Re: New CF Dad

[Guest guest]

Hi Joe,

I have a 19 month old with CF. She has been hospitalized twice with CF

related issues. Once when she was 5 months and once when she was 6 1/2

months. She has two copies of ^F508. We saw a geneticist who is supposed to

be world reknown. He told my husband and I that having two copies of ^F508

was the worst case scenario, that this was the severist of mutations and that

it was indeed a hard road to tow.

When I asked my daughter's CF doc about the issues with double copy of ^F508,

his eyes filled up and he said let's get through the challenges of the first

5 years and take it from there. I could not get a 100% concrete answer.

Said this does not necessarily determine the case scenario. Everyone is

different.

I know several people with the double copy of ^F508. Two of these people

have had double lung transplants, liver transplants. My sister-in-law is one

of these people. She is now 39. She has an unbelievable will to live every

minute to the fullest. She is a true miracle. The other woman is in her 50s

and is also doing quite well.

My daughter is a spit-fire and full of life. She drops fast asleep by 8:30

every night.

She is a fighter. She fights CPT like a son of a gun. The only time she

enjoys it is if she watches the Wiggles and she literally bounces up and down

while she having it done which is 7 days a week twice a day after pulmicort

nebulizer treatments.

I also have a 6 1/2 and 4 year old without CF.

This list is a wonderful site for CF. Everyone is kind and friendly and very

informative.

It makes life a lot easier. If you are having a bad day, they encourage you

and lift your spirits. It is truly a wonderful group of people.

Good luck,

Jenna

[Guest guest]

Joe,

Hello there! Sounds like you have had your hands full. Paige is three, and has

cultured Pa, but it disappeared on us after the first two times it

cultured...after tobi etc. MRSA has stayed with us now for over a year and a

half, and she is fighting with it now..but I think winning this round.

Hang in there DAD

Mom to KM 8 wocf, and Paige 3 wcf, and author of " Juggling Cystic Fibrosis. A

Health Journal "

New CF Dad

Hi,

I'm the father of 3 girls: Kelsi, 6, Chloe 2, and Abby, 8mos. Abby

was a surprise to us in more ways than one. She started with a

stuffy nose after Christmas at 4 1/2 mos., was given Augmentin which

helped temporarily - then developed a bad cough. She was

hospitalized 12/30 & 31 for bronchiolitis. Xray showed patchy mucus.

Discharged 1/1. Retracting badly, 55-65 resp./min. followed up 3x

the next week with ped. I finally demanded to know why she was

retracting so badly so ped. sent us to hospital for bronch to ck. for

tracheo-malacia. Bronch showed she did have it and would grow out of

it and we were headed home. Pulm. dr. said the sweat test was

routine for all resp. admits. Said he wasn't suspicious since,

although she'd had some foul, greasy stools they'd cleared up and it

was attributed to formula switch. She was gaining wt. and eating

good. Test was very positive for CF. Grew PA on culture from fluid

taken in bronchoscopy. Hospitalized for 18 days. Tobra and Timentin

(?) in IV Tobi in neb - began cpt. Throat culture neg. for PA at

discharge. Also neg. on March clinic throat swab. Has had 1 junky

cough since which cleared after 1 course Aug. and 1 week Tobi. Still

on Tobi (28day cycle) Has stuffy nose a lot. Back on Aug. and

fairly clear now. When other 2 tested negative 5 days after init.

diagnosis, we began to climb out of the grief and started getting

educated and involved. Raised over 6000 for local cf walk. Abby is

^ F 508. Both copies. Everything I've read says the mutation can

tell something about dig. problems but not lung. I know that about

half cfrs are both f508 but I want to know what the stats are on this

half in terms of severity and life expectancy. Do the non-f508s make

the overall stats look better? I know these are morbid questions but

every time I hear of these that are doing so well it seems as though

they always have at least one different mutation. I haven't met many

families - just a handful. Also, is the fact that she had PA that

young an indicator at all? The docs, which we like a lot are a

little evasive on this subject but have said it's just the luck of

the draw - in getting PA. We want to know about these things in case

we may decide to see other docs for other opinions on treatment

strategies. I found out about this list from a nice cf mother at the

walk. Abby is the picture of health right now and is much stronger

than the other two were at this age - maybe from fighting cpt? I

also have questions regarding cpt but will ask later. Any advice

will be very appreciated. Thank you

Joe Reams

[Guest guest]

Hi Joe,

Glad you found the list. I'm sure you will get lots of helpful info. from

the wonderful people on this list.

