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Cystic Fibrosis

  • Cystic fibrosis (CF) is a genetic disorder that affects the lungs, pancreas, and other organs, leading to severe respiratory and digestive problems.
  • 162,428 people are living with CF around the world.
  • 105,352 (65%) have been diagnosed
  • 57,076 individuals undiagnosed (Summary by Perplexity AI - March 2024)


Oral vitamin D helps cystic fibrosis - meta-analysis Jan 2024

Vitamin D deficiency in patients with cystic fibrosis: a systematic review and meta-analysis
Journal of Health, Population and Nutrition https://doi.org/10.1186/s41043-024-00499-2
Nazanin Farahbakhsh, Somaye Fatahi, Armin Shirvani, Monireh Sadat Motaharifard, Masoumeh Mohkam, Seyed Ahmad Tabatabaii, Ghamartaj khanbabaee, Shirin Yaghoobpoor, Seyedeh Zahra Davoodi & Amir hossein Hosseini

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WMD = Weighted Mean Difference

Aim
Vitamin D is a prominent modulator of immunity and respiratory function. It plays a vital role in respiratory diseases such as cystic fibrosis (CF). S. However, there is a dearth of information on patients with CF. The purpose of the meta-analysis is to highlight the importance of following the existing guidelines regarding maintenance of Vitamin D serum levels in patients with CF.

Methods
The systematic search was conducted without utilizing any time or language limitations in original database from the beginning until March 2022. The meta-analysis was performed using a random-effects model. Heterogeneity was determined by I2 statistics and Cochrane Q test.

Results
Pooled analysis using the random-effects model of the 8 case–control studies with 13 effect sizes revealed that the serum 25-OH-vitamin D in participants with cystic fibrosis was significantly lower than controls in pediatrics and adolescences (WMD: − 3.41 ng/ml, 95% CI − 5.02, − 1.80, p =  < 0.001) and adults (WMD: − 2.60 ng/ml, 95% CI − 4.32, − 0.89, p = 0.003). Based on data from 12 studies (21 effect sizes) with a total of 1622 participants, the prevalence of vitamin D levels of 20–30 ng/ml in CF patients was 36% among pediatrics/adolescents and 63% among adults. In addition, 27% of pediatric/adolescent CF patients and 35% of adult CF patients had vitamin D levels of below 20 ng/ml.

Conclusions
As a result, according to the existing guidelines, our results proved the need to pay attention to the level of vitamin D in these patients.
 Download the PDF from VitaminDWiki


CF treated by Vitamins D, A, C, E, and zinc, omega-3 (DHA), garlic, ginseng and curcumin - Mercola May 2024

  • "Vitamin D deficiency is notably prevalent among CF patients, affecting their lung function and overall disease progression; maintaining a vitamin D level of at least 60 ng/mL is recommended for mitigating these effects"

 Download the PDF from VitaminDWiki


Some CF studies in VitaminDWiki

51 Cystic Fibrosis items     Highlights


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Cystic Fibrosis articles in VitaminDWiki


Cystic Fibrosis treatment Trikafta costs $300,000/year - March 2024

THE CYSTIC-FIBROSIS BREAKTHROUGH THAT CHANGED EVERYTHING -The Atlantic
Can live to age 82 with the tripple drug.
Click here for the many side effects of Trikafta


See also web

  • Vitamin D supplementation in patients with cystic fibrosis:
    • A systematic review and meta-analysis - Dec 2020  PDF
    • Mortality. 2 trials studed CF deaths in the 12-month follow-up: 1 intervention, 5 placebo; p=0.03
    • Meta-analysis said Vitamin D did not consistently help - mainly because trials had different outcome measures (other than death)
  • Costly Vertex Drug Is Denied, and Medicaid Patients Sue July 2014
    • Kalydeco, for cystic fibrosis costs $300,000 per year;
    • average lung function improvement of 10.1% after about 11 months, compared with a decline of 0.4% in patients taking placebo….”
    • Note by VitaminDWiki - Vitamin D appears to provide a much better improvement and is 30,000 X less expensive
  • CF trial to use 250,000 IU bolus dose of vitamin D July 2017
    To be followed by maintenance dose of 50,000 IU Vitamin D every 2 weeks
     Download the PDF from VitaminDWiki
  • Patient Voices: Cystic Fibrosis New York Times Oct 2017
    Four CF youths are profiled
    Two are running, one is surfing,
    One stays indoors and takes 50 pills per day
    Another hint that Vitamin D helps (not mentioned by the article)

Google Scholar had 354 studies with CF and Vitamin D in their title as of March 2024

  • Vitamin D and Prebiotics for Intestinal Health in Cystic Fibrosis: Rationale and design for a randomized, placebo-controlled, double-blind, 2 × 2 trial of administration of prebiotics and cholecalciferol (vitamin D3) (Pre-D Trial) in adults with cystic fibrosis - Jan 2024 FREE pre-print PDF
    • pre-biotic and 50,000 IU of Vitamin D weekly
  • Safety and efficacy of a new supplementation protocol in patients with cystic fibrosis and vitamin D deficiency - April 2023 FREE PDF
    • Up to 8,000 IU of vitamin D did not get CF patients to 30 ng
  • Vitamin D supplementation in patients with cystic fibrosis: A systematic review and meta-analysis - Sep 2021, https://doi.org/10.1016/j.jcf.2020.12.008 FREE PDF
  • Systematic review and meta-analysis: Associations of vitamin D with pulmonary function in children and young people with cystic fibrosis April 2023, https://doi.org/10.1016/j.clnesp.2023.02.006 FREE PDF
  • METABOLISM AND PHARMACOKINETICS OF VITAMIN D in PATIENTS with Cystic Fibrosis - May 2023 https://doi.org/10.1016/j.jsbmb.2023.106332 PDF behind paywall
  • Development and Evaluation of a Pharmacist-Driven Vitamin D Protocol for a Cystic Fibrosis Clinic - 2022 FREE PDF
  • A Clinician’s guide to vitamin D supplementation for patients with cystic fibrosis - Dec 2021 FREE PDF daily or weekly

