Ok, after getting about a million pages of abstracts from Val on the yahoo group, i decided to go through a bunch of them and list some that we can use now, or potentially use in the near future. Valerie Hudson is an amazing women, and her dedication to this disease is so inspirational. Well here they are. I hope you enjoy.
144. L-arginine deficiency contributes to impairment
of airway relaxation in CF mice. Pulmonary
exacerbations are associated with an increased
systemic arginase activity and L-arginine deficiency
which may contribute to airway obstruction in CF
196. It is likely that CF cultures do not produce
more mucus than the WT cultures, rather the high rate
of NA absorption simply desiccates the apical surface
and the mucus adheres to the surface.
201. Genistein improved Cl- secretory response in
delF508/delF508 cell lines to 95.6% of corrected cell
lines.
214. I've reprinted this abstract before in its
entirety, but it is about high dose oral NAC given in
the Stanford study. It increased whole blood GSH,
blood neutrophil GSH, reduced sputum IL-8 and HNE
levels, and reduced # of neutrophils. The dose was
1.8-3.0 grams, divided into three doses a day, given
for 4 weeks. The company was BioAdvantex
215. This team gave CF pts an antioxidant mix
containing beta-carotene, Co-Q10, vitamins D and K,
and various tocopherols. They normalized
beta-carotene levels in serum and improved PFTs and
inflammatory markers in sputum. They noted that the
controls were patients on ADEKs and ABDEKs, whose
beta-carotene levels were never normal even with this
supplementation.
222. Sildenafil (which I believe is Viagra) is a type
5 cyclic nucleotide phosphodiesterase (PDE5) inhibitor
that is active as a delF508 trafficking drug, moving
the mutant protein to its correct location in the
apical cell membrane of human nasal epithelial cells.
224. High dose IV colistin once a day is a safe and
effective treatment of multi-resistant PA, and is not
nephrotoxic.
234. The StcE mucinase is a protease from E. coli
that can reduce saliva viscosity by 66%. It was able
to solubilize 78% of CF sputum samples, and took 20-30
minutes to do so.
239. This team treated CF pts with severe
mycobacterium abcessus lung infection with GM-CSF.
Treatment was well tolerated. One pt came off the
lung transplant list; the others had their lung
function stabilized. White blood cell count remined
normal. It is believed that GM-CSF increased the
activity of alveolar macrophages, with resulted in
improved cellular imminuty and higher IFN-gamma
production.
243. Bisaminomethylbithiazoles can correct delF508
trafficking.
245. Cyclic compressive stress (CCS) mimicking normal
breathing restores the ASL layer and mucus transport
in cultured CF airway epithelia. It does so by
inhibitng NTPDase 1. This effect can be reproduced by
ebselen. Ebselen may constitute a practical
alternative to mechanicall-induced lung clearance for
CF pts.
GSNO treatment of cells from CF pts increased
significantly the % of ciliated cells that stained for
CFTR from 17% to 26%. GSNO also increased the
chloride efflux from delF508 cell lines
There is no viable treatment for the highly
transmissible B. dolosa. But silver compounds have
been used for centuries as antimicrobials, most
recently as anti-pseudomonals in burn wards. Silver
carbene complexes are suitable for inhalation, and
this team tried inhalation in mice with B. dolosa.
Silver carbene complexes reduced # of neutrophils,
bacterial load, and likelihood of dissemination of
bacteria to the spleen.
299. In pts on AZM for 3 years, no significant
hepatic or renal effects were noted
This study suggests that 4 weeks of TOBI was
more effective in fighting PA than nebulized colistin
and oral Cipro.
339. Inhalation of nebulized L-arginine increases
exhaled NO and improved pulmonary function in CF pts.
NO augmentation therapy may be a valuable treatment
option for CF pts.
