Nutritional Well-Being After Transplant Measure of Likely Lung Health

By Joana Carvalho

The study, “Impact of nutritional status on pulmonary function after lung transplantation for cystic fibrosis,” was published in the United European Gastroenterology Journal.

CF is the third most common cause for lung transplants worldwide (16.8 percent of all cases). Although the disease is mostly associated with respiratory symptoms, gastrointestinal complications are also known to afflict patients, such as diarrhea, constipation, malnutrition, and inflammation in the pancreas, liver and intestines.

Previous studies suggest that malnutrition is linked to a poor prognosis in those needing a lung transplant. However, data on the impact of nutritional status on pulmonary function in those who have received a transplant is still quite limited.

In a retrospective study, a team of researchers at the Medical University of Vienna set out to evaluate the impact of nutritional status on pulmonary function of CF patients who underwent a double lung transplant within a median of 2.3 years.

Patients’ nutritional status was assessed using two different criteria: body mass index (BMI; kg/m2), and body composition measured by bioelectrical impedance analysis (BIA) — a technique that allows researchers to estimate body composition, especially fat content, by calculating the resistance posed by body tissues to the passage of an electrical current.

Lung health was analyzed by spirometry, a common test based on the amount of air a person can inhale and quickly exhale.

Investigators analyzed a total of 147 spirometries and BIAs performed on 58 CF patients (median age, 30.1), who were divided into four groups depending on their BMI scores. These groups were set according to BMI the guidelines defined by the World Health Organization (WHO), were: malnutrition (less than 18.5 kg/m2), normal weight (18.5–24.9 kg/m2), overweight (25.0–29.9 kg/m2), or obese (more than 30 kg/m2).

Data showed that malnourished patients (27.6%) had a significantly poorer in lung function than those of normal weight (63.8%) or overweight (8.6%), as measured by the percentage of forced expiratory volume in one second (FEV1% predicted, 57% vs 77%), and the percentage of maximum vital capacity (percent predicted, 62% vs 75%).

Investigators also found that lung function measured by FEV1% worsened over time in malnourished patients (decreasing by up to 15%), unlike normal weight and overweight individuals. In these patients, FEV1% remained stable throughout the observation period (median of 10.3 months).

Further analysis also showed that the ratio of extracellular mass (ECM) over body cell mass (BCM), as measured by BIA, accurately predicted lung function over time in CF transplant recipients, suggesting that BIA is superior to BMI in predicting patients’ pulmonary function.

The team concluded “nutritional status assessed by BIA predicted lung function in CF transplant recipients,” and suggested that “BIA represents a non-invasive, safe, fast, mobile, and easy-to-use procedure to evaluate body composition. Thus, it may be used in everyday clinical practice and bears the advantage of repeatability at every patient follow-up.”

The researchers also emphasized the importance of multidisciplinary patient care provided by dietitians and gastroenterologists to try and prevent or diminish malnourishment in CF patients, and so help preserve lung function after a transplant.

Original article here. 

Mutations in Genes Regulating Digestion Prevalent in CF Patients with Pancreatitis

By Vijaya Iyer

In addition to mutations in the CFTR gene, cystic fibrosis (CF) patients with pancreatitis also have a high prevalence of mutations in genes regulating pancreatic function, according to researchers.

Conducted by a research team in Italy, the study, “Trans-heterozygosity for mutations enhances the risk of recurrent/chronic pancreatitis in patients with Cystic Fibrosis,” was published in the journal Molecular Medicine Continue reading Mutations in Genes Regulating Digestion Prevalent in CF Patients with Pancreatitis

Advancing the GI frontier for patients with CF

The care of patients with Cystic Fibrosis (CF) has seen amazing advances in the past few years, made in part through the development of CFTR modulators. However, the recognition of the frequency of gastrointestinal (GI) symptoms in our patients is just beginning to emerge. Only recently have publications noted the excessively high frequency of GI issues. Continue reading Advancing the GI frontier for patients with CF

You are invited to attend a free web-cast event SATURDAY, APRIL 22, 2017

RARE LUNG DISEASES PATIENT EDUCATION DAY ON CYSTIC FIBROSIS, CHILDREN’S INTERSTITIAL LUNG DISEASE AND PRIMARY CILIARY DYSKINESIA

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This event is co-sponsored by the American Thoracic Society, the Cystic Fibrosis Foundation, the Children’s Interstitial Lung Disease Foundation and the Primary Continue reading You are invited to attend a free web-cast event SATURDAY, APRIL 22, 2017

Gastroparesis and Cystic Fibrosis – By Meranda Sue Honaker

By Meranda Sue Honaker

Gastroparesis (delayed gastric emptying) is a common complication for many with CF; however, the condition is exceptionally hard to treat due to lack of promotility agents in the United States. In my early 20’s I began to experience Continue reading Gastroparesis and Cystic Fibrosis – By Meranda Sue Honaker

OWN IT: Five Years with the G-Tube

This May marks five years since I had my feeding tube placed, or to put it another way, my G-tube has been with my for 1/5 of my life.

It’s a strange thought. For years I rejected the idea of having a feeding tube – Continue reading OWN IT: Five Years with the G-Tube

UDCA Supplementation Enhances Lipid Digestion and Absorption in Pancreatic Insufficient Patients with Cystic Fibrosis

http://www.hcplive.com/

Results from a trial involving 23 cystic fibrosis patients with pancreatic insufficiency and mild liver involvement indicate that supplementation with ursodeoxycholic acid can increase the digestion and absorption of fat. Continue reading UDCA Supplementation Enhances Lipid Digestion and Absorption in Pancreatic Insufficient Patients with Cystic Fibrosis

Anthera, Patheon to Co-Manufacture Sollpura for CF Phase 3 Trial

Anthera Pharmaceuticals, Inc., a biopharmaceutical company that specializes in research and development of novel treatments for serious and fatal diseases such as lupus, lupus with nephrotic affectation, and exocrine pancreatic insufficiency (EPI) secondary to cystic fibrosis Continue reading Anthera, Patheon to Co-Manufacture Sollpura for CF Phase 3 Trial

Pitt-led study suggests cystic fibrosis is 2 diseases, 1 doesn’t affect lungs

Cystic fibrosis (CF) could be considered two diseases, one that affects multiple organs including the lungs, and one that doesn’t affect the lungs at all, according to a multicenter team led by researchers at the University of Pittsburgh School of Medicine. The research, published online today in PLOS Continue reading Pitt-led study suggests cystic fibrosis is 2 diseases, 1 doesn’t affect lungs

Pancreatic Enzyme Products May Benefit Cystic Fibrosis Patients with Malabsorption

After the Food and Drug Administration (FDA) issued rules requiring approval of pancreatic enzyme products (PEPs) in 2004, the cost and availability of the products added another hardship to the lives of cystic fibrosis patients, some of whom use PEPs to prevent uncomfortable and Continue reading Pancreatic Enzyme Products May Benefit Cystic Fibrosis Patients with Malabsorption