, and actually disappear.  To accomplish this, we use medicines which suppress the body’s immune system (immunosuppressive medications).  Corticosteroids, or steroids for short, which can rapidly suppress inflammation, are frequently the first medication given for treatment of vasculitis, and may work quickly to help you feel better.    

Very often, additional immunosuppressive medications are added to the treatment program to more completely quiet down the inflammation associated with vasculitis.  These can include medications which are effective and have been used for many years, such as cyclophosphamide, methotrexate, and azathioprine.  Other medications used to treat childhood vasculitis include those developed over the past one to two decades and which target a specific part of the immune system.  Examples of targeted medications, also known as biologics, are rituximab, infliximab and tocilizumab.  The choice of medication will vary based on your vasculitis diagnosis and which organs may have been affected by the vasculitis.  For example, rituximab is often given to treat ANCA-associated vasculitis, such as GPA and MPA, because it decreases circulating antibodies found in these diseases.  Infliximab and tocilizumab may be given to patients with Takayasu arteritis 

For more information on medications used to treat vasculitis visit our Treatments page. 

Most of the knowledge that we have on how to treat vasculitis is based on studies performed in adult vasculitis patients.  Sometimes patients as young as 16 years old are included in these studies, but most of the time the studies include only patients who are 18 years of age or older. This means most of the treatments we use for pediatric patients (age < 18 years at age at onset of disease) are based on adult studies. Some registries (data collected at multiple pediatric hospital sites) have been established and studies are currently being conducted to evaluate the dosage and use of medications in children. For example, pediatric rheumatologists from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) have developed treatment protocols to treat ANCA-associated vasculitis in children.

You might also be invited to participate in specific research registries that either take place in your center or sometimes internationally.  Although participation might not help you directly, the ability to look at your response to medication could help someone diagnosed with vasculitis 5 or 10 years from now.

Besides treatment for your primary disease (systemic vasculitis), you may also need additional medications to decrease possible side-effects of some of the treatments or to protect the body from some of the problems that vasculitis may have caused.  For example:

• If you are treated with glucocorticoids such as prednisone, you will usually be asked to also take calcium and vitamin D supplements. This is because we know treatment with glucocorticoids can affect your bone health and we want to make sure your bones stay as healthy and as strong as possible so you can participate in activities you enjoy now and in the future.
• If you are on a medication which suppresses your immune system, you are at an increased risk of developing an infection. The risk might only be minimal and in that case, you will be asked to be vigilant and if you show any signs of infection, you should be seen to be evaluated for infection. Some medications bring a specific risk with them (for instance, treatment with rituximab or cyclophosphamide may increase risk of infection with a certain bacteria, PJP) and in those cases you might be asked to take a preventative antibiotic which is usually taken three days a week (Mon/Wed/Fri).
• Sometimes the vasculitis has already caused an injury to an organ and we will want to protect the organ from further damage. For example, if the kidney has been affected by the vasculitis, it may leak protein or lead to high blood pressure. In this case, you will likely be started on a medication which helps to control your blood pressure and also may help to protect the kidney from further injury.  Although it sometimes is difficult to take additional medications, these treatments are very important to prevent further injury to vital organs already affected by the vasculitis, even if you do not notice a difference or feel any differently.  The goal is to protect the health of your organs so that you will not develop more issues when you are older.
• Some medications can cause stomach pain or nausea.  If this happens, you might understandably think about lowering or avoiding your medication, but this can backfire and harm you.  If you feel unwell, please reach out to your health care provider, who can prescribe treatments to protect your stomach and help you to feel better while you remain on these medications. For example, if higher doses of glucocorticoids cause a stomach ache, you can be given medication to prevent or treat these symptoms.

As a child or teen living with vasculitis, you likely will be prescribed medication to manage your disease. Some of the medications used to treat vasculitis may have unpleasant side effects. For example, glucocorticoids may be associated with unwanted changes in your appearance and mood.

It is important to acknowledge how you feel about taking your medication and remember that it is normal to experience negative feelings when you are dealing with challenging side effects. Keep in mind, the importance of taking your medication as your doctor recommended and also the reason that you are taking your medication (to slow or stop your disease and get back to doing the things you love most).

As a parent to a child taking medication, you can support your child by validating their feelings and maintaining expectations for medication adherence.

Find helpful resources for dealing with fatigue and mental health challenges on our Managing Symptom & Side Effects page. 

Learn tips for dealing with the side-effects of prednisone and other glucocorticoids on our Prednisone page. 

Some medications for vasculitis are taken by mouth and need to be swallowed. This can be a challenge if you have never swallowed a pill before or if you are not used to swallowing pills. When learning how to swallow pills, your parent may break the task down into smaller goals, working toward the larger goal of taking the medicine. You should be proud of every step of progress that you achieve toward the goal of swallowing pills. It is often recommended that your parent have you first start with a sip of water and then start to swallow candies of different sizes, increasing the size from very small to larger. For example, start out with smaller candy, such as sprinkles, Nerds®, and mini M&M’s®, and work towards larger candy like regular M&M’s® or Good “N Plenty.® Be proud of each step that you take toward pill-swallowing. Until you are able to swallow all the necessary pills, your parents may decide to put the pill (usually crushed) in a spoonful of pudding, ice cream or peanut butter; or they may cover the pill in a sticky, enjoyable treat such as Fruit Roll-Ups® or Airheads.® Remember, practice is key for pill-swallowing. You will become more skilled and self-confident the more you do it!

Some medications require a needle poke. When receiving an injection, relaxing your body and distracting your mind can help you to calm down your stress level and decrease the discomfort you may feel. You can try taking deep belly breaths, in through your nose and out through your mouth, while inflating your belly with air. Other relaxing activities include: tensing up different muscle groups in your body and relaxing them, or thinking of the place that you want to be. Watching your favorite show or video, or listening to music, can be a great distraction. Even blowing bubbles can distract your mind and relax your body at the same time. 

The things we say to ourselves matter too! Remember to stay positive in the way you think about your injections with thoughts like “I can do this” or “I’m an expert with shots now,” as this will make the experience easier and reduce pain. Sometimes, you may want to put off your injection or ask your parents to do it later, but this will likely make it more difficult for you to cope. Instead, we recommend setting a plan to receive your injection, including a specific day and time to take it and things you can do to help you through it, and sticking to the plan. Receiving special treats or rewards after taking your medication can be motivating and help you stay on track with the plan!

Recommendations for Parents

It will be most helpful for you to stay calm, demonstrate confidence in your child’s ability to cope, promote use of positive coping strategies, and praise your child’s successes. If your child experiences needle fears or has trouble complying with pokes, you can use a comfort hold to help them feel secure and stay safe during the procedure. The nurse on your rheumatology team may be very helpful in reviewing the technique of giving the injection, increasing your confidence, and also can provide some tips for calming your child. For those children who are very fearful of needles, it may be helpful to gradually face the fear of needles by looking at needle pictures or videos of other children getting pokes, giving a practice injection, and then, progressing towards receiving the injection. If these tips do not provide enough relief, your child may benefit from consulting with a psychologist or counselor.