Re: Severity of the disease/Sara

[Guest guest]

Sara,

Nowhere have I read that a positive Pa culture implies that the

disease is progressing. I think progression could only be measured

by the decline of lung function or other actual clinical

manifestations. I am curious to know more about this subject as

well. I even did a poll a few months ago to see the ages of the

first Pa culture of the members kids. I kept thinking people

(including Abby's doctors) were sugar-coating things in order to keep

us from freaking out. Like I said earlier, the articles I read were

from trials that began years ago. I have read that Pa is

an " opportunistic " pathogen. It attacks damaged tissue like in burn

patients etc. This immediately made me ask our team if this meant

that Abby already had some damage. They assured me this was not the

case. Her lungs were " pristine " as described by the doctor that

performed her bronch four months ago.

If you get any good info on this subject I would be very interested.

Thanks.

Joe

> > Thanks so much Joe,

> >

> > We are at clinic this Friday, I assume they will do another

> culture. I

> > guess I can expect the inhaled Tobi to continue for what, about

28

> days?

> > Seems to be the time period that I have read so much about here.

> >

> > I have to admit, I am terribly afraid of the future now that he

has

> > cultured PA. I am not in a good place these days. I had to take

a

> > leave from work due to stress. I had enough sick days banked to

> get

> > thru til Jan. But it is going by so fast and I don't feel any

> better

> > emotionally. But money needs to be made. I am so lost.

> >

> > Cheryl, mom to 3 boys, Zac 7 yrs. wocf, Jake 3 yrs. wocf,

> 21

> > mos. wcf

> >

> > Re: Severity of the disease

> >

> > Cheryl,

> >

> > I looked at a few older studies that did suggest that the kids

> that

> > cultured Pa early didn't do as well - BUT - I now have a better

> > understanding of why it seemed to look like more of a

> correlation. I

> > think that it's just because Pa is one of the main trouble makers

> for

> > CF'rs and they didn't have a lot of the knowledge or tools that

we

> > have now. Basically the simple fact that they weren't able to

> > eradicate intermittent Pa or manage mucoid Pa as well as they do

> now

> > made these subjects suffer problems sooner than those who didn't

> > culture it as early. Some say it is the difference in the

mucous,

> > that some is more hospitable to it than others while others say

> > getting it is just the luck of the draw. I have heard from quite

> a

> > few that cultured it early but weren't colonized with the mucoid

> > variant until much later. I talked a long time to a CF doctor

who

> is

> > in my family about this subject and he was very reassuring about

> it.

> > He said that even if our Abby did culture some mucoid Pa that it

> > wasn't such a huge deal these days.

> >

> > Abby cultured Pa from a bronch when she was diagnosed at 5 mos.

> old,

> > was treated aggressively with IV tobra, timentin and Tobi for 2

> > weeks. Her throat culture was clear at discharge. She had one

> clear

> > culture 3 mos. later then after 3 more mos. she cultured it

> again.

> > We did 2 weeks Tobi and Cipro. At the end of that course she

> > cultured it again. They then performed a bronch a few days later

> > which revealed she had no Pa in her lungs and they were very

clear

> > with hardly any inflammation. A small amount of Pa was obviously

> in

> > her upper airways causing the positive culture. We just had a

> > culture done Friday and are awaiting the results. She is

> completely

> > asymptomatic now - no cough, wheeze and in 97th percentile in wt.

> > 90th in ht.

> >

> > If she cultures it this time I may try to get her doctor to put

> her

> > on an azithromycin regimen. Maybe go for a full blown IV

course.

> > I'm not sure. There are differing schools of thought on this.

> They

> > do know that it comes and goes at first normally then comes to

> stay.

> > In Denmark they have demonstrated that they can eradicate

> > intermittent non-mucoid Pa and can delay mucoid Pa by treating

> very

> > aggressively with antibiotics - IV and inhaled. Ask your doc

> about

> > the Danish model.

> >

> > This subject is one that has horrified us at first but have

grown

> > much more optimistic. Your post looks like one of mine verbatim

a

> > few months ago. This disease is in it's " short-rows " , which is

an

> > old farming expression meaning it is about finished. In the

> > meantime, with good care, our kids are going to kick butt - Pa or

> no

> > Pa. Don't fear it.

> >

> > Joe

> > (Dad to 3 girls: Kelsi,7; Chloe 2 3/4 & Abby,15mos.w/cf and fat

> legs.

> >

> >

> >

> >

> >

> > -------------------------------------------

> > The opinions and information exchanged on this list should IN NO

> WAY

> > be construed as medical advice.

