I am very new to the CF world. My 3 month old daughter Aven, was diagnosed with CF through newborn screening at 7 days old and was confirmed with DDF508 through genetic testing as well as sweat chloride test with result of 98 and 120 at 3 weeks of age.Eventhough we were experiencing loose and foul smelling diapers, she was gaining weight very well on breast milk without the enzymes.Our CF center is very proactive and went ahead and prescribed Zenpep as well as Aquadeks and salt. Even though her lung scans are clear and Oxygen levels have also been at 100%, we will be starting chest physiotherapy in August. I know there is not a crystal ball and that every individual case is different, but I find myself worrying about every little cough ( usually just from the milk going down wrong - nothing that lingers). I have heard and read that having both mutations of the deltaF508 is one of the severest forms of CF and I just feel that I am waiting for the infections to start at any minute. From your experience when did the infections start for your child? Were they born with them? Did it seem to be when their siblings got sick or when they started school? Thank You -
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What can we expect?
- Thread starter palmera
- Start date
We were in your steps 13 years ago. What to expect - who knows!? Our son is age 13 and we are always waiting for the other shoe to drop!
13 years ago, the Director of ouf CF Center firmly believed and told us he expected a cure in 10 years - didn't happen. We as well were told DD FF08 was a severe form. DS was diagnosed at 3 mos. First 6 months rough, but we didn't have the benefit of diagnosis through newborn screening. Getting the diagnosis was very difficult and a story not relevant here. Anyway, DS is DD F508. At 3 mos. DS cultured humufales influenza (sp?) which has reoccurred on and off over the years. He was home for 3 and 1/2 years. Then went to daycare - one of the healthiest kids there. He went to an owner-occupie day care. It was a new facility and the owners were incredibly attentive to DS' needs. Thrived! Fast forward, DS is now 13, attends public school, misses very few days of school, is a straight A student, Jr. Olympiad swimmer (ironically, does very well in distance events), cross-country runner (top 1/3 in county), and leading scorer on his soccer team. He also plays French horn in the school band. I tell you these things not to brag about DS, albeit I am very proud, but to show you that although CF will, without doubt, impact your lives, your daughter's life can still be filled with the normal "stuff".
13 years ago, the Director of ouf CF Center firmly believed and told us he expected a cure in 10 years - didn't happen. We as well were told DD FF08 was a severe form. DS was diagnosed at 3 mos. First 6 months rough, but we didn't have the benefit of diagnosis through newborn screening. Getting the diagnosis was very difficult and a story not relevant here. Anyway, DS is DD F508. At 3 mos. DS cultured humufales influenza (sp?) which has reoccurred on and off over the years. He was home for 3 and 1/2 years. Then went to daycare - one of the healthiest kids there. He went to an owner-occupie day care. It was a new facility and the owners were incredibly attentive to DS' needs. Thrived! Fast forward, DS is now 13, attends public school, misses very few days of school, is a straight A student, Jr. Olympiad swimmer (ironically, does very well in distance events), cross-country runner (top 1/3 in county), and leading scorer on his soccer team. He also plays French horn in the school band. I tell you these things not to brag about DS, albeit I am very proud, but to show you that although CF will, without doubt, impact your lives, your daughter's life can still be filled with the normal "stuff".
kimberlya421
New member
My daughter was also diagnosed at birth and she is almost 4 years old now. She has one DF508 and one other more rare mutation (N1303K). My daughter has zero pancreatic function, so she lost weight in the first weeks and finally starting coming back up after she started taking enzymes. I remember feeling exactly the same in the beginning, worrying about every cough, runny nose, etc. and just waiting for her first occurrence of Pseudomonas. We did the chest therapy from the beginning, using one of those little infant oxygen mask and tongue depressor contraptions. She graduated to a vest when she was about a year and a half, and she has been doing that twice a day ever since. She had always had good lung function, you can't really know how that aspect of the CF will turn out so you have to keep up with chest therapy to be proactive. It's definitely better than just waiting and seeing how her lung function will turn out!She has had one run in with the dreaded Pseudomonas when she was a little over two, and she had a feeding tube placed at the same two week hospital stay.
