Who Defines Me? A book about SJIA.

Who Defines Me? A book about SJIA.

• 3. Who Defines Me? By: Beatriz Peixoto

• 5. Message This book is dedicated to all of the kids who suffer from JIA. To let you know that you are not alone and that everybody who truly loves you and cares will always care for you and support your final decisions. I also want you to remember that everything is going to be OK and that there is a light at the end of the tunnel. I would like to say a special thanks to: My Mom: Words can not describe how much I love you and how much I thank you for everything you have done in the past few years. I am so grateful to have you in my life and wanted to let you know that you are my hero and that we can get through anything together. I love you mom! My Dad: Thank you for everything you have done. From making me laugh, to writing thousand of emails and paying my bills. We have gone through this journey together and have created a relationship between us which is unbreakable. I love you dad! Rheumatology Team at Kiderspital Zurich: Thank you for all of the patience that you have had with me. You all are very kind and hav helped through all the hard times. Words can not even describe how much I thank you. Keirra: When I was sad, you would pick me back up. You are the most kindhearted people I know and I truly have no idea why you came into this crazy ride along with me. I love you and hope we will always be friends! Cora: Here it is! Thank you for everything and all of your hard work! I truly appreciate it! Mr. Grover: Thank you for all of your support throughout the years. You are the most amazing person I know! You rule! Thank you for everything! Mr. Loughrin: Thank you for helping me write this book and for supporting me in every step of the way.

• 7. Contents Page Chapter 1: My Journey - A Brief History........................................ Chapter 2: The Basics................ Chapter 3: Symptoms................. Chapter 4: Treatments............... Chapter 5: Organizations and Events........................................ Chapter 6: My Future - Where we go from Here......................... 15 27 33 53 45

• 8. SJIA made me stronger. It made me want to keep fighting. It motivates me to say, 'I know I can do this.' Haley, Age 19 Having people who appreciate you and support you means everything! Beatriz, 15 Don’t give up. Keep fighting through and don’t let Arthritis stop you. Tess, 16

• 9. Chapter 1: My Journey - A Brief History I am Beatriz, and I have Systemic Juvenile Idiopathic Arthritis. Being a child with this condition is not easy in many different aspects but there are always ways in which people support you and give advice, especially in the beginning when the change is a big part of your transition. However, you eventually learn to cope with the disease and see yourself like everybody else. You try and think constantly that you are not different, but the thin you need to learn is that, being different is what makes you special. When a child is diagnosed with JIA, it means that their life will change in some way, no matter if it is big or small, positive or negative. You learn that you will have to face new challenges but you also have to remember that every challenge also comes with a benefit later on, even if you might think that benefit is not that good.

• 10. For me, it all started in the beginning of September 2013, at the age of 11 when my symptoms started to appear. It first started with a rash on my legs and then quickly progressed to high fevers (40 ) that would always come in the morning and in the evening around the same time. Whenever the fever came, it felt like it was sucking all of the energy out of my body and eventually all I would do is sleep the entire day. After the fever, I started to feel pain, it was so intense that I could not get up from bed or even move on the bed to change the side that I was on. Then, after many days of going to the emergency room, the doctors decided to hospitalize me to find out what was going on. This was what I feared the most because I had never gone through a hospitalization before and it was completely new territory for me. When I was in hospital, the doctors already knew that it was not a virus so antibiotics would not have helped in any way. They knew this because no signs would show up in the blood work only my CRP (C - Reactive Protein) was high. This result shows

• 11. inflammation signs in the body. By this time I was already on painkillers for me to at least be able t get around. After 7 days in hospital, the doctors found out that I had Systemic Juvenile Idiopathic Arthritis. As I was 11, a lot of excitement came to mind as I would start my secondary school that same year but then that was quickly shattered when the first few weeks of school I had to spend at home. Once I was out of the hospital, doctors started me on corticosteroids as well as other medicines to reduce the anti - inflammatory drugs side effects. And by the end of September 2013, I already was diagnosed and at home and we started reducing the steroids until February of 2014 when we were able to reach a dose of 2.5mg per day. This was a very good sign and it made the entire family think that the disease had finally gone into remission but that was not the case. By March 2014, the rash started to appear again. The doctors then decided to go up with the corticosteroid dose and start with the

• 12. 10 methotrexate treatment. Once I was stable, in November 2014, the doctors decided to try again and start to reduce the steroids because it is not good idea to be a long time on steroids as it can cause many side effects. While I was reducing the steroids, I was still taking subcutaneous Methotrexate injections once a week. In December 2014, my fever started to spike up again so the doctors decided to increase the dose of steroids I was taking again. A short time later, in February 2015, I started a new biological called actemra which was an infusion at the hospital once every two weeks. At this time my schedule was pretty easy as I was in grade 7 and did not have a lot of work to do. In the beginning, actemra looked like was going to be the medicine that would work, that was until May 2015 came. May 2015 was one of the most uncomfortable months I had in my entire life. Everything was OK until my body started to have an allergic reaction to the actemra biological that I was taking. Not only that but it was also no longer working. Once my doctor found this out, in June