> >

> > PLEASE CONSULT YOUR PHYSICIAN BEFORE CHANGING ANY MEDICATIONS OR

> > TREATMENTS.

> >

> > ------------------------------------

> >

> >

> >

[Guest guest]

The whole issue of " Current Opinions in Pulmonary Medicine " is about

CF in November. There is an article that I read that is all about

culturing PA in young children. What they know and what they don't

know. It was very good but it shows they don't really know the

answers.

However it did change the opinion of my pulmonary doc. My 23 month

old son with CF has cultured non mucoid PA since 3month old. We

had done one course of TOBI early on that had only cleared the

culture for a month. So he held off since has been healthy to

avoid antibiotic resistance. Because of the new article and studies

out of Europe my doctor decided mucoid PA is a bigger problem than

antibiotic resistance at this point and we are trying TOBI again.

I'm sorry I don't know how to print the link right here but that

article is very helpful for all of us with little ones.

Tina Mertz mom to Zoe 3 wo CF and almost 2!! with CF and a NICU

RN too

[Guest guest]

Joe,

Yes, progression is measured in terms of declining lung function.

Below are three abstracts from PubMed that link chronic pseudo

colinization to decline.

You can search abstracts on pubmed at

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed

~

mother of 3, one with cf

>

> Nowhere have I read that a positive Pa culture implies that the

> disease is progressing. I think progression could only be

measured

> by the decline of lung function or other actual clinical

> manifestations. I am curious to know more about this subject as

> well. >

> If you get any good info on this subject I would be very

interested.

> Thanks.

>

> Joe

Curr Opin Pulm Med. 2003 Nov;9(6):492-7. Related Articles, Links

Pseudomonas acquisition in young patients with cystic fibrosis:

pathophysiology, diagnosis, and management.

Rosenfeld M, Ramsey BW, Gibson RL.

Department of Pediatrics, University of Washington, Seattle, USA.

PURPOSE OF REVIEW: To summarize the pathophysiology of, risk factors

for, and outcomes of early Pseudomonas aeruginosa (Pa) infection in

CF; to review the results of trials of early intervention and to

describe treatment options for early Pa infection. RECENT FINDINGS:

Chronic lower airway Pa infection is associated with significant

morbidity and mortality among CF patients. However, first

acquisition of Pa does not appear to cause an immediate and rapid

decline in lung function. Early Pa isolates are generally non-

mucoid, antibiotic-sensitive, and present at low density, suggesting

a possible " window of opportunity " for early intervention. SUMMARY:

Anti-pseudomonal therapy for early infection results in transient Pa

eradication, but re-infection with Pa appears inevitable despite

early aggressive treatment. There are no controlled trials

demonstrating clinical benefit in young children. There is a

critical need for further investigation of the clinical outcomes

associated with early intervention, the long-term safety profile,

and the optimal drug regimen.

PMID: 14534401 [PubMed - in process]

+++++++++++++++++++++++++++++++++++++++++++++++++++++++

Pediatr Pulmonol. 2002 Jun;33(6):483-91. Related Articles, Links

Predictors of deterioration of lung function in cystic fibrosis.

Schaedel C, de Monestrol I, Hjelte L, Johannesson M, Kornfalt R,

Lindblad A, Strandvik B, Wahlgren L, Holmberg L.

Department of Pediatrics, University Hospital, Lund, Sweden.

Charlotta.Schaedel@...

The severity of lung disease in cystic fibrosis (CF) may be related

to the type of mutation in the cystic fibrosis transmembrane

conductance regulator (CFTR) gene, and to environmental and

immunological factors. Since pulmonary disease is the main

determinant of morbidity and mortality in CF, it is important to

identify factors that can explain and predict this variation. The

aim of this longitudinal study of the whole Swedish CF population

over age 7 years was to correlate genetic and clinical data with the

rate of decline in pulmonary function. The statistical analysis was

performed using the mixed model regression method, supplemented with

calculation of relative risks for severe lung disease in age

cohorts.The severity of pulmonary disease was to some extent

predicted by CFTR genotype. Furthermore, the present investigation

is the first long-term study showing a significantly more rapid

deterioration of lung function in patients with concomitant diabetes

mellitus. Besides diabetes mellitus, pancreatic insufficiency and

chronic Pseudomonas colonization were found to be negative

predictors of pulmonary function. In contrast to several other

reports, we found no significant differences in lung function

between genders. Patients with pancreatic sufficiency have no or

only a slight decline of lung function with age once treatment is

started, but an early diagnosis in this group is desirable.

