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Study 4 Citation: Defining the Impact of Hemophilia: The Academic Achievement in Children With Hemophilia Study

 

Journal

PEDIATRICS Vol. 108 No. 6 December 2001, pp. e105
Authors
Amy D. Shapiro, MD*, Sharyne M. Donfield, PhD, Henry S. Lynn, PhD, Valerie A. Cool, PhD, James A. Stehbens, PhD, Scottie L. Hunsberger, Sharon Tonetta, PhD, Edward D. Gomperts, MD and the Academic Achievement in Children with Hemophilia Study Group

 

Institutions: 

* Indiana Hemophilia and Thrombosis Center, Indianapolis, Indiana
Rho, Inc, Chapel Hill, North Carolina
University of Iowa Hospitals and Clinics, Iowa City, Iowa
|| Baxter Healthcare Corporation, Hyland Immuno Division, Glendale, California

 

Abstract:
The authors looked at the impact severe hemophilia was postulated to have on academic achievement. To do this the authors investigated a population-based group of children with regard to their method of treatment one year prior to study inclusion (on-demand or prophylactic), and their number of bleeding episodes during this time as well. They also examined the relationship between the number of bleeding episodes during the year before enrollment and their academic achievement, taking into account other precipitating factors known to have an effect on achievement. Additionally, the mechanisms thought to be present between bleeding episodes and academic achievement were considered.

Study Design: Boys with severe hemophilia (factor VIII—clotting factor <2%) were enrolled in this US-based study from multiple investigation centers. Several factors disallowed children from enrolling, including non-fluency in English, psychiatric disorders, developmental disorders and a demonstrated history of inhibitor. For this study the researchers chose to measure academic achievement using the Wechsler Individual Achievement Test and quality of life was measured by the Child Health Questionnaire. Limitations to the amount of physical activity, the kinds and amount of schoolwork or the amount of social activities engaged in, and any measurable pain were quantified by the Physical Summary (PhS) portion of this questionnaire, and were found to be of particular interest.
 

Key words:

WIAT, Wechsler Individual Achievement Test • WISC-III, Wechsler Intelligence Scale for Children-Third Edition • PhS, Physical Summary • HGDS, Hemophilia Growth and Development Study

 

 Discussion:

Children were stratified into two distinct groups: those that had never received prophylaxis before study enrollment, and those that had. The correlation was made as to prophylaxis being the more effective treatment regimen for controlling bleeds, and as such, reducing the impact on academic achievement. Significantly, children that had fewer bleeding episodes had better PhS scores than those that had more bleeds. In fact, children in the low bleeding group had PhS scores similar to that of the general US population. Children in the higher bleed group had a PhS score nearly a full standard deviation lower than the the mean for the US population.

Hemophilia is a disease that affects a broad range of circumstances among school-aged children, namely their physical, social and academic activities. Many of these points of impact have been linked to the disease’s effects on the musculoskeletal system. Currently, alternative treatments that limit these musculoskeletal complications are under investigation1-4. These effects can have a distinct influence on academic achievement due to increased school absences. Hemophilia is seen by several researchers as causing the highest rates of school absenteeism among several chronic diseases. In other studies, researchers have reported lower than expected academic achievement scores, and found that physical changes attributed to hemophilia have a pronounced effect on academic achievement as well (coordination and gait abnormalities).

The Hemophilia Growth and Development Study (HGDS), that most of this previous research belongs to, has now spawned the Academic Achievement in Children with Hemophilia study group that investigates hemophilia-rated psychological data within U.S. school children. This group investigates the collection of social, demographic data and it’s relationship to academic achievement.

 

Study Data

# of children

131

 

mean age

9.6 years

 

on prophylaxis at enrollment

62%

 

 

 

 

on-demand at enrollment

38%

 

 

 

 

presently on prophylaxis

9%

 

 but on-demand in past

 

 

 

 

 

 

 

 

treatment duration

2.7 months - 7.7 years

 

 

 

 

# of bleeding episodes

prophylaxis group

on-demand group

 1 year before enrollment

6

25.5
 

Academic Achievement

Score*

 

reading

math

total achievement

</=11 bleeding episodes

104

105.2

104.4

>11 bleeding episodes

98.6

99.6

100.6

 

 

 

 

*after adjustment for child's IQ and parents' education

 

 
 
 

The study had 3 objectives:

  1. Characterize the cohort with respect to type of treatment (on-demand vs. prophylaxis) and frequency of bleeding episodes in the year before enrollment;

  2. Determine the association between hemophilia-related morbidity, measured by number of bleeding episodes, and academic performance after adjustment for other factors known to have an effect on achievement13,14;

  3. Explore the mechanisms of the association between frequency of bleeding episodes and academic achievement levels.

 

The investigators proposed 2 mechanisms for the association between bleeding episodes and academic achievement levels. First, bleeding episodes could lead to diminished ability to take advantage of academic opportunities, in part because of school absenteeism. Second, factors that limit or interfere with physical functioning could have an effect by reducing the ability to complete schoolwork and participate in school-related activities. Examples of these factors include pain or discomfort such as that which accompanies a bleeding episode or is associated with chronic arthropathy.

