Childhood Disintegrative Disorder

Dame Stephanie Shirley discusses her experiences of CDD (Childhood Disintegrative Disorder).

When my son Giles was about 2½ years old, it was already clear that he was learning disabled. But whereas up to then he had progressed day by day, a really scary thing happened. He started – over a very short period – to regress.

I use the word in the sense of losing skills. The little speech Giles had just seemed to dry up. Previously he had kept up a modest stream of appropriate words ‘up’ ‘more’ ‘car’ to express his wishes: I remember him going quieter and me trying to stimulate his speech with “What’s this?” and “What do you want?” questions. But within a few days, Giles was silent.

Except, that is, for the screaming and tantrums stemming from his frustration.

I got him into the Oxford Park hospital then operating as a psychiatric diagnostic unit for children. Patients lived there Monday to Friday usually for two weeks after which a diagnosis was made. But with Giles they couldn’t decide what was wrong so he was there for 8½ months (for part of which I was trying to run my business from the associated Mother’s Unit). Then the learned Professor came out with his bombshell diagnosis: we should expect our son to lose his other senses of hearing, sight and motor skills. Over what timescale? He couldn’t, or wouldn’t, say.

Although he did not call it CDD (Childhood Disintegrative Disorder) it was such an appalling prognosis that, even after 8½ months, we asked for a second opinion. So, Giles was transferred to Great Ormond Street Hospital who diagnosed severe autism (then considered a rare disorder affecting 1 in 20,000 boys and not germane to girls).

That was in the 1960’s. And Giles did NOT lose his other faculties.

So what is CDD? It is also known as Heller’s syndrome after the remedial teacher Theodor Heller working early in the 20th century in Vienna (where else?). My charitable foundation funded a CDD study in 2015 to find out very basic facts starting with how many new cases were seen each year and what treatments, if any, doctors were using. The 13-month study was run through BPSU (British Paediatric Surveillance Unit) and CAPSS (Child and Adolescent Psychiatry Surveillance System) fully anonymised, of course. Follow up at one and two years is leading to a CDD workshop at Glasgow’s 2018 Annual Child Psychiatry conference on 21 September.

CDD is defined in terms of abnormal reciprocal society interaction and social communication (of the type defined by Autism Spectrum Disorders); restrictive, repetitive and stereotyped behaviour; and a general loss of interest in what is around them. It is classed as very rare (1 in 50,000 children both boys and girls) and we do not know what causes this horrendous condition.

We only know some negatives: CDD is not attributable to acquired aphasia with epilepsy; selective mutism; schizophrenia; Rett Syndrome; neuro degenerative diagnosis; or acquired brain injury.

The great Leo Kanner believed otherwise but I do wonder if CDD isn’t just a delayed variant of regressive autism.