Copyright 2002 Wiley-Liss, Inc.

PMID: 12001283 [PubMed - indexed for MEDLINE]

++++++++++++++++++++++++++++++++++++

Infect Immun. 1999 Sep;67(9):4744-50. Related Articles, Links

Pulmonary outcome in cystic fibrosis is influenced primarily by

mucoid Pseudomonas aeruginosa infection and immune status and only

modestly by genotype.

Parad RB, Gerard CJ, Zurakowski D, Nichols DP, Pier GB.

Division of Pulmonary Medicine, Ina Sue Perlmutter Cystic Fibrosis

Research Laboratory, Children's Hospital, Boston, Massachusetts, USA.

Whether allelic variants of the cystic fibrosis (CF) transmembrane

conductance regulator (CFTR) independently contribute to pulmonary

outcome in CF patients has not been resolved. We used both cross-

sectional and mixed-model longitudinal analyses of data from CF

patients that were at least 12 years old to determine the influence

on pulmonary function (percent predicted forced expiratory volume

[FEV(1)]) of the CFTR gene genotype, gender, mucoid Pseudomonas

aeruginosa (MPA) infection status, presence of total opsonic

antibody to MPA, and, separately, the opsonic antibody activity

specific to the mucoid exopolysaccharide (MEP) surface antigen. Two

different factors were independently associated with the lack of MPA

infection: a high level of MEP-specific opsonic activity (MSOA),

implicating an immunologically based mechanism of resistance to

infection, and a lack of any type of opsonic antibody to MPA,

indicative of no significant exposure or infection. This latter

phenotype was found in a subset of CF patients who carried at least

one uncommon CFTR gene allele suggestive of a genetic basis for

resistance to infection in this group of older CF patients. For CF

patients in whom both CFTR gene alleles were identified by screening

for the 12 most common variants (75% of alleles), cross-sectional

analysis showed that MPA infection was best correlated with lower

percent predicted FEV(1), while genotype (two versus one DeltaF508

CFTR gene allele) and a low level of MSOA were associated with

increased risk of infection. A mixed-model analysis of longitudinal

spirometric measurements that considered multiple risk factors to

derive regression equations was used to determine which clinical

parameters had the greatest effect on the annual rate of decline in

percent predicted FEV(1). This analysis showed that the CFTR gene

genotype only modestly modified the constant (y intercept) of the

derived equations, while gender and MPA infection status had the

largest effects on annual rates of decline in percent predicted FEV

(1). These results indicate that the CFTR genotype is usually not a

primary determinant of pulmonary function in most CF patients, but

gender and MPA infection status are. Infection status is potentially

influenced by both immunologic (a high level of MSOA) and genetic

factors, such as carriage of a CFTR gene allele that leads to a

diagnosis of CF but still confers resistance to infection that is

comparable to that of the wild-type CFTR gene.

PMID: 10456926 [PubMed - indexed for MEDLINE]

[Guest guest]

Do you have to pay to read the article?

Thanks,

Gale

> The whole issue of " Current Opinions in Pulmonary Medicine " is

about

> CF in November. There is an article that I read that is all about

> culturing PA in young children. What they know and what they don't

> know. It was very good but it shows they don't really know the

> answers.

> However it did change the opinion of my pulmonary doc. My 23 month

> old son with CF has cultured non mucoid PA since 3month old.

We

> had done one course of TOBI early on that had only cleared the

> culture for a month. So he held off since has been healthy to

> avoid antibiotic resistance. Because of the new article and studies

> out of Europe my doctor decided mucoid PA is a bigger problem than

> antibiotic resistance at this point and we are trying TOBI again.

> I'm sorry I don't know how to print the link right here but that

> article is very helpful for all of us with little ones.

> Tina Mertz mom to Zoe 3 wo CF and almost 2!! with CF and a

NICU

> RN too

[Guest guest]

Joe,

I guess I just assumed that he would have PA without the disease

progressing, but it does make sense that if his lungs are not

damaged then he still might be very healthy.

I consulted my favorite book I call 's Big Book of Everything

CF ( the Orenstein book) and he says that lung damage is the

biggest concern.

Zach cannot do any pft tests until he is six. Our clinic does not

do them until the kids are six. He doesn't seem to have any

breathing trouble so that is good.

Thanks for the pep talk!

Sara

> > > Thanks so much Joe,

> > >

> > > We are at clinic this Friday, I assume they will do another

> > culture. I

> > > guess I can expect the inhaled Tobi to continue for what,

about

> 28

> > days?

> > > Seems to be the time period that I have read so much about

here.

