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Late Consequences of Pediatric Chronic Illness

Last updated: 05-25-2020

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Late Consequences of Pediatric Chronic Illness

Late Consequences of Pediatric Chronic Illness
b
Susan Turkel
a Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California
Find articles by Susan Turkel
Maryland Pao
b National Institute of Mental Health, Office of the Clinical Director, National Institutes of Health, Bethesda, Maryland
a Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California
b National Institute of Mental Health, Office of the Clinical Director, National Institutes of Health, Bethesda, Maryland
Corresponding author.
Susan Turkel, MD, Childrens Hospital Los Angeles, University of Southern California, Keck School of Medicine, 4650 Sunset Blvd #82, Los Angeles, CA 90027, Phone: 323-361-2471, email: ude.csu@lekrutbs
Maryland Pao, MD, National Institute of Mental Health, Building 10, Room 6-5340, Bethesda, MD 20892, Phone: 301-435-5770, Fax: 301-402-2588, Email: vog.hin.liam@moap
Keywords: cystic fibrosis, congenital heart disease, juvenile rheumatoid arthritis, pediatrics, consultation-liaison psychiatry, child psychiatry
The publisher's final edited version of this article is available at Psychiatr Clin North Am
See other articles in PMC that cite the published article.
Introduction
With the advent of new treatments for chronic pediatric disorders such as cystic fibrosis, juvenile rheumatoid arthritis, and congenital heart disease, more children and adolescents are surviving into adulthood than ever before. Seventy years ago, individuals with cystic fibrosis survived an average of 5 years, while currently the life expectancy for cystic fibrosis is over 30 years [ 1 ]. Increased survival has brought new morbidities [ 2 ], and may affect psychosocial outcomes of adult life [ 3 ]. The prevalence of children suffering from a chronic illness varies widely, but the overall rate is 10% to 20% [ 4 ]. Children with chronic illnesses are more likely to have emotional, behavioral and psychiatric symptoms than healthy children [ 5 ] and may be psychologically affected or traumatized by medical treatments [ 6 ]. On the other hand, resilience is common [ 7 ] and chronically ill children do not inevitably develop psychiatric difficulties. This chapter is aimed at helping psychiatric consultants understand how medical, developmental and psychosocial needs are altered in adults who have grown up with chronic pediatric illnesses. Three childhood conditions, congenital heart disease, cystic fibrosis, and rheumatologic disorders, will be discussed in detail, using these conditions as models to illustrate the impact of congenital malformations, genetic disorders, and typically adult disorders occurring in the pediatric age group.
Evaluating Chronically Ill Children and Adolescents
Three aspects of psychiatric consultation in the medically and surgically ill that are specific to working with youth include: 1) an awareness of the cognitive and emotional developmental levels of the patient, 2) the essential role of the family, and 3) a focus on facilitating coping and adjustment to illness in order to follow an optimal developmental trajectory, rather than focus on psychopathology. Clinicians need to be familiar with normal physical, motor, language, cognitive, sexual, and emotional development in chronically ill children in order to distinguish normal responses to stress from unhealthy ones [ 8 ]. Understanding a child’s cognitive ability to process information is essential when communicating about his/her disease. Clinicians cannot assume that chronologic age is equivalent to mental age. Children with medical illness may not develop at the same rate as healthy children because of delayed neurocognitive development, disruptions in education and limited social experiences in addition to the medical condition and treatment influences on intellectual and somatic growth and maturation [ 9 ].
The hospital or clinic environment is often distressing or even traumatic for the chronically ill child. Injections, procedures, and surgeries are highly stressful experiences for children. Pain from both medical conditions and treatments can provoke anxiety and affect later pain sensitivities and neurological development [ 10 ]. Post-traumatic stress disorder (PTSD) is a risk from traumatic injury or intense hospital experiences such as transplantations. Identifying and easing potentially traumatic situations may decrease the child’s stress and improve medical outcomes [ 11 ]. Adult clinicians should inquire into a patient’s childhood medical disorders and treatments as these early experiences surely influence the patient’s trust and utilization of the medical system as they develop into adults.
Impaired growth and development and Impact of Chronic Steroid Use
Physical growth is a dynamic process that starts at conception and ends after full pubertal development [ 13 ]. Chronic illness may lead to growth retardation, either because of the illness itself, or because of treatments required for it. Short stature is commonly perceived to be associated with social and psychological disadvantage [ 14 ]. Parents often attribute behavioral disorders, anxiety, depression, social and attentional problems to short stature, and are concerned that their children are subjected to height related stressors of being teased or infantilized [ 14 ]. However, it is difficult to determine if problems in psychosocial functioning are due to the underlying illness, treatment, or resultant effects such as impaired growth.
Long term administration of systemic corticosteroids (e.g. dexamethasone, prednisone) is a major cause of impaired growth but is often required for children and adolescents with a range of chronic inflammatory, autoimmune, and neoplastic diseases. They are also often used to treat inflammatory bowel disease, asthma, cystic fibrosis, bone marrow and solid organ transplants, nephrotic syndrome and other causes of renal failure, systemic lupus erythematosus, and juvenile arthritis. Children and adolescents with these conditions are at high risk for growth failure, both from their underlying disease and from glucocorticoid therapy.