368. Subjects with chronic lung disease have
incontinence that appears not to be due to differences
in pelvic floor muscle strength, but reduced endurance
with prolonged coughing. Recommendations include:
tightening pelvic floor muscles prior to coughing,
endurance training of pelvic floor muscles.
377. 20 mins of CPT significantly accelerates
mucociliary clearance in CF adults
379. The Frequencer is a digitally controlled
electro-acoustical transducer device invented by a CF
adult and manufactured by Dymedso. A power head
transmits vibrations to the airways. The user adjusts
the frequency until a sympathetic resonance in the
thorax is felt. The Frequencer induced similar
clearance to that of CPT.
382. All airway clearance modalities were equally
effective if performed correctly with good patient
participation. But one-on-one training of RTs is
necessary to ensure correct performance.
391. Health care workers were surveyed on how they
reacted to recommendations for infection control in CF
clinics. Although 66-90% agreed with the
recommendations, only 22-53% were adherent to them.
90% agreed that exam rooms should be cleaned between
pts, but only 44% reported this happened for at least
3/4 of the rooms. Only 53% discouraged socialization
between pts. (And they complain that CF pts are not
adherent!!!!!!)
434. Plasma zinc status has been associated with poor
growth and nutritional status in CF infants. For
older children, this association did not hold.
450. This team used an anti-psychotic drug,
Olanzapine, to try and induce weight gain in CF
adults. Some subjects experienced liver dysfunction.
After 6 months, average BMI went from 16 to 18.
501. Self-care independence was unrelated to the pt's
adherence. Self-care independence was related to
psychosocial adjustment, whereas adherence was related
to physical health. (So does that mean you get to
choose between being well-adjusted and being
healthy??)
507. Reported rates of adherence to CPT among CFers
is 40-47%. Adherence to enzymes was <50%.
28. Curcumin rescues delF508 via keratin 18 network.
Curcumin aided delF508 to make it to the plasma
membrane, and also increased chloride efflux. They
feel this happened via an increase in K18 Ser52
phosphorylation.
36. Salmeterol is able to improve the hydration of
mucus and thus may facilitate the process of mucus
exocytosis in CF.
38. Polyunsaturated fatty acids (PUFAs) increased the
basal chloride efflux in CF cells, the most efficient
of which was DHA. This efflux is not mediated by
CFTR, but by another, probably Ca-dependent channel.
46. Inhaled dry powder mannitol (Bronchitol) improves
FEV1 in CF. FEV1 increased 7%, FEF 25-75 by 15%, FVC
increased 4%.
47. Doses of denufosol (a P2Ys receptor agonist which
is supposed to improve mucociliary clearance) up to 60
mg given 3 x a day were generally well tolerated, and
had favorable effects on lung function and
exacerbation rates over 28 days.
50. AZM reduces the ability of CF neutrophils to
produce oxidants and may represent one of its key
anti-inflammatory actions.
69. This study examined what factors seemed to
present a higher risk of one's PA turning mucoid:
factors included persistent PA in sputum, inhaled
bronchodilators, and inhaled colimycin.
Interestingly, isolation of SA, Hib, or S. pneumoniae
in sputum was associated with a lower risk of mucoid
transition.
80. This Italian team is trying out a PA vaccine, and
found good presence of antibodies over several months
after vaccination.
91. IV antibiotics work best in exacerbations when
the exacerbations are severe. Interestingly, FEV1
improves even if the bacteria are supposedly
resistant
93. In UK, they sometimes have routine 3-monthly IV
therapy for CF pts. They compared routine use to "on
demand" use and found there was no difference in
health outcomes, and moving to "on demand" reduced
treatment burden and possible side effects.
105. Double Abx therapy was more effective than
single therapy against resistant PA. Most effective
combos included cipro plus inhaled tobramycin and
meropenem plus amikacin.
111. In this study, a year's treatment with AZM
improved weight and resulted in fewer exacerbations
than before treatment, but there was no change in
FEV1.