> > >

> > > I have to admit, I am terribly afraid of the future now that

he

> has

> > > cultured PA. I am not in a good place these days. I had to

take

> a

> > > leave from work due to stress. I had enough sick days banked

to

> > get

> > > thru til Jan. But it is going by so fast and I don't feel any

> > better

> > > emotionally. But money needs to be made. I am so lost.

> > >

> > > Cheryl, mom to 3 boys, Zac 7 yrs. wocf, Jake 3 yrs. wocf,

> > 21

> > > mos. wcf

> > >

> > > Re: Severity of the disease

> > >

> > > Cheryl,

> > >

> > > I looked at a few older studies that did suggest that the kids

> > that

> > > cultured Pa early didn't do as well - BUT - I now have a

better

> > > understanding of why it seemed to look like more of a

> > correlation. I

> > > think that it's just because Pa is one of the main trouble

makers

> > for

> > > CF'rs and they didn't have a lot of the knowledge or tools

that

> we

> > > have now. Basically the simple fact that they weren't able to

> > > eradicate intermittent Pa or manage mucoid Pa as well as they

do

> > now

> > > made these subjects suffer problems sooner than those who

didn't

> > > culture it as early. Some say it is the difference in the

> mucous,

> > > that some is more hospitable to it than others while others

say

> > > getting it is just the luck of the draw. I have heard from

quite

> > a

> > > few that cultured it early but weren't colonized with the

mucoid

> > > variant until much later. I talked a long time to a CF doctor

> who

> > is

> > > in my family about this subject and he was very reassuring

about

> > it.

> > > He said that even if our Abby did culture some mucoid Pa that

it

> > > wasn't such a huge deal these days.

> > >

> > > Abby cultured Pa from a bronch when she was diagnosed at 5

mos.

> > old,

> > > was treated aggressively with IV tobra, timentin and Tobi for

2

> > > weeks. Her throat culture was clear at discharge. She had

one

> > clear

> > > culture 3 mos. later then after 3 more mos. she cultured it

> > again.

> > > We did 2 weeks Tobi and Cipro. At the end of that course she

> > > cultured it again. They then performed a bronch a few days

later

> > > which revealed she had no Pa in her lungs and they were very

> clear

> > > with hardly any inflammation. A small amount of Pa was

obviously

> > in

> > > her upper airways causing the positive culture. We just had a

> > > culture done Friday and are awaiting the results. She is

> > completely

> > > asymptomatic now - no cough, wheeze and in 97th percentile in

wt.

> > > 90th in ht.

> > >

> > > If she cultures it this time I may try to get her doctor to

put

> > her

> > > on an azithromycin regimen. Maybe go for a full blown IV

> course.

> > > I'm not sure. There are differing schools of thought on

this.

> > They

> > > do know that it comes and goes at first normally then comes to

> > stay.

> > > In Denmark they have demonstrated that they can eradicate

> > > intermittent non-mucoid Pa and can delay mucoid Pa by treating

> > very

> > > aggressively with antibiotics - IV and inhaled. Ask your doc

> > about

> > > the Danish model.

> > >

> > > This subject is one that has horrified us at first but have

> grown

> > > much more optimistic. Your post looks like one of mine

verbatim

> a

> > > few months ago. This disease is in it's " short-rows " , which

is

> an

> > > old farming expression meaning it is about finished. In the

> > > meantime, with good care, our kids are going to kick butt - Pa

or

> > no

> > > Pa. Don't fear it.

> > >

> > > Joe

> > > (Dad to 3 girls: Kelsi,7; Chloe 2 3/4 & Abby,15mos.w/cf and

fat

> > legs.

> > >

> > >

> > >

> > >

> > >

> > > -------------------------------------------

> > > The opinions and information exchanged on this list should IN

NO

> > WAY

> > > be construed as medical advice.

> > >

> > > PLEASE CONSULT YOUR PHYSICIAN BEFORE CHANGING ANY MEDICATIONS

OR

> > > TREATMENTS.

> > >

> > > ------------------------------------

> > >

> > >

> > >

[Guest guest]

I couldn't get a copy online but I got a photocopy from the librarian

at our Children's Hospital. She would do this for anyone staff or

family.

Tina Mertz

[Guest guest]

Hi ,

I had no idea that they could do pft tests on babies. I thought

they had to be able to understand to blow into the machine. I will

check with our clinic about having a pft test done at another

clinic. How is this test done anyway?

Sara

> > > > Thanks so much Joe,

> > > >

> > > > We are at clinic this Friday, I assume they will do another

> > > culture. I

> > > > guess I can expect the inhaled Tobi to continue for what,

> about

> > 28

> > > days?

