1. Chapter 28 The New Face of Developmental Disabilities
Overview
Effects of silent stroke and overt stroke on children with sickle cell disease
Role of cranial irradiation in treatment of acute lymphocytic leukemia and brain tumors and long-term effects on cognitive function
Impact of multidrug therapy and C-section on outcome in mother-to-child transmission of HIV
Effects of chronic kidney failure on neurodevelopment
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

2. Sickle Cell Disease Sickle Cell Disease (SCD)
Autosomal recessive disorder, results in the production of an abnormal hemoglobin
Chronic hemolytic anemia, crescent-shaped cells
Most common in those with sub-Saharan African ancestry
1 in 2,647 births, almost all states screen newborns
Clinical manifestations
Anemia, jaundice, enlarged spleen, gall stones, restrictive lung disease, pulmonary hypertension, destruction of bone from microvascular occlusion, retinopathy, leg ulcers, and delays in physical growth and sexual maturation
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

3. Sickle Cell Disease (continued)
Abnormal immune function, risk for bacterial infections (leading cause of mortality and morbidity)
Neurological complications
Developmental and behavioral manifestations of SCD
Developmental disability (cognitive and academic domains)
High proportion born into low socioeconomic status
Family stress, increased school absenteeism
Behavior problems (aggression, oppositional defiant disorder, conduct disorder, depression)
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

4. Sickle Cell Disease (continued)
Treatment and management
Prophylactic penicillin to prevent bacterial infections
Bone marrow transplantation and hydroxyurea therapy
Regular doctor visits, immunizations, documentation of spleen size/baseline blood counts
Pain management
Heating pads, acetaminophen/ibuprofen
Stronger medications, hospitalization if more severe
Life expectancy significantly increases with
Early identification
Disease modifying therapies
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

5. Cancer: Acute Lymphocytic Leukemia and Brain Tumors
Most common: leukemias (blood cell cancers) and central nervous system (CNS) tumors
Clinical manifestations
Acute lymphocytic leukemia (ALL): 2,000 children under 15 diagnosed in U.S. each year
More frequent in boys
Peak incidence: 2–6 years of age
Anorexia, fatigue, bone pain, irritability, fever
Depends on tumor location, tumor type, child’s age
Classical symptoms: persistent and severe headache, vomiting
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

6. Cancer: Acute Lymphocytic Leukemia and Brain Tumors (continued)
Treatment:
Induction of remission by irradicating leukemic cells from bone marrow
CNS prophylactic treatment to prevent relapses
Maintenance phase of chemotherapy, 2–3 years
Development and behavior (depends and varies)
Academic progress in reading, math impaired
Poorer neurocognitive outcomes, psychological adjustment problems
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

7. Cancer: Acute Lymphocytic Leukemia and Brain Tumors (continued)
Outcomes
Survival rate for children with ALL: nearly 80%; for CNS cancers, depends on tumor type
Greatly decreased mortality and morbidity, but still challenges in education (repeat grades) and adult employment
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

8. Human Immunodeficiency Virus
Viral infection transmitted by
Sexual contact, blood exposure/needles, mucous membrane exposure, mother-to-child, contaminated blood products
Vertical transmission prevented in 98% of cases
Severe clinical presentation: AIDS
Clinical manifestations
Infancy: enlargement of lymph glands, liver, and spleen, poor weight gain, diarrhea, pneumonia, thrush
Children: recurrent bacterial infections, chronic swelling of parotid, lymphocytic interstitial pneumonia (LIP), progressive neurologic deterioration
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

9. Human Immunodeficiency Virus (continued)
Development and behavior in children infected with HIV
Biomedical factors: the viral load, age at time of infection, clinical symptoms at diagnosis, and treatment
Biosocial factors: prenatal drug exposure, malnutrition, prematurity, poverty, neglect, family stress, developmental disabilities
HIV-related encephalopathy: different profiles, varying impact on cognitive and motor milestones and development
Common behavior problems: psychosomatic symptoms, learning deficits, hyperactivity, impulsive-hyperactive behavior, conduct disorder, and anxiety
Challenges related to stigma
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

10. Human Immunodeficiency Virus (continued)
Treatment and outcomes
Highly active antiretroviral therapy (HAART or ART): greatly decreased mortality/morbidity
Good prognosis related to sustained suppression of the blood viral load and a high CD4 lymphocyte count
Infants and toddlers with lower neuropsychological functioning: at higher risk for later disease progression
In developing countries
ART not available
Prognosis worst in children who have opportunistic infections, encephalopathy, or wasting syndrome
75% mortality rate before age 3
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

11. Chronic Kidney Disease
Chronic kidney disease (CKD): progressive and irreversible loss of renal (kidney) function
Chronic Kidney Disease in Children study: to determine progression and effect on neurodevelopment/cognitive function
Can result from congenital causes, acquired diseases, and genetic disorders
Pediatric incidence: 15 per million, twice as many boys as girls
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

12. Chronic Kidney Disease (continued)
Clinical manifestations
Progression: kidney no longer able to balance electrolytes, filter waste from blood, and regulate blood pressure
Stages 1 and 2: generally asymptomatic until renal function deteriorates
Polyuria (excessive passage of urine), oliguria (decreased passage of urine), anuria (absence of urine), edema (abnormal accumulation of fluid in body tissues), hypertension (elevated blood pressure), hematuria (blood in urine), proteinuria (protein in urine)
Symptoms of severe renal impairment: anorexia, vomiting, weakness, fatigue, deficits in neurocognitive function
See the textbook Children with Disabilities, Seventh Edition, for full citation and reference information.
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

13. Chronic Kidney Disease (continued)
Neurodevelopment and behavior
Increased risk for delays in neurocognitive development
Historically: high rates of mortality and morbidity
Lower IQ, memory difficulties, problems with executive functioning
Lower health-related quality of life
Poorer physical, social, emotional, and school functioning
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.

14. Chronic Kidney Disease (continued)
Treatment and management: slow progression of kidney failure, correction of metabolic disturbances, nutritional and hormonal support for growth failure, and preparation for renal replacement therapy
When a child reaches end-stage renal disease (ESRD), renal replacement therapy must be initiated
Ultimate goal for children with ESRD is kidney transplantation
Outcome: no cure, but improvements in treatment = improved neurocognitive function and slowed renal disease progression
Chapter 28 slides in Children with Disabilities, Seventh Edition, Online Companion Materials. Copyright © 2013 Paul H. Brookes Publishing Co., Inc. All rights reserved.