The best advice I can give you is to just roll with it. It all becomes normal; the chest therapy, nebulizers, enzymes (if needed), vitamins, etc. It all becomes so routine that you don't think twice about it being part of your day. Also, understand that things will come in steps. In our experience, we had time to adjust to each new thing that was thrown our way. It began with enzymes, iron supplement, and chest therapy. Then we added the vest. Then an inhaled antibiotic. Then hypertonic saline (also inhaled), then a feeding tube, then ANOTHER inhaled med. It sounds overwhelming but I promise you will get it under control if you take it one day, one treatment, one cuddling/vest/reading session at a time. And explaining things to your daughter will pay off in the long run. She's too little to understand now, but even a four year old can understand that she needs to do her vest and medicine before school and bed so her lungs stay healthy and she doesn't have to go in the hospital. She even reminds her teachers at preschool that she needs enzymes every time she eats.
ALSO, I hope you've heard about the amazing new treatments that are "curing" CF. They have found a way to reverse the basic gene defect and make the symptoms obsolete - with what looks like just a pill a day. It has been shown to work on one defect, and the DDF508 is the next one going through trials. That's your daughter! There's more info on that here,-- http://www.cff.org/aboutCFFoundation/NewsEvents/2012NewsArchive/6-28-Final-Results-Phase-2-Kalydeco-and-VX-809.cfm -- check it out because it's super exciting!
There is so much more I could tell you... we practically kept Chelsea in a bubble for the first year or so, carrying hand sanitizer everywhere and making everyone use it when they were around her. We did manage to keep her pretty cold free. After a certain point, though, you have to let them slowly adjust to germs. Unless you plan on keeping them out of school forever, they're going to have to get exposure or they're going to be in big trouble come kindergarten. I put Chelsea in a home daycare that had four other children at it when she was three and moved her up to a very clean Montessori preschool last November. She has gotten some minor colds and one case of strep throat, but we keep up with the treatments and she gets over things and I know it's strengthening her immune system.
Please take what I'm telling you with a grain of salt, it's just my experience and your daughter's doctor could have different suggestions. One of the most helpful things I've found is to have constant contact with other CF parents because if nothing else, they know exactly what you're going through and they can offer moral support which is invaluable.
Good luck with everything, take deep breaths when you're overwhelmed, and surround that little girl with strength and love!
The best advice I can give you is to just roll with it. It all becomes normal; the chest therapy, nebulizers, enzymes (if needed), vitamins, etc. It all becomes so routine that you don't think twice about it being part of your day. Also, understand that things will come in steps. In our experience, we had time to adjust to each new thing that was thrown our way. It began with enzymes, iron supplement, and chest therapy. Then we added the vest. Then an inhaled antibiotic. Then hypertonic saline (also inhaled), then a feeding tube, then ANOTHER inhaled med. It sounds overwhelming but I promise you will get it under control if you take it one day, one treatment, one cuddling/vest/reading session at a time. And explaining things to your daughter will pay off in the long run. She's too little to understand now, but even a four year old can understand that she needs to do her vest and medicine before school and bed so her lungs stay healthy and she doesn't have to go in the hospital. She even reminds her teachers at preschool that she needs enzymes every time she eats.
ALSO, I hope you've heard about the amazing new treatments that are "curing" CF. They have found a way to reverse the basic gene defect and make the symptoms obsolete - with what looks like just a pill a day. It has been shown to work on one defect, and the DDF508 is the next one going through trials. That's your daughter! There's more info on that here,-- http://www.cff.org/aboutCFFoundation/NewsEvents/2012NewsArchive/6-28-Final-Results-Phase-2-Kalydeco-and-VX-809.cfm -- check it out because it's super exciting!
