Post by Amy Lombardi on Mar 31, 2009 12:15:21 GMT -5
Subject: Freaking Out!
Name: Michelle
Date Posted: Nov 17, 08 - 5:44 PM
Email: Click here to Email
Message: My son Zayne, 9yrs. old. He has late infantile batten's. This is the first time I have looked into the discussion forums. I'm reading through and I'm coming across a lot of you with more the one child with Battens. I was told in the beginning that my 12yr. old has the same percentage that Zayne has. I was told that I could have him tested, but never did. Why? so then if he was, wait for the disease to do what it does. Zayne's regression started around 13 months old. Did the disease start around the same time for you parents that have more then one child with Battens??..... Zayne is up every night, I've tried everything to calm him. Usually, around 2-4 am he'll finally go to sleep for a few hours. He can't seem to take naps during the day either. It seems like the disease is worse during the night. This has been like this for about 5months now. The Dr. says he has to get a sleep study before considering sleep meds. Did your kids have to go through a sleep study?? The Dr. is always cautious of suppressing his breathing with meds. Thanks. Bless you and your sweet angels
Name: Anonymous
Date Posted: Nov 17, 08 - 7:20 PM
Message: For families with more than one child with Batten's, usually the symptoms present within a very close age range. Late Infantile Batten Disease is usually symptomatic between 2-4 years of age, though there are some exceptions of later onset. However, it's usually a fairly close age onset within famlies.
I believe when the doctors said "same chance" your child had the same chance to inherit the disease. Since they're 12 without symtpoms the chances of them having Batten Disease are pretty slim, but there is a 50/50 chance they might be a carrier for the disease so, for having children, it might be advisable to find out if that child is a carrier or not.
I hope this helps
Name: Michelle
Date Posted: Nov 17, 08 - 8:31 PM
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Message: Thank you so much. I have talked to him about being a possible carrier.
Name: Corrina Dahl
Date Posted: Nov 17, 08 - 10:03 PM
Message: My son Clifford is age 11 with juvenile. When he strted having sleep issues they would not give him sllep meds without doing a sleep study in order to rule out any sleep disorders. He was only sleeping about 2 to 3 hours at a time and had even gone a couple of nights w/o sleeping.
The sleep study proved normal and he was put on a sleep med but its been too long and I don't remember the actual med off the top of my head. The sleep meds seemed to work ok. He would sleep about 6 hours at a time, still not normal but definately better than two. After about a year though his behaviour became more and more hyper and impulsive. He as having absence seizures so we got him on meds for that but the psychiatrist said the sleep meds can make some children hyper. So we took him off the sleep meds and put him on seroquel. The seroquel combined with the depakote has let him sleep fairly normally for the last 3 1/2 years.
I would also agree with anonymous that your 12 year old is most likely healthy. When my son was diagnosed at age 6 1/2 his younger brother was only 8 months old. We were really scared for him but held off testing him until he was 4. We decided to test at that time so he could get the school services he would need before kindergarten rather than later like his brother. Fortunately he tested negative.
At any rate I don't regret not testing him right away. I could not have taken any more bad news at that point and I just wanted to enjoy watching my baby grow like I had his brother. Everybody has to decide for themselves the right time to test a sibling.
Name: Michelle
Date Posted: Nov 18, 08 - 9:20 AM
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Message: Corrina,
Thank you for sharing.I appreciate the info. I don't know what took me so long to go to this discussion forum.
Name: Nancy Peterson
Date Posted: Nov 18, 08 - 9:37 AM
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Message: Michelle,
I did not have a child with Late Infantile, so I can't address those issues. My son had juvenile and my daughter is fine. She was blessed to not even be a carrier. Testing for carrier I do know about. It needs to be done with great care and at the appropriate age. The Batten organization assist sibs with carrier testing, but not until they are a minimum of 18. My daughter 21 before she was cleared, but that was partly due to the science and available of identifying mutations. My gene was a standard JNCL deletion, but my husband carried a mutation. They did not have the ability to find the mutation until 4 years ago. Talk to Amy at the head office about this subject. It really isn't an issue in your son's life now. I do agree with other posts that your 12 is more than like not going to have Battens. Families with multiples do typically have a come parameter of onset. As to the sleep issue again contact the main office and talk to Nancy our nurse. Take care.
