Clinician-to-Clinician Update Clinician-to-Clinician Update

Specialized Team Care, Nutritional Support Aid in Management of Cystic Fibrosis

August 2016

Contributed by Dr. Terri Laguna

In 2014, 14% of infants with cystic fibrosis (CF) under age 1 were diagnosed with meconium ileus, a complication of CF, or other intestinal obstruction, and 21.1% of those infants had bowel perforation.1 In this case study, the combination of specialized pediatric gastroenterological care tailored to address the complications of CF and a dedicated and knowledgeable pediatric CF team dramatically improved the outcome for a young patient with CF and meconium ileus.

Patient

An infant girl born at 32 weeks and weighing 4.34 pounds was diagnosed with meconium ileus. She required an emergency operation to address the resulting bowel obstruction and was transferred to the University of Minnesota Masonic Children’s Hospital’s neonatal intensive care unit (NICU) where she could receive specialized care. The next day, pediatric surgeons removed a large section of her intestine to relieve the obstruction. A genetic counselor within the Minnesota Cystic Fibrosis Center was notified that this infant had a new diagnosis of CF, and the genetic counselor mobilized the CF team to work with surgeons and NICU staff to provide guidance on CF issues. Four days after the patient’s birth, the patient’s CF diagnosis was confirmed. When the patient left the NICU, she was fully transitioned to the care of the University of Minnesota Masonic Children’s Hospital’s multidisciplinary CF team.

MH Loc UMMCH 0001-v.2
— University of Minnesota Masonic Children’s Hospital provides interdisciplinary care for patients with cystic fibrosis, including nationally ranked pulmonology, gastroenterology, and GI surgery specialty services.

Management

Dysfunction of the gastrointestinal tract and pancreas are known complications of CF, as the majority of CF patients require supplemental pancreatic enzymes in order to absorb the fat and protein they eat. Following the procedure to address the bowel obstruction, this patient acquired “short-gut syndrome,” which added further challenges in absorbing the adequate nutrition needed to gain weight and grow. Optimizing the patient’s nutritional intake in order for her to achieve normal height and weight, became the multidisciplinary care team’s main objective.

The CF pediatric team, including its dietician, worked closely with a University of Minnesota Health physician specializing in the gastrointestinal complications of CF and the clinical methods to address them. Each of the patient’s conditions causes malabsorption of nutrients, and their management required specialist input on nutrition, CF, and short gut syndrome. Physicians placed a gastrostomy tube for delivery of supplemental formula, and the patient also received intravenous nutrition to further help her gain weight, given her issues with malabsorption. Total parenteral nutrition was used as needed to drive growth. The patient continued with the nutritional regime as she transitioned from the NICU and hospital care. The care plan included working closely with the family to monitor the patient’s daily growth and to adjust tube feedings for their fat and protein and overall calorie content.

At 8 months of age, her weight had risen to 12.19 pounds, a spectacular increase over a relatively short period of time. The patient’s weight for her age and height, however, has remained low, at the 8th percentile, and below the ultimate clinical care goal of a growth and development measurement at the 50th percentile. The team plan includes ongoing communication with the patient’s mother, typically several times a week by phone or email in addition to meeting with her at monthly visits to the clinic. The CF pediatric team continues consulting with the specialist in CF and gastrointestinal care to address the patient’s nutritional needs.

Discussion

An important goal in pediatric CF care is for each child to reach the optimal growth percentile. Optimal nutrition correlates directly with healthy lungs and longer life expectancy.1 A multidisciplinary team approach to cystic fibrosis care, including routine visits and follow-up, provides the best opportunity to improve the nutritional status and overall health of the CF patient, especially in infancy. A dietician and a gastrointestinal physician with a specialty in CF are crucial members of the multidisciplinary team in the care of a CF patient.

References

  1. Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013 Mar;162(3):530-535.e1. doi: 10.1016/j.jpeds.2012.08.040. Epub 2012 Oct 11.
Related Articles

August 2016

New Therapies and Early Care Benefit Patients with Cystic Fibrosis

Early aggressive treatment of cystic fibrosis, enabled by newborn screenings, has markedly improved health outcomes for patients. Now promising new therapies target the gene defects linked to the condition.

Continue reading

August 2016

Pediatric Specialty Updates

New studies aim to preserve the health and lung function of patients with CF, including research into markers of lung disease and efforts to better define the links between body composition and lung function.

Continue reading