Subject: Re: Mental Retardation

Hi Martha,

Let me give a little different perspective on the "developmental delay" label verses the "mental retardation" label. Even before our daughter was diagnosed with AS, our neurologist described her as being DDeed. Our immediate question was, "If she is only delayed does that mean that she will eventually catch up?" He responded that she would be DDeed all her life, given her microcephaly. We got frustrated hearing that term because we found it to be an evasive "politically correct" term.

Whenever we would talk with people about our daughter, when we mentioned that she was developmentally delayed we would get reactions ranging from blank stares to, "Oh, that's nice." Nobody seemed to grasp the nature of our daughter's disability by describing it in this way. When I tell people that she has a disorder that causes a lack of speech, seizures, and mental retardation I get a completely different reaction.

It is true that there are varying degrees of mental retardation among AS kids, especially UPD and deletion negative. Nothing I am saying here is meant to downplay the need for increased therapy to see our children develop to their fullest. I think the term "mental retardation" has fallen into disuse because of the way people have mistreated and derided people who fall into this category. The problem is not the term, the problem is people's ignorance and insensitivity (which makes me want to call a few of THEM developmentally delayed).

Kevin Ditamore

Subject: Re: Mental Retardation

Kevin, Well put...I always felt the same way about the term "developmentally delayed" as well as "developmentally disabled" as too "pc" and not meaningful. Marianne (Emily, almost 7, del+)

Subject: Re: Mental Retardation

Dear Kevin:

I like your attitude. I guess what's finally gotten to me is that mental retardation (or severe developmental delay) is featured prominently in every clinical description of AS that I've ever seen. Over this summer, the first in years that Sara wasn't away at Easter Seals camp every day for 9 hours, I got to see more of what my daughter is like in a learning situation. We have a full-time TSS (therapeutic support staff) through Medicaid who worked with Sara all day every day on many of the same things she tackles at school. I began to think, when I saw how quickly Sara learned some things (and I am operating from the old mental retardation = slow to learn standpoint), that maybe we should take the situation as it is and try to come at it from a different perspective.

For example, it is easy--too easy--for professionals not familiar with AS to see the clumsy gait, the drooling, the inappropriate smiling, the lack of speech, etc., as classic characteristics of MR people. But without changing the facts of Sara's gait, drooling, etc. , why can't we explain them in other ways? It's a lot simpler to lump these characteristics together, but I believe that it might be more helpful to our kids to talk about physical and neurologic causes rather than intellectual or mental ones. I believe now that there are also really urgent behavioral issues (no duh) that should be addressed on more of a hyperactivity model than an intellectual deficit model. I also believe now that our kids have to have suffered some emotional damage from not have appropriate responses all their lives to their attempts to communicate.

It really isn't that I'm offended by the term MR--or DD, for that matter. I just think they may be inaccurate for all AS kids; maybe not for some--there are, after all, retarded and developmentally delayed people in all populations, and maybe what we have are some AS kids who are also retarded, or maybe they have AS with MR--but I would like to see some investigation as to whether MR or DD is central to the diagnosis. I am convinced that my daughter is not MR, and that her DDs are attributable to physical and neurologic causes. This is not just a political statement. It makes a big difference in how she will be educated and what our plans for the rest of her life must accommodate.

I did not mean to upset anyone. My fervor is uncontrollable because these ideas are to new to me and I'm really excited. And, after all, the AS/MR position is the establishment position; it's not like I'm a big threat to anyone's kids' getting the treatment they need, or that their parents want them to have.

Martha

Subject: Mental retardation

Kevin and Martha,

It may be boring to discuss definitions of MR (=mental retardation) and DD (= developmental delay), yet in this case I feel there is a point. You might want to check the definition of MR given by the American Association on Mental Retardation at:

http://www.ici.net/cust_pages/nbhsi/mr-def.htm#definition

As regards the definition of DD, I think we all know. I guess different people attach different connotations to these concepts. For instance, DD is often used, as Kevin mentioned, in cases where no diagnosis has been established, and that, of course, is an unsatisfactory state of affairs. DD may also lead people think that the person in question would be otherwise normal, but he/she has received insufficient attention, education, or whatever. In my mind, however, explaining that DD is associated with AS would make things clear.

