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Long-term nutritional rehabilitation in cystic fibrosis
Volume 108 Number 3
Editorial correspondence
increments that the tempo conditional charts make the difference. With them~ the clinician begins to understand how the child is growing and how to interpret th~it growth in relation to the possibility of his or her being an early, mid, or late maturer. If the clinician uses tempo unconditional charts (for those over nine years) he simply gets it wrong. However, the proof of our charts' usefulness must lie in t h e Using. Pediatricians a n d endocrinologists have been using the British charts for many years, not only in Europe and Israel but in several academic departments in the United states, as the only tempo conditional charts available. It seemed to Us that American pediatricians had waited long enough for tempo conditional standards of their own.
J. M. Tanner P. S. IV. Davies Department of Growih and Development Institute of Child Health 30 Guilford St. LondOn WCIN I EH, England
A n o t h e r cause of neonatal Cholestasis To the Editor." In his excellent review article on neonatal cholestasis, Balistrcri 1 neglected to mention neonatal cholestasis caused by the marked hepatic h~fpertrophy with islets of extramedullary i:rythropoiesis seen in severe erythroblastosis fetalis, as so eloquently described by Dunn. 2,3 As a referral center for severely atioimmunized women (e.g., anti-Rh, anti-c), We find intrahepatic biliary duct obstruction from the above cause to be one of the commonest reasons for neonatal cholestasis? This syndrome may last for 2 to 4 months, but is invariably transient and benign, leaving no sequelae of hepatic damage. Altlaough total bilirubin levels may exceed 680 ~mol/L (40 mg/dl), with conjugated biiirubin levels as high as 510 ~tmol/L (30 mg/dl), extensive investigative procedures are not warranted.
John M. Bowman, M.D. Professor Department of Pediatrics and Child Health Department of Obstetrics, Gyneeology. and Reproductive Sciences Faculty of Medt'cine University. of Manitoba Medical Director Rh Laboratory 735 Notre Dame Ave. Winnipeg, Manitoba, Canada R3E OL8 REFERENCES
1. 2.
Balistreri WF. Neonatal cholestasis. J PEDIATR 1985; 106:171-184. Dunn PM. Obstructive jaundice and haemolytic disease of the newborn. Arch Dis Child 1963;38:54.
3.
4.
489
Dunn PM. Obstructive jaundice, liver damage and Rh hemolytic disease of the newborn. Jewish Mere Hosp Bull 1965;10:94. Bowman JM. Neonatal management. In: Queenan JT, ed. Modern management of the Rh problem. Hagerstown, Md.: Harper & Row, 1977:200-239.
Long-term nutritional rehabilitation in cystic fibrosis To the Editor: We would like to comment on the article by Levy et al. t The optimum method to evaluate a new therapy is a randomized control trial. Inasmuch as Levy et al. state they believe there m~iy be serious practical and ethical problems in conducting a randomized trial, we assume that they made no attempt at randomization. Their matched comparison subjects were in a similar nutritional state as the patients, but were noi. selected for the study. We are interested in how they selected their patients from among those who satisfied the entrance criteria. They note changes in percent weight~height (% wt/ht) oI: 5.0 _+ 5.0 (mean _+ SD) over a mean of 1.2 years. These changes are statistically significant, but are they biologically significant? Six of the 14 treated patients are now dead. What was the % Wt/ht at death'? Did the patients sustain their weight gain, or was there weight loss prior to death? For 3 years we have been studying the problem of undernutrition in cystic fibrosis in a similar fashion to that of our colleagues in Toronto. We have been using supplemental jejunostomy feeding of a noneiemental formMa (cost of which is 25% that of an elemental formula). This method also leads t o weight gain, but there are major problems with evaluation of such a feeding program. We agree that there are ethical problems with randomization. In our clinic of approximately 65 patients, the families form a close community and follow the decline of their sickest members With concern. After the first patient given jejunostomy feeding achieved a normal weight for height and a visible improvement in activity, any attempt at randomization of our subjects would have failed. Activist parents wanted the treatment, and others who were more fatalistic refused this intervention; often denying the existence of the problem~ Some families also have difficulties wi}.h managing routine therapy and could not cope with this extra burden. To use this latter group for comparison is to invite unjustified conclusions. Recognizing the problems of comparison, we examined the changes in % w t / h t in our jejunostomy treatment group and in all other patients in our clinic whose % wt/ht since 1982 has fallen below 90% for >--6 months. Our criterion for admission to the study is a sustained % w t / h t <86%, so some of our comparison group have not yet been offered this intervention. The stai'ting % Wt/ht values were higher for the comparison group (86.9 • 3.1) than for the treatment group (80.4 • 8.4), but the oscillations were greater in the treatment group. Precipitate falls in weight for height (>5% over 3 months) signaled death in the comparison