129. Looking at their pts colonized with PA, this
team found that 42% of them had one strain, and the
rest had more than one strain. Interestingly, there
was greater mutation of PA strain in pts who did
better (compared to pts who died), and this team
speculated tha the increased evolution of the PA
strain allowed pt and bacteria to co-exist better
together!
142. The survival of PA within aerosol can be reduced
by reducing air humidity and increasing air
temperature. (Hot, dry air--come to Utah; it's 102
degrees with almost no humidity today.)
161. Eradication of PA infection with the aggressive
use of Abx leads to increased risk of infection with
unusual fungi and yeasts. They looked at Scedosporium
apiospermum and Esophiala dermatididis, and while
these cause serious infection in immunocompromised
pts, they did not find decline in nutritional status
or respiratory function over 12 months in CF pts with
these fungi.
167. In CF lungs, there is insufficient production of
surfactant protein D (SP-D), and this team suggests
supplementation with SP-D to prevent infection and
inflammation.
174. The AKITA inhalation system allows more
effective drug utilization. Therefore, lower dose
Gernebcin formulation of tobramycin is as sufficient
as TOBI, and reduces bronchial irritation. Using
Gernebcin and AKITA for tobramycin inhalation saves 80
euros a day per patient
179. Physiotherapy with flutter and inhaled
salbutamol immediately before TOBI inhalation makes
the deposition LESS efficient in CF pts.
182. The Pari LC_Star neb has a smaller particel size
and a higher respirable fraction than the Pari LC-Plus
nev. Despite this, some CF centers recommend the
LC_Plus for the delivery of rhDNase because of faster
output than the LC-Star. But in a head-on trial
between the two nebs, there was virtually no
difference in neb time. So the LC-Star would be
preferable.
185. Bramitob is a new tobramycin preservative-free
sterile solution for nebbing. In this study, Bramitob
performed similarly to TOBI, and peak concentration in
sputum was actually higher with Bramitob
186. This was very interesting: they compared how PA
positive pts did over 10 years, divided into pts who
received no therapy and pts who received aggressive
Abx therapy (FEV1 is being compared):
No therapy: Baseline 92 After 5 yrs 84 After 10
years 83
Therapy: Baseline 82 After 5 yrs 81 After
10 yrs 76
191. CF pts, especially those with low lung function,
may have a significant positive reponse to salbutamol.
194. In Bristol, UK, their CF pts use a remote
monitoring device, which is a portable computer/mobile
phone attached to a spirometer. Pts use the device
once a day and data is sent automatically on a
real-time basis and is read via a secured web site.
(Now, this might definitely help prevent
cross-infection!!)
203. This Polish study showed a significant
correlation between BMI and FEV1. There was a
significant inverse correlation between FEV1 and
sputum IL-8 levels. High IL-10 was correlated with
higher FEV1. High levels of IL-8 were correlated with
decreased BMI.
215. All CF pts with FEV1 < 30% predicted and
baseline O2-sat <95% require in-flight oxygen. Pts
with one of these two risk factors are at risk when
exposed to hypoxic challenge such as air travel.
221. 48 CF children ages 6-10 were followed for 13
years. They found that abnormally low weight for
height increased from 19% in the youngest ages to 40%
at the oldest. These pts with low BMI had
significantly lower FVC and FEV1, and had a greater
yearly loss in lung function than pts with normal
weight for height.
242. ALTU-135 is a microbially derived enzyme therapy
incorporating novel cross-linking and protein
crystallization to create better enzymes. ALTU-135
significantly decreases stool weight while improving
fat absorption in CF pts
250. In this study, 67% of CF pts surveyed said they
never missed anzymes with meals; 35% said they never
missed enzymes with snakcs. Interestingly, there was
no correlation between FEV1 and frequency of missed
enzymes.