There is so much more I could tell you... we practically kept Chelsea in a bubble for the first year or so, carrying hand sanitizer everywhere and making everyone use it when they were around her. We did manage to keep her pretty cold free. After a certain point, though, you have to let them slowly adjust to germs. Unless you plan on keeping them out of school forever, they're going to have to get exposure or they're going to be in big trouble come kindergarten. I put Chelsea in a home daycare that had four other children at it when she was three and moved her up to a very clean Montessori preschool last November. She has gotten some minor colds and one case of strep throat, but we keep up with the treatments and she gets over things and I know it's strengthening her immune system.
Please take what I'm telling you with a grain of salt, it's just my experience and your daughter's doctor could have different suggestions. One of the most helpful things I've found is to have constant contact with other CF parents because if nothing else, they know exactly what you're going through and they can offer moral support which is invaluable.
Good luck with everything, take deep breaths when you're overwhelmed, and surround that little girl with strength and love!
O
ohiosmom
Guest
My daughter is now 24 years old. I <em>remember when she was diagnosis</em> . I also remember how i felt the same way as you do . Always watching never letting my guard down , waiting to see how long it would be before she became sick. What i can tell you is it does get better but it takes a lot of work and never let your guard down. She got sick a lot mostly with weather change and still does. Keeping them active and a healthy diet and doing their treatments at the same time everyday is very important. That's what our doctor told use to do and we did that and it worked for us. we lived around treatments, med's and meals times. When she got into school she was in swimming, band, dance, soccer. So when she was born they told me she would not make it to age 10, then when 10 came they said puberty,then when that came they told me 21, now they say she will live to be 5o to 55 years old if we keep doing everything as we have always done. Others look at her and can not tell she has this disease. we have never had to have feeding tubes. i was very strict about the foods she eat. she never had junk foods till she was a teenager never drank soda but sprite till she was 9 years old.
DS is 9 years old and was diagnosed due to a bowel obstruction. It's difficult to tell when the lungs are going to be affected. We were very proactive from day one -- CPT and nebs 4 times a day and we were told about psuedomonas as if it was something we might deal with when ds was MUCH older --- teens maybe. Big shock at less than 3 months, him still being home with me while I was on maternity leave and he developed bronchitis and cultured pseudmonas.
Basically, just take it day by day, one step at a time. Get into a routine. Only so much you can control.
DS baseline is NO cough. We do treatments three times a day, he's on a number of meds, but pretty much leads a normal life -- goes to school, is in several different sports activities. He still gets colds, last fall he got a stomach bug and DH and I were pretty much flabbergasted because we just weren't quite sure how to deal with a normal childhood bug because DS really doesn't get sick all that often....
Basically, just take it day by day, one step at a time. Get into a routine. Only so much you can control.
DS baseline is NO cough. We do treatments three times a day, he's on a number of meds, but pretty much leads a normal life -- goes to school, is in several different sports activities. He still gets colds, last fall he got a stomach bug and DH and I were pretty much flabbergasted because we just weren't quite sure how to deal with a normal childhood bug because DS really doesn't get sick all that often....
Aven - I love her name! Congrats!
DD was diagnosed via newborn screening - the results came during a hospitalization at two weeks old. She is also homozygous dd508. She was hospitalized due to an exacerbation possibly brought on by aspiration during delivery - may not have been CF-related at all - we really don't know. She has always cultured staph and I do constantly think about when the pseudomonas diagnosis will occur. One thing is certain - there are no absolutes with CF. I pray for my daughter all the time - and worry all the time - but, in truth, the only thing we can do is take the best care of her and teach her how to take care of herself. We are very diligent about her treatments - some individuals can go above and beyond their 'required' treatments and still get sick often. Others can slack and seldom get sick. Everybody is different. Know that diligence is key with this disease - regardless of how it presents itself.
