Robert Karp, MD
Joseph Sleiman, MD
SUNY-Downstate Medical Center
Brooklyn, NY
Steven Bachrach, MD
Nemours/A. I. duPont Hospital for Children
Thomas Jefferson University
Pretest:
Q1. True or False
There is no relation between malnutrition in infancy and increased risk of infection later in childhood.
Q2. Best answer.
To maintain ideal weight for height (or BMI):
a. An athetoid 17-year-old boy would need same number of calories as a normal 17-year-old boy.
b. A 17-year-old boy with spastic cerebral palsy would need about 1500 kilocalories per day.
c. A normal 17-year-old boy needs approximately 2000 kilocalories per day.
d. Children with different types of cerebral palsy need the same daily caloric requirements.
Q3. Best answer.
Deficiency of which of the following nutrients has been implicated in the development of bronchopulmonary dysplasia in the premature infants:
a. Vitamin E
b. Vitamin D
c. Zinc
d. Folate
Q4. True or False
Broad spectrum antibiotics commonly used in pulmonary infections can change the colonic flora resulting in malabsorption of vitamin k.
True
Q5. Best answer.
Prevention of malnutrition in chronic illness includes:
a. Keeping child on a restrictive diet.
b. Assessment of nutritional status and a complete nutritional history and physical examination.
c. Separation of children from their parents in case of disturbed parental care.
d. Assessment of tissue nutrient levels in every routine evaluation.
Q6. True or false
Metabolic rates can be profoundly increased or decreased by various illnesses, thus altering the total metabolic needs of the patient.
Q7. Best answer
In cystic fibrosis malnutrition is caused by:
a. Increased metabolic needs and decreased intake.
b. Fat malabsorption.
c. Recurrent pulmonary infections.
d. All of the above.
Learning Objectives
At the conclusion of this section, residents and physicians will able to:
1. Identify the mechanisms by which malnutrition occurs in chronically ill children with special reference to pulmonary disease.
2. Review the forms of malnutrition likely to occur.
3. Understand the biologic effect of chronic illness on nutritional status.
4. Provide a conceptual framework to link biologic and social aspects of nutrition as related to the care of chronically ill children.
5. Prevent malnutrition in the chronically ill child.
Facilitator Preparation
This module is derived from Karp RJ, Bachrach SJ, Moskowitz S. Malnutrition in Chronic Illness of Childhood with Special Reference to Pulmonary Disease. Clinics in Chest Medicine, 1980;1:375-383 It addresses many of the unique cultural as well as biologic and nutritional responses to chronic illness.
Contemporary comments on these issues can be found in:
- Sanjur D. (1982) Social and cultural perspectives in nutrition. Prentice Hall. Engelwood Cliifs, NJ.
- Reilly JJ. Proc Nutr Soc. 2002 Aug;61(3):321-7.
- Thomsen C. Respir Med. 1997 May; 91(5): 249-54.
- Two researchers who have explored food culture extensively are Paul and Elizabeth Rozin. A paper is available through Paul Rozin's web-site "Rozin, P. (1999). Food is fundamental, fun, frightening, and far-reaching. Social Research, 66, 9-30."
- Elizabeth Rozin has published Crossroads cooking (1999) Viking Press as part of a set of writings exploring her family's engagement with food and flavor.
- Karp RJ, Bachrach SJ, Moskowitz S. Malnutrition in Chronic Illness of Childhood with Special Reference to Pulmonary Disease. Clinics in Chest Medicine, 1980;1:375-383
- Davison, S (ed) 1988) Davidson and Passmore Human nutrition and dietetics 8th edition. Churchill Livingstone, London.
Registered Dieticians are trained to pay attention to the broad scope of nutrition related problems of chronically ill patients. Krause's Food, Nutrition and Diet Therapy, for example, is a highly respected RD's text.
INTRODUCTION:
Chronic illness has a multiplicity of nutritional consequences including any of four consequences of a diet inappropriate for need. These are
1. obesity,
2. protein-energy malnutrition,
3. specific nutrient deficiency, and
4. nutrient imbalance. [Hyperlink to Nutritional Assessment]
TEACHING CAPTION: these four characterizations of malnutrition often occur together. An obese child may have a specific nutrient deficiency and be at high risk for nutrient imbalance.