For the rest of you, Joe and his family team raised the most money for our

Great Strides walk last Sat.

Angie P. (mom to JC, 3yo, wcf) and the " nice cf mother " Joe refers to.

New CF Dad

> Hi,

> I'm the father of 3 girls: Kelsi, 6, Chloe 2, and Abby, 8mos. Abby

> was a surprise to us in more ways than one. She started with a

> stuffy nose after Christmas at 4 1/2 mos., was given Augmentin which

> helped temporarily - then developed a bad cough. She was

> hospitalized 12/30 & 31 for bronchiolitis. Xray showed patchy mucus.

> Discharged 1/1. Retracting badly, 55-65 resp./min. followed up 3x

> the next week with ped. I finally demanded to know why she was

> retracting so badly so ped. sent us to hospital for bronch to ck. for

> tracheo-malacia. Bronch showed she did have it and would grow out of

> it and we were headed home. Pulm. dr. said the sweat test was

> routine for all resp. admits. Said he wasn't suspicious since,

> although she'd had some foul, greasy stools they'd cleared up and it

> was attributed to formula switch. She was gaining wt. and eating

> good. Test was very positive for CF. Grew PA on culture from fluid

> taken in bronchoscopy. Hospitalized for 18 days. Tobra and Timentin

> (?) in IV Tobi in neb - began cpt. Throat culture neg. for PA at

> discharge. Also neg. on March clinic throat swab. Has had 1 junky

> cough since which cleared after 1 course Aug. and 1 week Tobi. Still

> on Tobi (28day cycle) Has stuffy nose a lot. Back on Aug. and

> fairly clear now. When other 2 tested negative 5 days after init.

> diagnosis, we began to climb out of the grief and started getting

> educated and involved. Raised over 6000 for local cf walk. Abby is

> ^ F 508. Both copies. Everything I've read says the mutation can

> tell something about dig. problems but not lung. I know that about

> half cfrs are both f508 but I want to know what the stats are on this

> half in terms of severity and life expectancy. Do the non-f508s make

> the overall stats look better? I know these are morbid questions but

> every time I hear of these that are doing so well it seems as though

> they always have at least one different mutation. I haven't met many

> families - just a handful. Also, is the fact that she had PA that

> young an indicator at all? The docs, which we like a lot are a

> little evasive on this subject but have said it's just the luck of

> the draw - in getting PA. We want to know about these things in case

> we may decide to see other docs for other opinions on treatment

> strategies. I found out about this list from a nice cf mother at the

> walk. Abby is the picture of health right now and is much stronger

> than the other two were at this age - maybe from fighting cpt? I

> also have questions regarding cpt but will ask later. Any advice

> will be very appreciated. Thank you

> Joe Reams

>

>

>

>

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

> 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]

Wow, Joe! Congratulations on being such a great Dad and having three

wonderful girls - being a great Dad, you must have wonderful kids :-).

You have educated yourself well in a short time and done a wonderful job

of fundraising. I don't underestimate the emotional toll involved in the

fundraising efforts, so you get lots of extra points for that. Our local

walk for CF (across Canada) is coming up on May 25th.

Regarding the double DeltaF508s: that particular mutation is considered

to present the 'classic' symptoms of CF, as you have learned. However,

more and more the research is coming to the conclusion that there are

modifier genes that affect the severity of any of the CFTR mutations.

There is a large, multi-centre trial just beginning in Canada to search

more comprehensively for the modifiers. It helps explain why some people

with double DeltaF508 mutations do well by CF standards and do 'outlive

the stats'. You are right, though, that two copies of it are almost

always associated with pancreatic insufficiency. The severity of

nutritional problems can affect lung health.

Just remember that stats are only good for predicting trends in the

overall population and cannot easily be applied to any one individual.

In addition to modifier genes that may affect her progress with CF, Abby

has the advantage of you on her side!

M

abbygirlsdad wrote:

>Hi,

>I'm the father of 3 girls: Kelsi, 6, Chloe 2, and Abby, 8mos. Abby

>was a surprise to us in more ways than one. She started with a

>stuffy nose after Christmas at 4 1/2 mos., was given Augmentin which

>helped temporarily - then developed a bad cough. She was

>hospitalized 12/30 & 31 for bronchiolitis. Xray showed patchy mucus.