• 13. 11 2015 I started the Ilaris which is also known as canakinumab. After spending June, July and part of August with the Ilaris injection, at the end of August 2015, my doctors and I decided to change to the kineret (anakinra). At this time I was taking one injection a day until September 2015 which was when I had my first steroid pulse which lasted for 3 days. In the middle of September 2015, at the age of 13, I did a blood work exam and some of the results did not come out so good so I had to go into hospital again. During this time around in the hospital, I was diagnosed with MAS which is also known as Macrophage Activation Syndrome. After this diagnosis was made I started therapy with cyclosporine straight away as well as doubled the dose of the kineret. Instead of 1 shot, I would tak 2. The doctors also decided to increase the steroids because my inflammation was starting to flare up again. This time of the year was also when I had to make one of the hardest decisions of my life. My body was not well enough for me to do sports and for me to go back being the

• 14. 12 energetic girl that I always was so with that I had to stop playing football for the school team. However new opportunities were starting to knock on the door, I was offered an exchange to go to Singapore and was also offered a position to help the Varsity Boys Basketball Team. In June 2016, we failed again to reduce the steroids so we resorted back to going to the canakinumab medication. We also changed because my legs were really hurt from taking 2 injections a day. However, in April 2017 we failed to reduce the steroids again so we then decided to go ahead and start the again with the double dose of kineret and steroids even though my experience with it in the past was not very good but this time around, it was not as bad. In the end of May and beginning of July 2017, the kineret was starting to affect my liver functions causing my liver values to go through the roof. When the doctor saw this in my bloodwork, she completely removed the kineret and I was admitted into hospital until my liver values went back to normal. After this phase was

• 15. 13 over, we never decided on another treatment. Then in the beginning of October 2017, it felt like a miracle had happened. My family was invited to a conference in the United States on Systemic Juvenile Idiopathic Arthritis that was being hosted by the SJIA Foundation. This was a true blessing because we were able to discuss new treatment methods in the future and also discuss what are the next steps for my treatment.

• 16. 14 Take the medicine your doctor prescribes and don’t forget to take it. Don’t give up an keep moving. Kaitland, 12 Stay positive and have a heating pad. Daniel, 9 It’s tough and you miss a lot of school but if you stay positive, you get through it. Beatriz, 15

• 17. 15 Chapter 2: The Basics Arthritis is a disease that is present in the lives of many people in our world today. However not much research is being done on it in terms of what are its causes and why it happens. Arthritis found in children is different from the arthritis found in adults which is what this book will mainly focus on. Juvenile Idiopathic Arthritis, also known as JIA, is a chronic illness found in children under the age of 16. A chronic illness means that the conditions persists, there is no cure, only treatments in which will help the symptoms go away and also to improve with your health. Also, it is almost impossible to say how long a person will be ill for when a person has a chronic illness (how long the condition will stay active). Juvenile Idiopathic Arthritis is characterized by joint inflammation which leads to pain, swelling and

• 18. 16 limitation of movement in many children. The word idiopathic in the term means that the cause in why the chronic illness happens is unknown. JIA is a very rare disease to find in children toda but at least 1 - 2 children in every 1,000 have it. it is not really clear why this disease happens. Everybody has an immune system that protects your body from infections. Microbes like viruses and bacterias, are seen as foreign things in your body and your immune system can distinguish them from your own cells and notice that they are different. This releases a signal to your brain for you to create other blood cells so that you can protect yourself. JIA creates an abnormal response from your immune system. Your immune system loses the capacity to distinguish your foreign cells from your healthy-self cells which in the end is what leads to the inflammation, because your body is attacking your own body this is why JIA is defined as an autoimmune disease. However, the mechanisms that cause JIA to occur in a child are actually unknown.

• 19. 17 Juvenile Idiopathic Arthritis is not a very common disease for kids to have so, there is not a lot of research done on it. What scientists have already found out is that this chronic illness is not hereditary. This means that it can not be transferred through genetics or transmitted from parents to the child. As far as research goes to tr and find out how this disease occurs, scientists have gone into thinking that it might be because of exposure to the environment. This means that things like viruses, infections or bacteria can be the reason. Another hypothesis that scientists have been looking at is genetic predisposing factors, this is when there has been a very small change in genetics from you to your parents that may have caused this disease to happen. Diagnosing Juvenile Idiopathic Arthritis is an extremely complex diagnosis as there are so many different types of this disease. When doctors are making a diagnosis, they look at many factors like medical history, laboratory tests (blood work) and physical examinations. JIA tends to affect children before the age of 16 and symptoms

• 20. 18 can last for more than 6 weeks at a time. When trying to diagnose as doctors have to rule out every type of bacteria, virus or infection. In children, it is easier to make an arthritis diagnosis through physical examination as a child normally should not have any pain or joint problems. The exact type of arthritis is only discovered after a clear confirmation that the disease the child has is arthritis. This means that some sort o treatment can start to make the child feel more comfortable and more like themselves. Also, the only way to be able to tell what specific type of Juvenile Arthritis a kid has is to look for medical help (a doctor) as a child would need a physical examination. JIA is a disease that normally targets the synovial membrane which is also known as the inner lining of all of your joints. When inflammation occurs, this lining becomes thicker due to the increase in inflammatory cells and tissues. As there is an increase in those 2 things, it will increase the fluid inside the joint causing swellin pain and also limitation in movement. Most kids