Multiple mechanisms play a role in glucocorticoid impact on bone development and growth. In the short term, bone loss and deterioration depend on the type and dose of glucocorticoid, and occur most prominently in the first six months of treatment. Treatments directed at preventing bone loss during this period are more effective than attempts to compensate for lost growth later on [ 15 ]. Glucocorticoids have direct effects on the growth plate and disrupt growth plate vasculature. They have a suppressive effect on osteoblastogenesis, and promote apoptosis of osteoblasts and osteocytes. This may lead to decreased bone formation and osteonecrosis or avascular necrosis of bone. Glucocorticoids may promote calcium loss through the kidneys and gastrointestinal tract, increasing bone remodeling and osteoclastic activity due to secondary hyperparathyroidism. High dose glucocorticoid therapy can attenuate physiologic growth hormone secretion and increase somatostatin tone, and may also impair attainment of peak bone mass and delay growth through direct effects on gonadotrophin and sex steroids [ 16 ].
Growth retardation has been reported in children with chronic inflammatory bowel disease (IBD), including ulcerative colitis (UC) and especially in those with Crohn’s disease (CD) [ 17 ]. Typically children with IBD grow more slowly before diagnosis and when disease is active. Growth retardation has been reported in 15% to 40% of children with IBD [ 18 ]. Decreased height velocity may be the earliest indicator preceding the diagnosis of CD. Chronic low nutrition is generally considered an important reason for growth impairment. Treating IBD may restore growth velocity, but ultimately the prolonged use of glucocorticoids may itself lead to growth retardation. Eventual height is usually normal in UC and near normal in CD, and delayed puberty may compensate for the period of poor growth earlier in life [ 17 ]. In pediatric patients with IBD treated with glucocorticoids, there is also a higher incidence of osteoporosis, glaucoma, and cataracts compared to adult patients [ 19 ]. Steroid sparing agents, such as 6-mercaptopurine may prevent growth retardation associated with chronic steroid use [ 18 ]. Mercaptopurine and its prodrug, azathioprine, are effective in maintaining remission in children with CD and may improve growth velocity and final adult height by controlling the disorder and sparing the child long term glucocorticoid treatment [ 20 ].
Children with chronic renal disease may also have growth retardation, and are often dependent on glucocorticoid treatment to control their disease. Prednisone is associated with impairment of growth and body height in a dose dependent fashion [ 21 ]. Children with severe steroid-dependent nephrotic syndrome are at risk of permanent growth retardation due to prolonged courses of steroid treatment [ 22 ]. Suboptimal final height and marked weight gain are common after renal transplant, and may result in significant obesity. After transplant, some children show improved growth, but height remains suboptimal, and steroids needed to maintain the transplant contribute to obesity [ 23 ]. Management of growth retardation before transplantation and further reduction in the steroid dose after transplantation may increase final height of children with chronic renal failure [ 24 ].
In children with severe rheumatic disorders, treatment with glucocorticoids is frequently needed and is associated with growth retardation and osteopenia [ 25 ]. Growth hormone treatment may improve growth and lean body mass, but these benefits disappear when growth hormone therapy is stopped. Long term growth hormone treatment is necessary to maintain a potential positive effect on bone density and metabolism [ 26 ]. Children with mild or moderate juvenile idiopathic arthritis disease and lower medication requirements responded better to growth hormone therapy than those with active disease [ 27 ]. Using growth hormone earlier may prevent growth deterioration and metabolic complications induced by chronic inflammation and prolonged steroid therapy [ 28 ]. Chronic inflammation and prednisone therapy may adversely affect growth, and final height may be closely dependent on both severity of growth retardation during the active phase of the disease and on linear growth after remission [ 29 ]. After remission of active disease and discontinuation of prednisone treatment, 70% of children will show catch-up growth, but 30% show persistent loss of height [ 30 ]. This has led to the recommendation that early initiation of growth hormone therapy may prevent growth deterioration and other metabolic complications induced by chronic inflammation and long term steroid therapy [ 30 ]. Wider use of growth hormone in children and adolescents with rheumatic disorders is not without risk, however, and growth hormone may lead to a flare of previously well controlled systemic lupus erythematosus [ 31 ].
Children with bronchial asthma, allergic rhinitis and atopic dermatitis have a 2–5 times higher incidence of short stature, skeletal retardation and delayed puberty. This is likely secondary to the severity and underlying mechanisms of their disorder. Local growth factor prostaglandin E(2) (PGE(2)), which is important in bone mineralization, is a messenger substance for both the immediate and late allergic reaction. The platelet-activating factor (PAF) is one of the strongest mediators in the pathogenesis of allergic disorders and it influences the PGE(2) synthesis in osteoblasts [ 32 ]. Inhaled and nasal glucocorticoids rarely suppress adrenal function, though they may decrease prepubertal growth [ 33 ].
Children with CF have reduced growth velocity and delayed adolescent growth spurt [ 35 ]. This may result from a combination of poor nutrition, pancreatic insufficiency, chronic inflammatory lung disease, and intestinal disease. Even with vigorous treatment of these problems, including dietary interventions to provide calories and fat soluble vitamins in excess of usual recommended amounts [ 35 ], severe CF is associated with poor weight gain and slower growth [ 36 ]. Relative insulin deficiency rather than nutritional deprivation or poor clinical status may be implicated in the poor linear growth of children with relatively stable lung disease [ 36 ].
Abnormal growth in CF may also be related to the primary dysfunction of a cyclic-AMP-regulated chloride channel. The gene for this chloride channel, CFTR, is found in the thalamus, hypothalamus, and amygdala of the brain, sites related to regulation of appetite, energy expenditure, and sexual maturation [ 35 ]. Inhibition of CFTR inhibits secretion of gonadotropin-releasing hormone in cell lines [ 34 ]. Failure of CFTR function may be related to inhibition of pubertal maturation as well as growth [ 34 ].
Twenty percent of all children in the 1993 National CF Patient Registry were


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