> > > > Seems to be the time period that I have read so much about

> here.

> > > >

> > > > I have to admit, I am terribly afraid of the future now that

> he

> > has

> > > > cultured PA. I am not in a good place these days. I had to

> take

> > a

> > > > leave from work due to stress. I had enough sick days

banked

> to

> > > get

> > > > thru til Jan. But it is going by so fast and I don't feel

any

> > > better

> > > > emotionally. But money needs to be made. I am so lost.

> > > >

> > > > Cheryl, mom to 3 boys, Zac 7 yrs. wocf, Jake 3 yrs. wocf,

>

> > > 21

> > > > mos. wcf

> > > >

> > > > Re: Severity of the disease

> > > >

> > > > Cheryl,

> > > >

> > > > I looked at a few older studies that did suggest that the

kids

> > > that

> > > > cultured Pa early didn't do as well - BUT - I now have a

> better

> > > > understanding of why it seemed to look like more of a

> > > correlation. I

> > > > think that it's just because Pa is one of the main trouble

> makers

> > > for

> > > > CF'rs and they didn't have a lot of the knowledge or tools

> that

> > we

> > > > have now. Basically the simple fact that they weren't able

to

> > > > eradicate intermittent Pa or manage mucoid Pa as well as

they

> do

> > > now

> > > > made these subjects suffer problems sooner than those who

> didn't

> > > > culture it as early. Some say it is the difference in the

> > mucous,

> > > > that some is more hospitable to it than others while others

> say

> > > > getting it is just the luck of the draw. I have heard from

> quite

> > > a

> > > > few that cultured it early but weren't colonized with the

> mucoid

> > > > variant until much later. I talked a long time to a CF

doctor

> > who

> > > is

> > > > in my family about this subject and he was very reassuring

> about

> > > it.

> > > > He said that even if our Abby did culture some mucoid Pa

that

> it

> > > > wasn't such a huge deal these days.

> > > >

> > > > Abby cultured Pa from a bronch when she was diagnosed at 5

> mos.

> > > old,

> > > > was treated aggressively with IV tobra, timentin and Tobi

for

> 2

> > > > weeks. Her throat culture was clear at discharge. She had

> one

> > > clear

> > > > culture 3 mos. later then after 3 more mos. she cultured it

> > > again.

> > > > We did 2 weeks Tobi and Cipro. At the end of that course

she

> > > > cultured it again. They then performed a bronch a few days

> later

> > > > which revealed she had no Pa in her lungs and they were very

> > clear

> > > > with hardly any inflammation. A small amount of Pa was

> obviously

> > > in

> > > > her upper airways causing the positive culture. We just had

a

> > > > culture done Friday and are awaiting the results. She is

> > > completely

> > > > asymptomatic now - no cough, wheeze and in 97th percentile

in

> wt.

> > > > 90th in ht.

> > > >

> > > > If she cultures it this time I may try to get her doctor to

> put

> > > her

> > > > on an azithromycin regimen. Maybe go for a full blown IV

> > course.

> > > > I'm not sure. There are differing schools of thought on

> this.

> > > They

> > > > do know that it comes and goes at first normally then comes

to

> > > stay.

> > > > In Denmark they have demonstrated that they can eradicate

> > > > intermittent non-mucoid Pa and can delay mucoid Pa by

treating

> > > very

> > > > aggressively with antibiotics - IV and inhaled. Ask your

doc

> > > about

> > > > the Danish model.

> > > >

> > > > This subject is one that has horrified us at first but have

> > grown

> > > > much more optimistic. Your post looks like one of mine

> verbatim

> > a

> > > > few months ago. This disease is in it's " short-rows " , which

> is

> > an

> > > > old farming expression meaning it is about finished. In the

> > > > meantime, with good care, our kids are going to kick butt -

Pa

> or

> > > no

> > > > Pa. Don't fear it.

> > > >

> > > > Joe

> > > > (Dad to 3 girls: Kelsi,7; Chloe 2 3/4 & Abby,15mos.w/cf and

> fat

> > > legs.

> > > >

> > > >

> > > >

> > > >

> > > >

> > > > -------------------------------------------

> > > > The opinions and information exchanged on this list should

IN

> NO

> > > WAY

> > > > be construed as medical advice.

> > > >

> > > > PLEASE CONSULT YOUR PHYSICIAN BEFORE CHANGING ANY

MEDICATIONS

> OR

> > > > TREATMENTS.

> > > >

> > > > ------------------------------------

> > > >

> > > >

> > > >