- February 1, 2000 - American Academy of Family Physicians

Editorials

Hypercholesterolemia in Children

RAE-ELLEN W. KAVEY, M.D.
State University of New York Health Science Center
Syracuse, New York

See article in this issue.

Over the past two decades, convincing evidence has emerged linking defined risk factors with atherosclerosis, the pathologic basis for cardiovascular disease. Postmortem studies have clearly shown that elevated total and low-density lipoprotein (LDL) cholesterol levels are associated with strong evidence of accelerated atherosclerosis beginning in childhood.^1,2 In this issue of American Family Physician, Shamir and Fisher³ review the guidelines for lipid testing in children and provide a complete and practical guide to the initial approach to dietary treatment.

The guidelines for lipid measurement were developed by the Pediatric Panel of the National Cholesterol Education Program (NCEP) in 1991.⁴ It is important to emphasize that universal screening of children was not recommended. Cholesterol levels measured in childhood correlate statistically with levels measured in young adults, but the correlation is not strong. Less than one half of children with total cholesterol levels above the 90th percentile in childhood will remain at that level as young adults. For an individual, only 25 percent of an adult cholesterol level can be predicted from the pediatric value.⁵

In response to this information, the NCEP Pediatric Panel recommended a policy of selective screening with lipid determination only in children with a positive family history of premature cardiovascular disease in an expanded first-degree pedigree (i.e., to include not only parents and siblings, but also aunts, uncles and grandparents), or a history of parental hypercholesterolemia. This algorithm aims to identify the majority of children at greatest risk of having high cholesterol as adults, without inappropriately labeling normal young people as "diseased." Overall, this conservative approach appears reasonable, but it has some limitations. As in the original screening protocol for adults, the focus is exclusively on identification and modification of elevated total and LDL cholesterol levels. An important missing element is the role of high-density lipoprotein (HDL) cholesterol.

HDL cholesterol levels vary inversely with risk for cardiovascular disease: an isolated low HDL cholesterol is a powerful independent predictor of premature coronary artery disease.⁶ Among men younger than 45 years of age hospitalized with a myocardial infarction, a low HDL cholesterol level is identified as the major lipid abnormality more often than an elevated LDL cholesterol level. HDL levels are largely unaffected by diet composition, but they decrease significantly with obesity and exposure to cigarette smoke, and increase with habitual exercise and alcohol intake. Identification of low HDL cholesterol levels in a child from a family with a positive history of premature coronary disease is as critical as identification of elevated total and LDL cholesterol levels. It is a significant limitation of the NCEP Pediatric Panel guidelines that the issues surrounding HDL cholesterol are not addressed.

With a dramatic increase in prevalence over the past 20 years, obesity is now one of the most serious health problems facing young persons in this country. Depending on definition, as many as 25 percent of U.S. children and adolescents are overweight.⁷ The increased prevalence of childhood obesity parallels the large increase in adult obesity that has occurred over the past two decades: from NHANES III, with data collected between 1960 and 1994, the prevalence of obesity in adults increased from nearly 13 percent to 22.5 percent of the U.S. population, with most of that increase occurring in the past decade.⁸

Almost all coronary risk factors are increased with obesity, including elevated total and LDL cholesterol levels, reduced HDL cholesterol levels, hypertension and insulin resistance.⁹ In an autopsy study of children dying traumatically, elevated body mass index was the risk factor that correlated most highly with increased histologic extent of atherosclerosis.¹⁰ Importantly, even small amounts of weight loss have been shown to result in normalization of the lipid profile in obese adolescents.¹¹ In comparison, in children with genetically based lipid abnormalities, diet interventions are often only minimally effective.