 

Results:

The results, according to the authors, were measured by looking at the number of bleeding episodes and achievement. When children were categorized as above or below the median by the number of bleeding episodes they had in the previous year, those who had a low number of bleeding episodes had higher total achievement and mathematics scores than children in the higher bleeding episode category. Most children in the low bleeding episode group were being treated with prophylaxis. Children who had been treated on a regimen of long-term prophylaxis (>40% of their lifetime) and reported 11 bleeding episodes in the year before enrollment had significantly higher scores in mathematics, reading, and total achievement than all other children reporting 12 bleeding episodes in the same time period.

Summary of Study 4

People with severe hemophilia have long been known to suffer morbidity from the musculoskeletal complications associated with recurrent bleeding events. These complications have led to investigation of replacement therapy methods to minimize or prevent these sequelae. Primary prophylaxis converts  hemophiliacs from a state of severe clotting factor deficiency, to a moderate or mildly deficient state. Although prophylaxis has known treatment benefits for severe hemophilia,15,16,17,18 it is not universally practiced. Product availability as well as cost, patient acceptance and compliance, and difficulty in delivering frequent infusion therapy because of venous access problems remain as the major issues to be faced today. 

This study has identified an important association between the number of bleeding episodes experienced and academic achievement in a cohort of school-aged children with severe hemophilia. The data supports the assertion that therapeutic care programs in this population must not be evaluated only in terms of financial cost to achieve adequate musculoskeletal outcomes. Other considerations are the individual and societal benefits of child's increased academic accomplishments if adequate suppression of hemorrhagic events can be attained.

References:

  1. Manco-Johnson MJ, Nuss R, Riske B, et al. Optimal therapy for severe factor VIII deficiency: study design considerations for a US prospective, randomized controlled trial. Haemophilia (book of abstracts from the XXIII International Congress of the World Federation of Hemophilia). 1998;4:97
  1. Risebrough NA, Oh PI, Feldman BM, Dean J, Manco-Johnson M, Blanchette V. Cost-effectiveness model for factor VIII prophylaxis strategies in hemophilia A. Haemophilia (book of abstracts from the XXIII International Congress of the World Federation of Hemophilia). 1998;4:202
  1. Feldman BM, Rivard G, Israels S, et al, for the Association of Hemophilia Clinic Directors of Canada. Preliminary results from the Canadian Hemophilia Prophylaxis Trial. Haemophilia (book of abstracts from the XXIV International Congress of the World Federation of Hemophilia). 2000;6:272
  1. Manco-Johnson M, Numms R, Abshire T, Richardson L, Shapiro A, Valentinos L, for the Hemophilia Joint Preservation Study Group. Port infections in children with haemophilia. Haemophilia (book of abstracts from the XXIV International Congress of the World Federation of Hemophilia). 2000;6:275
  1. Weitzman M. School absence rates as outcome measures in studies of children with chronic illness. J Chronic Dis.1986; 39:799–808
  1. Markova I, McDonald K. Integration of hemophilic boys into normal schools. Child Care Health Dev.1980; 6:101–109
  1. Woolf A, Rappaport L, Reardon P, Cibrorowski J, D’Angelo E, Besette J. School functioning and disease severity in boys with hemophilia. J Dev Behav Pediatr.1989; 10:81–85
  1. Olch D. Effects of hemophilia upon intellectual growth and academic achievement. J Genet Psychol.1971; 119:63–74
  1. Colgrove RW, Huntzinger RM. Academic, behavioral, and social adaptation of boys with hemophilia/HIV disease. J Pediatr Psychol.1994; 19:457–473
  1. Loveland KA, Stehbens J, Contant C, et al. Hemophilia Growth and Development Study: baseline neurodevelopmental findings. J Pediatr Psychol.1994; 19:223–239
  1. Sirois PA, Usner DW, Hill SD, et al, and the Hemophilia Growth and Development Study. Relationships between neuropsychological, neurological, and MRI findings at baseline. J Pediatr Psychol.1998; 23:45–56
  1. Usner DW, Donfield SM, Sirois PA, Gomperts ED, Bale JF, Mitchell WG, and the Hemophilia Growth and Development Study. Hemophilia morbidity, cognitive functioning, and academic achievement. J Pediatr.1998; 133:782–787
  1. Sattler JM. Assessment of Children. 3rd ed. San Diego, CA: Jerome M. Sattler, Publisher; 1988
  1. Kemp LCD. Environmental and other characteristics determining attainments in primary schools. Br J Educ Psychol.1955; 25:66–77
  2. Nilsson IM, Berntorp E, Lofqvist T, Pettersson H. Twenty-five years’ experience of prophylactic treatment in severe haemophilia A and B. J Intern Med.1992; 223:25–32
  3. Aledort LM, Haschmeyer RH, Pettersson H, and The Orthopaedic Outcome Study Group. A longitudinal study of orthopaedic outcomes for severe factor-VIII–deficient haemophiliacs. J Intern Med.1994; 236:391–399
  4. Manco-Johnson MJ, Nuss R, Geraghty S, Funk S, Kilcoyne R. Results of secondary prophylaxis in children with severe hemophilia. Am J Hematol.1994; 47:113–117
  5. Liesner RJ, Khair K, Hann IM. The impact of prophylactic treatment on children with severe haemophilia. Br J Haematol.1996; 92:973–978

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