With MR, there certainly are problems like those Kevin mentioned, but to me at least - and I guess to Martha, too - the main problem is that it is the FINAL JUDGMENT: DD in AS is due to a severe intellectual disability (low IQ). I think this still remains to be proven. Indeed, the paper "Angelman Syndrome", written by Charles A. Williams, Harry Angelman and others, touches this point and offers some reservations. They use DD in their list of clinical features.

As I understand it, DD is a more comprehensive concept which also encompasses MR. It leaves open the possibility that tests could be developed which measure AS children's intellectual ability more accurately, or that it could become recognized that therapies, new communication devices, or some medication could improve their condition significantly.

Let me elaborate on my own standpoint. I do think that our kids could achieve much more if given sufficient attention in the form of therapies and special education. (Martha - that 66 hours is way beyond what would become available even if I voted the local (Social) Democrats. I think I understand why the U.S. has the highest proportion of national income allocated to health care in the world !) Indeed, I've often wondered if really a special educator constantly on her side would be something that Liisa should get. My wife and I suffer from bad conscience, because we're not doing enough, but we try to do everything we can. It is possible that, sometime in the future, a communication device would be invented which enables her to express herself in much the same way as we do - but then, a lot of damage has already occurred, because it would come so late.

Another thing is, what are the genetic, chemical, physiological and other mechanisms that lead to the speech impairment and low measured IQ. Just my own view: I expect that when the AS gene is found and its functions becomes under- stood, they will have something to do with metabolism and neurotransmitters in the brain. It is then conceivable that some medication and/or nutritional adjustments would improve the functioning of the brain. Whether that would show up as higher results in IQ tests, remains to be seen. I really don't believe that any AS people would learn to speak as they now are, but perhaps we would see much more of it, if my optimistic vision should ever materialize.

Heikki Taimio, dad to Liisa (6, del-) Finland

Subject: Mental retardation

Dear Heikki:

I think we are in accord. I feel sad that I apparently am causing guilt/wrath/unhappiness with all my blabbering about Sara's progress and questioning MR status (or DD status, if you prefer). I question DD status because it is not clear to me that a diagnosis of developmental delay--the catchall for undiagnosed special needs children--does not mean intellectual delay and nothing but intellectual delay, whether it is measured in terms of IQ or achievement of physical milestones. I contend that with AS children there are so many physical problems (my God, read the letters posted here; I had no idea) which I feel could just as well explain the delayed or missed physical milestones, as well as perhaps the intellectual "deficit."

I am truly sorry I am making people feel sad. I love and respect everyone who is caring for an Angelman child, and I hate to cause extra hardship. But I feel strongly that all Angelman kids are entitled to anything and everything, at least as a trial, to see how we can help them attain their highest functioning. In sharing Sara's experience with TSS's, I hope to encourage other parents and caregivers to press their governmental agencies to help more. This is a political issue. We need to be vocal, visible, and persistent.

Martha

p.s. I haven't found anything here boring, but maybe that's just me.

Subject: Mental retardation

By the way, Heikki, the "national income allotted to health care" is not paying for this. The reason the US population spends so much per capita on health care is that we have to pay for it through private insurance, which does not cover expenses like Sara's TSS. What is paying for Sara's TSS is tax money allocated to mental health treatment, an infinitesimal part of the national health-care budget. We pay a lot for health care because a)costs of doctors, drugs, hospitals, therapists are obscenely high and b)there is widespread resistance to a national health care plan. We have massive, rich insurance companies who provide no care but skim big profits from what we pay before the providers get paid. That's why I keep saying that this is a political issue. We need a national plan, at least for chronic conditions, that provides equal benefits for patients no matter where in the US they live. Sara gets so much government- funded help because we happen to live in Pennsylvania (but we now have a Republican governor who wants to cut this program, and Sara's social worker tells me that while we are probably safe for the time being, new cases are unlikely to be accepted). So on top of everything else, AS parents have to rely on the shifting sands of politics.