As Shamir and Fisher state in their article,³ obesity in and of itself should be considered an indication for lipid measurement in children. Appreciating the difference in response to diet therapy means that practitioners can confidently recommend diet change in obese children. Pediatric care professionals are in a unique position to attempt to prevent obesity by identifying a positive family history for obesity in infancy. Early identification of weight for height disproportion as it develops, especially in the setting of family obesity, can lead to diet and exercise recommendations that may help prevent the development of progressive obesity.¹²

Finally, nutrition counseling requires knowledge, training, experience and time, all of which physicians frequently lack.¹³ Shamir and Fisher³ provide some excellent aids for evaluation of current diet and handouts to assist families in making recommended diet changes. Referral to a registered dietitian is an option in helping families learn to translate prescribed levels of fat and saturated fat intake into practical recommendations for shopping and eating.¹⁴ In particular, referral to a registered dietitian is useful for institution of the step II diet, where fat intake is less than 20 percent of total calories and saturated fat is less than 7 percent. In managing hypercholesterolemia in childhood, recommended diet changes represent a potential lifetime commitment so all available resources including referral to a registered dietitian need to be considered.

Beginning in childhood, elevated cholesterol levels represent a major risk factor for premature cardiovascular disease. Cholesterol-lowering diets have been shown to be a safe and effective method of reducing total and LDL cholesterol levels without impairment of growth and development in children. Shamir and Fisher³ provide a very useful guide to the identification and management of hypercholesterolemia in childhood.

REFERENCES

Pathobiological Determinants of Atherosclerosis in Youth Research Group. Relationship of atherosclerosis in young men to serum lipoprotein cholesterol concentrations and smoking. JAMA 1990;264:3018-24.

Berenson GS, Srinivasan SR, Bao W, Newman WP 3d, Tracy RE, Wattigney MS. Association between multiple cardiovascular risk factors and atherosclerosis in children and young adults. The Bogalusa Heart Study. N Engl J Med 1998;338:1650-6.

Shamir R, Fisher EA. Dietary therapy of hypercholesterolemia in children. Am Fam Physician 2000: 61:675-82,685.

National Cholesterol Education Program Expert Panel on Blood Cholesterol Levels in Children and Adolescents. Report of the Expert Panel on Blood Cholesterol in Children and Adolescents. Bethesda, Md.: National Heart, Lung, and Blood Institute. NIH Publication no. 91-2732, 1991.

Lauer RM, Clarke WR. Use of cholesterol measurements in childhood for the prediction of adult hypercholesterolemia. The Muscatine Study. JAMA 1990;264: 3034-8.

Gordon DJ, Probstfield JL, Garrison RJ, Neaton JD, Castelli WP, Knoke JD, et al: High-density lipoprotein cholesterol and cardiovascular disease. Four prospective American studies. Circulation 1989;79: 8-15.

Troiano RP, Flegal KM. Overweight children and adolescents: Description, epidemiology, and demographics. Pediatrics 1998;101:497-504.

Kuczmarski RJ, Flegal KM, Campbell SM, Johnson CL. Increasing prevalence of overweight among US adults. The National Health and Nutrition Examination Surveys, 1960 to 1991. JAMA 1994;272: 205-211.

The American Pediatric Society and the Society for Pediatric Research annual meeting. San Diego, Calif., May 7-11, 1995. Pediatr Res 1995;37:108A.

Berenson GS, Srinivasan SR, Bao W, Newman WP 3d, Tracy RE, Wattigney WA. Association between multiple cardiovascular risk factors and atherosclerosis in children and young adults. N Engl J Med 1998;338:1650-6.

Becque MD, Katch VL, Rocchini AP, Marks CR, Moorehead C. Coronary risk incidence of obese adolescents: reduction by exercise plus diet intervention. Pediatrics 1988;81:605-12.

Flodmark CE, Ohlsson T, Ryden O, Sveger T. Prevention of progression to severe obesity in a group of obese schoolchildren treated with family therapy. Pediatrics 1993;91:880-4.

Young EA. National Dairy Council Award for Excellence in Medical/Dental Nutrition Education Lecture, 1992: perspectives on nutrition in medical education. Am J Clin Nutr 1992;56:745-51.

Rhodes KS, Bookstein LC, Aaronson LS, Mercer NM, Orringer CE. Intensive nutrition counseling enhances outcomes of National Cholesterol Education Program dietary therapy. J Am Diet Assoc 1996;96:1003-12.

Dr. Kavey is a professor of pediatrics and preventive medicine in the Division of Pediatric Cardiology at the State University of New York Health Science Center.

