Comparison of the Very Young Child (ages 0 through 5) With the Young Child (ages 6 through 10) in a Prolonged Low Response State Following Severe Brain Injury

By: Julia Buch-Wamstad, Psy.D., Lisa Cantore, NP, RN, BSN, MSN, CCRN, Kenneth Norwood, M.D., Jodi Darring, B.S., James Blackman, M.D. M.P.H., Peter D. Patrick, PhD

No studies have investigated the differences between young children, specifically the differences in presentation of the very young child (ages 0 through 5) with the young child (ages 6 through 10) after sustaining a traumatic brain injury.

Subjects

Twenty-one children who remained in a prolonged low response (LR) state for longer than 30 days post injury were studied. The subjects were 11 children, ages 0 through 5, and 10 children ages 5 through 10.

Methods

This descriptive study of children with acquired brain injury investigates the differences between the very young children (VYC) and young children (YC) in a prolonged low response state. Data was gathered during the patient’s inpatient rehabilitation admission to Kluge Children’s Rehabilitation Center (KCRC). The study also begins to explore potential differences between patients who sustained inflicted injuries compared to patients who did not.

Results

The VYC in a LR state progressed more slowly than the YC in an LR state. When compared to YC the VYC are more likely to be in a vegetative state upon admission to KCRC, and at discharge, the VYC are less likely to have progressed beyond a vegetative state than YC. The VYC are also less likely to be in a low response state as a result of a TBI and more likely to be in a low response state as a result of a non-traumatic injury. The VYC are much more likely to be in a low response state as a result of an inflicted injury than the YC.  The VYC in LR state may not qualify or are less likely to progress to rehabilitation than the YC in an LR state resulting in a shorter hospital stay for the VYC.

Conclusions

There appears to be important differences in etiology, presentation, and outcome between the VYC and YC. There may also be differences between children who sustained inflicted injuries compared to those who did not. These potential differences should be explored further.

Introduction

The study of disorders of consciousness in children focuses primarily on diagnostic criteria, epidemiology, etiology, pathology, and prognosis (particularly life expectancy), as well as medical aspects and ethical and legal controversies related to the child in a vegetative or minimally conscious state (Ashwal 2002, Ashwal 2003, Strauss 2000, Ashwal 1994). No studies, however, have investigated the differences between young children, specifically the differences in presentation of the very young child (ages 0 through 5) with the young child (ages 6 through 10). These studies have not investigated short term outcome based on etiology of the vegetative or minimally conscious state, specifically whether the brain injury was due to an inflicted injury (i.e. non-accidental trauma) or not.

This descriptive study of children with acquired brain injury due to traumatic brain injury (TBI) or non-traumatic injury (NTBI) investigates the differences between very young and young children in a prolonged low response state (does not reach RLA IV or more within 30 days post injury). This study also lays the foundations for future research to explore differences between children who are in a low response state as a result of an inflicted injury with children who did not sustain an inflicted injury.

Overview of low response program for severe pediatric acquired brain injury

At the University of Virginia Children’ Hospital, Kluge Children’s Rehabilitation Center (KCRC), a primary focus is on addressing the needs of children and adolescents who cannot, or do not, regain functional arousal, awareness, and communication following a severe brain injury. These children remain in a static, prolonged low response state (LRS) characteristic of Rancho Los Amigos (RLA) Levels I, II and III.

The majority of the patients that we see who are in a LRS are over the age of 11; however, the presentation of the very young child (ages 0-5) in LRS appears to be different from the young child (ages 6-10) in a LRS. Assessing consciousness in very young children is difficult. Although the RLA scale has limitations for use with the very young child, we have not found a more reliable alternative to assess consciousness in the very young child. 

Table 1 provides the percentages of children who would meet criteria for two traditional groupings, namely, vegetative and minimally conscious (Cartlidge, 2001) upon admission and discharge to KCRC rehabilitation.