Obesity itself is a laying down of excess fat. In its usual context in a community setting, exogenous obesity results from inappropriate responses to appetite. This definition is used because it emphasizes the importance of appetite control, which may be disrupted in chronic illness. Children with diseases that lessen the level of caloric expenditure, such as spina bifida, are often obese.
Caloric and/or protein intake below needs is expressed as protein-energy malnutrition (p-e m). Two forms of this disorder exist, kwashiorkor and marasmus with a spectrum of abnormalities in between depending on the age and nutritional needs and health of the individual. Marasmus is caused by lack of both proteins and calories. It is expressed by wasting and stunting, and edema is not present. Kwashiorkor is another form of protein calorie malnutrition in which edema is present. As discussed in the section on nutritional assessment, (and contrary to common teaching) there is invariable deficit in energy intake with kwashiorkor. Whether edema occurs depends more on the rapidity of caloric deficit occurrence and the internal milieu of the affected individuals. [Hyperlink to Nutritional Assessment]
Both kwashiorkor (p-e m with edema) and marasmus (p-e m without edema) may occur at all ages and result in loss of parietal and visceral proteins, growth retardation, interference with brain growth early in infancy and an increase incidence of infection and death later in childhood. Moreover, prolonged malnutrition may result in nutritional dwarfism later in life, even when recovery from an underlying illness is complete.
Caloric intake is the vehicle for carrying specific nutrients. Any aberration in the diet can lead to vitamin or mineral deficiencies if there is a reduction in nutrient/energy ratio of the food consumed. Nutrient imbalance indicates that the body is getting too much of a nutritious substance in either absolute or relative quantities.
Thus, it is evident that every child with a chronic illness is "at risk" for malnutrition and development of an inappropriate food consumption pattern-a food culture of chronic illness. In this context "at risk" means that there are social and biologic precursors to malnutrition present in the life of the chronically ill child that make the occurrence of malnutrition likely.
Assessment of the risk for malnutrition is best accomplished by active teamwork among physicians, nurses, clinical dieticians, and other health professionals. [Hyperlink To Nutritional Assessment] Careful review of the history, physical examination and laboratory data is recommended as in any clinical setting. Changes in anthropometric measurements are an early indicator of malnutrition, and further studies such as measurements of nutrient levels are indicated.
Chronic illnesses themselves are often a direct cause of malnutrition. The effects of disease processes on the nutrition are divided into two categories: the changes in nutrient needs, which are caused by illness, and the effects of illness on availability of nutrients to the patient. Metabolic rates can be profoundly increased or decreased by various illnesses, thus altering the total metabolic needs of the patient. For this reason, underweight and obesity are frequent nutritional problems in handicapped children. Overall caloric needs are clearly reduced in patients with physical handicaps that impede walking, such as spina bifida, where obesity is a common occurrence. In addition to their inactivity, these children may receive excessive calories through overprotective feeding or eating out of frustration, a mechanism that one can see in any chronic illness. It was estimated that an athetotic 17-year-old boy would need 6000 kilocalories per day to maintain ideal weight for height. BY contrast a child of the same age and weight with spastic cerebral palsy would need only 1500 kilocalories per day. A normal 17-year-old boy needs approximately 3000 kilocalories per day.
Physical impediments interfere with normal oral food intake, such as neurological diseases that result in swallowing dysfunction or in children with tracheoesophageal fistula or who are intubated for respiratory failure. Anorexia can be a direct result of illness itself or secondary to medications or depression. Medications also have multiple effects, which may interfere with the availability of nutrients.
A Case study :
Tony T is a 2 year-old white Caucasian boy with cystic fibrosis, diagnosed recently secondary to chronic diarrhea, failure to thrive and 3 episodes of broncho-pulmonary infections. He was admitted to the hospital many times for recurrent pneumonia and was treated by broad-spectrum antibiotics. His nutritional assessment showed a weight of 8.5 kg, which is less than the 5th percentile for age and sex. His height of 82 cm places him in the 10th to 25th percentile. He came in today with his parents who are concerned about his nutritional status and have many questions about his growth and development and what can be done at this point to help him gain weight. His weight to length ratio is below the 5th [XXXX] percentile for gender and age.