490
Editorial correspondence
group unless some major improvement in therapy was made. The jejunostomy fed patients, despite initially being less well nourished, were better able to recover from such losses (10 of 11), subsequently regaining or even surpassing the former % wt/ht. The rate of weight gain was also different in the treatment group. They could increase % w t / h t at >_5% over 3 months; such a change was not seen in the other patients. Four deaths have occurred in the 15 comparison patients, and one in the treated group; this last patient did not achieve the weight gain expected because of intolerance of more than minimal jejunal feeding. The conclusion we draw is that enteral feeding appears to alter the prognostic significance of precipitate weight loss in undernoui'ished patents with cystic fibrosis and is capable of supporting faster rates of recovery. Enterostorny feeding in most cases gives a degree of control over body weight that was previously impossible. The pattern of weight change, particularly the capacity to recover from precipitate losses, is different between the two groups. As lung function deteriorates, the capacity to excrete the CO2 load associated with a normal meal is lost. With an enterostomy tube in place, feeding time can be progressively extended so that weight can be maintained for some time without the COz load increasing the degree of hypercapnia. This treatment seems to improve patient well-being. What we lack are the tools to measure quality of life. It is in this area that any advantage or disadvantage of enteral feeding must be evaluated, and the answer will probably be obtained using techniques other than a randomized control trial. Margaret P. Boland, M.D. Research Fellow Deanna S. S(oski, R.N. Research Assistant John Patrick, M.D. Associate Professor of Biochemistry and Pediatrics University of Ottawa Children's Hospital of Eastern Ontario 401 Smyth Road Ottawa, Ontario, Canada KIH 8Ll REFERENCES
1.
Levy LD, Durie PR, Pencharz PB, Corey ML. Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis. J PEDIATR 1985;107:225.
The Journal of Pediatrics March 1986
Reply To the Editor." We stated in our article that patients whose weight as a percentage of ideal (% wt/ht) fell below 90%, or who had growth retardation, or who failed to regain weight lost during chest infections were referred to the Division of Clinical Nutrition for a formal nutritional assessment. The final decision to refer a patient was up to the responsible chest physician. Of the total number of patients in our CF Clinic (n = 550), approximately 150 were identified as having % w t / h t <90%. Most of these patients are referred to the dietitian; only a small proportion are later referred on to our service. Thus our matched comparison subjects were, t'or the most part, not referred to us for assessmerit and management. Three of the 10 comparison subjects were not referred to our service, because they were judged to be psychologically unsuitable to receive gastrostomy feeding. One was referred and was offered a gastrostomy, but refused. In those patients who died, nutritional status had worsened, as might be expected. With regard to the feasibility of a randomized study, we are pleased that Dr. Patrick's group also recognizes the inherent difficulties. Thus, at present, it is not possible for us to determine the effects of any enteral tube feeding program on the duration of survival of patients with cystic fibrosis. On the other hand, we are attempting, within subjects, to determine whether we can detect improvements in the quality of life. Lance D. Levy, M.B.; Ch.B. Peter R. Durie, M.D. Paul B. Pencharz, M.D., Ph.D. Mary L. Corey, M.Sc. The Research Institute The Hospital for Sick Children 555 University Ave. Toronto, Ontario, Canada M5G IX8 Department of Paediatrics and Nutritional Sciences The University of Toronto
Editorial correspondence
increments that the tempo conditional charts make the difference. With them~ the clinician begins to understand how the child is growing and how to interpret th~it growth in relation to the possibility of his or her being an early, mid, or late maturer. If the clinician uses tempo unconditional charts (for those over nine years) he simply gets it wrong. However, the proof of our charts' usefulness must lie in t h e Using. Pediatricians a n d endocrinologists have been using the British charts for many years, not only in Europe and Israel but in several academic departments in the United states, as the only tempo conditional charts available. It seemed to Us that American pediatricians had waited long enough for tempo conditional standards of their own.
