He has a 96% pancreas failure right now.... What does this mean for him. Please tell me that he has a chance for a long life... what do I need to do to help them... they are 7 hours away, but I will do whatever I can to help. I am so CF ignorant that I have no Idea what it will mean for his future, thanks for any help...
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My best friend called and her 3 year old son has been diagnosed with CF..
- Thread starter anonymous
- Start date
Hi. You're off to a good start helping your friend by just researching CF. There is so much to this disease that each case is so different. My son has lung difficulties, needs pancreatic enzymes to help digest his food and also takes liver medications. He does therapy where we clap his back and chest to break up mucus in his lungs. I am not sure what 96% pancreatic failure means for him. Hopefully there will be more posts tonight for you. His enzymes help digest the food due to the blockage his pancreas has. Since he's been on them, he has gained 15 pounds in just a year. (He was diagnosed 1 year ago today)
Just know that the advancements made with disease have come so far. Don't panic and be a good ear for your friend. She will be going through a wave of emotions and just having someone a phone call away that understands what she is talking about will be a great help.
Best wishes. <img src="i/expressions/rose.gif" border="0">
Just know that the advancements made with disease have come so far. Don't panic and be a good ear for your friend. She will be going through a wave of emotions and just having someone a phone call away that understands what she is talking about will be a great help.
Best wishes. <img src="i/expressions/rose.gif" border="0">
Hi, I know this is tough for you but you seem like a wonderful friend adn are off to a good start as Shelly said. Have you told your friend about this site? She can come here to get some answers as well.
As far as the pancreas failure goes, I am not sure exactly what is meant by that but I am sure you or your friend will be able to clairify it as you learn more about CF. Digestive enzymes are given to those with CF who have digestive issues. This is because the pancreas in some CFers doesn't work properly and produce the enzymes that break down foods in the body. Therefore, a CFer with pancreatic insufficiency isn't getting all the protein, calories, carbs, fats and vitamins that the body needs. This frequently results in very little weight gain despite a HUGE appetite and them eating a lot. Once your friends son (I can imagine he's like a nephew to you) starts on pancreatic enzymes and they get the dosage right for him (sometimes it takes some adjustments) he will gain weight. Making sure that he is healthy with his weight will assist in keeping the other aspects of his CF in check-if that makes sense.
For some basics on CF, CF is a genetic disorder. Both parents have to be carriers of a CF mutation in order to end up with a child with CF. Many carriers don't realize they are such until they have a child or a family member born with CF. There can be absolutely no traceable CF in the family history but it is still possible. Just in case you were curious, or your friend wanted to know and some you may already know as basic biology so it might be repetative-sorry, the CF mutation is located on Chromosome 7 of the 23 pairs of chromosomes. Every person has 46 chromosomes, arranged in 23 pairs. In each pair, one chromosome is contributed by the mother and one is contributed by the Father. If a person only has 1 CF gene on chromosome 7 (from either mom or dad-it doesn't matter) they are just a carrier (as you best friend and her signifigant other/husband are). If a person has 2 CF genes on chromosome (one from each parent) they have CF. There are currently over 1200 CF mutations that have been detected and named and there are new ones being found all the time. A person's CF mutations does not determine the severity of their CF, it is different for everyone, even those with the same mutations. There is currently research going on as to what exactly determines somebody's severity. Some believe there are modifying genes, some don't, so they are researching it but as to date there is really no explanation.
CF can involve the digestive system, the lungs, sometimes the liver and sometimes the reproductive system. Does your friends son have any lung problems? Has he ever had bronchitis or pneumonia or been diagnosed has having "sever allergies" or "athsma"? If not, I won't bother explaining it now as I know this is a lot of info for both of you to take in and that question can be answered at any point. Let us know if he's had some respiratory involvement too.
-A little personal story to answer the long life question and what it might mean for his future. Everybody's CF affects them differently, even siblings in a family who ovbiously have the EXACT same mutations. I am sure many others will provide some personal background too (It usually helps me to relate to people, I hope it does the same for you).
My husband was diagnosed in 1982 because of failure to thrive (wasn't gaining weight despite eating every 2 hours like a little piggy) and had been in and out of the hospital with respiratory issues. After his diagnosis he was put on enzymes and immediately started eating less and finally gaining weight. His parents started manual chest PT because of his respiratory issues. He was put on antibiotics and they tested his sputum (deep spit from the lungs-have to work hard to cough it up) to see what kind of bacteria he was growing in his lungs. It is very common for CFers to "grow" Psuedomonas and Staph. Aureaus. There are a few other common ones but I cannot think of them right now, I am sure others will mention them.
