Valkenburg, A.J.

Without Uttering a Word: Pain assessment and management in intellectually disabled children (Doctoral Thesis)

2012-11-30T00:00:00Z

This thesis addressed several studies on pain assessment and management, as well as general anesthesia and sedation, in intellectually disabled children with a focus on children with Down syndrome. The pain sensitivity of children and adults with Down syndrome has been widely debated but rarely studied. Parents rated their children with Down syndrome as less sensitive to pain, but this was not confirmed by quantitative sensory testing. Children with Down syndrome will remain dependent of pain assessment by proxy, since self-report was not adequate. Previous studies found that intellectually disabled children receive lower doses of analgesics during general anesthesia. On the other hand, children with Down syndrome are often described more agitated and “difficult to sedate” after surgery. We found no reasons for different morphine dosing after cardiac surgery in children with Down syndrome compared to children without Down syndrome. As pain behavior of intellectually disabled children and neonates is different from what caregivers would expect, more objective methods for the assessment of pain and distress are badly needed. This asks joint effort of medical professionals, researchers, the industry and even parents. More than a million intellectually disabled children, children below the age of 4, and demented elderly in the Netherlands will benefit from improved objective methods to measure pain, as self-report may be unreliable or impossible.

The COMFORT-Behavior scale is useful to assess pain and distress in 0- to 3-year-old children with Down syndrome (Article)

2011-06-02T00:00:00Z

Many pediatric intensive care units use the COMFORT-Behavior scale (COMFORT-B) to assess pain in 0- to 3-year-old children. The objective of this study was to determine whether this scale is also valid for the assessment of pain in 0- to 3-year-old children with Down syndrome. These children often undergo cardiac or intestinal surgery early in life and therefore admission to a pediatric intensive care unit. Seventy-six patients with Down syndrome were included and 466 without Down syndrome. Pain was regularly assessed with the COMFORT-B scale and the pain Numeric Rating Scale (NRS). For either group, confirmatory factor analyses revealed a 1-factor model. Internal consistency between COMFORT-B items was good (Cronbach's α = 0.84-0.87). Cutoff values for the COMFORT-B set at 17 or higher discriminated between pain (NRS pain of 4 or higher) and no pain (NRS pain below 4) in both groups. We concluded that the COMFORT-B scale is also valid for 0- to 3-year-old children with Down syndrome. This makes it even more useful in the pediatric intensive care unit setting, doing away with the need to apply another instrument for those children younger than 3.

Pain management in intellectually disabled children: Assessment, treatment, and translational research (Article)

2010-12-01T00:00:00Z

The primary focus of pain research in intellectually disabled individuals is still on pain assessment. Several observational pain assessment scales are available, each with its own characteristics, its own target group and its own validated use. Observational studies report differences in the treatment of intra- and postoperative pain of intellectually disabled children and almost all children with intellectual disability have comorbidities that need to be addressed. The scope of research has started to broaden. In this review we aim to answer the question: Can we integrate validated ways of pain assessment and postoperative pain treatment in intellectually disabled children to develop specific analgesic algorithms? Regrettably there is little knowledge on possible interaction effects and other relevant pharmacological issues. Possible genotype-phenotype associations related to pain in children with Down syndrome have several promises as six possible candidate genes are located on chromosome 21. In conclusion, the pain assessment tools for intellectually disabled children are there. We should now focus on tailoring the pain treatment. To this aim we need to perform pharmacokinetic and pharmacodynamic studies of analgesics and obtain information about the genotype-phenotype relationships for pain. This can lead to the development of specific analgesic algorithms.

Lower bispectral index values in children who are intellectually disabled (Article)

2009-11-01T00:00:00Z

BACKGROUND:: Very few data are available on the use of bispectral index (BIS) monitoring in children who are intellectually disabled. Epileptiform electroencephalogram activity, underlying cerebral pathology, or anticonvulsant/spasmolytic therapy might influence BIS monitoring.Our aim in this exploratory study was to first compare BIS values at 4 different stages of anesthesia between intellectually disabled children and controls. Our second aim was to investigate the discriminative properties of BIS between consciousness and unconsciousness for intellectually disabled children and for controls. METHODS:: Eighteen intellectually disabled children and 35 control children, aged 2-13 yr, were included. BIS values, landmark events, and standard monitoring values of vital functions were recorded throughout the whole procedure. The performance of BIS in distinguishing between a conscious and unconscious state was assessed from receiver operating characteristic curves. RESULTS:: Median (interquartile range) BIS values for the intellectually disabled group were significantly lower than those for controls in the awake state (72 [48-77] vs 97 [84-98], P < 0.001), during stable intraoperative anesthesia (34 [21-45] vs 43 [33-52], P = 0.02), and during return of consciousness (59 [36-68] vs 73 [64-78], P = 0.009). The discriminative properties of the BIS monitor for the state of consciousness were comparable between the 2 groups according to the receiver operating characteristic curves. Nevertheless, the optimal cutoff BIS value for discrimination between conscious and unconscious state was 28 points lower for the intellectually disabled group. CONCLUSIONS:: We advise anesthesiologists to be alert to possible lower BIS values in intellectually disabled children. There is a risk that they will inadvertently misinterpret the state of consciousness in intellectually disabled children. New multicenter studies must find the optimal manner of evaluating (un)consciousness in intellectually disabled patients with documented and confirmed specific etiologies of their intellectual disability. Copyright

Extremely low preanesthetic BIS values in two children with West syndrome and lissencephaly (Article)

2008-05-01T00:00:00Z