J. M. Tanner P. S. IV. Davies Department of Growih and Development Institute of Child Health 30 Guilford St. LondOn WCIN I EH, England
A n o t h e r cause of neonatal Cholestasis To the Editor." In his excellent review article on neonatal cholestasis, Balistrcri 1 neglected to mention neonatal cholestasis caused by the marked hepatic h~fpertrophy with islets of extramedullary i:rythropoiesis seen in severe erythroblastosis fetalis, as so eloquently described by Dunn. 2,3 As a referral center for severely atioimmunized women (e.g., anti-Rh, anti-c), We find intrahepatic biliary duct obstruction from the above cause to be one of the commonest reasons for neonatal cholestasis? This syndrome may last for 2 to 4 months, but is invariably transient and benign, leaving no sequelae of hepatic damage. Altlaough total bilirubin levels may exceed 680 ~mol/L (40 mg/dl), with conjugated biiirubin levels as high as 510 ~tmol/L (30 mg/dl), extensive investigative procedures are not warranted.
John M. Bowman, M.D. Professor Department of Pediatrics and Child Health Department of Obstetrics, Gyneeology. and Reproductive Sciences Faculty of Medt'cine University. of Manitoba Medical Director Rh Laboratory 735 Notre Dame Ave. Winnipeg, Manitoba, Canada R3E OL8 REFERENCES
1. 2.
Balistreri WF. Neonatal cholestasis. J PEDIATR 1985; 106:171-184. Dunn PM. Obstructive jaundice and haemolytic disease of the newborn. Arch Dis Child 1963;38:54.
3.
4.
489
Dunn PM. Obstructive jaundice, liver damage and Rh hemolytic disease of the newborn. Jewish Mere Hosp Bull 1965;10:94. Bowman JM. Neonatal management. In: Queenan JT, ed. Modern management of the Rh problem. Hagerstown, Md.: Harper & Row, 1977:200-239.
Long-term nutritional rehabilitation in cystic fibrosis To the Editor: We would like to comment on the article by Levy et al. t The optimum method to evaluate a new therapy is a randomized control trial. Inasmuch as Levy et al. state they believe there m~iy be serious practical and ethical problems in conducting a randomized trial, we assume that they made no attempt at randomization. Their matched comparison subjects were in a similar nutritional state as the patients, but were noi. selected for the study. We are interested in how they selected their patients from among those who satisfied the entrance criteria. They note changes in percent weight~height (% wt/ht) oI: 5.0 _+ 5.0 (mean _+ SD) over a mean of 1.2 years. These changes are statistically significant, but are they biologically significant? Six of the 14 treated patients are now dead. What was the % Wt/ht at death'? Did the patients sustain their weight gain, or was there weight loss prior to death? For 3 years we have been studying the problem of undernutrition in cystic fibrosis in a similar fashion to that of our colleagues in Toronto. We have been using supplemental jejunostomy feeding of a noneiemental formMa (cost of which is 25% that of an elemental formula). This method also leads t o weight gain, but there are major problems with evaluation of such a feeding program. We agree that there are ethical problems with randomization. In our clinic of approximately 65 patients, the families form a close community and follow the decline of their sickest members With concern. After the first patient given jejunostomy feeding achieved a normal weight for height and a visible improvement in activity, any attempt at randomization of our subjects would have failed. Activist parents wanted the treatment, and others who were more fatalistic refused this intervention; often denying the existence of the problem~ Some families also have difficulties wi}.h managing routine therapy and could not cope with this extra burden. To use this latter group for comparison is to invite unjustified conclusions. Recognizing the problems of comparison, we examined the changes in % w t / h t in our jejunostomy treatment group and in all other patients in our clinic whose % wt/ht since 1982 has fallen below 90% for >--6 months. Our criterion for admission to the study is a sustained % w t / h t <86%, so some of our comparison group have not yet been offered this intervention. The stai'ting % Wt/ht values were higher for the comparison group (86.9 • 3.1) than for the treatment group (80.4 • 8.4), but the oscillations were greater in the treatment group. Precipitate falls in weight for height (>5% over 3 months) signaled death in the comparison
