Impact of lactation on women with cystic fibrosis and their infants: A review of five cases

doi:10.1016/0002-8223(94)90240-2

Journal of the American Dietetic Association

Volume 94, Issue 2, February 1994, Pages 159-165

https://doi.org/10.1016/0002-8223(94)90240-2 Get rights and content

Abstract

Objective To evaluate the impact of lactation on the health status of women with cystic fibrosis and their infants.

Design Data were acquired through a retrospective review of pulmonary, obstetric, and pediatric medical records.

Setting A medical school-based Pediatric Cystic Fibrosis Center in Philadelphia, Pa.

Subjects Five women with cystic fibrosis and their respective infants.

Main outcome measures For the women, measures of pregravid, gravid, and postpartum height, weight, pulmonary function test results, and energy intake were determined. For the infants, birth Apgar scores were obtained, as were measures of growth and development.

Statistical analyses performed Means and standard deviations were calculated. Data were compared with published standardized references.

Results Mean maternal age at conception was 25.4±4.1 years. Mean pregravid weight was 60.0±10.7 kg of standard body weight for height (range = 50.8 to 76.7 kg; median = 58 kg). Two women consumed up to two enteric-coated supplemental enzyme capsules per meal, three women consumed none. Pulmonary disease was mild in four of the five women. Mean weight gain during pregnancy was 10.2±7.4 kg. Mean gestation was 37.4±1.5 weeks; mean birth weight was 3.0±0.5 kg. Sweat tests performed on all infants were negative. Duration of breast-feeding was 3 to 30 weeks. Four of the five infants maintained appropriate growth velocity during breast-feeding. Mean maternal energy intake during lactation was above the Recommended Dietary Allowance for lactating women. Four of the five women were at or above their standard body weight during lactation.

Conclusions/applications We conclude that women with the clinical diagnosis of mild cystic fibrosis disease can maintain a normal pregnancy with appropriate weight gain and can deliver infants of normal weight for gestational age. During lactation, women with mild cystic fibrosis disease can maintain their own weight and support growth in healthy infants. The dietitian needs to be an integral member of the health care team in assessing, monitoring and managing, women with cystic fibrosis during their child-bearing years.

Section snippets

METHODS

Our subjects were five women with cystic fibrosis, as confirmed by the sweat pilocarpine iontophoresis test, and their five unaffected infants. Data were acquired through a retrospective review of pulmonary, obstetric, and pediatric medical records. The women gave written permission for researchers to review their medical records and their infants' pediatric records. The women's records from the Cystic Fibrosis Center were examined for weight, pulmonary function values, and medical status

RESULTS

Descriptive data pertaining to the women are provided in Table 1. Subjects' mean (± standard deviation) age at diagnosis of cystic fibrosis was 9.0±6.8 years. Subjects' mean age when their child was conceived was 25.4±4.1 years. Subjects 1, 3, and 5 required no supplemental pancreatic enzyme capsules; subjects 2 and 4 required a maximum of two supplemental enzyme capsules (Pancrease, 8,000 units of lipase, McNeil Pharmaceutical, Springhouse, Pa) per meal to achieve normal bowel movements.

DISCUSSION

Our evaluation of these five adult women with cystic fibrosis indicates that pregnancy and breast-feeding had no long-term adverse effect on their medical and nutritional status. Corresponding to well-accepted principles of obstetric care for women without cystic fibrosis (1), adequate weight before and during pregnancy is associated with positive pregnancy outcomes both for women with cystic fibrosis and for their children. Gravid and postweaning evaluation of the SK score, PFTs, and weight

APPLICATION

Women with cystic fibrosis often ask dietitians to make recommendations regarding breast-feeding. It is important that the recommendations be based on scientific fact. This is difficult because very little information is available describing the nutrient content of breast milk from women with CF. To our knowledge, no data are available describing the impact of breast-feeding on women with cystic fibrosis and their infants. The intent of our report is to help dietitians counsel patients with

THE JOURNAL TALKS WITH…

Patients with cystis fibrosis (CF) are living longer and dietitians are encountering new issues in working with adults with this disease. The idea for this study began back in 1988 when Suzanne H. Michel, MPH, RD, and Donna H. Mueller, PhD, RD, were commiserating about the lack of information they could provide their adult, female patients with CF who wanted to become pregnant and breast-feed.

Journal: What particular nutrition problems do women with CF encounter in pregnancy and lactation?

