My JRA Heros

My JRA Heros
My JRA Heros- Jake & Hannah

Saturday, November 3, 2012

NEW DIAGNOSIS, BUT NOT SO NEW DISEASE ACTIVITY....


Thought it was about time to share with those friends and family who keep our children in your prayers and follow The Sloan Siblings Journey. About 6 weeks ago, we were informed by Jake's Pediatric Rheumatologist at Shands Hospital that he has a new diagnosis....... Juvenile Ankylosing Spondylitis.  It has taken a few weeks for us to process this information, learn about the disease and prepare ourselves as how we are going to help Jake manage this complicated, chronic disease, that yes, children can get, too!
 
We thought Juvenile Rheumatoid  Arthritis- Systemic type with Polyartiucular features  was a mouthful for Jake.  Now.....he's got new vocabulary  words and more than a 10 year old should ever have to accept and learn to cope with.  We have not shared all the facts with Jake yet, but he is aware of his new diagnosis, MRI findings and understands this disease primarily focuses on his spine. Juvenile Ankylosing Spondylitis (JAS) is basically a systemic autoimmune disease affecting the joints, heart, lungs, bowels, kidneys and eyes. It is a painful, progressive  type of arthritis that aggressively  affects the spine and  large joints.  Although it can affect any joint, more common joints include: hips, knees, ankles, toes, ribs and jaws. The heels may also be affected(Iachilles tendinitis and  Plantar fasciitis), making it uncomfortable to stand or walk on hard surfaces.  Inflammation in JAS  also occurs in the areas where muscles, tendons, and ligaments are attached to the bone. To help break down the word, "Ankylosing" means stiff or rigid, and "spondyl" means spine and "itis" refers to inflammation.  Over time, the joints and bones may grow together ( fuse), causing the spine to become rigid and inflexible.  These fused bones are brittle and vulnerable to fractures. The lower neck is the most common area for these fractures.  Bones in the chest may also fuse ,causing breathing difficulty, and limits lung capacity. The fusion of the spine can lead to a forward curvature of the spine, a forward-stooped posture. Complications from the disease varies from each child, and JAS does not follow the same course in everyone.

Jake's new diagnosis still shocks us and I tend to try to figure out 'why"?? Juvenile Ankylosing Spondylitis is to be considered a multifactorial condition, meaning  that "many factors" are involved in causing it.  There is no known way to prevent JAS.    The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait. It is also highly associated with the antigen/protein called HLA-B27. JAS strikes young people, typically between 17 and 35 years old and more common to affect males ( 2-3x more common with males than females).Our family does not have any genetic role (nobody diagnosed with AS) and Jake was not born with the HLA-B27 gene. So I often ask, why, why Jake?

Some of the early symptoms of juvenile ankylosing  spondylitis include:
(However, each child with JAS may experience symptoms differently and the severity of symptoms and disability vary from each child.)
  • back pain, usually most severe at night, during rest
  • early morning stiffness
  • stooped posture in response to back pain ( bending forwatrd tends to relieve some pain)
  • inability to take deep breath ( when joints between ribs and spine are affected)
  • appetite loss
  • weight loss
  • fatigue
  • fevers
  • anemia
  • enthesitis ( pain at the site of attachment of muscles, ligaments and tendons to bone)
  • joint pain
  • vague pain, usually in the buttocks, thighs, heels and shoulders
  • painful eye inflammation
  • organ damage, such as the heart, lungs and eyes 

There is no cure for JAS, and the course of the disease is unpredictable.  It is often said that when a child is diagnosed with JAS, it usually results in a much more severe disease than adult onset of AS. .The children who have JAS have a worse functional outcome (in terms of work disability, functional impairment, quality of life, and spinal involvement) compared to adult onset of AS.  Treatment for Juvenile Ankylosing Spondylitis is individualized but overall the goal is to reduce pain and stiffness, prevent deformities, and to help children like,Jake, maintain as normal lifestyle as possible. Educating ourselves and appropriate mild exercise  is very important. Swimming is ideal,as it avoids jarring impact of the spine.  Exercise programs designed by physical therapists with regular back and chest exercises are crucial to help improve posture, spine mobility, and lung capacity. Hot baths, heat and massages are also beneficial. We are very thankful for Jake's gift of his hot tub from Make-A-Wish Foundation. Jake continues to get weekly full body massages from Ms. Patrice.  ohhhh......we just can't begin to say enough wonderful things about her. Jake would go everyday, if he could.
    
