Having another baby- how was your journey?

So I'm interested to know what people's experiences are with deciding to have more children after you've had one diagnosed with CF. I always thought we'd try naturally knowing that there was a possibility of having another with CF, but I've recently changed my mind to thinking IVF with PGD is probably the more sensible option. Our daughter (DDF508) is 3.5 now, and though I wouldn't change her or our journey so far, I think having any more children with CF would stretch us too far and impact what we are capable of giving to our children, to each other, and to our lives in general. Another option that my SO has put on the table is adoption, but I'm not sure how I feel about that yet. I think in all honesty I'd like to try for one more of our own, and then maybe consider adoption after that. I can't see us having more than 3 total, but I don't pretend to know what the future holds... So what was your decision-making process? And if you did go for IVF/PGD, what was it like?
Source: Cystic Fibrosis Families Forum - Category: Respiratory Medicine Authors: Tags: Families Source Type: forums

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Publication date: 15 June 2019Source: New Scientist, Volume 242, Issue 3234Author(s): Clare Wilson
Source: New Scientist - Category: Science Source Type: research
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Source: Acta Clinica Belgica - Category: General Medicine Authors: Source Type: research
The objectives of this study were to characterize the clinical outcomes and clearance of Mycobacterium abscessus complex (M. abscessus) from respiratory cultures in children with CF M. abscessus PD. Methods: This retrospective longitudinal cohort analysis evaluated the first course of treatment for M. abscessus PD in 33 children in Queensland, Australia between 2001 and 2015. Spirometry and nutritional outcomes 2 years pretreatment and 1 year posttreatment were compared with clearance or relapse/persistence of Mycobacterium abscessus complex from respiratory cultures. Results: Nine of 18 children who completed therap...
Source: The Pediatric Infectious Disease Journal - Category: Infectious Diseases Tags: Original Studies Source Type: research
AbstractThe past decade has witnessed a surge in research centered around exploring the role of the enigmatic innate immune-like lymphocyte MAIT cell in human disease. Recent evidence has led to the elucidation of its role as a potent defender at mucosal surfaces including lungs due to its capacity to mount a formidable immediate response to bacterial pathogens. MAIT cells have a unique attribute of recognizing microbial ligands in conjunction with non-classical MHC-related protein MR1. Recent studies have demonstrated their contribution in the pathogenesis of chronic pulmonary disorders including asthma and chronic obstru...
Source: Inflammation Research - Category: Research Source Type: research
ConclusionOur results highlight the importance of screening for large rearrangements in CF cases with no or only oneCFTR mutation defined.
Source: Molecular Genetics & Genomic Medicine - Category: Genetics & Stem Cells Authors: Tags: ORIGINAL ARTICLE Source Type: research
Calcium-activated anion secretion is expected to ameliorate cystic fibrosis, a genetic disease that carries an anion secretory defect in exocrine tissues. Human patients and animal models of the disease that present a mild intestinal phenotype have been postulated to bear a compensatory calcium-activated anion secretion in the intestine. TMEM16A is calcium-activated anion channel whose presence in the intestinal epithelium is contradictory. We aim to test the functional expression of TMEM16A using animal models with Cftr and/or Tmem16a intestinal silencing. Expression of TMEM16A was studied in a wild type and intestinal Tm...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
We report a case of cystic fibrosis (CF) in a 15-year-old female patient who is a compound heterozygote for CFTR gene, with delta F508 and Tyr109Glyfs mutations detected. This is the first detailed description of such a case in the medical literature. The primary CF presentation occurred at the age of 9 in the form of gastrointestinal symptoms including greasy, bulky, and foul-smelling stool. The patient exhibited delayed growth, with her height and weight being below the 5th centile for age according to the World Health Organization growth curves. Pancreatic enzyme supplement treatment was started immediately, alongside h...
Source: Croatian Medical Journal - Category: General Medicine Authors: Tags: Croat Med J Source Type: research
CONCLUSIONS: We found insufficient evidence from the literature to determine an effective strategy for antibiotic therapy for treating chronic BCC infection. PMID: 31194880 [PubMed - as supplied by publisher]
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
CONCLUSION: SLC6A14-mediated L-arginine transport augments residual F508del-CFTR channel function via a non-canonical, NO pathway. This effect is enhanced with increasing pharmacological rescue of F508del-CFTR to the membrane. The current study demonstrates how endogenous pathways can be utilized for the development of companion therapy in CF. PMID: 31189070 [PubMed - as supplied by publisher]
Source: Mol Biol Cell - Category: Molecular Biology Authors: Tags: Am J Respir Cell Mol Biol Source Type: research
Staphylococcus aureus is the most commonly identified airway colonizer of cystic fibrosis (CF) patients, and infections with methicillin-resistant S. aureus (MRSA) are associated with poor outcomes. Yet, little is known about the intrahost evolution of S. aureus among CF patients. We investigated convergent evolution and adaptation of MRSA among four CF patients with long-term respiratory carriage. For each patient, we performed whole-genome sequencing on an average of 21 isolates (range: 19-23) carried for a mean of 1,403 days (range: 903-1,679), including 25 pairs of isolates collected on the same day. We assessed intrah...
Source: Frontiers in Genetics - Category: Genetics & Stem Cells Source Type: research
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