• 21. 19 with JIA wake up with morning stiffness in their joints as they did not move a lot at night but this usually goes away after 10 - 30 minutes of moving around in the morning. Normally, a child with JIA sleeps with their joints in a semi - flexed positio as it can reduce pain. In the morning however, this can also lead to muscle contraction which is the shortening of the muscle. This can also lead to joint damage which can be defined in 2 ways; the synovial membrane can become too thick, which causes the loss of joint cartilage and bone but thi can only be seen in x - rays. Another damage that it can lead to is muscle atrophy which is the waste or loss of a muscle meaning that your movement would be restricted in some way. JIA normally is distinguished by the number of joints that affects a young child’s body but tha is not always the case as sometimes, the disease does not only attack the joints but also the organs However, it is distinguished by the number of joints affected the child in the first 6 months of diagnosis.If less than 5 joints were affected in th first 6 months of the child's diagnosis with no

• 22. 20 fever, it is normally called Oligoarticular JIA and if more than 5 joints were affected in the first 6 months with no fever, it is called Polyarticular JIA. Symptoms can also help doctors to diagnose the type of arthritis by the type of rash the child has and how high fever spikes. Systemic JIA is one of the rarer types of JIA found in children as it affects less than 10% of al JIA patients around the world. This is because it i mostly found present in toddlers and preschool children but a child at any age can still get this disease. This type of arthritis is harder to diagnose as organs are also involved and blood work results can be easily manipulated if inflammation from the disease surges. When inflammation occurs, it can happen in your joints as well as your organs like the spleen, liver, lymp nodes, and membranes around the heart (pericarditis) and lungs (pleuritis). Symptoms like a rash and fever come along with the disease but the rash would only be present when the fever is present as well (fever comes in certain periods of time of the day) as well as muscle pain. Half of th

• 23. 21 Half of the patients diagnosed with systemic JIA have long term prognosis meaning that it will take the disease a long time to go away and the other half, the disease tends to subside. Polyarticular JIA is a type of juvenile arthritis that affects 5 or more joints in the first 6 months of the disease after all of the symptoms are under control. Polyarticular JIA is measured by 2 different blood evaluation in the rheumatoid factor (RF). RF negative is present in 15 - 20% of all polyarticular JIA cases and it can affect a child o any age and also any joint can be affected (big or small). RF positive is more of a rare type of arthritis to see in children as it affects less tha 5% of JIA patients. RF positive is more commonly seen in adults and it normally only affects small joints and it is symmetrical meaning that it would happen to both sides of your body. If Polyarticular JIA is discovered at an early age, the earlier the treatment is in place the better because there is a higher potential of the treatment working, but doctors also have to keep in mind that every child reacts differently to a certain treatment.

• 24. 22 Another type of JIA is Oligoarticular JIA and this is the most common type of arthritis in children affecting 50% of all of the cases. This type of arthritis can be found in 2 different ways; persistent or extended. When Oligoarticular JIA is persistent, it means that the first joints that wer affected in the first 6 months are the only joints that will be affected throughout the disease and when a child has extended Oligoarticular JIA, it means that the joints can constantly be affected and there is nothing the child can do to stop it bu to try new treatments. This subtype of JIA normally manifests itself before a child reaches the age of 6 and it is also more commonly seen in girls. One way to detect this type of arthritis is by noticing eye complications like anterior uveitis bu in most cases, this problem passes by unnoticed due to the many other problems happening to the child’s body. Psoriatic arthritis is another type of JIA. This type of JIA is associated with psoriasis which is a skin inflammatory disease which causes patches of scaling skin, usually on the elbows and the

• 25. 23 knees and the elbows are not affected, the nails are affected instead. JIA can be discovered in psoriasis when there is also the swelling of the finger and the toe (dactylitis) or nail pitting. If the arthritis in onset when a child has this type of arthritis, they also have to get their eyes regularly checked as uveitis can occur. This type of arthriti is very tricky to treat as children all over the world have different types of skin meaning that the treatment will vary from person to person but scientist have found out that if the child has Psoriatic arthritis but less than 5 joints affected the child can be treated with the same treatment as a child with oligoarticular arthritis and if mor than 5 joints are affected, the treatment is the same as the one to a child with the polyarticular type of arthritis. Arthritis can also be associated with another disease called enthesitis which most commonly manifests itself in the larger joints of the lower limbs or other places like heels and soles of feet. The enthesitis is the place in which the inflammation occurs causing the person a lot of

• 26. 24 pain. What scientists have discovered however is that if a family tests positive for a HLA B27 laboratory test, it most likely means that the family has had the predisposition to the disease. This type of arthritis normally does not really affect children under the age of 6 but if a child does have it, doctors do have to be careful as the inflammation can grow out towards the lower spine and affect the sacroiliac joints which will then limit the child’s movement on the back. If a child is affected with this type of pain in their lower back, it is called ankylosing spondylitis. JIA does not only cause problems in the joints and some organs, sometimes it can lead to other types of autoimmune diseases like iridocyclitis which is a type of eye inflammation. This is normally seen in patients diagnosed with IA (intestinal atresia) or a positive ANA test. The ANA is a test taken to find out if a person has an autoimmune disease or not, it is normally done by taking blood from the patient. The link of this to the disease is unknown but even if the arthritis has been said to be remission, the patient should

• 27. 25 still get regular check up on their eyes as it can relapse at any point (even without inflammation). JIA patients who take cortisone also need to get their eyes regularly checked as the use of steroids can lead to a cataract causing blurry vision and sensitivity to light and also high blood pressure. Arthritis in children is different to arthritis in adults as so many different factors come into place when talking about symptoms and also how it affects a person’s movement. Arthritis has a Rheumatoid Factor (RF) to it. This means that there are certain things that are visible in blood work and others that are not. The difference is that on adults, the RF affects at least 70% of them while it affects less than 5% in kids. This is because as you get older, it is more typical to fin problems in blood work rather than physical exams. However, systemic arthritis is a type of arthritis that can only be diagnosed through blood work.