Nancy
Name: Jocelyn
Date Posted: Nov 18, 08 - 3:26 PM
Email: Click here to Email
Message: Hi Michelle,
I have two children with late infantile battens. My son was diagnosed when he was around 3 1/2 (he is now nearly 8)we where also told that as parents of battens children any other siblings have a one in four chance of actually having the disease. Your 12 year old would well and truly be showing signs if he had the disease. We had our younger daughter Mckenzie tested not long after finding out about Seth and she too tested positive to having the disease. At the time she was showing no signs what so ever of having the disease but as time has gone on she too is now getting sicker. We have never had any trouble with Seth as far as sleeping but Mckenzie used to do everything you are experiencing at the moment, she would be awake most of the night, have night terrors and spend hours crying or screaming. We tried numerous things to help her but our doctor eventually prescribed us Cloral Hydrate at night time, some people don't have any success with this but from my expereince it was a life saver. We would give her a dose at night after all her normal meds. We started off just giving 2ml then another 1ml each 1/2 hour until she would fall alseep we where able to all get a good nights sleep. Some nights it would only take the 2ml, other nights it would be 5ml. I have expereinced alot of ups and downs in the past 4 years since the kids have been diagnosed and see a pattern that the things they experience do pass, almost like stages, we now don't have to give Mckenzie anything for her to sleep and she has become a very peaceful, placid child, not anything like the screaming child we used to have. Hope this is helpful to you
Kind Regards
Jocelyn
Name: Deb
Date Posted: Dec 5, 08 - 9:41 AM
Message: 2 of my 3 bio children have/had batten's my oldest is fine (he might be a carrier but has not been tested yet).
As for sleep my kidos had juvenile but they had did not sleep good so the doctor put JJ on clonidine and Hilly on risperdol which help with behavior and sleep.Both worked great on each child even though the risperdol did not help JJ. I would ask the doc about those. No we did not need a sleep study to get them th emeds they needed.
Name: Michelle
Date Posted: Nov 17, 08 - 5:44 PM
Email: Click here to Email
Message: My son Zayne, 9yrs. old. He has late infantile batten's. This is the first time I have looked into the discussion forums. I'm reading through and I'm coming across a lot of you with more the one child with Battens. I was told in the beginning that my 12yr. old has the same percentage that Zayne has. I was told that I could have him tested, but never did. Why? so then if he was, wait for the disease to do what it does. Zayne's regression started around 13 months old. Did the disease start around the same time for you parents that have more then one child with Battens??..... Zayne is up every night, I've tried everything to calm him. Usually, around 2-4 am he'll finally go to sleep for a few hours. He can't seem to take naps during the day either. It seems like the disease is worse during the night. This has been like this for about 5months now. The Dr. says he has to get a sleep study before considering sleep meds. Did your kids have to go through a sleep study?? The Dr. is always cautious of suppressing his breathing with meds. Thanks. Bless you and your sweet angels
Name: Anonymous
Date Posted: Nov 17, 08 - 7:20 PM
Message: For families with more than one child with Batten's, usually the symptoms present within a very close age range. Late Infantile Batten Disease is usually symptomatic between 2-4 years of age, though there are some exceptions of later onset. However, it's usually a fairly close age onset within famlies.
I believe when the doctors said "same chance" your child had the same chance to inherit the disease. Since they're 12 without symtpoms the chances of them having Batten Disease are pretty slim, but there is a 50/50 chance they might be a carrier for the disease so, for having children, it might be advisable to find out if that child is a carrier or not.
I hope this helps
Name: Michelle
Date Posted: Nov 17, 08 - 8:31 PM
Email: Click here to Email
Message: Thank you so much. I have talked to him about being a possible carrier.
Name: Corrina Dahl
Date Posted: Nov 17, 08 - 10:03 PM
Message: My son Clifford is age 11 with juvenile. When he strted having sleep issues they would not give him sllep meds without doing a sleep study in order to rule out any sleep disorders. He was only sleeping about 2 to 3 hours at a time and had even gone a couple of nights w/o sleeping.