252. In CF pts with PI, even with adequate enzyme
therapy, significant steatorrhea still occurs. The
reasons for this are unclear, but it has been
suggested that CF is associated with mucosal
inflammation. This may explain the increased
incidence of Crohn's disease and a 5-fold increase of
small bowel cancer in this populaiton. CF PI pts in
this study were asked to undergo colonoscopy. These
showed a high incidence of small intestinal mucosal
pathology correlating with elevated levels of
calprotectin. In the absence of other known causes of
small bowel pathology, it seems likely that the
observed damage represents a hitherto unknown integral
part of the disease--CF Enteropathy. This may explain
in part the malabsorptive symptoms seen in CF pts.
(Val--another good reason to take oral GSH!!!)
273. In CF children with normal fasting glucose but
abnormal OGTT, the use of glargine insulin helped halt
the progressive decline in lung function.
289. Liver triglyceride accumulation is common in CF
and is due to choline deficiency. They found that CF
children had higher plasma homocysteine, SAM/SAH
ratio, and GSH/GSSG ratio than normal children.
INTERESTINGLY, all these were significantly IMPROVED
by supplementation with lecithin, choline, or betaine.
Choline especially helped the GSH/GSSG ratio.
(Val-This seems like an easy intervention to make.
Many CF children are already using lecithin to help
fat absorption, and it looks like it has far-reaching
effects beyond that.)
296. This clinic used zinc sulfate as a supplement
when their pts had low serum zinc or poor appetite.
In addition to serum levels improving, the group also
had fewer infections than before, as well as caloric
intake and weight-for-height improving. (Val--again,
this is a simple, but possibly very important
intervention!)
297. In 5 adult CF pts, they used 70 mg DHA/kg body
weight for 6 weeks. This treatment significantly
increased the level of DHA in intestinal mucosa.
308. For malnourished infants with CF, a
semi-elemental formula with MCT for a month to help
them catch up is useful
347. This study suggests that there are no
significant differences among the airway clearance
regimens of the active cycle of breathing technique,
autogenic drainage, cornet, flutter, or PEP over a one
year period. Thus, greater consideration should be
given to patient choice of method.
364. Severe changed in posture were observed in 48%
of the CF pts studied. In 84% of these, there was a
difference in the levels of the shoulders. In 77%
there were alterations in the spine, kyphosis,
depressed chest, and externally rotated feet. In 69%,
the head was slightly inclined to one side and the
shoulders protruded. 61% presented lumbar lordosis as
well as cervical and abdominal protrusions. All pts
studied had a difference in the level of the hips.
The incidence of chest alterations was high, which
meant that the respiratory pattern was affected.
397. When CF teens in this study felt well, adherence
rate to therapy was 40%; when they felt unwell,
adherence was 90%
402. They monitored phone calls to the nurse
specialist at their ADULT CF clinic. They discovered
that 1/3 of all inquiries were from parents, and in
79% of these cases, it was the pt's mother who called.
407. In this survey done at an adult CF center in
France, 46% used complementary and alternative
medicines. 57% of these thought CAM helped a lot; 27%
thought it helped a moderate amount. Only 51% of
these pts said they had told their doctor about their
CAM use.
425. CF children were given a questionnaire, and it
was found that there was no association between level
of knowledge about CF and patient health, and no
correlation between knowledge of CF and adherence to
therapies. (!!)
434. This study looked at the exercise habits of
adult CF males. Healthy adult CF males engaged in as
much rigorous activity as their healthy peers.
However, once they became ill, they were less likely
to participate in any physical activity--even milder
activity such as walking. The researchers suggest
that the social discourse of masculinity may
predispose young adult males not to participate in
lighter exercise such as walking, because it is not
seen as manly.
445. They looked at 24 CF teens. 10 of the pts did
less than 50% of their prescribed inhalations of meds.
497. Mean daily physical activity status measured as
steps/day with a pedometer in CFpts does not differ
from healthy subjects.