• 28. 26 • I feel broken • I feel as though everyone knows I am different • I wonder what it is like to be “normal” without arthritis • I always feel tired and worn out, I wonder what i is like to feel happy and lively like all my friend Jazmin, Age 11 Shots hurt at first but as you get used to it, it starts to stop hurting. If you are doing the shot, you can as them to numb your leg or count to 3 or to whatever number you prefer. Another thing you could ask is t hold their hand and squeeze it. Caroline, 10 Never give up because you are never alone. Laughing and being joyful will always make you believe. Miranda, 17

• 29. 27 Chapter 3: Symptoms The symptoms of Juvenile Idiopathic Arthritis are very tricky to detect in many times as they can be thought to be something else and they can also change day to day. There are many different symptoms that can lead to a doctor to a JIA diagnosis on a child but each kid will have different symptoms. Some characteristics of the symptoms of JIA help lead to a diagnosis of a subtype of JIA (a specific type). Doctors normally never diagnose JIA right away as they wait to see if there are patterns in the symptoms which will then help confirm that the disease is really JIA. The symptoms that are most commonly seen in JIA are rashes, fever, eye problems, pain, weight loss or gain, swelling and stiffness. Rash: The most common symptom of JIA is a faint pink rash which can normally be found over the knuckles of your fingers, cheek, nose arms

• 30. 28 and legs. It does not normally itch and it also tends to come and go either with a fever or fade out throughout some days. The hard thing about detecting a JIA rash is that it can be confused wit an allergic reaction, eczema or poison Ivy. Fever: Another very common symptom that is seen in almost every single case of JIA is fever. The fevers normally tend to go up as high as 40 (104 ) and be very frequent. Sometimes they have a schedule and only come at certain times of day and then leave without any medication but that is normally only in rare cases. Eye Problems: JIA can also be diagnosed through recognizing eye complications in a child such as iritis which is an inflammation in the eye or uveitis which is an inflammation in the middle layer in the eyes. However these are not the only symptoms that the eye can show as a child can all of a sudden start complaining about blurred vision due to light or even pain in the eyes. In some cases, the eye also might even present to have a little redness which many doctors may also confuse as pinkeye.

• 31. 29 Pain: Arthritis can cause 2 different types of pain. The first type of pain is joint pain which usually will happen after the child has had a long day of activity. The second type of pain is muscle pain which normally presents itself in the morning. The JIA pain can affect your entire body and it can develop slowly over time. The problem with this JIA symptom is that not always pain medication like ibuprofen and acetaminophen work. Weight Loss and Weight Gain: If the child used to be a healthy kid and was not really a picky eater but now is, this could be one of the symptoms of JIA. It all mainly starts of by the child saying that they are not hungry or even just saying they might not like the food. The child's weight would go down increasingly fast and also their energy levels go down. However, it is not only weight loss that can cause the diagnosis but also weight gain and the child is overeating because of the urge of constantly feeling like they are hungry. Swelling: Not all types of arthritis show swelling in the joints however, the skin can also feel hot o

• 32. 30 warm to the touch. Swelling can also be seen by showing redness on the skin but it is not necessarily a rash. When swelling does actually happen, it usually is one of the strongest symptoms and it happens in bigger joints like hands, feet and knees. Stiffness: Stiffness is one of the symptoms that might come later on after the first few weeks. It i usually present in the morning when the child first wakes up and can get either better or worse throughout the course of the day. You can notice stiffness if the child is holding their joint in th same position or even struggling to perform normal movement like getting up from bed or holding a spoon.

• 33. 31 Sometimes you will get bullied but that’s ok. You are you. Nothing can break you. If you are in a sad situation, ask someone for help. Angie, 10 I now know that there are other kids like me and that makes me happy. Crystal, 13 My Mom keeps up on everything medical with me. I am lucky it was caught early and nothing will stop me. Drake, 14 Sometimes you will get bullied but that’s ok. You are you. Nothing can break you. If you are in a sad situation, ask someone for help. Angie, 10

• 34. 32 I was diagnosed with JA in 2009 and life has pretty much been flipped upside down. But my advice to you is to find someone who has JA as well. Because it will most likely be that the stuff that you are going through, they have gone through as well. Anna, 16 When you hurt remember you are not alone. There are many kids who are dealing with the same things. The thing to remember is that arthritis gives you strength. Allie, 16 I've had arthritis now for about a year and here are some things I’ve learned. When you have shots that make you feel sick, usually a cold drink and nausea medicine helps. Also, if you are feeling down and really tired it helps to watch a movie with family and friends or even cuddling with a pet. Emily, 15

• 35. 33 Chapter 4: Treatments There is no cure for JIA however, there are existing medications and treatments in which help control the symptoms like pain and inflammation which then leads to the improvement in the quality of life of the patient. One thing that has shown significant results in JIA is that the earlier you start a treatment, the higher the chances are that the child will go into remission. JIA has many differen types of treatment as a certain treatment might work for one child but might not work for the other When treating JIA, what doctors try to do to make the immune system more relaxed so that it does not attack the good cells of your body which is what would have been causing the inflammation. However, making your immune system more relaxed, it means that the risks for infections and viruses increase because your defense system is not as good.