Subject: Re: Mental retardation

At 10:24 4.9.1996 -0500, Martha S. Sprowles wrote:

>Dear Heikki:

>I think we are in accord. I feel sad that I apparently am causing guilt/wrath/unhappiness with >all my blabbering about Sara's progress and questioning MR status (or DD status, if you >prefer). I question DD status because it is not clear to me that a diagnosis of developmental >delay--the catchall for undiagnosed special needs children--does not mean intellectual delay >and nothing but intellectual delay, whether it is measured in terms of IQ or achievement of >physical milestones. I contend that with AS children there are so many physical problems (my >God, read the letters posted here; I had no idea) which I feel could just as well explain the >delayed or missed physical milestones, as well as perhaps the intellectual "deficit."

Dear Martha,

To me at least, DD has been very obvious ever since we noticed that Liisa wasn't developing at a normal pace, although, of course, we had to allow for a certain range. DD doesn't mean intellectual delay and nothing but intellectual delay, it's just that the person can't perform tasks which are normally expected in his/her age group. Does anyone have the same impression of DD ? There are a number of lists and tables describing the things that "normal" children are expected to do. DD may be due to low IQ (i.e., MR), but it may also be caused by illness, neglect or other special circumstances.

Moreover, I forgot to mention in my earlier message that, if the speech impairment and low measured IQ in AS is attributable to something which has happened to the brain - and I'm pretty sure that this is the case - then it is quite possible that the damage is irreparable, even if some medication could be devised. For instance, it could be something going wrong with a neurotransmitter called serotonin, which, incidentally, also takes part in the production of melatonin. But I don't really know much about the biology of the brain - and I understand that the scientists also have a lot to learn - to provide any estimate of whether the damage is reparable or not.

Some people might say that expecting the progress in the medical sciences to bring such improvements is only sci-fi, or wishful thinking on the part of those parents who haven't really come to terms with their child having AS. But even if it is easy to go over the board in this respect, I don't think that is the case with me. In the not too distant future, we are certainly going to see new medications applied to "diseases" which have genetic origins, so why not to AS as well ?

Heikki Taimio, dad to Liisa (6, del-) Finland

Subject: Re: Mental retardation

Dear Heikki,

I understand your point about DD, and I'm really and truly not in denial about Sara's intellectual abilities. I do not have the vocabulary to explain this very well when I say that to me DD is all about intellectual function, no matter what is being measured. If a child's speech is delayed or absent because of a profound deafness, that child is not described as DD. If a child is born with damaged limbs and cannot walk or hold a spoon on schedule, that child is not described as DD. A child who cannot read because of a traumatic head injury is not DD. Our kids have physical and neurological/chemical deficits which lead to delays in development just as those kids do, but ours are described as DD. To me, that means that an assumption of intellectual deficit is being made. Sure, it's semantics, but labels do make a difference as to what services and expectations are available. I am not sure that all professional dealing with our kids do not perhaps unconsciously and automatically "shorthand" them as MR when they see DD in a description.

I am not contending that these are necessarily treatable situations, either, although research is promising in substances like melatonin. And we are just beginning to explore chemical approaches to all kinds of MH like schizophrenia. I am sure that many AS parents like me rely on antidepressant medication to help our own functioning, so that we won't be DD with regard to our children.

I have no problem with anyone's labelling his or her AS child as retarded, or DD. I just object to MR/DD being assumed without any probing of the child's actual, individual status.

Does Liisa like to watch TV? Sara does, but she is very discriminating. She has outgrown many cartoon shows but continues to like Barney and other programs geared to younger children but with lots of singing and dancing. Sara watches opera and ballet, too, as well as '50s musicals. Like most 10 year olds over here, she is a fan of Jim Carrey and the Three Stooges and other physical comedians. To me, this behavior is indicative of intelligence. Other children in her class were invited to Sara's birthday party last year at the movies. We took them to see "Toy Story." The other kids in her class have a variety of disabilities, many of them functioning scholastically at a higher level than Sara. I was shocked to see that none of the kids except Sara could sit attentively for the whole movie, entranced, just as the non-disabled kids sitting near us were.

But when Sara went to respite, they saw MR and made no effort to engage her at an appropriate level. They put on cartoons that she was not interested in and then complained that she was whiney. That's the sort of thing I want to change. If her records showed something like "neurological deficit" I think people would deal with her with more respect.