Non-steroidal anti-inflammatory drugs may provide pain relief and decrease morning stiffness, however do not alter the course of the disease. With taking these meds routinely, it can also cause stomach upset, nausea, abdominal pain, diarrhea and bleeding ulcers. Jake has been dealing with these GI issues for a very long time, even after stopping Methotrexate. For those children like Jake who is unresponsive to anti-inflammatory meds, the  use of biologic agents {TNF ( tumor necrosis factor) blocking medications}, such as: Enbrel, Humira, and Remicade are necessary part of treatment. Unfortunately, Jake has been on all of these biologic' s and has continued to fail and not respond in treating his disease effectively. Jake and his sister, Hannah, have both been on Actemra bi-weekly infusions  for the past 16 months.  Actemra was the newest biologic approved by the FDA and is a IL-6 receptor drug. Unfortunately, it has lost it's effectiveness and Jake is no longer responding to it as he had in the past. His fevers are back almost every evening, multiple joints are inflamed and he literally has joint pain in EVERY joint in his body { toes (sausage toes), ankles, knees, hips, back, neck, shoulders, elbows, wrists, fingers, jaws and ribs}. Jake's recent cervical MRI showed "thickening", and his doctor believes it's been there for awhile. It is time to try another drug, but there is nothing new available to try. This is what is so hard to accept.......the disease is not being controlled and we have ran out of options at 10 years old. As of today, we are TRYING to get Rilonacept  (IL-1)approved as a "Fever Syndrome " medication. {It is currently in a closed study- treating Systemic JA.} We are unsure if insurance is going to approve this medication, but we got HOPE on Friday that it just might be a option! Praying hard and anxiously awaiting for a updated phone call from Shands. Jake will not stop any of his current medications, he will be adding Rilonacept weekly  as a second biologic .  This scares me too! For those of you who understand the risks involved in using biologic' s, and the "Black Boxed Warnings" and the lovely side effects of these drugs, especially when used with combing TWO biologic' s.......  I won't even begin to express my concerns and worries there.....     I am fully aware of the serious conditions/infections they can lead to, and even death.  I have had a lot of restless nights and heavy heart trying to process our upcoming changes to control these monster diseases!  Hannah is adding a second biologic- Rituxan this Wednesday, despite her re-occurring impetigo/staph infections. Her cardiologist gave the approval to go ahead and proceed with new treatment plan.{ Rituxan aims to help stop the activation of a certain type of white blood cell- called B cells. With fewer B cells, the over-activity of the immune system deceases}.
So..... we are adding a second new biologic for both Jake and Hannah and praying to get more control of these diseases with no serious infections or problems. Trying not to give up HOPE!

Jake keeps going - pushes through his pain & challenges everyday!
Not much more to share, new fancy name (Juvenile Ankylosing Spondylitis), but this name didn't change how Jake has been feeling or change his treatment plan.  Sometimes it takes a long time to see how disease progresses and to watch for patterns. Unfortunately, JAS is difficult to treat, but we know we are in good hands at Shands and ultimately God is in control. We never give up Hope that God will completely heal Jake and Hannah.  Until then, we only do what we know to do and try to make everyday special! 


Thank you for taking the time to learn a bit more about Juvenile Ankylosing Spondylitis. It's a learning curve for us, and we will continue to support the Arthritis Foundation. As Jake and Hannah and the other 300,000 children need better medication options and a cure!  Our family appreciates the prayers and the support.given by those in our lives!

12 comments:

  1. Heidi, I am glad you have a name for all that has been happening and I hope this additional treatment gets approved and helps Jake. It has certainly helped Parker. I am always keeping you and your family close to my heart and know that as you journey we are with you praying, hoping, and living the fullest with each new day. Great blog and much love! ~Rochelle

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  2. I`m feel so sorry for Jake and Hannah.
    Really pray for that the mew biologics gonna work.
    I`m 14 old and on Orencia,taked three dosages (I.V week 0,2,4)
    Have Juvenile Arhritis and Psoriatic arthritis and
    Its have affcted nearlly every joints ; Toes,ankles, knees, hips, back, neck, shoulders, elbows, wrists, fingers, jaws and heels. I`m also coping with a fever and Salamon Pink colored rash thats often come in the evening and the fever are at worst when the rash are there , but it appears after just a while. The doctors don`t know what it is but the rash they says are likely with the systemic type.. But beacuse it started at my joints they say it isnt that. But I`m still worried, the rash and fewer kicks me out and get severe fatique. Its there nearlly daily. ( the rash are in my face and chest and its goes from shoulder to shoulder. Its have been like this since 2010/2011 (?) but its so much worse now..