Q1. Why is Tony smaller than children of the same age?
A1. Children with chronic pulmonary disease, as a group, tend to be smaller than their healthy counterpart, probably because of a combination of increased metabolic needs and decreased intake. The overall metabolic needs of these infants are increased because of the increased work of breathing as well as the increased needs associated with infections, congestive heart failure, and countless other stresses.
In cystic fibrosis, fat malabsorption plays a prominent role in the lack of availability of sufficient calories, as well as specific nutrients, such as fat-soluble vitamins. However, cystic fibrosis patients with severe pulmonary disease only can be more growth-retarded than those with slight pulmonary involvement and malabsorption. Other factors inherent in the disease (e.g. chronic hypoxemia, recurrent pulmonary infections) interfere with nutrient availability in some way, resulting in growth retardation.
With Tony, length and now height have been protected by vigorous nutritional therapy [Hyperlink to CF module]. Because he has not become stunted, a regain of weight will assist in maintaining linear growth.
Q2. Are there specific nutrient deficiencies to be considered with a chronic illness such as cystic fibrosis?
A2. Chronic illnesses may cause changes in nutrient availability as well as in nutrient needs. Each illness must be considered separately. Is there a hypermetabolic state as in hyperthyroidism? Is there a malabsorbtion as in cystic fibrosis? Is hepatic or renal metabolism affected as in hereditary forms of rickets or in chronic organ specific disease?
Tony's cystic fibrosis provides an example of an illness that affects multiple systems. Pancreatic insufficiency (present in 85 to 90 % of patients) results in the malabsorption of fat and protein. Though children with cystic fibrosis are often described as having voracious appetite, studies have shown that they actually ingest 80 to 90 % of the total number of calories recommended for age, usually because of reduced fat intake. Furthermore calories that are ingested are frequently lost as stool fat secondary to malabsorption. These children need energy supplements in some readily absorbable forms.
There are also specific nutrient deficiencies in cystic fibrosis, some of which may be responsible for growth failure. One is linoleic acid deficiency, which causes desquamating skin lesions, thrombocytopenia, poor wound healing, increased susceptibility to infection, and growth retardation. There are also deficiencies of the fat-soluble vitamins, A, D, E, and K and children with cystic fibrosis should routinely receive supplements of these vitamins, although they may or may not correct the deficiency. There are also reports of zinc and vitamin B12 deficiency in some children with cystic fibrosis.
Q3. What are the factors other than the disease itself that can lead to malnutrition?
A3. Malnutrition in patients with chronic illness such as Tony's cystic fibrosis may be a consequence of intervention resulting from:
TEACHING CAPTION: These three phenomena are likely with any chronic illness. Simply treating the disease process without addressing the consequences of treatment is a sure prescription for treatment failure.
An example for medications that affect nutrient metabolism, is the use of broad-spectrum antibiotics with pulmonary infections. They often change the colonic flora, resulting in malabsorption of vitamin K. Other examples, commonly used to treat pulmonary diseases, are corticosteroids, antihistamines, and isoniazid.
Dietary manipulations often result in poor nutrition. Classic examples of this used to be treatment for milk allergy with a milk-free diet putting the patients at risk of becoming deficient in both calcium and vitamin D, and the use of elimination diets in nonspecific diarrhea of infancy, which can lead to protein calorie malnutrition.
Q4. What do you mean by "cuisine of the chronically ill child" and how could it be a cause of malnutrition?
A4. A child is born into a family and a food culture. Usually the family resides within a community of people who share this food culture and provide the essential food stuffs and flavorings for its maintenance. A cuisine is the food culture of a community considered in its entirety, its elements being:
TEACHING CAPTION: Food is consumed in a social setting. What and how one eats is highly constrained by the family and community food culture.
Of these, the third, "flavor principal" provides the most characteristic label for a cuisine and the culture group that maintain it. Thus food is more than nutrient.