Acknowledgements

The authors gratefully acknowledge the participation of the women of the Hahnemann University Cystic Fibrosis Center and Douglas Holscalow, MD, Center Director. They also thank Bonita Falkner, MD, for her expert editorial advice and Ms Gloria Rosado for manuscript preparation.

References (22)

BW Ramsey et al.
Nutritional assessment and management in cystic fibrosis: a consensus report
Am J Clin Nutr
(1992)
RM Kotloff et al.
Fertility and pregnancy in patients with cystic fibrosis
Clin Chest Med
(1992)
A Whitelaw et al.
High breast milk sodium in cystic fibrosis
Lancet
(1977)
SE Alpert et al.
Normal electrolyte and protein content in milk from mothers with cystic fibrosis: an explanation for the initial report of elevated milk sodium
J Pediatr
(1983)
ML Shiffman et al.
Breast-milk composition in women with cystic fibrosis: report of two cases and a review of the literature
Am J Clin Nutr
(1989)
SG Babson et al.
Growth graphs for the clinical assessment of infants of varying gestational age
J Pediatr
(1976)
L Cohen et al.
Cystic fibrosis and pregnancy: a national survey
Lancet
(1980)
CWB Corkey et al.
Pregnancy in cystic fibrosis: a better prognosis in patients with pancreatic function?
Am J Obstet Gynecol
(1981)
M Wilnick
Nutrition, Pregnancy, and Early Infancy
National Patient Registry
(1990)

E Luder et al.

Current recommendations for breast-feeding in cystic fibrosis clinics

Am J Dis Child

(1990)

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More aggressive nutritional intervention ranging from oral calorie supplementation to enteral or parenteral nutrition support should be considered in the absence of adequate weight gain [23,37]. Vitamin supplementation is often required and should be adjusted based on serum and plasma micronutrient levels with the goal of maintaining levels in the normal range [38]. Iron and folate supplementation is similar to that of women without CF though some literature supports high dose folate supplementation in CF particularly in those at high risk for deficiency [23].
Cystic fibrosis (CF) was historically a disease largely afflicting children. Due to therapeutic advancements, there are now more adults with CF than children. In the past decade, medications including Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulators became available that treat the underlying cause of CF and are dramatically improving lung function as well as quality and quantity of life for people with CF. As a result, more women with CF are becoming pregnant. We gathered a panel of experts in CF care, family planning, high risk obstetrics, nutrition, genetics and women with CF to review current literature on pregnancies and to provide care recommendations for this unique population.
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Women with cystic fibrosis (CF) now regularly survive into their reproductive years in good health and wish to have a baby. Many pregnancies have been reported in the literature and it is clear that whilst the outcome for the baby is generally good and some mothers do very well, others find either their CF complicates the pregnancy or is adversely affected by the pregnancy. For some, pregnancy may only become possible after transplantation. Optimal treatment of all aspects of CF needs to be maintained from the preconceptual period until after the baby is born. Clinicians must be prepared to modify their treatment to accommodate the changing physiology during pregnancy and to be aware of changing prescribing before conception, during pregnancy, after birth and during breast feeding. This supplement offers consensus guidelines based on review of the literature and experience of paediatricians, adult and transplant physicians, and nurses, physiotherapists, dietitians, pharmacists and psychologists experienced in CF and anaesthetist and obstetricians with experience of CF pregnancy. It is hoped they will provide practical guidelines helpful to the multidisciplinary CF teams caring for pregnant women with CF.
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Severity of lung disease predicted preterm delivery, and no congenital malformations were noted.73 A case series reported two women on pancreatic supplementation who had successful pregnancies and breast-fed their infants with appropriate growth.74 Practice points: Pancreatitis
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^b: Currently, S. H. Michel is an instructor in the Department of Pediatrics and a nutritionist with the Adult Cystic Fibrosis Center at the Medical College of Pennsylvania, Philadelphia, PA 19129

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ResearchImpact of lactation on women with cystic fibrosis and their infants: A review of five cases

Abstract

Section snippets

METHODS

RESULTS

DISCUSSION

APPLICATION

THE JOURNAL TALKS WITH…

Acknowledgements

Am J Clin Nutr

Clin Chest Med

Lancet

J Pediatr

Am J Clin Nutr

J Pediatr

Lancet

Am J Obstet Gynecol

Nutrition, Pregnancy, and Early Infancy

National Patient Registry

Current recommendations for breast-feeding in cystic fibrosis clinics

Am J Dis Child

Research
Impact of lactation on women with cystic fibrosis and their infants: A review of five cases