• 36. 34 When a child first receives the diagnosis of JIA, the doctor will probably prescribe medication to manage the symptoms. Further treatment would then be discussed. In the beginning, the objective is to hit the disease hard so that all inflammation signs go down and to prevent any further damage to happen. Next, further discussion about what treatment will take place has to be done to try and prevent the disease from progressing because it can cause the destruction of the child's joints, bones, cartilages, and soft tissues like muscles, tendons and joint capsules. Most of the time, the medication the child has to take will be given in a pill form. NSAIDs is one of the treatments most commonly used when first diagnosing JIA. NSAID stands for non - steroidal anti inflammatory drugs. What this treatment basically does is that it relieves the inflammation symptoms in your body (anti - inflammatory part of drug). It also works a an antipyretic which is what helps keep fevers down. The only down side to NSAIDs is that they can not induce remission to the disease, it can

• 37. 35 only control it. Other more inexpensive drugs like ibuprofen or naproxen are not used as much today as they have a high risk of toxicity which can cause liver toxicity and high blood levels. When a patient has oligoarticular arthritis and have one or more joints affected, the treatment option they would go for is joint injections. This is because it will relieve the pai and allow them to move a lot better as when they have a large amount of inflammation in the joint, i restricts their movement quite a bit. The injection is a long acting corticosteroid preparation which i injected in a place with local anesthesia. This typ of treatment can be repeated more than once but it is not recommended that a child does it more than 3 times in 1 year. Joint injections are also used as a combination with other treatments in JIA like bridging agents as they can achieve rapid improvements in a short period of time. When a child shows to have a severe or a progressive case of polyarthritis, it is recommended that they take a second level drug alongside their NSAID therapy. However, these

• 38. 36 second level drugs only show results after several weeks or months of use. One of the most popular second level drug is called methotrexate which is the first choice in which doctors go to if they see that other JIA treatments are not working. It is a medication that has now been used for several years and it is easy to monitor as the patient only has to take it 1x per week and also safe to use as the history of the drugs effectiveness has progressed throughout the years. The maximum effective dose is 15mg and this is either given in oral form (a pill) or in a subcutaneous injection. Methotrexate is normally the first option of a second level drug for a patient with polyarticular JIA but it has proven to be effective with other types of arthritis as well mainly because of it’s anti - inflammatory characteristics. One interesting thing about this drug is that in some cases, it has shown to reduce the disease progression however, scientist do not know the reason why this is. When taking methotrexate, patients do have to make laboratory examinations very

• 39. 37 regularly as the drug could cause some liver function problems. By combining another drug called folic acid with methotrexate, it reduces the risks of side effects on the liver making the medication even more successful for JIA patients. An alternative to methotrexate is a medication called leflunomide which is similar to methotrexate but it is only given to children if it is proven that the methotrexate is not working or the child can not tolerate it (if they are taking subcutaneous injections). Leflunomide is given orally and the treatment efficiency has been proven but the medication shows to be more expensive than methotrexate. Other medications like salazopyrin or cyclosporine are used as non - biologic drugs to treat JIA even though not as much study has been done about it. This treatment today is not used as much as the experience is more limited because not many people have used it. Also, countries in which have access to biologic agents also prefer not to go forward with this treatment as other treatments can show to be more effective. When

• 40. 38 When cyclosporine treatment is decided to be put to the test, it is normally used with patients diagnosed with systemic JIA and the medication would be used along with corticosteroids. This medication is normally only used when the patient has macrophage activation syndrome which is a life threatening complication of systemic JIA. Corticosteroids are one of the most effective anti-inflammatory drug in JIA as well as in other diseases however, it is very limited because of its long-term side effects like stunted growth or even osteoporosis which is when the bone is in a very fragile condition. The drug is known to treat the systemic symptoms that resist to other JIA therapies. Corticosteroids today is being used more as a “bridge” drug to control acute diseases while the doctors wait until the second level drug to take effect. However, corticosteroids can also be used alone before tying it alongside a second level drug. The newest group of drugs introduced to JIA are called biologic agents and they are drugs with biologic engineering that help to block the

• 41. 39 inflammatory process of JIA. What is different about these drugs compared to other JIA drugs is that they are not directed to a specific molecule, they are directed overall to stop the entire process. As these drugs are relatively knew, a final conclusion as to if they are successful or no has not yet been made and scientists have said that some modifications to the drugs might also have to take place. A list of drugs that are also relatively new to JIA are anti - TNF drugs. These drugs are used to block the TNF which are supposedly the mediators of the inflammatory process. They can be used alone or alongside methotrexate. Since the start of their usage in treatment for JIA, they have shown success however, the long-term side effects are still unknown as these drugs have just started to be put to the test. So far there are 3 drugs that have been approved. The first drug option is a subcutaneous injection 1 - 2 times a week called Etanercept. The second drug option is also a subcutaneous that has to be taken 2 times a week called adalimumab. Finally, the third