Don't compare your daughter to anybody else with CF. There are modifier genes at work that alter the disease progression from individual to individual. Try to avoid reading too much on the Internet - you'll stress yourself out! <img src="i/expressions/face-icon-small-smile.gif" border="0">
DD was diagnosed via newborn screening - the results came during a hospitalization at two weeks old. She is also homozygous dd508. She was hospitalized due to an exacerbation possibly brought on by aspiration during delivery - may not have been CF-related at all - we really don't know. She has always cultured staph and I do constantly think about when the pseudomonas diagnosis will occur. One thing is certain - there are no absolutes with CF. I pray for my daughter all the time - and worry all the time - but, in truth, the only thing we can do is take the best care of her and teach her how to take care of herself. We are very diligent about her treatments - some individuals can go above and beyond their 'required' treatments and still get sick often. Others can slack and seldom get sick. Everybody is different. Know that diligence is key with this disease - regardless of how it presents itself.
Don't compare your daughter to anybody else with CF. There are modifier genes at work that alter the disease progression from individual to individual. Try to avoid reading too much on the Internet - you'll stress yourself out! <img src="i/expressions/face-icon-small-smile.gif" border="0">
LittleLab4CF
Super Moderator
Delta F508, is the most common CF mutation with 66% of CFers having it. To be sure some DF508 are severe, considering the number of patients the complete range of presentations from the most sick to the least will be observed. Kalydeco is G551D specific to 4% of the CF population. I hope soon we will see a genetically based drug for DF508. This is the future.
G
Gorf
Guest
Delta F508 here, I was diagnosed at 3 years old, because not gaining weight and loose stools. Started enzymes, did therapy. My mother made me do aerosol and chest therapy religiously, never had a lung problem due to CF until 21, which I owe to her I think. It's not like when I was a kid on special occasions I didn't skip them to camp or something else, but if I was home, I did them period. At the time I was told I had the worst case, over the years that has been proven wrong.
I will say that DS started daycare at age 3 months and he was probably one of the healthiest kids there. The director would warn us if something was going around. Other than a couple ear & sinus infections and a stomach bug when he was a year and a half old, he was fine. Just have to be proactive with CPT to keep those lungs clear. Remember your child is a normal kid who just needs a little extra stuff to keep her happy and healthy.
We also make sure DS and anyone close to him gets their flu shots each year. Also, when he was a baby, anyone who wanted to hold him had to wash hands or use hand sanitizer.
We also make sure DS and anyone close to him gets their flu shots each year. Also, when he was a baby, anyone who wanted to hold him had to wash hands or use hand sanitizer.
LisaGreene
New member
You are right, each person's CF is different. What you are are going through is very normal- the worries and fears, concern about every cough. I think I was at our pediatrician's office every week after my son got out of the hospital (he was in for about a month at birth due to meconium ileus which is caused by CF).
Like others have said, take one day at a time. Odds are very good for your baby that she will live a long, healthy life. There is so much hope right now. Focus on that, take good care of yourself, and enjoy your baby. I wish I had that advice when Jake was born. I spent so much time and energy worrying about him. He is now 14 (hard to believe!) and is doing great- almost an Eagle Scout and is training for a Seattle to Vacouver, BC bike ride.
Here are links to two free pamphlets (written in part by me). One is for Newly Diagnosed parents and the other is Caring for Your Infant with CF. They are short, easy to read, practical and hopeful. CF centers around the world hand them out to patients. Let me know if you want me to mail you hard copies. Hugs and Hope! Lisa
Newly Diagnosed: http://www.happyheartfamilies.com/newlydiagnosed.html
Infants: http://www.happyheartfamilies.com/Infant.html
Like others have said, take one day at a time. Odds are very good for your baby that she will live a long, healthy life. There is so much hope right now. Focus on that, take good care of yourself, and enjoy your baby. I wish I had that advice when Jake was born. I spent so much time and energy worrying about him. He is now 14 (hard to believe!) and is doing great- almost an Eagle Scout and is training for a Seattle to Vacouver, BC bike ride.
