Therapies: What do you do each day?
Hey there! I think it would be interesting to read what kind of therapy everyone is doing, especially concerning age, which mutations and where you come from. If you are also interested, please share! I will make a start: 22 months old, with f508del and r553x, living in Germany morning: Sultanol, after that 4ml HTS 6% midday: 2,5ml Pulmozyme evening: Sultanol, after that 4ml HTS 6% after/during each neb session doing stretching exercises, blowing games etc. Taking Kreon, AntraMups, Aquadeks, Vitamin D and if necessary oral Antibiotics
To describe the poorly understood burden of pulmonary exacerbations experienced by primary caregivers of children (aged 2-17 years) with cystic fibrosis (CF), who frequently require prolonged hospitalizations for treatment of pulmonary exacerbations with intravenous (IV) antibiotics.
The symptomatology and clinical manifestations of cystic fibrosis (CF) lung disease and primary ciliary dyskinesia (PCD) are quite similar so that the diseases can masquerade as one another. Hannah et al evaluated the frequency of CFTR variants, the genetic basis for CG, in 1021 individuals referred for genetic testing for PCD and compared the results with 91 777 controls. The prevalence of single pathogenic CFTR variants was similar in the 2 groups so a single CFTR variant probably does not cause typical PCD symptoms.
Conditions: Staphylococcus Aureus Pneumonia; Lung Diseases; Cystic Fibrosis; Lung Function Decreased Intervention: Sponsor: University Hospital Muenster Recruiting
Drugs that effectively increase functioning of the cystic fibrosis transmembrane conductance regulator (CFTR) protein have revolutionized the treatment and anticipated health outcomes of many people with cystic fibrosis (PwCF). Collectively known as CFTR modulators, ivacaftor (IVA), lumacaftor/ivacaftor (LUM/IVA), and tezacaftor/ivacaftor (TEZ/IVA) improve lung function, reduce pulmonary exacerbation (PEx) risk, and alleviate respiratory symptoms of CF lung disease [1 –3]. As evidence of a systemic biological effect, these drugs can reduce sweat chloride levels towards the normal range in some individuals.
Pancreatic exocrine insufficiency occurs when there is insufficient production or delivery of pancreatic enzymes required for the digestion and absorption of food. It is associated with relatively poor outcomes, including reduced quality of life and survival.1 Common causes are cancer of the head/body of the pancreas, chronic pancreatitis, pancreatic resection and cystic fibrosis. It can occur following duodenectomy, gastrectomy or untreated coeliac disease via a reduction in cholecystokinin and thereby post-prandial pancreatic stimulation.
Conditions: Cystic Fibrosis; Bronchiectasis Intervention: Other: Comprehensive Respiratory Physiotherapy Sponsor: Bezmialem Vakif University Not yet recruiting
Authors: Brennan GT, Saif MW Abstract Pancreatic enzyme replacement therapy is safe and effective at treating pancreatic exocrine insufficiency. There are multiple causes of pancreatic exocrine insufficiency including chronic pancreatitis, cystic fibrosis and pancreatic cancer. Testing fecal elastase-1 level is useful for the diagnosis of pancreatic exocrine insufficiency. Starting doses of pancreatic enzyme replacement therapy should be at least 30-40,000 IU with each meal and 15-20,000 IU with snacks. pancreatic enzyme replacement therapy should be taken in divided doses throughout meals. Patients who do not resp...
We examined the association between the 8.1 ancestral haplotype and delayed colonization in Cystic Fibrosis, postulating that downregulation ofRNF5 expression is the likely causal mechanism. Our study provides insights into the genetic architecture of the MHC region and pinpoints disease associations that are due to differential expression of HLA genes and non-HLA genes.
Abstract TMEM16A (anoctamin 1) is an important calcium-activated chloride channel (CaCC) in airway smooth muscle (ASM). We have previously shown that TMEM16A antagonists such as benzbromarone relax ASM and have proposed TMEM16A antagonists as novel therapies for asthma treatment. However, TMEM16A is also expressed on airway epithelium, and TMEM16A agonists are being investigated as novel therapies for cystic fibrosis. There are theoretical concerns that agonism of TMEM16A on ASM could lead to bronchospasm making them detrimental as airway therapeutics. The TMEM16A agonist Eact induced a significant contraction of ...
Streptococcus pseudopneumoniae, first described in 2004, belongs to the “mitis group” within the viridans group of streptococci (VGS) . An extremely high level of similarity, especially noted for Streptococcus pneumoniae, Streptococcus mitis, and S. pseudopneumoniae, characterizes the species of the mitis group. The identification of S. pseudopneumoniae remains t herefore challenging [1–3].