Take what you want from it. It is long, and there is a lot of stuff, but there are a lot of things we can start taking now, and that are going to be available pretty soon (ie surfactants, manitol etc). Just keep a notepad and write down some of the suppliments available now and seem of interest.
ok enough of that, take it easy
Kiel
144. L-arginine deficiency contributes to impairment
of airway relaxation in CF mice. Pulmonary
exacerbations are associated with an increased
systemic arginase activity and L-arginine deficiency
which may contribute to airway obstruction in CF
196. It is likely that CF cultures do not produce
more mucus than the WT cultures, rather the high rate
of NA absorption simply desiccates the apical surface
and the mucus adheres to the surface.
201. Genistein improved Cl- secretory response in
delF508/delF508 cell lines to 95.6% of corrected cell
lines.
214. I've reprinted this abstract before in its
entirety, but it is about high dose oral NAC given in
the Stanford study. It increased whole blood GSH,
blood neutrophil GSH, reduced sputum IL-8 and HNE
levels, and reduced # of neutrophils. The dose was
1.8-3.0 grams, divided into three doses a day, given
for 4 weeks. The company was BioAdvantex
215. This team gave CF pts an antioxidant mix
containing beta-carotene, Co-Q10, vitamins D and K,
and various tocopherols. They normalized
beta-carotene levels in serum and improved PFTs and
inflammatory markers in sputum. They noted that the
controls were patients on ADEKs and ABDEKs, whose
beta-carotene levels were never normal even with this
supplementation.
222. Sildenafil (which I believe is Viagra) is a type
5 cyclic nucleotide phosphodiesterase (PDE5) inhibitor
that is active as a delF508 trafficking drug, moving
the mutant protein to its correct location in the
apical cell membrane of human nasal epithelial cells.
224. High dose IV colistin once a day is a safe and
effective treatment of multi-resistant PA, and is not
nephrotoxic.
234. The StcE mucinase is a protease from E. coli
that can reduce saliva viscosity by 66%. It was able
to solubilize 78% of CF sputum samples, and took 20-30
minutes to do so.
239. This team treated CF pts with severe
mycobacterium abcessus lung infection with GM-CSF.
Treatment was well tolerated. One pt came off the
lung transplant list; the others had their lung
function stabilized. White blood cell count remined
normal. It is believed that GM-CSF increased the
activity of alveolar macrophages, with resulted in
improved cellular imminuty and higher IFN-gamma
production.
243. Bisaminomethylbithiazoles can correct delF508
trafficking.
245. Cyclic compressive stress (CCS) mimicking normal
breathing restores the ASL layer and mucus transport
in cultured CF airway epithelia. It does so by
inhibitng NTPDase 1. This effect can be reproduced by
ebselen. Ebselen may constitute a practical
alternative to mechanicall-induced lung clearance for
CF pts.
GSNO treatment of cells from CF pts increased
significantly the % of ciliated cells that stained for
CFTR from 17% to 26%. GSNO also increased the
chloride efflux from delF508 cell lines
There is no viable treatment for the highly
transmissible B. dolosa. But silver compounds have
been used for centuries as antimicrobials, most
recently as anti-pseudomonals in burn wards. Silver
carbene complexes are suitable for inhalation, and
this team tried inhalation in mice with B. dolosa.
Silver carbene complexes reduced # of neutrophils,
bacterial load, and likelihood of dissemination of
bacteria to the spleen.
299. In pts on AZM for 3 years, no significant
hepatic or renal effects were noted
This study suggests that 4 weeks of TOBI was
more effective in fighting PA than nebulized colistin
and oral Cipro.
339. Inhalation of nebulized L-arginine increases
exhaled NO and improved pulmonary function in CF pts.
NO augmentation therapy may be a valuable treatment
option for CF pts.
368. Subjects with chronic lung disease have
incontinence that appears not to be due to differences
in pelvic floor muscle strength, but reduced endurance
with prolonged coughing. Recommendations include:
tightening pelvic floor muscles prior to coughing,
endurance training of pelvic floor muscles.