Address correspondence to Rae-Ellen W. Kavey, M.D., State University of New York Health Science Center, Syracuse College of Medicine, Division of Pediatric Cardiology, 725 Irving Ave., Suite 804, Syracuse, NY 13210.

Family Physicians Should Be Experts in Palliative Care

ROBERT B. GWINN, D.O.
Hospice of Coshocton County,
Coshocton, Ohio

I will not endure chemotherapy. I will not go through that indignity. If this is the way I am to end my life, that's fine. I don't want to retch and writhe in nausea. I don't want to lose my hair and dignity. I am ready to go if that is what is demanded of me.

--Doris Pareson, 19221999

See article in this issue.

Regardless of the wisdom of the choices Doris made as she faced the diagnosis of metastatic breast cancer, one thing is clear: she was adamant that she would retain her dignity and control of her life. She did not waver from this stand. Several months later, Doris died quietly and free of pain in her own bed, attended by her sister and her niece. The death of this patient is a demonstration of recent advances in end-of-life care.

The World Health Organization has defined end-of-life care, typically referred to as hospice care or palliative care, as "the active total care of patients whose disease is not responsive to curative treatment. Control of pain, of other symptoms, and of psychologic, social and spiritual problems is paramount. The goal of palliative care is achievement of the best quality of life for patients and their families."¹

Terminally ill patients in the United States are increasingly choosing hospices for their end-of-life care. In 1998, 3,100 hospice programs served approximately 540,000 dying patients in this country.²

By virtue of their lifelong relationship with patients, most family physicians practice some form of end-of-life care. It is a fact that many of our patients will die while under our care. Consequently, the sole goal of saving life is unrealistic. When confronted with a patient who is approaching the end of life, we need to change our treatment goal from saving life to ensuring that the patient has the best quality of life for as long as possible. This is the essence of palliative care. By virtue of our training and practice ideals, family physicians are in a unique position to champion this philosophy of terminal care.

Because family physicians provide care throughout life, they become family friends. Thus, they are skilled advocates for patients and their families in all aspects of health care. There have been some studies suggesting that longitudinal care may have some benefit over tertiary care.

A 1995 survey of end-of-life care in five tertiary care centers, conducted as part of the Study to Understand Prognoses and Preference for Outcomes and Risks of Treatment (SUPPORT),³ found that 38 percent of dying patients spent more than 10 days in the intensive care unit and that only 47 percent of the treating physicians knew that their patients preferred to avoid resuscitation.

A similar but smaller study⁴ of terminally ill patients treated by primary care internists found that these physicians had cared for the patients for an average of over one year and had been the primary medical care providers for the majority of them. In this community carebased model, 78 percent of the patients' families recalled discussing end-of-life preferences with their physician. Furthermore, only 5.5 percent of the patients received ventilatory support and only 2.4 percent received cardiopulmonary resuscitation. The findings of these studies suggest that variations exist in different health care settings regarding degree of technologic intervention at the end of life.

The challenges are to determine the extent to which the primary care physician helped improve end-of-life care and whether the longitudinal relationship with a family physician could improve patients' understanding of and access to palliative care, regardless of the type of facility.

From the inception of their specialty, family physicians have appreciated the role of the health care team. Nowhere is this more important than in the provision of hospice care. The multidisciplinary team that is the core of hospice end-of-life care integrates the disciplines of nursing, social work, counseling, ministry and medicine. Many family physicians have demonstrated their commitment to this concept of care by serving as hospice medical directors.

Several areas of end-of-life care remain of concern in the United States. The most significant of these is the generally accepted definition of terminal illness as one in which a patient has six months or less to live. In particular, this is the definition accepted by Medicare. However, predicting the end of life is extremely difficult, and several studies have demonstrated little predictive value for existing systems of evaluating the possible duration of life.^5,6

The six-month criterion also eliminates from hospice care many patients who have terminal diseases but a life expectancy of greater than six months, such as patients with chronic obstructive pulmonary disease or Alzheimer's disease. Uncertainty surrounding the six-month criterion is also a significant barrier to physician referrals of terminally ill patients to hospice care.⁷ Because life expectancy for many terminal diseases may be longer than six months and determining exactly when life will end is so difficult, many hospice programs are working to develop palliative care services or units to serve patients with these particular needs.