    What do do`?

    Was on Prednisolon for some weeks ago and was on it for more than two months, was feeling great but gained weight. Take MTX weekly, and failed Humira and the must of NSAIDS. Cortisone Injections have been done but beacuse that many joints are affacted they either but me on Prednisolon, under 15mg day I feeling bad again and at 10mg daily I`m back at there I started. But at least my immflamation goes down. I don`t now what do do if the Orencia dont help.. feel scared. My ``name`` is my epost. please message me.

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  3. Thanks for your response and taking the time to think about Jake & Hannah when you are facing similar issues yourself. Do you live in US? Are you in contact with local Arthritis Foundation or a support group of some type? I hope you have an opportunity to share your feelings, concerns with another child who also has JA. My children are a bit younger, but absolutely cherish these friendships with other JA children.

    Im not a doctor but only have experience to go on.... Many times it takes LOTS of time to watch patterns and see how the disease progresses before you really find out what's going on. And just when you think you know what's happening...your immune system can go crazy and change things in different paths! There's no easy answers, no quick fixes. I know nobody like to be on steroids, the weight gain is undesirable for anyone at any age. Do you keep a journal or log on your disease activity? Monitoring fevers, taking pictures of rashes? The fatigue is very difficult to live with. I also have JA ( Since i was 3 years old) and the fatigue bothers me mostly! Certainly hope Oriencia works well for you and you get much needed relief. I will talk to Jake and see if he'll email you and share about his experiences. He also gets fevers ( late afternoons/ evenings). Take care of self and will keep advocating and raising $$ for kids like you! We need better treatment options and a CURE!

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  4. Thanks for a quickly reply.

    At first I want say Thank You for answering, feel sorry for you, how are your JA doing now?
    Wish for a CURE and at least I hoping for bether treatment options.
    I don`t live in the US, but
    some off my family does so Ive
    been there. Yes I have taked pictures
    of the rash and I showed it to the doctor
    and thats when he told it looked like the rash from the systemic type but then he said if it was it would have started different. And I also have a diary there I write down the pain activity.

    Thanks for talking to Jake, hope he messages me but its his own choose.
    Send hope and prayers to our all and I`m going to follow your blog. :)

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  5. If you can't get Rilonocept, inquire about Kineret. It's another IL-1 used for fever syndromes and sJIA patients. It is a daily injection, but supposedly does wonders for certain people. I'm in Tallahassee and have been considering Shands for rheumatology-- I would love to chat about your experiences there if you would be willing :)


    Warm regards,

    Emily
    chroniccurve.com

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  6. Emily-
    I am happy to share Jake got Rilanacept approved and I our hands. It's a miracle it got approved!! I'd be happy to share our experiences at Shands :-) ( email me at Heidisloan@verizon.net).

    ReplyDelete
  7. Emily-
    I am happy to share Jake got Rilanacept approved and I our hands. It's a miracle it got approved!! I'd be happy to share our experiences at Shands :-) ( email me at Heidisloan@verizon.net).

    ReplyDelete
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  9. Your beautiful children are going through so much at such a young age and are extremely strong and extraordinary kids for it! They will always be in my thoughts and I hope nothing but the best!! I really hope they find some pain relief soon! I was diagnosed with JRA at 18 months and have a blog also. I was shown yours because I was asked to have mine as a featured blogger as well. Your kids are truly an inspiration. Feel free to visit my blog and see if anything on there can be of any help! Juliesjrajoint.com. Feel free to contact me anytime! You are a strong woman for what you have to go through, as well. I know it can't be easy but you are doing everything you can! =)

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  10. Just wondering if you have tried any natural treatments for your kids? My son has had JRA since was 2. The doctor told us an elimination diet wouldn't do anything, but it helped tremendously. We are now doing the Paleo autoimmune protocol diet and trying homeopathy to help settle his immune system down. If you are at he end of your rope it might be something to consider, its hard, but worth it!

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    Replies
    1. Hi I have a daughter with arthritis in the jaw. I have chosen not to put her on methotrexate until I have tried some natural treatments to get her inflamation down. She is currently being weaned off prednisone, it has helped tremendously but as we all know not a long term answer. I am afraid that when she is off the steroid the damage and stiffness will continue or get worse. Our doctor has suggested jaw injections but I know that has its own side effects. Has anyone had positive experiences with the jaw injections? I would love some ideas on diet and supliments that moms have had success with giving their children. ~ Shelly

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