377. 20 mins of CPT significantly accelerates
mucociliary clearance in CF adults
379. The Frequencer is a digitally controlled
electro-acoustical transducer device invented by a CF
adult and manufactured by Dymedso. A power head
transmits vibrations to the airways. The user adjusts
the frequency until a sympathetic resonance in the
thorax is felt. The Frequencer induced similar
clearance to that of CPT.
382. All airway clearance modalities were equally
effective if performed correctly with good patient
participation. But one-on-one training of RTs is
necessary to ensure correct performance.
391. Health care workers were surveyed on how they
reacted to recommendations for infection control in CF
clinics. Although 66-90% agreed with the
recommendations, only 22-53% were adherent to them.
90% agreed that exam rooms should be cleaned between
pts, but only 44% reported this happened for at least
3/4 of the rooms. Only 53% discouraged socialization
between pts. (And they complain that CF pts are not
adherent!!!!!!)
434. Plasma zinc status has been associated with poor
growth and nutritional status in CF infants. For
older children, this association did not hold.
450. This team used an anti-psychotic drug,
Olanzapine, to try and induce weight gain in CF
adults. Some subjects experienced liver dysfunction.
After 6 months, average BMI went from 16 to 18.
501. Self-care independence was unrelated to the pt's
adherence. Self-care independence was related to
psychosocial adjustment, whereas adherence was related
to physical health. (So does that mean you get to
choose between being well-adjusted and being
healthy??)
507. Reported rates of adherence to CPT among CFers
is 40-47%. Adherence to enzymes was <50%.
28. Curcumin rescues delF508 via keratin 18 network.
Curcumin aided delF508 to make it to the plasma
membrane, and also increased chloride efflux. They
feel this happened via an increase in K18 Ser52
phosphorylation.
36. Salmeterol is able to improve the hydration of
mucus and thus may facilitate the process of mucus
exocytosis in CF.
38. Polyunsaturated fatty acids (PUFAs) increased the
basal chloride efflux in CF cells, the most efficient
of which was DHA. This efflux is not mediated by
CFTR, but by another, probably Ca-dependent channel.
46. Inhaled dry powder mannitol (Bronchitol) improves
FEV1 in CF. FEV1 increased 7%, FEF 25-75 by 15%, FVC
increased 4%.
47. Doses of denufosol (a P2Ys receptor agonist which
is supposed to improve mucociliary clearance) up to 60
mg given 3 x a day were generally well tolerated, and
had favorable effects on lung function and
exacerbation rates over 28 days.
50. AZM reduces the ability of CF neutrophils to
produce oxidants and may represent one of its key
anti-inflammatory actions.
69. This study examined what factors seemed to
present a higher risk of one's PA turning mucoid:
factors included persistent PA in sputum, inhaled
bronchodilators, and inhaled colimycin.
Interestingly, isolation of SA, Hib, or S. pneumoniae
in sputum was associated with a lower risk of mucoid
transition.
80. This Italian team is trying out a PA vaccine, and
found good presence of antibodies over several months
after vaccination.
91. IV antibiotics work best in exacerbations when
the exacerbations are severe. Interestingly, FEV1
improves even if the bacteria are supposedly
resistant
93. In UK, they sometimes have routine 3-monthly IV
therapy for CF pts. They compared routine use to "on
demand" use and found there was no difference in
health outcomes, and moving to "on demand" reduced
treatment burden and possible side effects.
105. Double Abx therapy was more effective than
single therapy against resistant PA. Most effective
combos included cipro plus inhaled tobramycin and
meropenem plus amikacin.
111. In this study, a year's treatment with AZM
improved weight and resulted in fewer exacerbations
than before treatment, but there was no change in
FEV1.
129. Looking at their pts colonized with PA, this
team found that 42% of them had one strain, and the
rest had more than one strain. Interestingly, there
was greater mutation of PA strain in pts who did
better (compared to pts who died), and this team
speculated tha the increased evolution of the PA
strain allowed pt and bacteria to co-exist better
together!