Here are links to two free pamphlets (written in part by me). One is for Newly Diagnosed parents and the other is Caring for Your Infant with CF. They are short, easy to read, practical and hopeful. CF centers around the world hand them out to patients. Let me know if you want me to mail you hard copies. Hugs and Hope! Lisa
Newly Diagnosed: http://www.happyheartfamilies.com/newlydiagnosed.html
Infants: http://www.happyheartfamilies.com/Infant.html
Aboveallislove
Super Moderator
Dear Lisa,
First, I really do love your work--I've recommended your book so many times and have read it so many times. So please know I don't mean this comment as confrontational at all, but I'm wondering: Do you really think that if someone had told you to "enjoy and not worry," you would have been able to? I read so many people saying that and think that really it is the natural part of the "mourning" process for CF. The worry we have at week 1 is different than week 10 and year 1. And that by trying to tell ourselves to "not worry, relax, enjoy," all we do is add another stressor. What helped me most actually in the early stages is another CF mom who said for 2 years she never thought life would be good again. I never felt like that, but was so uplifted to know how calm and peaceful she was after feeling like that in the beginning was truly uplifting. As was knowing what I felt was natural and a stage (which would change, but always be there in some way) helped me so much. And telling myself not to worry would be like saying "don't look at the elephant in the room." Everyone copes differently and some handle worries better than others--I think its best to know how each of us operates and figure out how we cope best. For me, it was knowing I'll worry and let that aspect of me "be," while I naturally evolved to handling the worry better, while focusing on the hope by researching and keeping current of everything out there in the pipeline.
Not sure if this makes sense but something I've been meaning to write for ages. Thanks again for all your wonderful work and Godspeed to you and your precious children.
First, I really do love your work--I've recommended your book so many times and have read it so many times. So please know I don't mean this comment as confrontational at all, but I'm wondering: Do you really think that if someone had told you to "enjoy and not worry," you would have been able to? I read so many people saying that and think that really it is the natural part of the "mourning" process for CF. The worry we have at week 1 is different than week 10 and year 1. And that by trying to tell ourselves to "not worry, relax, enjoy," all we do is add another stressor. What helped me most actually in the early stages is another CF mom who said for 2 years she never thought life would be good again. I never felt like that, but was so uplifted to know how calm and peaceful she was after feeling like that in the beginning was truly uplifting. As was knowing what I felt was natural and a stage (which would change, but always be there in some way) helped me so much. And telling myself not to worry would be like saying "don't look at the elephant in the room." Everyone copes differently and some handle worries better than others--I think its best to know how each of us operates and figure out how we cope best. For me, it was knowing I'll worry and let that aspect of me "be," while I naturally evolved to handling the worry better, while focusing on the hope by researching and keeping current of everything out there in the pipeline.
Not sure if this makes sense but something I've been meaning to write for ages. Thanks again for all your wonderful work and Godspeed to you and your precious children.
LisaGreene
New member
Hi Aboveallislove (love your name, by the way). Thank you so much for your kind words about my work and your wise observations. You make some very good points and I am so glad you brought them up!
I totally agree that it is a normal part of the mourning process to worry and be afraid. Everyone has to walk through the “valley of the shadow of death” at some point in life. And, we would never want to discount someone’s feelings and experiences when in the midst of the darkness by telling them: “Don’t worry.” Like, hello, really? ;-) As you say, if someone had said that to me, I’d be pretty mad! Unless of course (for me), it was someone who I deeply respected, who had been in the same place, who was compassionate and gentle, and who also provided “education” about why “I shouldn’t worry” (like knowledge of a possible cure looming on the forseeable horizon). Not that I wouldn’t still worry, but I might worry a little bit less.
So, all of that being said, I do want to clarify that I did not specifically say: “Don’t worry.” I did say: “Take one day at a time, focus on the hope, take good care of yourself, and enjoy your baby.” These are all things that, research shows, are positive ways to cope with difficult life challenges. A great reference for this is a book called “Strengthening Family Resilience” by Froma Walsh. I have been studying it as part of my master’s degree and it is so relevant for us parents as we learn to cope with CF and be the models that our children need in order to be positive, hopeful, and resilient themselves.
Thanks again for your thoughts. It is such a good reminder that we each experience things so differently and that compassion, listening, and just being there for another person in their journey is often the best response rather than providing advice.
