Re: Re: Severity of the disease/Sara

[Guest guest]

In a message dated 11/11/2003 2:27:00 AM Central Standard Time,

reamsfarm@... writes:

Joe

Sometimes people can also culture pseudomonas in a throat culture but it

doesn't necessarily mean they have pseudomonas in there lung yet. My oldest

keeps

culturing pseudomonas in a culture but we have done two bronchoscopes and she

has not yet cultured it in her lungs. We have done cultures in her sinuses and

are finding it there. So even though they culture pseudomonas does not mean

it is in there lungs it is just in there airways. This is why our doctor is

doing everything he can to get the gargle for my oldest so it doesn't get into

her lungs. We are hoping it happens by the first of the year. Deb A

[Guest guest]

Sara,

We do PFT's across town at the other Clinic. You know our son is 21 months

old and has had two pfts done so far.

Maybe your clinic can refer Zach to the other clinic for a PFT.

_____

From: dwson2

Sent: Tuesday, November 11, 2003 10:29 AM

To: cfparents

Subject: Re: Severity of the disease/Sara

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]

Hi Sara,

I just wanted to comment on the PFT...Ellie had her first one at 6

months and they are doing another next month. If you are interested in

monitoring her lung function prior to Zach turning 6, you may want to

check and see if there is another CF clinic nearby that offers them. My

understanding is that it's one of the best ways to see how the disease

is progressing.

Mom to Ellie, almost a year old! wCF and Jack 3yrs noCF

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]

ANNOUNCING OUR NEWARRIVAL

The Pulmonary Function Lab ANNOUNCING OUR NEWARRIVAL

The Pulmonary Function Lab at St. Louis Children's Hospital is pleased to

announce our latest arrival, the IPL or Infant Pulmonary Lab from

Medical. The IPL is a devise that will permit very sophisticated

measurements of lung volumes and airflow mechanics in infants from 2 months

old to approximately 3 years old. The PFT Lab at SLCH has extensive

experience in performing infant PFTs since 1990; however, the techniques now

available are far more accurate, and will significantly impact our ability

to assess lung function in infants and guide therapy. Reduction in pulmonary

function tests during infancy may be associated with early lung inflammation

that is not detected by routine exam or chest X-ray. Several key researchers

in the CF field have convincing evidence that aggressive early intervention

with antibiotic and/or anti-inflammatory medicines during infant years will

slow the progressive of lung disease in young childhood. The decision as to

the choice of drug therapy remains with the physician caring for your child.

The IPL has been chosen by the North American Cystic Fibrosis Foundation as

the devise of choice to be used by hospitals designated as TDNs (Therapeutic

Development Network). The SLCH Cystic Fibrosis Center has been selected as a

TDN site and will be eligible effective November 2003 to participate in

shared TDN projects. Your child's doctor and nurses will, therefore, be

requesting permission to schedule all infants with CF for this testing as

part of routine and standard care.

Pulmonary function testing has been a standard care of care for older

children with cystic fibrosis for many years. In children, 4 years and

older, the testing requires cooperation and significant effort. In infants,

this, of course, is not possible. It is necessary, therefore, to sedate the

baby with a very standard and safe medicine. The drug, called chloral

hydrate, is classified as a conscious sedating agent and is given by mouth.

This differs from other sedating agents, such as narcotics, that are

administered via IV or intramuscularly. The advantage of chloral hydrate

aside from it safety record is that is does not alter normal breathing.

Additionally, it can be administered by laboratory personnel. The child can

be awoken from sleep with strong stimulation if necessary. Babies given any

sedating medicine are always monitored carefully, watching heart rate and

oxygen level, and attended to by several technicians. Children sleep

approximately 2 hours which gives the laboratory ample time to complete a

study. The child will wake up from chloral hydrate on their own, and are

able to drink and eat with 15 minutes. Side effects are minimal; however,

all medicines have potential side effects. The taste of the chloral hydrate

is unpleasant, and can be difficult for some children to swallow. Excessive

crying can lead to upset stomachs and gastric cramping. Diarrhea with a

later bowel movement is possible, but not common. In general, the potential

side effects are considered minimal and are strongly outweighed by the value

of the testing. Discontinuing solid and liquid feeds for a prescribed time

prior to testing is required for all sedated children, and significantly

reduces potential side effects associated with the medicine and/or

procedure.

Some parents ask to be with their child during the testing, while others

prefer to wait in a separate room. For those who wish to be present, they

should understand that a mask will be placed over the child's face with a

special seal to prevent leaks. The infant receives fresh air (and oxygen if

necessary) at all times. For a short period, a see - through plexiglas tube

slides over the baby's body to make measurements of lung volume. The child

is observed through the glass for any movement, and this tube is retracted

once the measurements are complete. A second type of test involves placing a

cloth vest around the baby's chest. During this procedure, the child takes

several very deep breaths, following by a rapid forced exhalation. This

procedure is not at all harmful, and actually relaxes the child and improves

oxygen levels. If your child remains asleep, a bronchodilator, such as

albuterol, may also be administered with some testing repeated. Once the

infant awakens from sleep, the testing is discontinued. The total time for

the study including sedation, testing, and recovery time is between 2-3

hours.

_____

From: dwson2

Sent: Tuesday, November 11, 2003 6:45 PM

To: cfparents

Subject: Re: Severity of the disease/Sara

.. How is this test done anyway?

Sara

[Guest guest]

Here is more information on the IPL.

My last post was on OCR scan of a paper I received at the last CF parents

day. I couldn't find it in electronic format.

http://www.ferraris.co.uk/news/2002/1July2002.htm

_____

From: dwson2

Sent: Tuesday, November 11, 2003 6:45 PM

To: cfparents

Subject: Re: Severity of the disease/Sara

Hi ,

How is this test done anyway?

Sara

[Guest guest]

Thank YOU!

Love to all at cfparents,

n Rojas

Re: Severity of the disease/Sara

>

> Hi ,

> How is this test done anyway?

> Sara

>

>

>

>

>

[Guest guest]

Thank you for posting this!!! I was just about to type all this in for

Sara. This is a really good explanation about the procedure.

Re: Severity of the disease/Sara

.. How is this test done anyway?

Sara

[Guest guest]

The post on the new arrival of the IPL at St.Louis Children's Hosp. is a

really good explanation of the test. If you have any other questions

after reading it, let me know. It sounds a little scary with the idea

of having your child sedated but it was fine. Ellie did great and the

only side effect we noticed was she was groggy and a little unbalanced

for a few hours after the test.

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.

> > > >

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

> > > >

> > > >

> > > >