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
- 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.
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!|
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!