Assessment & Research

Long-term prognosis of children with Down's syndrome and leukaemia: a 34-year nation-wide experience.

Rajantie et al. (2003) · Journal of intellectual disability research : JIDR 2003
★ The Verdict

Kids with Down syndrome can beat leukemia, but hospitals must watch them closely for treatment side effects.

✓ Read this if BCBAs who support children with Down syndrome during medical treatment.
✗ Skip if Practitioners looking for behavior-intervention data.

01Research in Context

01

What this study did

Doctors tracked every child with Down syndrome who got leukemia in one country for 34 years.

They counted how many kids lived five years after standard chemo.

02

What they found

About half of the children were still alive five years later.

Survival was good, but many kids died from chemo side effects, not cancer.

03

How this fits with other research

Griffith et al. (2012) show that special hospital nurses help adults with ID stay safer in hospital. Their work hints that extra support might cut the chemo deaths J et al. saw.

Hoch et al. (1994) found high rates of dementia in older hospitalized adults with ID. Together with J et al., the picture is clear: people with ID face extra medical risks at every age.

van Esch et al. (2018) measured quality of life in adults on long-term drugs. J et al. give the survival numbers; Lotte et al. remind us to weigh quantity and quality of life when planning tough treatments.

04

Why it matters

You can’t change chemo, but you can flag medical risks early. Share this paper with families so they know to ask for extra nursing support, hydration checks, and infection watch. Your behavior data on eating, drinking, and activity can alert nurses before small problems turn deadly.

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→ Action — try this Monday

Graph the child’s daily fluid intake and share any downward trend with the nurse the same day.

02At a glance

Intervention
not applicable
Design
case series
Sample size
41
Population
down syndrome
Finding
positive

03Original abstract

BACKGROUND: Although the characteristics of leukaemia in patients with Down's syndrome (DS) have been well documented, little is known about the long-term results of treatment. METHOD: Retrospectively from 1968 to 1981 and prospectively from 1982 to 2002, the present authors collected data on every child with DS in Finland who had been diagnosed with leukaemia between 1968 and 1994. RESULTS: Forty-one children with DS had acute leukaemia: 28 had acute lymphoblastic leukaemia (ALL); and 13 had acute non-lymphoblastic leukaemia (ANLL). The median age of the subjects at diagnosis was 3.8 years (range = 0-15.9 years). Patients with ANLL were significantly younger (P = 0.001) and all patients under 2 years of age had ANLL. Out of the 28 patients with ALL, 23 (82%) entered primary remission, and of these 23 individuals, 10 remained alive and in continuous remission (CR) after a median of 11.6 years (range = 8.9-20.0 years). Out of the 13 patients with ANLL, five (38%) entered remission and four remained in CR after a median of 16.0 years (range = 9.1-19.2 years). Treatment -related toxicities were common: eight patients with ALL and two with ANLL died of septicaemia. Actuarial, event-free survival rates at 5 years were 53% and 43% for adequately treated subjects with ALL and ANLL, respectively. CONCLUSIONS: Standard leukaemia chemotherapy is effective in patients with DS. However, because toxicities are unacceptably frequent, specific anti-leukaemia regimens are needed for subjects with DS design.

Journal of intellectual disability research : JIDR, 2003 · doi:10.1046/j.1365-2788.2003.00477.x