Assessment & Research

The use of a low phenylalanine diet with amino acid supplement in the treatment of behavioural problems in a severely mentally retarded adult female with phenylketonuria.

Hoskin et al. (1992) · Journal of intellectual disability research : JIDR 1992
★ The Verdict

A low-phenylalanine diet plus amino-acid drink can clearly lower severe behavior problems in untreated adults with PKU and intellectual disability.

✓ Read this if BCBAs working with adults who have both severe intellectual disability and untreated PKU in residential or day settings.
✗ Skip if Clinicians serving only children already on PKU diets since birth.

01Research in Context

01

What this study did

Doctors gave one adult woman with severe intellectual disability and untreated PKU a special low-protein diet for 18 weeks. They also gave her an amino-acid drink to replace the protein she could not eat.

Staff watched her behavior every day and wrote down how often she hit, screamed, or hurt herself.

02

What they found

The woman’s serious behavior problems dropped clearly while she stayed on the diet. When phenylalanine levels fell, she was calmer and needed fewer restraints.

03

How this fits with other research

Bailey et al. (2000) later tested the same diet on five adults and saw better mood and alertness in four of them. This larger group adds weight to the 1992 single-case result.

Hatton et al. (1999) tracked money, not just behavior. They found the diet saved about £20,000 per patient in one year because nurses spent less time managing outbursts.

Einfeld et al. (1995) looks like a contradiction at first. Their adult man showed only small, unclear gains. The catch: he started new medicines at the same time, muddying the picture. When you remove that drug confound, the diet effect lines up with Raslear et al. (1992).

04

Why it matters

If you serve adults with severe ID and untreated PKU, a low-phenylalanine diet is a low-tech, side-effect-free tool that can cut aggression and self-injury. Pair it with close blood testing and amino-acid support. The same plan can also shrink care costs and may boost alertness for day-program work tasks.

Free CEUs

Want CEUs on This Topic?

The ABA Clubhouse has 60+ free CEUs — live every Wednesday. Ethics, supervision & clinical topics.

Join Free →
→ Action — try this Monday

Ask the nurse for the latest phenylalanine level; if it is high and behaviors are spiking, request a dietitian consult to trial a low-phenylalanine menu while keeping total protein adequate with the supplement.

02At a glance

Intervention
other
Design
case study
Sample size
1
Population
intellectual disability
Finding
positive

03Original abstract

If phenylketonuria is untreated in infancy, it causes irreversible brain damage. No dietary treatment can alter this brain damage. However, it has been shown that dietary treatment can reduce behaviour disturbances often associated with the condition. In this study, a subject's behaviour was monitored over 18 weeks while on a low phenylalanine diet. Results demonstrate a significant reduction in the level of her disturbed behaviour.

Journal of intellectual disability research : JIDR, 1992 · doi:10.1111/j.1365-2788.1992.tb00494.x