See also WikiPedia

  • Cystic fibrosis (CF), also known as mucoviscidosis, is a genetic disorder that affects mostly the lungs but also the pancreas, liver, kidneys and intestine.[1][2] Long-term issues include difficulty breathing and coughing up sputum as a result of frequent lung infections.
  • Other symptoms include sinus infections, poor growth, fatty stool, clubbing of the finger and toes, and infertility in males among others.
  • Different people may have different degrees of symptoms.
  • Occurance: 1 in 30,000 in US, 1 in 1,300 in Ireland
  • Primarily genetic, incureable
  • Congenitally unable to process Vitamin K very well - wonder if vitamin K injections would help
  • "While infants born with it 70 years ago would have been unlikely to live beyond their first year, infants today are likely to live well into adulthood"
  • "Many people with CF are on one or more antibiotics at all times, . . . "

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UK Cystic Fibrosis recommendations to be released in 2017

 Download the UK NHS 2014 draft guidelines of Cystic Fibrosis from VitaminDWiki
 Download the Comments on NHS guidelines - see page 16 for Vitamin D and CF from VitaminDWiki
It is interesting to note that the comments document is 2X larger than the draft document


Average annual cost of CF = $16,000 (March 2013)

Understanding the costs of care for cystic fibrosis: an analysis by age and health state March 2013
US $ Costs for patients with mild =$10,151, moderate, =$25,647 and severe =$33,691
Costs: hospital inpatients (58%), followed by pharmaceuticals (29%)
 Download the PDF from VitaminDWiki
_"Cure" for Cystic Fibrosis - Trikafta - Costs $311,000 for a year
Washington Post Oct 2019 a tripple drug
improves lung function by about only 14%
unknonw how many years it needs to be take


Role of vitamin D on gut microbiota in cystic fibrosis - 2018

Journal of Steroid Biochemistry – Jan 2018 Pages 82-87
Mansi Kanhere, Benoit Chassaing, Andrew T. Gewirtz, Vin Tangpricha
Highlights

  • Vitamin D plays a vital role in maintaining the integrity of the gut epithelium.
  • The mechanism underlying this association involves the reduction of pro-inflammatory cytokines such as IL-8 and inhibition of inflammation-induced apoptosis of intestinal epithelial cells by vitamin D.
  • Intestinal dysbiosis is prevalent in CF owing to multiple factors including malabsorption of nutrients and recurrent antibiotics.
  • VDR regulates the composition and functions of the gut microbiota and decreased expression of intestinal epithelial VDR disrupts microbial homeostasis.
  • Vitamin D could be a potential treatment option for altering dysbiosis in CF, but additional trials are needed to evaluate this.

 Download the PDF from VitaminDWiki


CF results in pancreatic insufficiency-induced malabsorption - which reduces the response to standard Vitamin D - Jan 2021

Factors Contributing to Vitamin D Status at Hospital Admission for Pulmonary Exacerbation in Adults With Cystic Fibrosis
Am J Med Sci . 2021 Jan;361(1):75-82. doi: 10.1016/j.amjms.2020.08.020
Should consider gut-friendly forms of Vitamin D, such as nanoemulsions which can be absorbed in the mouth, rather than the gut


Associated with categories: Intervention - any 7, Breathing 5, Intervention - non daily 3, Magnesium 3, Genetics 3, Diabetes 2, Hypertension 2, Vit D Binding Protein 2, Tuberculosis 2, Virus 2 (May 2024)

Cystic Fibrosis        
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Attached files

ID Name Comment Uploaded Size Downloads
21166 natural-therapies-for-cystic-fibrosis-pdf.pdf admin 05 May, 2024 235.58 Kb 21
20936 CF meta table.png admin 08 Mar, 2024 78.31 Kb 75
20935 CF Meta_CompressPdf.pdf admin 08 Mar, 2024 195.34 Kb 60
14737 Cystic FIbrosis Meta-analysis.pdf admin 19 Dec, 2020 1.12 Mb 637
10388 59351-159663-1-PB.pdf admin 21 Aug, 2018 808.63 Kb 1014
8933 Role-of-vitamin-D-on-gut-microbiota_2018_The-Journal-of-Steroid-Biochemistry.pdf admin 14 Dec, 2017 741.29 Kb 1495
7989 CF trial 250,000.pdf admin 15 May, 2017 432.31 Kb 1435
7911 CF costs.pdf admin 10 Apr, 2017 449.15 Kb 1812
7910 CF Wikipedia.jpg admin 10 Apr, 2017 89.61 Kb 4746
7887 Vitamin D CF PHD 2014 Pincikova.pdf admin 04 Apr, 2017 6.18 Mb 3884
7536 CF lungs.jpg admin 20 Dec, 2016 62.55 Kb 39815
5194 CF UK draft 2014 comments.pdf admin 18 Mar, 2015 426.40 Kb 2525
5192 CF UK draft 2014.pdf admin 18 Mar, 2015 178.59 Kb 2870
5185 CF Wikipedia.jpg admin 17 Mar, 2015 47.10 Kb 7056