When physicians, nurses, dieticians, and other health professionals advise dietary changes, it is certain that the subtle (or not so subtle) changes in food that are used, how they are prepared and flavored and the social setting in which they are served can lead to a cuisine of a chronically ill child that separates him from his family. Thus prevention of this cause for malnutrition begins with identification of the nature of the home life of the affected child and an attempt to maintain the integration of the food culture of the child with that of his family. The learning of an acceptable flavor early in life for the benefit of identifying nutritious foods is part of the developmental pattern. It is in this phase of child development that the child is most accepting of new flavors. The process is expansive in its early stages and the child is willing to try new foods as every food is new to him. The negativism of later childhood would be very destructive if expressed too early.
There are no universally accepted or rejected food flavors. For example, early exposure to rancid fish oils allows Eskimo children to accept foods containing vitamin D that protect them from rickets but that would be exceedingly distasteful to American children. [Hyperlink To Nutrition Notes]
When dietary restrictions are made among chronically ill children, the normal period of encouragement may be lost and a child may enter into older childhood and adult life without ever becoming a part of the food culture of his parents. An important developmental event is missed. The effect on a chronically ill child of missing this developmental milestone will differ according to the age of the child and the severity of the illness. Children who have early severe restrictions placed on their free intake of food are more likely to be malnourished than children with respiratory or other chronic illnesses that develop later in childhood.
Q5. What influence does the social setting of a chronically ill child have on his nutritional status?
A5. In addition to the biologic mechanisms we have discussed, malnutrition in the children with early chronic illness may be exacerbated by the severe disruption in family life.
Three related elements of the social setting of feeding of children with chronic illness deserve emphasis:
TEACHING CAPTION: Chronically ill children showing signs of malnutrition may require the services of a skilled family counselor or therapist. Having an experienced RD as part of the health care team often serves to help. Social workers are often able to support families. If needed however, it may be necessary to have psychologists involved.
The effects of separation are evident. Restriction in itself interferes with the normal socialization of the child to the culture of his family, of which food is an important element. While in the hospital, many different people are in charge of what the child eats, how much of it, and how often. In infants, where most of the interaction with the family is eating-centered, this separation disrupts the normal maternal-infant bonding process, often manifesting itself in a neglected or abused child after the child goes home to the family. In older children with chronic illnesses, similar disturbances are seen. Inappropriate and/or disturbed parental care, in the form of neglect or overprotection and inability to set limits, often is manifested around eating, leading to either overnutrition or under nutrition in many of these children.
The response of the child to his restrictions can be placed on a scale from over acceptance to non-acceptance. By easy acceptance of limitations, the affected child does not have to confront disapproval of physician or parent. Passive acceptance is easier for physicians and parents to deal with but developmentally is inappropriate as the child grows. He remains dependent on his family, and finds it difficult to separate. In an adolescent struggling with issues of dependence and independence, and who already has less control over his life than his peers because of his illness, issues of compliance with diet or medications will often be seen. One needs to prescribe dietary limitations as necessary, but one also needs to allow for as much fostering of growth and independence as possible.
The Case study continues
Resident exercise: Look closely at the roles played by non - physicians. Role play how they contribute to the care of these children
The physician team working with Tony included a dedicated (full time) Registered Dietician and a Social Worker available from the ambulatory care center. Psychological services were available, but were not needed. The RD created a diet for Tony that met nutritional needs and gave the boost needed for weight gain. She also addressed the dependency issues that were affecting his behavior and d food consumption. The social worker helped the family get additional; resources to feed Tony. She also discussed some of the very personal and emotional issues affecting the parents as likely when a child has a hereditary disease. The physicians monitored the health status and kept pulmonary problems at a minimum.
Q6. How can we prevent malnutrition in chronic illness?
A6. The essential difficulty in confronting the problem of malnutrition in chronically ill children is the integration of a social with a biologic approach to the patient. Integrating these two approaches will influence success in preventing or at least modifying the consequences of malnutrition. Every patient with chronic illness is "at risk" for malnutrition. The nature of that risk should be assessed at the initial contact with the patient and the family and then periodically. Alterations in nutrient needs and consequences of treatment must be recognized and addressed at the time of initial contact. Problems related to feeding and nutrition should be discussed with the patient and his family from the very beginning.