Hugs and hope to you and your family.
PS: Here is an article that I wrote some time ago about "Keeping Hope Alive" that talks about some of these issues (needing the space to grieve) and tips for moving forward. Has some great quotes from others dealing with CF, too. http://www.happyheartfamilies.citymax.com/articles/article/4494324/118290.htm
I totally agree that it is a normal part of the mourning process to worry and be afraid. Everyone has to walk through the “valley of the shadow of death” at some point in life. And, we would never want to discount someone’s feelings and experiences when in the midst of the darkness by telling them: “Don’t worry.” Like, hello, really? ;-) As you say, if someone had said that to me, I’d be pretty mad! Unless of course (for me), it was someone who I deeply respected, who had been in the same place, who was compassionate and gentle, and who also provided “education” about why “I shouldn’t worry” (like knowledge of a possible cure looming on the forseeable horizon). Not that I wouldn’t still worry, but I might worry a little bit less.
So, all of that being said, I do want to clarify that I did not specifically say: “Don’t worry.” I did say: “Take one day at a time, focus on the hope, take good care of yourself, and enjoy your baby.” These are all things that, research shows, are positive ways to cope with difficult life challenges. A great reference for this is a book called “Strengthening Family Resilience” by Froma Walsh. I have been studying it as part of my master’s degree and it is so relevant for us parents as we learn to cope with CF and be the models that our children need in order to be positive, hopeful, and resilient themselves.
Thanks again for your thoughts. It is such a good reminder that we each experience things so differently and that compassion, listening, and just being there for another person in their journey is often the best response rather than providing advice.
Hugs and hope to you and your family.
PS: Here is an article that I wrote some time ago about "Keeping Hope Alive" that talks about some of these issues (needing the space to grieve) and tips for moving forward. Has some great quotes from others dealing with CF, too. http://www.happyheartfamilies.citymax.com/articles/article/4494324/118290.htm
Aboveallislove
Super Moderator
Dear Lisa,
Thanks for your thoughtful and charitable reply--given that I had put words in your mouth! I am so sorry for that. I re-read and you're advise was really spot on and I wish I had had that too when I was a new mom to the diagnosis!!!
I think what prompted my question was what you had said after that: "I wish I had that advice when Jake was born. I spent so much time and energy worrying about him." But both your comment and your reply made me really think more about what "would help" and it made me realize that it is so different for each of us. I remember early on in clinic asking a "what if, x" question and one of the staff said "we'll we find that it helps parents to take one day at a time and not think about contingencies," and I was like "lady, that is not how I cope. I want to know if the results are x, then what are our options." and then I'll go home and research it and have it mapped out. (Now of course, as I said, I've read your book like 10 times (starting at like 3 months!) so that I'm ready with response A to situation A, and response B to situation B, all the way to response Z when ds is 30!) But it was so very helpful for me to read your thoughts because it will remind me that my way isn't "the" way.
Again, so sorry.
Again, so sorry for the
Thanks for your thoughtful and charitable reply--given that I had put words in your mouth! I am so sorry for that. I re-read and you're advise was really spot on and I wish I had had that too when I was a new mom to the diagnosis!!!
I think what prompted my question was what you had said after that: "I wish I had that advice when Jake was born. I spent so much time and energy worrying about him." But both your comment and your reply made me really think more about what "would help" and it made me realize that it is so different for each of us. I remember early on in clinic asking a "what if, x" question and one of the staff said "we'll we find that it helps parents to take one day at a time and not think about contingencies," and I was like "lady, that is not how I cope. I want to know if the results are x, then what are our options." and then I'll go home and research it and have it mapped out. (Now of course, as I said, I've read your book like 10 times (starting at like 3 months!) so that I'm ready with response A to situation A, and response B to situation B, all the way to response Z when ds is 30!) But it was so very helpful for me to read your thoughts because it will remind me that my way isn't "the" way.
Again, so sorry.
Again, so sorry for the