Table 1. Admission status to and discharge status from the low response program

 

Ages 0-5

Ages 6-10

 

Admission

Discharge

Admission

Discharge

N

11

11

10

10

Vegetative State (RLA I-II)

73%

27%

60%

0%

Minimally Conscious State (RLA III)

27%

64%

40%

40%

Conscious (RLA IV)

0%

9%

0%

60%

The very young patients admitted with a RLA I or II, 62.5% progressed to a RLA of 3 or more during their inpatient hospitalization, while 37.5% did not progress beyond a RLA of 2. The young patients admitted with a RLA of 2, 100% progressed to a RLA of 3 or more during their inpatient hospitalization.

The KCRC low response inpatient program admits the child into an “acute medical bed” and initially addresses comorbid medical conditions or iatrogenic factors that may be contributing to their “complicated emergence” from the low response state. Once comorbid or iatrogenic factors are addressed, some children will spontaneously recover functional skills and move through the RLA levels (Figure 1).

The large majority of the children we see remain in a low response state even after these factors have been minimized or eliminated. These children are considered to be in a “neuropathic state” that is the primary influence in their failure to regain basic cognitive skills (Patrick, 2006). Children in a prolongedly low response state are considered for pharmacological trials to improve arousal and awareness. Clinical decision making in each case depends on clinical presentation, and we have also begun to reference MRI pattern to inform clinical judgment. See Patrick (2009) for more information.

Throughout the medication trial, the children are monitored for side effects. Changes are assessed using the Western NeuroSensory Stimulation Profile (Ansell, 1989) (administered by a speech-language pathologist) and serial neurobehavioral assessments (administered by a neuropsychologist). Pharmacological trials of dopamine enhancing medications are not initiated on all children in a low response state. Only 55% of very young children in this study received such medications, while 90% of young children were started on a dopamine enhancing medication. Our clinical experience has demonstrated a tendency for these children to become more agitated on dopaminergics, so other options are explored.

In addition to the pharmacological trial, the children receive a full array of medical, nursing and traditional therapies (occupational, physical, and speech therapy) as part of their activities. While addressing medical need, basic therapies also address range of motion, positioning and daily stimulation.

Figure 1. Emergent pathways following coma

 

 

Comparison of very young children with young children in a low response state

We currently have a database of 21 children under the age of 11 who were enrolled in the low response program. Characteristics of these children are described in Table 2.

Table 2. Characteristics of children in the low response program

 

0-5

6-10

N

11

10

Gender

7 males, 4 females

8 males, 2 females

Type of injury

45% TBI, 55% NTBI

70% TBI, 30% NTBI

Average age at injury

1.76

8.86

Average GCS

5.00 (N=1/11)

3.60 (N=5/10)

Average Grados

3.80 (N=10/11)

4.80

Progress to rehabilitation services?

27% Yes

80% Yes

Acute Length of Stay (LOS)

46.27 days

57.40 days

Rehab LOS

7.00 days

33.90 days

Total LOS

53.27 days

91.30 days

Inflicted Injury

27% Yes

0% Yes

The Glasgow Coma Scale (GCS) (Teasdale, 1974) is widely accepted as a measure of impaired consciousness. However, GCS for the very young child as well as the young child is not routinely recorded and caution is advised when interpreting means. In addition, Reilly (1988) highlights that, in its standard form, the GCS is not applicable to children under the age of 5, and a pediatric version of the GCS should be used.

According to Grados (2001), children with deep brain lesions after TBI are likely highly vulnerable to neurological and functional disability. We used the Grados classification system (Table 3) to determine the depth of lesion for each patient. Very young children had a mean Grados of 3.80, while young children had a mean Grados of 4.80. This indicates that although young children in this study had deeper brain lesions than very young children, other factors, such as etiology and mechanism of injury (which leads to differences in pathophysiology), likely contribute to outcome.