• 42. 40 Finally, the third drug that has been approved for JIA is infliximab which is an intravenous infusion once a month. Currently, there are 2 other medications that are under study which are called golimumab and certolizumab pegol. All of these can be used to treat JIA but these drugs have not showed as strong as an effect with patients with oligoarthritis but have shown effect in decreasing symptoms. Abatacept is the name of another medication to treat arthritis. It can also be known as Anti CTL4lg. This medication is directed to a specific type of white blood cell called T lymphocytes. Today, this treatment is only being used with children that have polyarthritis and have not responded well to methotrexate or biologic agents. A treatment that also exists but is normally only used to treat patients with systemic JIA is anti interleukin 1 and anti interleukin 6. However, these treatments are only put to trial when the patient with systemic JIA has already tried other treatments like corticosteroids and methotrexate.

• 43. 41 When looking at anti interleukin 1, there are 2 different medications, anakinra and canakinumab. These two medications are both subcutaneous injections that help treat systemic manifestation like fever and also help to treat arthritis. Tocilizumab is the name of the medication for the anti interleukin 6. This medication is also used in patients with polyarticular JIA if the patient show no response to biologic agents or methotrexate. Surgery is rarely used as a way to treat JIA as there is not really much you can do. It can be used as a method to fix damage made from the arthritis with things like replacing a damaged join or even to straighten a bent or a deformed muscle but it is never used as a form of treatment. If the JIA has made the child get an eye problem due to the disease itself or also as a side effect on any of the medicines, treatments and cures can be offered. If the child has uveitis, it will have to be accompanied by an ophthalmologist over a period of time. If the patient has a side effect (like a cataract), the patient can easily ge surgery to repair it but, keep in mind that in orde

• 44. 42 to do this, the patient has to be in remission. Dental care is important as well when looking at JIA because one of the joints that can be affected is the jaw joint. The child may feel pain and stiffness. This is because treatments from JIA can alter the growth of the joint making i smaller than it should. If this is the case, the child will have to experience more dental visits as some solution might be offered from the dentist himself. Splints can also be used in JIA as a way of helping the child to cope, they are mainly used in the joints that are most active throughout the day like knees, wrists and fingers. Physiotherapy might also be recommended to try and add flexibility to some joints so that they can bend better as well as help with muscle shortening and balance problems. When your doctor has told you or any other patient that their JIA is in remission, what they mean is that the disease is no longer active at the time in the child’s body. When a doctor says this, usually means that you are showing the signs that the American College of Rheumatology (ACR)

• 45. 43 have declared as signs of remission. The signs are that all the patients blood work has gone back to normal as well as their blood pressure. The patient should also feel back to themselves , so things like fatigue, and joint pain would still be signs that there is still some activation in your system. If the doctor has said you are in remission, physical appointments as well as blood work appointments might start to space out a little bit more.

• 46. 44 My advice to you is not to be ashamed / embarrassed of having arthritis. Once you feel like you can talk about it, it gets easier to deal with those who don’t understand. Some people don’t understand and you need to teach them. Just remember to have patience. Katherine, 15 If you have arthritis, go swimming because it is fun and good for you. Siniya, 9 When I exersiz it helps me feel happy. Leigha, 9

• 47. 45 Chapter 5: Organisations and Events There are many organisations around the world that exist today to raise awareness for both juvenile arthritis and arthritis but these are not recognised the way they should be mainly due to the lack of awareness and spreading the word. These organisation try and do many things to support people who suffer from arthritis. They host events like camps or even conferences so that people can talk more about the disease and raise awareness. Below are some organisations and some events that have been hosted in the past to raise awareness towards arthritis. Kids Get Arthritis Too Organisation: This organisation is possibly the most famous juvenile arthritis awareness organisation in the world. They are in link with the Arthritis Foundation who are actually their founders. This organisation was founded in 1948 because of the lack of research

• 48. 46 done on arthritis (for both children and adults). This organisation is considered to be the leader organisation of the arthritis community as it is th biggest one. Kids Get Arthritis Too is a non - profit organisation and their mission statement is “We exist to conquer arthritis. We are the champions of Yes”. The Kids Get Arthritis Too Organisations host many events like camps for kids as well as arthritis conferences. The website helps inform kids about their disease and also share other kids stories and how they dealt with the disease. As this organisation is also linked to the arthritis foundation, it is also worth knowing that they are the publishers of the award winning magazine arthritis today which has more than 4.2 million subscribers to it. The Kids Get Arthritis Too Organisation is an organisation where you can see it offers support even through the hard times. Juvenile Arthritis Association: Founded in 2012, the Juvenile Arthritis Association is one of the newest organisations to join the fight in raising awareness. It is a non - profit organisation mainly dedicating their time funding research towards

