Tag: CFRI

In Cystic Fibrosis Patients, Vitamin D Supplements Might Not Prevent Deficiencies. Study found low vitamin levels in young patients despite recommended supplements

A new study entitled “Vitamin D and Chronic Lung Colonization in Pediatric and Young Adult Cystic Fibrosis Patients” published in the Spanish-language journal Nutrición Hospitalaria, investigated vitamin D levels in the body and its association with chronic lung colonization. The study showed that vitamin D supplements did not improve the vitamin’s insufficiency in young cystic fibrosis patients.

Cystic fibrosis (CF) is a frequently inherited genetic disorder in the Western world, and most CF patients develop respiratory failure due to chronic pulmonary infection. Several factors, including environmental, nutritional, and socioeconomic, may affect the clinical manifestations of cystic fibrosis. Low levels of vitamin D are highly prevalent in CF patients, likely due to the combination of inadequate nutrient absorption, impaired metabolism, and lack of sun exposure. Vitamin D insufficiency is associated with high incidence of bone disease or osteoporosis in CF patients.

Food-with-Vitamin-DIn this study, researchers evaluated vitamin D levels in serum samples from 377 cystic fibrosis patients whose ages ranged from 2 months to 20 years. The patients, from several university hospitals in Spain, were evaluated over the course of two years. The researchers found that younger patients had lower levels of vitamin D as well as a higher number of pathogen colonizations. They showed that despite CF patients receiving vitamin D supplements — which are currently recommended for the disease — a high percentage showed insufficient levels of the vitamin. Moreover, they found that age, pancreatic function, type of diagnosis, and lung colonization modulate the levels of serum vitamin D in these patients. Regardless of age, diagnosis by screening or pancreatic status, chronic colonization by Pseudomonas aeruginosa in children and adolescents and by Staphylococcus aureus in infants and preschoolers increased their risk of developing vitamin D deficiency.

The authors, however, are aware of the limitations of their study.  The longitudinal nature of the study makes impossible to know whether vitamin D levels are the cause or consequence of chronic lung colonization in these patients. The multicenter nature of the study, while necessary to obtain a sample size sufficient in low-prevalence diseases as CF, could introduce biases when analyzing the dose and vitamin D levels due to lack of vitamin supplement standardization protocol among the centers. However, all patients received vitamin D doses as expected. The clinical centers were from different latitudes, but the sera vitamin D were collected during the same time period (fall-winter), decreasing but not eliminating the modifying effect of sun exposure on vitamin D levels.

These findings could encourage additional studies into the interplay of vitamin D, cystic fibrosis, and bacterial infections. New discoveries might help physicians develop new best practices for supplements that could better optimize vitamin D levels in CF patients.

What can you do?

How about ensuring you and your family have 2 serves of oily fish a week – try baked salmon or a tuna sushi roll?

How about serving eggs a couple of times a week – try an egg & cheese wrap for brekky or sneaking an egg into their mash at dinnertime?

 

You can see the original article here CFRI

What is the optimal anti-pseudomonal treatment when Pa is first isolated?

Pseudomonas aeruginosa causes lung infection and chronic lung infection is still the leading cause of lung infection and morbidity in CF. So as a CF community we must be vigilant about:

  1. Infection control
  2. Surveillance
  3. Early diagnosis
  4. Early treatment

Whereas the median age for chronic infection used to be 5 years, its now around 25 years in the top US centres.

Of course the million dollar question (there are many withn pseudomonas but the one we’re most concerned with) is which is the most effective course of treatment for eradication?

While inhaled Tobramycin has been shown to transiently clear the pseudomonas from  the lower airways – it doesn’t clear the lungs of the inflammation which is a key factor in the disease progression and continues the cycle of damage.

Study # 1

In a study cited in this preso, patients were randomised to receive one of two aggressive approaches of 4 weeks inhaled Tobi vs 2 weeks of IV Tobi and Ceftazadine (which is what we do at our clinic) and then they had did a bronch wash of the lower airways (the gold standard to see what’s kicking about down there).

The results showed that there was a difference in the inflammatory markers but it appears that the conclusions drawn were that the results were not significant enough to balance out the feasability, costs and side effects of the systemic IV antibiotic treatment.