The sleep study proved normal and he was put on a sleep med but its been too long and I don't remember the actual med off the top of my head. The sleep meds seemed to work ok. He would sleep about 6 hours at a time, still not normal but definately better than two. After about a year though his behaviour became more and more hyper and impulsive. He as having absence seizures so we got him on meds for that but the psychiatrist said the sleep meds can make some children hyper. So we took him off the sleep meds and put him on seroquel. The seroquel combined with the depakote has let him sleep fairly normally for the last 3 1/2 years.
I would also agree with anonymous that your 12 year old is most likely healthy. When my son was diagnosed at age 6 1/2 his younger brother was only 8 months old. We were really scared for him but held off testing him until he was 4. We decided to test at that time so he could get the school services he would need before kindergarten rather than later like his brother. Fortunately he tested negative.
At any rate I don't regret not testing him right away. I could not have taken any more bad news at that point and I just wanted to enjoy watching my baby grow like I had his brother. Everybody has to decide for themselves the right time to test a sibling.
Name: Michelle
Date Posted: Nov 18, 08 - 9:20 AM
Email: Click here to Email
Message: Corrina,
Thank you for sharing.I appreciate the info. I don't know what took me so long to go to this discussion forum.
Name: Nancy Peterson
Date Posted: Nov 18, 08 - 9:37 AM
Email: Click here to Email
Message: Michelle,
I did not have a child with Late Infantile, so I can't address those issues. My son had juvenile and my daughter is fine. She was blessed to not even be a carrier. Testing for carrier I do know about. It needs to be done with great care and at the appropriate age. The Batten organization assist sibs with carrier testing, but not until they are a minimum of 18. My daughter 21 before she was cleared, but that was partly due to the science and available of identifying mutations. My gene was a standard JNCL deletion, but my husband carried a mutation. They did not have the ability to find the mutation until 4 years ago. Talk to Amy at the head office about this subject. It really isn't an issue in your son's life now. I do agree with other posts that your 12 is more than like not going to have Battens. Families with multiples do typically have a come parameter of onset. As to the sleep issue again contact the main office and talk to Nancy our nurse. Take care.
Nancy
Name: Jocelyn
Date Posted: Nov 18, 08 - 3:26 PM
Email: Click here to Email
Message: Hi Michelle,
I have two children with late infantile battens. My son was diagnosed when he was around 3 1/2 (he is now nearly 8)we where also told that as parents of battens children any other siblings have a one in four chance of actually having the disease. Your 12 year old would well and truly be showing signs if he had the disease. We had our younger daughter Mckenzie tested not long after finding out about Seth and she too tested positive to having the disease. At the time she was showing no signs what so ever of having the disease but as time has gone on she too is now getting sicker. We have never had any trouble with Seth as far as sleeping but Mckenzie used to do everything you are experiencing at the moment, she would be awake most of the night, have night terrors and spend hours crying or screaming. We tried numerous things to help her but our doctor eventually prescribed us Cloral Hydrate at night time, some people don't have any success with this but from my expereince it was a life saver. We would give her a dose at night after all her normal meds. We started off just giving 2ml then another 1ml each 1/2 hour until she would fall alseep we where able to all get a good nights sleep. Some nights it would only take the 2ml, other nights it would be 5ml. I have expereinced alot of ups and downs in the past 4 years since the kids have been diagnosed and see a pattern that the things they experience do pass, almost like stages, we now don't have to give Mckenzie anything for her to sleep and she has become a very peaceful, placid child, not anything like the screaming child we used to have. Hope this is helpful to you
Kind Regards
Jocelyn
Name: Deb
Date Posted: Dec 5, 08 - 9:41 AM
Message: 2 of my 3 bio children have/had batten's my oldest is fine (he might be a carrier but has not been tested yet).
As for sleep my kidos had juvenile but they had did not sleep good so the doctor put JJ on clonidine and Hilly on risperdol which help with behavior and sleep.Both worked great on each child even though the risperdol did not help JJ. I would ask the doc about those. No we did not need a sleep study to get them th emeds they needed.