I'm having a hard time with the school's aides respecting Sara's wishes, too. She does not like to color, and I told Lisa, her TSS, that I didn't want any more hand-over-hand with crayons. Some kids just don't like to color, and with Sara's fine motor problems, it's just too difficult to be fun for her. I don't care if she never colors another picture in her life. Further, she loves to paint, so what's the problem. So at school, when Sara is given crayons, she independently makes a few marks and then gives the crayons back. She is asked (by Lisa) if she's through coloring, and she nods yes. Then the classroom aide comes over and over Lisa's protests that Sara said she was finished, forces Sara hand-over-hand to color more. What has been learned here? That "yes" doesn't mean anything? Sara certainly isn't learning to color, or to like to color. But if it were prominent in the record that Sara has physical difficulty holding crayons and applying pressure, a difficulty being worked on by therapists and not to be cured by brute force, I think this wouldn't have happened. Instead, Sara is DD and of course doesn't know what's good for her, or even what she wants to do.

Holy moly I'm long-winded.

I enjoy this service way too much.

Martha

Subject: Re: Mental retardation

At 09:45 5.9.1996 -0500, Martha S. Sprowles wrote:

>I understand your point about DD, and I'm really and truly not in denial about Sara's >intellectual abilities. I do not have the vocabulary to explain this very well when I say that to >me DD is all about intellectual function, no matter what is being measured. If a child's speech >is delayed or absent because of a profound deafness, that child is not described as DD. If a >child is born with damaged limbs and cannot walk or hold a spoon on schedule, that child is >not described as DD. A child who cannot read because of a traumatic head injury is not DD. >Our kids have physical and neurological/chemical deficits which lead to delays in development >just as those kids do, but ours are described as DD. To me, that means that an assumption of >intellectual deficit is being made. Sure, it's semantics, but labels do make a difference as to >what services and expectations are available. I am not sure that all professional dealing with >our kids do not perhaps unconsciously and automatically "shorthand" them as MR when they >see DD in a description.

Dear Martha,

The important thing on which we agree is that one should not use the label MR as long as there is no conclusive scientific evidence, in particular on IQ below 70. And I understand that not long ago, and apparently even today to many people, including professionals, DD and MR mean much the same thing. Maybe I'm wrong in thinking that DD is measured by failure to attain milestones and not necessarily by low IQ. Your examples relate to what I would call disabilities with known causes. For instance, if a deaf child doesn't speak properly, I understand that this must be allowed for, but if he/she doesn't learn alter- native means of communication like signing, say, because of neglect, then there is DD.

We have a psychologist, Tim Freeman, on line - hello, are you listening ? Maybe you could intervene here. The question is: how do you define and distinguish between developmental delay and mental retardation ?

>Does Liisa like to watch TV? Sara does, but she is very discriminating. She has outgrown >many cartoon shows but continues to like Barney and other programs geared to younger >children but with lots of singing and dancing.

Yes, she does and is discriminating, but we think she hasn't yet grown to really like cartoons. Also, she is very social and therefore often easily distracted from watching TV, if there is someone or something going on in the house she likes to join.

Heikki Taimio, dad to Liisa (6, del-) Finland

Subject: Re: Mental retardation

Martha,

Our discussion on MR and DD has kept bothering me so much that I couldn't wait for Tim Freeman's comments, so I called Liisa's psychologist. He is really an expert on autism, but he has helped us in behavioral and educational issues. As regards MR, many definitions refer to IQ, and there are, of course, a number of different tests. Another school, endorsed by Liisa's psychologist, doesn't want to give a general evaluation of the level of intelligence but, instead, tries to give a broader profile.

DD: earlier, this meant something that was not thought to be permanent. The new definition is a broader one, also including MR. A low level of intelligence is not necessary for this definition, although often evaluations tend to focus on cognitive disabilities.

I don't know what the U.S. law says on this, but it is interesting to note that the Finnish law doesn't recognize the definition of MR which refers to IQ. For all practical purposes, DD and MR have the same meaning.

Liisa's psychologist emphasized that there are different schools of thought, and because of this, you could get rather contradictory evaluations from different psychologists.

Heikki Taimio, dad to Liisa (6, del-) Finland