1STST and Muscle Weakness in CF Patients
Condition: Cystic Fibrosis
MeSH terms: Muscle Weakness; Cystic Fibrosis; Paresis; Fibrosis
Study type: Interventional

Summary: 
      The investigators know that peripheral muscle weakness and exercise intolerance are prevalent
      (56%) in cystic fibrosis (Trooster et al, 2009). Physical inactivity is likely to be an
      important underlying factor. Those conditions are associated with a poor prognosis (Nixon et
      al, 1992). The effect of intravenous antibiotherapy on peripheral muscle and physical
      activity remains unclear.

      The aim of the study is to evaluate the impact of intravenous antibiotherapy on peripheral
      muscular strength in patients with cystic fibrosis (adults and children) who receive
      intravenous antibiotherapy for an acute exacerbation or electively (decline in lung function
      without exacerbation).
    

Detailed description: 
      Background Peripheral muscle weakness and exercise intolerance are prevalent (56%) in cystic
      fibrosis (Trooster et al, 2009). Physical inactivity is likely to be an important underlying
      factor. Those conditions are associated with a poor prognosis (Nixon et al, 1992). The effect
      of intravenous antibiotherapy on peripheral muscle and physical activity remains unclear.
      Wieboldt et al showed that quadriceps strength at the hospital admission for an exacerbation
      was lower than before and one month after hospitalisation while Burtin et al showed that
      individual changes in quadriceps force were correlated with daily time spent activities of at
      least moderate intensity (Wieboldt et al, 2012; Burtin et al, 2013). The effect of
      intravenous antibiotherapy itself is poorly known in cystic fibrosis. Moreover, they did not
      study the impact on muscular strength of hospitalisation versus home treatment. In contrast,
      in COPD, it is well known that exacerbations are associated with a decline of muscle mass and
      strength and that repeated exacerbations lead to a more rapid decrease in fat free mass
      (Spruit et al, 2003; Jones et al, 2015; Joppa et al, 2016).

      Hypothesis We think that exacerbations aggravate factors underlying muscle weakness as
      physical inactivity, systemic inflammation and anabolic status. Moreover, hospitalisation
      itself might be a reason of inactivity as patients spent all the day inside their room
      compared to patients who follow their treatment at home and continue their usual life.

      Aim The aim of our study is to evaluate the impact of an intravenous antibiotherapy on
      peripheral muscular strength in patients with cystic fibrosis.

      Methods We would compare patients (adults and children) who receive intravenous
      antibiotherapy for an acute exacerbation, with patients who receive elective intravenous
      antibiotherapy (decline in lung function without exacerbation). We will also compare
      hospitalised patients who receive specific exercise training, with patients who follow their
      antibiotherapy at home without specific exercise training. We will have a stable patients
      group as control. To evaluate muscular strength we plan to measure quadriceps force at the
      beginning of the cure and at the end with a functional test, the 1-minute Sit-to-Stand test
      and a strength (isometric) test, with the Microfet2dynamometer. Those test are not invasive,
      easy to realise and have already been used in other studies (Ozalevli, 2005). We will
      quantify the physical activity level of the patients with an accelerometer that they will
      carry during 72 hours, during the first week of the treatment.
    

Intervention name(s): Microfet Dynamometer; 1 minute sit to stand test; Accelerometer
Intervention type(s): Device; Behavioral; Device
Intervention description(s): To evaluate muscular strength, the investigators will measure quadriceps force at the beginning of the cure and at the end with a functional test, the 1-minute Sit-to-Stand test, and a maximal isometric force test, with the Microfet2dynamometer. Those tests are not invasive and easy to realise.
The investigators will estimate the physical activity of the patients with an accelerometer that they will each carry 72 hours, during the cure.
Patients who undergo their cure at the hospital will follow a specific training program with the physiotherapist.; To evaluate muscular strength, the investigators will measure quadriceps force at the beginning of the cure and at the end with a functional test, the 1-minute Sit-to-Stand test, and a maximal isometric force test, with the Microfet2dynamometer. Those tests are not invasive and easy to realise.
The investigators will estimate the physical activity of the patients with an accelerometer that they will each carry 72 hours, during the cure.
Patients who undergo their cure at the hospital will follow a specific training program with the physiotherapist.; To evaluate muscular strength, the investigators will measure quadriceps force at the beginning of the cure and at the end with a functional test, the 1-minute Sit-to-Stand test, and a maximal isometric force test, with the Microfet2dynamometer. Those tests are not invasive and easy to realise.
The investigators will estimate the physical activity of the patients with an accelerometer that they will each carry 72 hours, during the cure.
Patients who undergo their cure at the hospital will follow a specific training program with the physiotherapist.
Allocation: Non-Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Primary purpose: Treatment