The initial assessment includes a complete nutritional history and an assessment of nutritional status. Anthropometric measurements are easily obtained. These measurements should be adjusted for age and sex and recorded on flow sheets and growth curves. The assessment of tissue nutrient levels should not be made as part of the routine evaluation. It should only be performed where there is a concern raised for nutrient adequacy from the nutritional history, the physical examination, or the nature of the illness. We cannot overemphasize the importance of addressing the problem of iatrogenic malnutrition with the parents and the child at the time when the patient/health professional relationship is being established.
Parents and physicians alike should recognize the consequences of intervention. While it is hoped that dietary recommendations will be based solely on the needs of the patient, it is not unfair to suggest that the advice of health professionals regarding feeding reflects their own cultures, in addition to their knowledge.
CONCLUSION
Alterations in nutritional status are likely with or without intervention. The potential for harm or benefit must be assessed when intervention is essential. Prevention of malnutrition requires integrating an understanding of nutrient needs of specific patients with the social and emotional needs of children and their families. Prevention, treatment, and rehabilitation protocols are most likely to succeed if they are able to enhance the pleasure of eating and not allow a cuisine of chronic illness to develop.
Post-test:
Post-Q1. Best answer
The elements of a cuisine are:
1. Foods that are consumed.
2. Means of preparation.
3. Distinctiveness of flavoring.
4. Social setting for food consumption.
5. All of the above.
Answer 5
Post-Q2. True or false.
The overprotected chronically ill child will rebel as he enters adolescence, becoming a defiant teenager.
True
Post-Q3. Best answer.
To maintain ideal weight for height:
1-An athetoid 17-year-old boy would need same number of calories as a normal 17-year-old boy.
2-A 17-year-old boy with spastic cerebral palsy would need about 1500 kilocalories per day.
3-A normal 17-year-old boy needs approximately 2000 kilocalories per day.
4Children with different types of cerebral palsy need the same daily caloric requirements.
Answer 2
Post-Q4. True or False.
Broad spectrum antibiotics commonly used in pulmonary infections can change the colonic flora resulting in malabsorption of vitamin k.
True
Post-Q5. Best answer.
Prevention of malnutrition in chronic illness includes:
1-Development of a cuisine of the chronically ill child.
2-Assessment of nutritional status and a complete nutritional history and physical examination.
3-Separation of children from their parents in case of disturbed parental care.
4-Assessment of tissue nutrient levels in every routine evaluation.
Answer 2
Post-Q6. True or False
Inappropriate and/or disturbed parental care, in the form of overprotection and inability to set limits, often is manifested around eating, leading always to overnutrition.
False
The REFERENCES are listed in the facilitator's preparation.
Annotated Answers:
A1. The answer is false. The single most important contributor to long tern survival in children with cystic fibrosis and other chronic diseases is preventing malnutrition.
A2. The answer is b. Children and adolescents with spastic CP do not need an inordinate amount of energy intake. It is important that they do maintain modest weight gain through childhood. It is athetoid CP that triggers a need for extra caloric intake
A3. The answer is a. Vitamin E is an anti-oxidant nutrient. Minerals are oxidants.
A4. The answer is True. The principal source of Vitamin K is from production in and absorption from the intestinal tract. Broad spectrum antibiotics will destroy the microorganisms needed to produce Vitamin K.
A5. The answer is b. It is essential to make assessment of nutritional status and a complete nutritional history and physical examination a part of every patient visit. Restrictive diets are often the source of malnutrition. The often occurring of parent child conflict should be resolved trough interaction with the health care team. Tissue levels are rarely needed.
A6. The answer is true. Different disease produce different metabolic responses.
A7. The answer is d. Cystic fibrosis is associated with fat malabsorption. The children are often feeling ill and do not want to eat, or perhaps they are rebelling against the restrictions. They also have chronic inflammation. Caloric needs can run quite high.