>Discharged 1/1. Retracting badly, 55-65 resp./min. followed up 3x

>the next week with ped. I finally demanded to know why she was

>retracting so badly so ped. sent us to hospital for bronch to ck. for

>tracheo-malacia. Bronch showed she did have it and would grow out of

>it and we were headed home. Pulm. dr. said the sweat test was

>routine for all resp. admits. Said he wasn't suspicious since,

>although she'd had some foul, greasy stools they'd cleared up and it

>was attributed to formula switch. She was gaining wt. and eating

>good. Test was very positive for CF. Grew PA on culture from fluid

>taken in bronchoscopy. Hospitalized for 18 days. Tobra and Timentin

>(?) in IV Tobi in neb - began cpt. Throat culture neg. for PA at

>discharge. Also neg. on March clinic throat swab. Has had 1 junky

>cough since which cleared after 1 course Aug. and 1 week Tobi. Still

>on Tobi (28day cycle) Has stuffy nose a lot. Back on Aug. and

>fairly clear now. When other 2 tested negative 5 days after init.

>diagnosis, we began to climb out of the grief and started getting

>educated and involved. Raised over 6000 for local cf walk. Abby is

>^ F 508. Both copies. Everything I've read says the mutation can

>tell something about dig. problems but not lung. I know that about

>half cfrs are both f508 but I want to know what the stats are on this

>half in terms of severity and life expectancy. Do the non-f508s make

>the overall stats look better? I know these are morbid questions but

>every time I hear of these that are doing so well it seems as though

>they always have at least one different mutation. I haven't met many

>families - just a handful. Also, is the fact that she had PA that

>young an indicator at all? The docs, which we like a lot are a

>little evasive on this subject but have said it's just the luck of

>the draw - in getting PA. We want to know about these things in case

>we may decide to see other docs for other opinions on treatment

>strategies. I found out about this list from a nice cf mother at the

>walk. Abby is the picture of health right now and is much stronger

>than the other two were at this age - maybe from fighting cpt? I

>also have questions regarding cpt but will ask later. Any advice

>will be very appreciated. Thank you

>Joe Reams

>

>

>

>

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

>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 Joe,

I'm glad you have found this list, it has helped me so much the past 4 years.

My almost 5 year old son, Jack is double DF508 too and what we have been told is

it means they are always pancreatic insufficient and TEND to have more

respiratory problems, however that is variable. Our doctor explained to us(

somebody help me out here, I can get confused) that there can be variations even

within the genes for D F508, which explains why children in the same family can

vary widely in how they are affected by CF. Jack has yet to have any lung

infections, pneumonia, bronchitis or any respiratory problems. Every time he

has been hospitalized it is due to bowel problems. He is in the 90th percentile

for weight and 75th percentile for height. In other words, he is a typical boy.

I too went thru the morbid stage, wondering when/if he was going to die etc. It

has only been in the past year or two that I feel I can finally relax(a

little)and take things as they come. As hard as it is to deal with at first,

you will learn to cope with it- It just takes time and you have to give yourself

that time. I told my husband after our last clinic vist that if someone would

have told me my life would be this " normal " when he was first diagnosed I never

would have believed them. Hang in there!

Kim

>

> Date: 2003/04/17 Thu AM 10:23:21 EDT

> To: cfparents

> Subject: New CF Dad

>

>

[Guest guest]

Welcome Joe to the cfparents list. Abby sounds like she is doing pretty well

now. It is good she was diagnosed when she was, of course. My daughter wcf

is age 19. She cultured pseudo around age 2 1/2. It was not treated

at the time. She was in the hospital as a baby when she was diagnosed (at 8

months) due to dehydration and electrolyte problems. She was not

hospitalized again until age 9 when she was in for IV antibiotics. I

couldn't say about the pseudo being diagnosed early. has 1 df 508

and one still unidentified, she had the Ambry test for over 900 mutations

and none matched. She had two positive sweat tests at 8 months and again at

age 13 when we changed centers. She is pretty mild but has had all the

symptoms. She used to need enzymes but doesn't currently. She is on

albuterol/intal, Tobi and Pulmozyme and the Vest for cpt.

Shes on some other meds for allergies and reflux. I guess this doesn't

really help you a whole lot, but I hope for the best for you. Thanks for

getting involved already and raising $$, You did WONDERFULLY!

love,

M