Study # 2

The next study compared the two different treatment methods in the US and the UK amongst asymptomatic children (ie. not sick but just positive culture)

Inhaled Tobramycin for 28 days (USA)

VS

Inhaled colistithemate + oral ciprofloxin for 3 months (UK)

After one year the groups had no significant differences in BMI, FEV1, pseudomonal-specific igg – none of the markers that we care about.

After 2 years, 10% of the patients had chronic pseudomonal infection in both groups.

You can watch it online here: http://www.ustream.tv/recorded/75462435

Study # 3

Comparing two more treatment protocols:

Inhaled Tobramycin + oral ciprofloxin

VS

Inhaled colistithemate + oral ciprofloxin

Again this study resulted in no major difference in the eradication profile.

Study # 4

The Standford University team also looked at the timing approach for pseudomonal eradication protocols over a 5-10 year period with their own clinic.

Group # 1 – After a positive Pa culture, they received the initial 28 days inhaled Tobramycin followed by a cycle of treatment for the next 5-6 months (presumably month on, month off).

Group # 2 – Group # 1 – After a positive Pa culture, they received the initial 28 days inhaled Tobramycin followed by a second cycle of treatment if they continued to culture Pa in their swabs.

That group that received the “cycle of treatment” irrespective of whether they continued to test positive or negative for Pa following the initial infection.

Patients on the “cycle therapy” got their 5 rounds of antibiotic therapy post infection regardless of whether the sputum was clear or positive Pa after the initial treatment did not seem to be protected from exacerbations.

So what is the gold standard of care when it comes to Pa?

  1. Inhaled antibiotic therapy – Tobramycin twice daily for 28 days
  2. Against the use of an anti-pseudomonal drug to avoid infection
  3. Routine surveillance sputum cultures to test for pseudomonas before symptoms set in
  4. Positive pseudomonas cultures should be treated quickly (inside of 4 weeks)
  5. There is robust evidence that pseudomonas eradication treatment is effective but there is no definitive result to suggest that one method is superior to another.

A few notes from the latest CFRI conference in California

Screen Shot 2015-08-13 at 8.43.51 AMLast week the annual CFRI conference was held in Redwood City California. For those of you who are not familiar with CFRI, it’s a terrific organisation that funds cystic fibrosis research, provides educational and personal support, and spreads awareness of cystic fibrosis.

Their website hosts a bunch of terrific content from research paper, presentations and key learnings to events and links with other key CF networks. It’s well worth your while getting on there and having a look. They also held a pediatric conference earlier in the year which had some incredible content specifically related to pediatrics. Do yourself a favour and get onto that content when you have a moment, its positive, uplifting but really informative. You can find that here

Screen Shot 2015-08-13 at 8.44.09 AMI travelled to the most recent conference last week which was fantastic. About 200 people went, a combination of speakers that included CF doctors and program directors, a physiotherapist, a social worker, an infection control expert and CF nurse / clinic co-ordinator who has devoted her life to CF; plus about 150 CF parents.

I took a heap of notes so that I could share the content with you all. You can download my notes here.

CFRI summary notes

Please keep in mind that there was a mountain of info so I’ve done my best to try and record / remember as much as I could in each session but there may be some things missing. There’s a bit more to put down on paper but this is the majority of the sessions as I remember them.

If you have any questions please give me a shout at jen@ecooutdoor.com.au

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Keeping CF lungs infection free

A must watch for any CF paediatric parent.

Different doctors give different advice. So do different clinics. There’s no getting around it. We do not have an agreement on modes and models of care from one country to the next or even one clinic to the next.

As parents we can’t avoid the responsibility of making the most informed decisions possible, which is why we all need to seek out information and experiences beyond our own as much as we can. The real reason is not just hearing other doctors speak but also listening to other parents and children with CF. These are the people on the battlegrounds at the forefront of day-to-day care of this disease, just as we are. The CFRI is a terrific organisation that offers loads of resources and they recently had a paediatric conference on “Tools to help your CF child thrive” – this is the first presentation from that series. I’m going to post them one by one with a short summary over the next few weeks.

So here’s a summary of the first session :

What are the things we need to do to keep CF lungs healthy: Tips from a CF researcher who is also the parent of a CF child  Continue reading “Keeping CF lungs infection free”