• 49. 47 finding a cure or a more convincing treatment for juvenile arthritis. Their main focus is CARE (Community, Awareness, Research, Empowerment), this is their motto and what they strive to do. As this is a relatively new organisation, not much involvement has been done but things like having an informative website and a good support base are what starts of a successful organisation. The Juvenile Arthritis Association are currently having a public service announcement campaign to try and see if it raises more awareness. The role of empowerment in this association is very important to them and is also one of their main priorities as they try and help kids to not be scared of their disease and to inspire them to do what they want to do and not let the disease stop them. The Juvenile Arthritis Association is a community where you can tell that everyone supports each other. Cure Arthritis Organisation: The Cure Arthritis Organisation was created in 1952 to raise awareness about arthritis and to raise funds for research. Today it is the second biggest arthritis

• 50. 48 awareness organisation in the world that is non - profit. The organisation mainly focuses on Juvenile Arthritis as the people from within the organisation see that more progress needs to be done around that area instead of only just focusing on adult arthritis. This organisation mainly raises money through events in which sometimes they try and collaborate with the Arthritis Foundation Organisation. However, this organisation also raises awareness with their own events that they host like the racing for a cure event which you can read more about later on in this chapter. Systemic JIA Organisation: The Systemic JIA organisation was founded in 2014 by parents who have a kid that is diagnosed with JIA. They are a non - profit organisation which is mainly based in the United states. Their mission statement is that they want to find a cure for Systemic Juvenile Idiopathic Arthritis (SJIA), a serious and sometimes lethal childhood disease. This organisation not only gives support to many parent through their Facebook group and

• 51. 49 conferences but they also help to inform other parents on clinical trials and how effective new research medicines are towards SJIA. Arthritis Awareness Month: Arthritis awareness month is celebrated every year in the month of July all over the world. June is arthritis awarenes month for the elderly in the united states while in July it is for both juvenile and elderly arthritis all over the world. Arthritis awareness month is not very advertised all over the world. An estimate of about 300,000 people in the US try and raise awareness but without the contribution of many people, there is not really a way in which people could spread the word quickly. In this month is when organisations take most of their time to organise events to try and raise funds. The raising of these funds is done through walks, donations or even volunteer work. Other countries in the world also celebrate arthritis awareness month in May but it is also not very advertised. Juvenile Arthritis Conference: The juvenile arthritis conference is hosted every year by the Kids Get Arthritis Too Organisation which is linked

• 52. 50 to the Arthritis Foundation. The objective of this conference is to let families bond and also let them share what they have gone through. There have been more than 24,800 families that have participated in this 4 day conference in the past 3 years. Parents think that it is a great opportunity to trade ideas with other families on what the best treatment option for the child will be. The juvenil arthritis conference is also a great way to meet other juvenile arthritis specialist as doctors are invited to participate as well. Juvenile Arthritis Camps: The Kids Get Arthritis Too Organisation also holds 1 week camps for kids who suffer from juvenile arthritis. These camps are normally hosted during the summer and are for both kids and teenagers. What this camp basically does is that it helps the kids understand more about their disease and their condition. It also helps the kids to grow in their self confidence as after a long and hard time of in and out of hospitals, they can find it hard to find their self confidence. The camp can be done as a family camp or even individually if the child feels

• 53. 51 comfortable to stay alone at the site. At the end, the camp owners hope that the kids have created new friends and lasting bonds with other camp members who have the same disease as them or even something different. Not all of the camps are aimed just towards JIA, some camps can also be focused on rheumatological diseases or something else. Racing For A Cure: The racing for a cure event is hosted by the Cure Arthritis Organisation in the month of July which is considered to be arthritis awareness month. Even though this event is considered to be hosted by Cure Arthritis Organisation, they also consider other organisations to take part because they think that it could help raise more awareness. You do not have to run if you want to participate, you could also just show up and support and maybe even donate as this event is a public event meaning that anybody can participate. The funds for this run are mainly raised for the Arthritis National Research Foundation (ANRF).

• 54. 52 Keep going, it gets better. And theres always tomorrow. McKenzie, 14 It can be hard to tell people , especially friends, about arthritis. I have found that the best way to do this is to be specific and to talk about a lot it in a lot of detail. Rachel, 13 I’m not going to lie, shots stink! However, even though there are tough times, there are happy ones too. Marisa, 14

• 55. 53 Chapter 6: My Future - Where we go from Here Today I am still waiting for my pathway regarding treatments. We are confronted with 2 options. The first option is to take a research dru which is a IL-18 blocker. This drug is a research drug because it has not been tested on many kids yet so it is not out in pharmaceutical markets. Thi drug was created because scientists found out that people with Systemic Arthritis have high IL - 18 levels in their blood so they created a drug to stop those levels from being too high. This means that if we are able to block it, I will be able to reduce my medications and maybe even letting the disease go into remission for the first time since my diagnosis. The other option is to do a stem cell transplant. Although this treatment option might be very tiring and aggressive, it has shown to have a 95% success rate if a 9/10 donor is found and fortunately we have already found