Table 3. Grados Depth of Lesion Ratings

Grados Rating

Depth of Lesion Group

1

Frontotemporal

2

Frontotemporal/Brain Stem-cerebellum

3

Corpus callosum

4

Basal ganglia

5

Thalamus

6

Brain stem-cerebellum

As is seen in Table 2, the very young child spends considerably less time, on average, in a formal inpatient rehabilitation program than the young child. If a patient progresses from an acute medical bed to rehabilitation, LOS at KCRC is generally determined by the patient’s ability to participate and make measurable gains. If a patient is not able to participate fully in inpatient rehabilitation or does not continue to make measurable gains, they are typically discharged home and outpatient therapies become the primary source of rehabilitation.

Almost all children had medical comorbidities that required treatment. This is not surprising, given the fact that TBI has been said to be a “multi-system disease” that can affect nearly every body function (Blackman 2009). In addition to medications to improve cognition, subjects were on a variety of other medications, including agents for spasticity, seizures, and storming. “Storming” medications were used for a cluster of symptoms including hypertension, tachycardia, and hyperthermia (see reference for Paroxysmal Autonomic Instability with Dystonia in Blackman, 2004 and Diagnosing Dysautonomia after TBI in Baguley, 2009). Data on differences in types of medications used for the different age groups are presented in Table 4.  

Table 4. Medical Comorbidities

 

Ages 0-5

Ages 6-10

Medical Comorbidity?

91% Yes

100% Yes

LR medication

55% Yes

90% Yes

Seizure medication

82% Yes

70% Yes

Spasticity Medication

45% Yes

70% Yes

Storming Medication

36% Yes

50% Yes

Inflicted vs. Not Inflicted

We are interested in whether or not children in low response states with inflicted injuries (most commonly due to non-accidental trauma) differ in their presentation and outcomes from children in low response states who did not sustain an inflicted injury. In our database of children in low response states, 3 very young children sustained an inflicted injury. This compares to 8 very young children in a low response state who did not sustain an inflicted injury. No child in our database over the age of 6 sustained an inflicted injury. While the number of subjects is too small to make definitive statements about differences between the two groups, directions for future research should expand upon this data to better investigate what differentiates the child in a low response state who sustains an inflicted injury compared to the child who did not sustain an inflicted injury. 

Table 5. Characteristics of children with inflicted vs. not inflicted injuries

 

Inflicted

Not Inflicted

N

3

8

Gender

2 males, 1 females

5 males, 3 females

Type of injury

100% TBI, 0% NTBI

25% TBI, 75% NTBI

Average age at injury

1.11

2.00

Average GCS

No data

5.00 (N=1/8)

Average Grados

3.50 (N=2/3)

3.88

Progress to rehabilitation services?

0% Yes

37.5% Yes

Acute LOS

39 days

49 days

Rehab LOS

0 days

10 days

Total LOS

39 days

59 days

Table 6. Admission and discharge status of children with inflicted vs. not inflicted injuries

 

Inflicted

Not Inflicted

 

Admission

Discharge

Admission

Discharge

Vegetative State (RLA I-II)

67%

0%

75%

37.50%

Minimally Conscious State (RLA III)

33%

100%

25%

50%

Conscious (RLA IV)

0%

0%

0%

12.50%

 

Table 7. Medical Comorbidities of children with inflicted vs. not inflicted injuries

 

Inflicted

Not Inflicted

Medical Comorbidity?

100% Yes

88% Yes

LR medication

67% Yes

50% Yes

Seizure medication

67% Yes

88% Yes

Spasticity Medication

67% Yes

38% Yes

Storming Medication

33% Yes

38% Yes

Conclusion and Lessons Learned

We have had great interest in understanding the course of events for the child in a prolonged low response state. Specifically, we are interested in exploring how children in low response states under the age of 11 respond to treatment, both pharmacological as well as non-pharmacological.  