Untreated pain is another significant end-of-life issue that was addressed in the SUPPORT survey.³ In this survey, 50 percent of families reported that their dying family members were in moderate to severe pain at least one half of the time. At the end of life, pain can exact a terrible toll through its direct effect on the patient and the fear it instills in both the patient and the family members. To address the problem of untreated pain, many states have developed cancer pain initiatives to improve the awareness and science of pain management. The prototype program has been the Wisconsin Cancer Pain Initiative, which has an excellent Web site⁸ that outlines provided services and gives links to programs in other states.

Nonetheless, there is still a crucial need for family physicians to be aware of untreated pain and to be proactive in alleviating it. In "Managing Pain in the Dying Patient,"⁹ the first article in American Family Physician's "End-of-Life Care" series, Whitecar and colleagues indicate that "following recommended guidelines on the use of analgesics (including narcotics), family physicians can achieve successful pain relief in nearly 90 percent of dying patients."⁹

Providing a patient with quality care to the end of life requires a knowledge of new information and techniques for symptom control. Virtually all end-of-life interventions use agents and techniques with which family physicians are familiar, although the dosages and delivery systems are sometimes different. That these skills are obtainable by primary care physicians is shown by a recent study in community-dwelling hospice patients.¹⁰ In this study, their levels of pain control were not significantly different from those obtained by oncologists. More importantly, family physicians must be willing to use the new technology.

The articles in the AFP series illustrate much of the science and art of end-of-life care. In addition, the American Academy of Palliative and Hospice Medicine has published an excellent self-study primer on palliative care.¹¹ Several textbooks on the subject are also available. Other powerful and willing resources are the nurses and physicians of local hospice programs.

Good end-of-life care is not assisted suicide. Rather, it is the relentless pursuit of symptoms that compromise quality of life to the moment of death. Hence, end-of-life care is actually a life-giving form of care. I urge all family physicians to become familiar with the science and art of end-of-life care and to use this care aggressively to help patients and their families have the best life possible to the very end.

REFERENCES

Cancer pain relief and palliative care. Report of a WHO Expert Committee. World Health Organ Tech Rep Ser 1990;804:1-75.

Hospice fact sheet. Retrieved November 10, 1999, from the World Wide Web: http://www.nhpco.org/.

A controlled trial to improve care for seriously ill hospitalized patients. The Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatment (SUPPORT). The SUPPORT Principal Investigators. JAMA 1995;274:1591-8.

Hanson LC, Earp JA, Garrett J, Menon M, Danis M. Community physicians who provide terminal care. Arch Intern Med 1999;159:1133-8.

Fox E, Landrum-McNiff K, Zhong Z, Dawson NV, Wu AW, Lynn J. Evaluation of prognostic criteria for determining hospice eligibility in patients with advanced lung, heart, or liver disease. Support Investigators. Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatments. JAMA 1999;282:1638-45.

Vigano V, Dorgan M, Bruera E, Suarez-Almazor ME. The relative accuracy of the clinical estimation of the duration of life for patients with end of life cancer. Cancer 1999;86:170-6.

Weggel JM. Barriers to the physician decision to offer hospice as an option for terminal care. WMJ 1999;98:49-53.

Wisconsin Cancer Pain Initiative Web site. Retrieved December 7, 1999, from the World Wide Web: http://www.wisc.edu/wcpi/.

Whitecar PS, Jonas AP, Clasesn ME. Managing pain in the dying patient. Am Fam Physician 2000; 61:755-64.

Nowels D, Lee J. Cancer pain management in home hospice settings: a comparison of primary care and oncologic physicians. J Palliative Care 1999;15:3,5-9.

Story P, Knight CF. Hospice/palliative care training for physicians. Gainesville, Fla.: American Academy of Hospice and Palliative Medicine, 1997.

Dr. Gwinn is in private practice in Coshocton, Ohio. He is also the medical director of Hospice of Coshocton County.

Address correspondence to Robert B. Gwinn, D.O., Hospice of Coshocton County, 440 Browns Lane, Coshocton, OH 43812.

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