490
Editorial correspondence
group unless some major improvement in therapy was made. The jejunostomy fed patients, despite initially being less well nourished, were better able to recover from such losses (10 of 11), subsequently regaining or even surpassing the former % wt/ht. The rate of weight gain was also different in the treatment group. They could increase % w t / h t at >_5% over 3 months; such a change was not seen in the other patients. Four deaths have occurred in the 15 comparison patients, and one in the treated group; this last patient did not achieve the weight gain expected because of intolerance of more than minimal jejunal feeding. The conclusion we draw is that enteral feeding appears to alter the prognostic significance of precipitate weight loss in undernoui'ished patents with cystic fibrosis and is capable of supporting faster rates of recovery. Enterostorny feeding in most cases gives a degree of control over body weight that was previously impossible. The pattern of weight change, particularly the capacity to recover from precipitate losses, is different between the two groups. As lung function deteriorates, the capacity to excrete the CO2 load associated with a normal meal is lost. With an enterostomy tube in place, feeding time can be progressively extended so that weight can be maintained for some time without the COz load increasing the degree of hypercapnia. This treatment seems to improve patient well-being. What we lack are the tools to measure quality of life. It is in this area that any advantage or disadvantage of enteral feeding must be evaluated, and the answer will probably be obtained using techniques other than a randomized control trial. Margaret P. Boland, M.D. Research Fellow Deanna S. S(oski, R.N. Research Assistant John Patrick, M.D. Associate Professor of Biochemistry and Pediatrics University of Ottawa Children's Hospital of Eastern Ontario 401 Smyth Road Ottawa, Ontario, Canada KIH 8Ll REFERENCES
1.
Levy LD, Durie PR, Pencharz PB, Corey ML. Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis. J PEDIATR 1985;107:225.
The Journal of Pediatrics March 1986
Reply To the Editor." We stated in our article that patients whose weight as a percentage of ideal (% wt/ht) fell below 90%, or who had growth retardation, or who failed to regain weight lost during chest infections were referred to the Division of Clinical Nutrition for a formal nutritional assessment. The final decision to refer a patient was up to the responsible chest physician. Of the total number of patients in our CF Clinic (n = 550), approximately 150 were identified as having % w t / h t <90%. Most of these patients are referred to the dietitian; only a small proportion are later referred on to our service. Thus our matched comparison subjects were, t'or the most part, not referred to us for assessmerit and management. Three of the 10 comparison subjects were not referred to our service, because they were judged to be psychologically unsuitable to receive gastrostomy feeding. One was referred and was offered a gastrostomy, but refused. In those patients who died, nutritional status had worsened, as might be expected. With regard to the feasibility of a randomized study, we are pleased that Dr. Patrick's group also recognizes the inherent difficulties. Thus, at present, it is not possible for us to determine the effects of any enteral tube feeding program on the duration of survival of patients with cystic fibrosis. On the other hand, we are attempting, within subjects, to determine whether we can detect improvements in the quality of life. Lance D. Levy, M.B.; Ch.B. Peter R. Durie, M.D. Paul B. Pencharz, M.D., Ph.D. Mary L. Corey, M.Sc. The Research Institute The Hospital for Sick Children 555 University Ave. Toronto, Ontario, Canada M5G IX8 Department of Paediatrics and Nutritional Sciences The University of Toronto