• 56. 54 and fortunately we have already found one. My family and I have decided that I will try the IL-18 first and then, if that doesnt work into putting th disease into remission, we will consider the stem cell transplant. JIA makes you confront many challenges in your life that many other children don’t have to face. One of the biggest challenges I face is the ability to have energy to do things like physical activity or even to go out for a walk as the medicines make me have anemia. The good thing is that over time, when I have tried to go into remission, the medicine symptoms have decreased. Another challenge I face is that I miss school more often due to more medical exams or even because of sickness as the medicines reduce my immune system causing me to have fevers very frequently. Finally, one of the challenges in which I know I faced the most is judgment. People at school might not know what is going on and might think that you are just missing school because you are lazy, this is where patience comes in because you need to

• 57. 55 learn to keep your calm and explain. People might also judge you by your looks as many of the times, corticosteroids have side effects which make your body swell up even though you might not gain weight. Over time, as this medicine is reduced, the side effects will also go away. Without a doubt, most parents think that children should never go through what a child goes through when they get JIA but parents also see that it transforms their child in some way. Even though there might be all of those challenges, benefits are also present in the life o a child with JIA, I know that my life has changed to the better in many ways. I, for example spend a lot of time in hospitals when the JIA inflammation is present, it normally inspires me to try new things meaning that I also find new hobbies like cooking, writing or medicine in my case. Also, it inspired me to want to do my best in anything that I need to do or am assigned to do in school. Finally, the benefit that I think is the best all, is the fact that you learn how to be patient with yourself and others. You learn that not always anything will

• 58. 56 go your way and that you have to understand that at some point, everything will be OK again and that there is a light at the end of the tunnel. I learned that it is patience and time and positive energy in which makes me happy and also what makes me who I am today. One of the most important things to keep in mind is that your doctors are there to help you make decisions in what the best treatment option would be for you but in the end, it is you who makes the decision. Also, don’t be scared to give your opinion about a treatment to your doctor, always tell them the truth because it might be important for them to know how you feel and how you are reacting to treatment. Another thing is that you should not worry about is school. Your health is your priority, not school when you are sick. Teachers understand that school is not your priority at the time when your disease is active and should not give you a lot of work. However, this also means that whenever you can, try your best because in the end you might not be able to have as many

• 59. 57 opportunities to improve your grades as other students in the school might have. The most important thing is also to always have positive energy around you and that normally comes from friends and family. You should always keep in mind that they are there to help you in every way, no matter if it is giving yo a shoulder to cry on or to give an opinion in what treatment to take. However, the most important thing is for you to be yourself around people you like and don’t be somebody you are not. Every child who is diagnosed with juvenile arthritis not only has to learn to live in a community where they might not always feel comfortable but they also have to learn to learn and deal with themselves. It’s important for every kid to know that it is ok to be in a bad mood at times and it is ok to feel down and that is one of the hardest things to try and understand because in the end the question of ‘Why me?’ always comes popping back into your head. When a child close to their teenage years is diagnosed, they need to learn that they are in control, that they are

• 60. 58 the ones who define who they will become and who they want to be and not the disease, so they should be the ones allowed to decide what path and what treatment they should take. A question that always stays in the back of my mind when making a decision that might change my life in any way is “Will I let my past define me or do I want to think about my future and who I want to become?”. This question also links to a quote that really inspired me and that I really think helps me to understand how to deal with things when they get hard. The quote is: “Yesterday is history, tomorrow is a mystery, but today is a gift. That is why it is called present” - Master Oogway (Kung Fu Panda). Since I have bin diagnosed with systemic JIA, I have been trying to find out different ways in which I could deal with my feelings when I could not take them anymore or was just fed up with everything. Of course, not all of these might work for everybody because each person has a different personality and each person deals with things in a different way. The way in which I found

• 61. 59 helped me the most when things got too overwhelming was writing a diary about how my day went and what I think at the time. This helps me get all of my thoughts out of my head and all of my frustrations of my chest. Another thing that helps when you feel overwhelmed is to take a shower or a bath alone and spending some time thinking and reflecting. This might mean that you might talk to yourself and that is ok because it is an output in which your body is finding to let out stress. Sometimes even crying or screaming, or even hitting something might help to get all of tha negative energy out of the body. I am not going to lie, sometimes I do feel like giving up. My mind goes into the mindset of why is it worth it? I also think a lot of the time, why me why did god choose me? What did I ever do to deserve this? Whenever I feel sad and want to pick myself back up, I pamper myself. I find close off one afternoon and that is me day. No matter if have a test the next day or whatever, I need to find a time to relax and let everything out because being emotionally unstable and keeping

• 62. 60 everything inside is what is going to ultimately break you from what I have learned. Keep positive people around, people who are your true friends and people that will support you through everything and will never judge you no matter what. In the end, the most important thing to keep in mind is that, everything happens for a reason and all you need to do is look forward and hope for the best in the future because there is always a light at the end of the tunnel.

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• 65. 63 Beatriz was born in Rio de Janeiro, Brazil. She was born on August 19th, 2002 and moved to Curitiba, Brazil when she was just 10 days old. At the age of 5, she moved to Miami, Florida with her parents and studied in Alexander Montessori School. Then, at the age of 6 Beatriz moved to Mexico City where she attended Greengates International School until the middle of 5th grade. She then moved to Zurich, Switzerland where she studied at the Inter Community School Zurich (ICSZ). It was there where Beatriz found out her passion for medicine after September 2013 when she was diagnosed with systemic juvenile idiopathic arthritis. About The Author