142. The survival of PA within aerosol can be reduced
by reducing air humidity and increasing air
temperature. (Hot, dry air--come to Utah; it's 102
degrees with almost no humidity today.)
161. Eradication of PA infection with the aggressive
use of Abx leads to increased risk of infection with
unusual fungi and yeasts. They looked at Scedosporium
apiospermum and Esophiala dermatididis, and while
these cause serious infection in immunocompromised
pts, they did not find decline in nutritional status
or respiratory function over 12 months in CF pts with
these fungi.
167. In CF lungs, there is insufficient production of
surfactant protein D (SP-D), and this team suggests
supplementation with SP-D to prevent infection and
inflammation.
174. The AKITA inhalation system allows more
effective drug utilization. Therefore, lower dose
Gernebcin formulation of tobramycin is as sufficient
as TOBI, and reduces bronchial irritation. Using
Gernebcin and AKITA for tobramycin inhalation saves 80
euros a day per patient
179. Physiotherapy with flutter and inhaled
salbutamol immediately before TOBI inhalation makes
the deposition LESS efficient in CF pts.
182. The Pari LC_Star neb has a smaller particel size
and a higher respirable fraction than the Pari LC-Plus
nev. Despite this, some CF centers recommend the
LC_Plus for the delivery of rhDNase because of faster
output than the LC-Star. But in a head-on trial
between the two nebs, there was virtually no
difference in neb time. So the LC-Star would be
preferable.
185. Bramitob is a new tobramycin preservative-free
sterile solution for nebbing. In this study, Bramitob
performed similarly to TOBI, and peak concentration in
sputum was actually higher with Bramitob
186. This was very interesting: they compared how PA
positive pts did over 10 years, divided into pts who
received no therapy and pts who received aggressive
Abx therapy (FEV1 is being compared):
No therapy: Baseline 92 After 5 yrs 84 After 10
years 83
Therapy: Baseline 82 After 5 yrs 81 After
10 yrs 76
191. CF pts, especially those with low lung function,
may have a significant positive reponse to salbutamol.
194. In Bristol, UK, their CF pts use a remote
monitoring device, which is a portable computer/mobile
phone attached to a spirometer. Pts use the device
once a day and data is sent automatically on a
real-time basis and is read via a secured web site.
(Now, this might definitely help prevent
cross-infection!!)
203. This Polish study showed a significant
correlation between BMI and FEV1. There was a
significant inverse correlation between FEV1 and
sputum IL-8 levels. High IL-10 was correlated with
higher FEV1. High levels of IL-8 were correlated with
decreased BMI.
215. All CF pts with FEV1 < 30% predicted and
baseline O2-sat <95% require in-flight oxygen. Pts
with one of these two risk factors are at risk when
exposed to hypoxic challenge such as air travel.
221. 48 CF children ages 6-10 were followed for 13
years. They found that abnormally low weight for
height increased from 19% in the youngest ages to 40%
at the oldest. These pts with low BMI had
significantly lower FVC and FEV1, and had a greater
yearly loss in lung function than pts with normal
weight for height.
242. ALTU-135 is a microbially derived enzyme therapy
incorporating novel cross-linking and protein
crystallization to create better enzymes. ALTU-135
significantly decreases stool weight while improving
fat absorption in CF pts
250. In this study, 67% of CF pts surveyed said they
never missed anzymes with meals; 35% said they never
missed enzymes with snakcs. Interestingly, there was
no correlation between FEV1 and frequency of missed
enzymes.
