ABSTRACT
BACKGROUND: Iron supplementation for hypoferremic anemia could potentiate bacterial growth in the cystic fibrosis (CF) lung, but clinical trials testing this hypothesis are lacking. METHODS: Twenty-two adults with CF and hypoferremic anemia participated in a randomized, double-blind, placebo-controlled, crossover trial of ferrous sulfate 325mg daily for 6weeks. Iron-related hematologic parameters, anthropometric data, sputum iron, Akron Pulmonary Exacerbation Score (PES), and the sputum microbiome were serially assessed. Fixed-effect models were used to describe how ferrous sulfate affected these variables. RESULTS: Ferrous sulfate increased serum iron by 22.3% and transferrin saturation (TSAT) by 26.8% from baseline (p<0.05) but did not affect hemoglobin, sputum iron, Akron PES, and the sputum microbiome. CONCLUSIONS: Low-dose ferrous sulfate improved hypoferremia without correcting anemia after 6weeks. We did not observe significant effects on sputum iron, Akron PES, and the sputum microbiome. Although we did not identify untoward health effects of iron supplementation, a larger blinded randomized controlled trial would be needed to fully demonstrate safety.
Subject(s)
Anemia, Iron-Deficiency/drug therapy , Cystic Fibrosis/complications , Ferrous Compounds/administration & dosage , Ferrous Compounds/adverse effects , Microbiota/drug effects , Adolescent , Adult , Anemia, Iron-Deficiency/etiology , Anemia, Iron-Deficiency/metabolism , Cross-Over Studies , Cystic Fibrosis/metabolism , Double-Blind Method , Female , Hepcidins/metabolism , Humans , Male , Middle Aged , Placebos , Sputum/drug effects , Sputum/microbiology , Treatment Outcome , Young AdultABSTRACT
BACKGROUND: Hypoferremia is a marker of disease severity in cystic fibrosis (CF). The effect of systemic antibiotics on iron homeostasis during CF pulmonary exacerbation (CFPE) is unknown. Our central hypotheses were that, by the completion of treatment, serum iron would increase, serum concentrations of interleukin-6 (IL-6) and hepcidin-25, two mediators of hypoferremia, would decrease, and sputum iron would decrease. METHODS: Blood and sputum samples were collected from 12 subjects with moderate-to-severe CF (median percentage-predicted forced expiratory volume in 1 second (FEV(1) %) = 29%; median weight = 56 kg) within 24 hours of starting and completing a course of systemic antibiotics. RESULTS: After treatment, subjects showed median FEV(1) % and body weight improvements of 4.5% and 2.0 kg, respectively (p < 0.05). Median serum iron rose by 2.4 µmol/L (p < 0.05), but 75% of patients remained hypoferremic. Median serum IL-6 and hepcidin-25 levels fell by 12.1 pg/mL and 37.5 ng/mL, respectively (p < 0.05). Median serum erythropoietin (EPO) and hemoglobin levels were unaffected by treatment. We observed a trend toward lower sputum iron content after treatment. CONCLUSIONS: Hypoferremia is a salient characteristic of CFPE that improves with waning inflammation. Despite antibiotic treatment, many patients remain hypoferremic and anemic because of ineffective erythropoiesis.
Subject(s)
Cystic Fibrosis/blood , Cystic Fibrosis/pathology , Disease Progression , Homeostasis , Iron/blood , Adolescent , Adult , Anti-Bacterial Agents/pharmacology , Anti-Bacterial Agents/therapeutic use , Antimicrobial Cationic Peptides/blood , Cystic Fibrosis/drug therapy , Cystic Fibrosis/physiopathology , Erythropoietin/blood , Female , Forced Expiratory Volume/drug effects , Hemoglobins/metabolism , Hepcidins , Humans , Interleukin-6/blood , Male , Middle Aged , Sputum/drug effects , Sputum/metabolism , Weight Gain/drug effects , Young AdultABSTRACT
Transition to age-appropriate care and transfer of care is a process that best occurs over time. Models to accomplish this are best designed at the local level because local factors weigh heavily on the model a center chooses. Ingredients for the successful transition must include focus on self-care and communication between cystic fibrosis (CF) teams, between pediatric and adult patients who have CF and the teams, and between parents of patients who have CF and the teams. A timeline for transition should begin years before transfer with the realization that one plan may not accommodate all patients' needs.
Subject(s)
Continuity of Patient Care , Cystic Fibrosis/therapy , Adolescent , Adult , Attitude of Health Personnel , Child , Humans , Patient Transfer , Referral and Consultation , Transfer AgreementABSTRACT
The natural history of cystic fibrosis (CF) is an evolving phenomenon. As such, it is continually being redefined as epidemiologic data is collected and processed and as advances in treatment for patients with CF improve the quality and quantity of life. The past year was no exception and has produced exciting new insights regarding outcomes in CF. We review the recent literature and focus our discussion on two specific areas with regard to their impact on the natural history of the disease: (1) low socioeconomic status and (2) wasting and cystic fibrosis related diabetes.