  • This study highlights the idea that not all children in a low response state are similar in their presentation, course of treatment, and outcome. Specifically, there appear to be age differences between the very young child and the young child in a low response state.
  • When compared to young children, very young children are more likely to be in a vegetative state (compared to a minimally conscious state) upon admission to KCRC. In addition, at discharge, the very young child is less likely to have progressed beyond a vegetative state than the young child.
  • The very young child is less likely to be in a low response state as a result of a TBI and more likely to be in a low response state as a result of a NTBI.
  • The very young child is less likely to progress to rehabilitation than the young child.
  • Because they often do not qualify for rehabilitation, the very young child has a shorter total length of stay than the young child.
  • The very young child is much more likely to be in a low response state as a result of an inflicted injury than the young child. 
  • The very young child is much less likely to be started on a dopamine enhancing medication than the young child.
  • Very young children and young children are comparable in whether or not they are started on seizure medication or medication to help with storming.
  • Very young children are less likely to be started on medication for spasticity than young children.
  • Future research should focus on the differences between children in a low response state who sustain an inflicted injury compared to children who do not sustain an inflicted injury.
  • Future research should explore long term outcomes (activities of daily living, quality of life, etc.) for the child who is injured either at a very young age or a young age.



References

  1. Ansell, B.J., Keenan, J.E., & De La Rocha, O. (1989). Western Neuro Sensory Stimulation Profile (Tustin, California: Western Neuro Care Center).
  2. Ashwal, S. (2003). Medical aspects of the minimally conscious state in children. Brain & Development, 25,535-545.
  3. Ashwal, S. & Cranford, R. (2002). The minimally conscious state in children. Seminars in Pediatric Neurology, 9(1), 19-34.
  4. Ashwal, S., Eyman, R.K., & Call, T.L. (1994). Life expectancy of children in a prolonged vegetative state.Pediatric Neurology, 10, 27-33.
  5. Baguley, I.J., Nott, M.T., Slewa-Younan, S., Heriseanu, R.E., & Perkes, I.E. (2009). Diagnosing dysautonomia after acute traumatic brain injury: Evidence for over responsiveness to afferent stimuli.Archives of Physical Medicine and Rehabilitation, 90(4), 580-586.
  6. Blackman, J.A. (2009). Medical aspects of the low response state after TBI. Brain Injury Professional 6(1), 16-19.
  7. Blackman, J.A., Patrick P.D., Buck, M.L., & Rust, R.S. (2004). Paroxysmal Autonomic Instability with Dystonia after brain injury. Archives of Neurology, 61, 321-328.
  8. Cartlidge, N. (2001). States related to or confused with coma. Journal of Neurology Neurosurgery, & Psychiatry, 71(supp I), i18-i19.
  9. Grados, M.A., Slomine, B.S., Gerring, J.P., Vasa, R., Bryan, N., & Denckla, M.B. (2001) Depth of lesion model in children and adolescents with moderate to severe traumatic brain injury: Use of SPGR MRI to predict severity and outcome. Journal of Neurology, Neurosurgery, & Psychiatry 70(3), 350-358.
  10. Patrick, P.D., Blackman, J.A., Mabry, J.L., Buck, M.L., Gurka, M.J., & Conaway, M.R. (2006) Dopamine agonist therapy in low-response children following traumatic brain injury. Journal of Child Neurology, 21(10), 879-885.
  11. Patrick, P.D., Wamstad, J.B., Norwood, K.W., Darring, J.G., Cantore, L., Blackman, J.A. (2009). Descriptive Analysis of Children and Adolescents in a Low Response State Following Severe Brain Injury.International Brain Injury Association Newsletter.
  12. Reilly, P.L., Simpson, D.A., Sprod, R., & Thomas, L. (1988). Assessing the conscious level of infants and young children: a paediatric version of the Glasgow Coma Scale. Child’s Nervous System, 4(1), 30-33.
  13. Strauss, D.J., Ashwal S., Day, S.M., & Shavelle, R.M. (2000). Life expectancy of children in vegetative and minimally conscious states. Pediatric Neurology, 23, 312-319.
  14. Teasdale, G. & Jennett, B. (1974). Assessment of coma and impaired consciousness. A practical scale.Lancet, 2, 81-84