252. In CF pts with PI, even with adequate enzyme
therapy, significant steatorrhea still occurs. The
reasons for this are unclear, but it has been
suggested that CF is associated with mucosal
inflammation. This may explain the increased
incidence of Crohn's disease and a 5-fold increase of
small bowel cancer in this populaiton. CF PI pts in
this study were asked to undergo colonoscopy. These
showed a high incidence of small intestinal mucosal
pathology correlating with elevated levels of
calprotectin. In the absence of other known causes of
small bowel pathology, it seems likely that the
observed damage represents a hitherto unknown integral
part of the disease--CF Enteropathy. This may explain
in part the malabsorptive symptoms seen in CF pts.
(Val--another good reason to take oral GSH!!!)
273. In CF children with normal fasting glucose but
abnormal OGTT, the use of glargine insulin helped halt
the progressive decline in lung function.
289. Liver triglyceride accumulation is common in CF
and is due to choline deficiency. They found that CF
children had higher plasma homocysteine, SAM/SAH
ratio, and GSH/GSSG ratio than normal children.
INTERESTINGLY, all these were significantly IMPROVED
by supplementation with lecithin, choline, or betaine.
Choline especially helped the GSH/GSSG ratio.
(Val-This seems like an easy intervention to make.
Many CF children are already using lecithin to help
fat absorption, and it looks like it has far-reaching
effects beyond that.)
296. This clinic used zinc sulfate as a supplement
when their pts had low serum zinc or poor appetite.
In addition to serum levels improving, the group also
had fewer infections than before, as well as caloric
intake and weight-for-height improving. (Val--again,
this is a simple, but possibly very important
intervention!)
297. In 5 adult CF pts, they used 70 mg DHA/kg body
weight for 6 weeks. This treatment significantly
increased the level of DHA in intestinal mucosa.
308. For malnourished infants with CF, a
semi-elemental formula with MCT for a month to help
them catch up is useful
347. This study suggests that there are no
significant differences among the airway clearance
regimens of the active cycle of breathing technique,
autogenic drainage, cornet, flutter, or PEP over a one
year period. Thus, greater consideration should be
given to patient choice of method.
364. Severe changed in posture were observed in 48%
of the CF pts studied. In 84% of these, there was a
difference in the levels of the shoulders. In 77%
there were alterations in the spine, kyphosis,
depressed chest, and externally rotated feet. In 69%,
the head was slightly inclined to one side and the
shoulders protruded. 61% presented lumbar lordosis as
well as cervical and abdominal protrusions. All pts
studied had a difference in the level of the hips.
The incidence of chest alterations was high, which
meant that the respiratory pattern was affected.
397. When CF teens in this study felt well, adherence
rate to therapy was 40%; when they felt unwell,
adherence was 90%
402. They monitored phone calls to the nurse
specialist at their ADULT CF clinic. They discovered
that 1/3 of all inquiries were from parents, and in
79% of these cases, it was the pt's mother who called.
407. In this survey done at an adult CF center in
France, 46% used complementary and alternative
medicines. 57% of these thought CAM helped a lot; 27%
thought it helped a moderate amount. Only 51% of
these pts said they had told their doctor about their
CAM use.
425. CF children were given a questionnaire, and it
was found that there was no association between level
of knowledge about CF and patient health, and no
correlation between knowledge of CF and adherence to
therapies. (!!)
434. This study looked at the exercise habits of
adult CF males. Healthy adult CF males engaged in as
much rigorous activity as their healthy peers.
However, once they became ill, they were less likely
to participate in any physical activity--even milder
activity such as walking. The researchers suggest
that the social discourse of masculinity may
predispose young adult males not to participate in
lighter exercise such as walking, because it is not
seen as manly.
445. They looked at 24 CF teens. 10 of the pts did
less than 50% of their prescribed inhalations of meds.
497. Mean daily physical activity status measured as
steps/day with a pedometer in CFpts does not differ
from healthy subjects.
Take what you want from it. It is long, and there is a lot of stuff, but there are a lot of things we can start taking now, and that are going to be available pretty soon (ie surfactants, manitol etc). Just keep a notepad and write down some of the suppliments available now and seem of interest.
ok enough of that, take it easy
Kiel