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Bailey's Life Welcome to Bailey's Web Page. It has been created by Todd, Chris and Brandon to keep friends and family updated about our child, his illness, and the family. Please check in frequently for updates. While we wish we could telephone each friend and family member with individual updates as things change, that is impossible. This site will help keep everyone informed and allow us to put our focus and energy into caring for the children and ourselves.
Please remember to sign the guest book after visiting! Thank you for your support.
Todd, Christine and Brandon Robinson
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We thought this first entry could serve as an explanation to the disease and what we are doing to treat it.
Adrenoleukodystrophy (X-ALD) is a serious progressive, genetic disorder, which affects the adrenal glands and the white matter of the nervous system.
X-ALD is an X-linked disorder, which means it affects only males and is transmitted by a female carrier. Such disorders are referred to as "X-linked" since the genetic abnormality involves the X-chromosome. Women have two X-chromosomes, men only one. In women, the affected X-chromosome, the one with the gene for X-ALD does not manifest because of the presence of a normal copy of the gene on the other X-chromosome. Men have one X-chromosome and one Y-chromosome. In men who have an X- chromosome for X-ALD, there is no other X- chromosome for protection, therefore symptoms may be seen in the male.
Bailey has been diagnosed with the childhood cerebral form. Thankfully Brandon does not test positive for the disease.
About half of women who are carriers for X-ALD develop mild neurologic symptoms in their later years. Clinical manifestations are usually limited to progressive paraparesis (a partial paralysis of the legs), moderate sensory loss, and peripheral neuropathy. Cerebral involvement is rare and adrenal function is almost always intact.
Chris has been diagnosed as a carrier of the disease and Britta will be evaluated at an older age.
X-ALD can present in several forms. The classic childhood form is the most severe with onset in boys usually between 4 and 10 years of age. Initially they may experience such difficulties as learning disabilities, perceptual problems, attention deficit disorder, short and long-term memory loss, impaired vision, coordination or gait, and various personality and behavioral changes.
It has been established by the experts at Johns Hopkins that some of the above may be occuring with Bailey. Chris had the opportunity to meet with Dr. Cox at Johns Hopkins and learned a lot about initial behaviors these children present with. You can liken the initial onset to that of a child with Autism, in that they are in a regular state of overload. We are learning how to apply discipline accordingly. You may say to your child "You're not listening!" for example. For an ALD child, there's just too much being said! They are listening, just not hearing it.
Along the same topic, we are learning what behaviors may be due to his disease, or a symptom of it, and how to deal with that. The most important thing we have learned so far is that it is imperative that Bailey not feel he is being punished for things he can not control.
Impaired adrenal function may cause increased skin pigmentation 'bronzing' (Addison's Disease), fatigue, and crises when stressed. Bailey has been diagnosed with Addisons and is on Adrenal replacement therapy, to which he will be dependent on for the rest of his life. The adrenal impairment can be managed by taking cortisone by mouth or a larger dose can be injected muscularly if needed. This medication is his life line to the extent that a Medic Alert bracelet specific to his condition with directions has been ordered in the event that Bailey is ill or in an accident in our absence.
It is, however, the nervous system involvement, which causes the main disability and which researchers are striving to understand and treat. The course of the fatal, childhood cerebral form is generally from one to ten years with nervous system deterioration leaving the patient bedridden.
We are planning and preparing for that time in Bailey's life, while living each day to its fullest extent! It's an interesting balance, and a huge thank you to our community for all the little and BIG ways they help us with this.
Extensive research is being done around the world in X-ALD. In 1993 the gene for X-ALD was identified through the combined efforts of Drs. Patrick Aubourg and Jean-Louis Mandel in France and Dr. Hugo Moser in the U.S. This has opened new doors for further study.
Although definitive treatments are not available for X-ALD, experimental studies are being done with the "Lorenzo oil" diet and bone marrow transplant. Simply put, there is currently no cure, but we won't dwell on that!
Dr. Moser and Dr. Raymond are the lead physicians in the clinical trial we are participating in, which is studying the effects of a therapeutic diet combined with Lorenzo's Oil and other medications and supplements.
Bailey is doing a very good job taking so many medications in so many forms many times a day. He also is doing a super job on his diet, while being confronted with foods he may not have almost everywhere! At school, while shopping, even driving through town he hears "NO" to requests for food.
While Bailey is very proactive where his health is concerned, he is still a six year old boy who wants what he wants when he wants it. To be fair his food requests are not that unreasonable, but many foods you might not expect are now forbidden to him. In order to support him it was decided weeks ago that Bailey's home would be the "safe food zone" and the one place where the answer would be "YES"!
The night before the diet began the house was emptied of all foods not allowed and replaced with acceptable alternatives.
In our home we all agreed this was a small sacrifice when faced with the alternative. Adhering to the plan will prolong Bailey's quality of life!
Well, that is where we are at and what we are doing. Again, please check in for updates. Scroll down for a link to our new family email adress and write with any questions, concerns, messages for the kids, or just to say hello!
Sign our guestbook and check out the links to learn more!
If you are a family with an ALD connection and have an opinion on treatment or advice to share please contact us. This disease is so "new", we appreciate hearing about anything you may have already learned.
Thank you for your support!
Todd, Christine and Brandon Robinson
*HUGS* TOTAL!
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Journal
Tuesday, January 15, 2013 8:21 PM CST Missing your smile, your hand in mine, dragging you through the base area and your LAUGH! Nobody could enjoy a day like today with me as you would have!
Baileys Mom
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