Since his diagnosis he has not been hospitalized, the only reason why he was in the hospital was to participate in some CF studies and new medication studies. In spite of his CF Mark has lived a very normal life. He played soccer in elementary all the way throught highschool, and recreationally in his first few years of college. He does cardio for about 15 minutes 3 times a week (too much causes him to start loosing weight) but it is necessary to help keep his lungs clear. He lifts weights at the gym 3-5 days a week and that is what he believes help's to keep his weight up and helps to keep him healthy (along with a diet VERY high in calories).
As far as his treatments go he has done chest PT 2 times a day for most of his life, he skipped some years between 13-19 but when we met I emphasized to him that it was very important to me-and our relationship for him to keep himself healthy. I agreed not to nag him about ANY of his treatment if he agreed to sit down with his docs, and draw up a treatment plan for daily usage when he isn't sick and STICK TO IT. At that time all he needed was 1/2hour chest pt every am and every PM. He used nebulized TOBI (Tobramycin is an antibiotic, TOBI is a registered name for the nebulized form) only when his culture reflected a bacterial growth which was about 1-2 times a year. Other than that, his treatments were pretty simple for quite a while and I was only allowed to ask, "what time do you want to do...?" I wasn't allowed to ask "have you done....." as he felt that was nagging. It has worked really well for us. Just last year after his 24th birthday he was put on Pulmozyme to help keep his lungs clear because last year he had about 7 lung infections. The doctor also started him on a regimine of 1 month on tobi, one month off-which is a very common treatment for CF. Additionally, he started using the vest (www.thevest.com) instead of manual Chest PT. Instead of an hour of chest PT every AM and PM, it is 20 mimutes and Mark can do it on his own without the vest so it allows me some extra time to sleep in and go to bed earlier. Mark really likes the vest and feels it is very effective. Some people like it, some hate it and some are neutral but if you can use it and you do think it's beneficial-it is a very wonderful thing! I have heard that some kids who are three are using the vest, and some situations I have heard the parents state the doc felt they needed to wait another year. It could be worth looking into for your friends son.
Mark is on his last "summer off" (as I call it). THis fall he starts his last full year of school and if all goes according to planned, he will be graduating next year from WSU with his BA. A full, normal life is very likely for CFers born nowdays.
If you have any more questions based off of anything we post, please ask more questions. I know this is tough for you and your friend, but it will all be ok.
Julie (wife to Mark 24 w/CF)
As far as the pancreas failure goes, I am not sure exactly what is meant by that but I am sure you or your friend will be able to clairify it as you learn more about CF. Digestive enzymes are given to those with CF who have digestive issues. This is because the pancreas in some CFers doesn't work properly and produce the enzymes that break down foods in the body. Therefore, a CFer with pancreatic insufficiency isn't getting all the protein, calories, carbs, fats and vitamins that the body needs. This frequently results in very little weight gain despite a HUGE appetite and them eating a lot. Once your friends son (I can imagine he's like a nephew to you) starts on pancreatic enzymes and they get the dosage right for him (sometimes it takes some adjustments) he will gain weight. Making sure that he is healthy with his weight will assist in keeping the other aspects of his CF in check-if that makes sense.
For some basics on CF, CF is a genetic disorder. Both parents have to be carriers of a CF mutation in order to end up with a child with CF. Many carriers don't realize they are such until they have a child or a family member born with CF. There can be absolutely no traceable CF in the family history but it is still possible. Just in case you were curious, or your friend wanted to know and some you may already know as basic biology so it might be repetative-sorry, the CF mutation is located on Chromosome 7 of the 23 pairs of chromosomes. Every person has 46 chromosomes, arranged in 23 pairs. In each pair, one chromosome is contributed by the mother and one is contributed by the Father. If a person only has 1 CF gene on chromosome 7 (from either mom or dad-it doesn't matter) they are just a carrier (as you best friend and her signifigant other/husband are). If a person has 2 CF genes on chromosome (one from each parent) they have CF. There are currently over 1200 CF mutations that have been detected and named and there are new ones being found all the time. A person's CF mutations does not determine the severity of their CF, it is different for everyone, even those with the same mutations. There is currently research going on as to what exactly determines somebody's severity. Some believe there are modifying genes, some don't, so they are researching it but as to date there is really no explanation.
CF can involve the digestive system, the lungs, sometimes the liver and sometimes the reproductive system. Does your friends son have any lung problems? Has he ever had bronchitis or pneumonia or been diagnosed has having "sever allergies" or "athsma"? If not, I won't bother explaining it now as I know this is a lot of info for both of you to take in and that question can be answered at any point. Let us know if he's had some respiratory involvement too.
-A little personal story to answer the long life question and what it might mean for his future. Everybody's CF affects them differently, even siblings in a family who ovbiously have the EXACT same mutations. I am sure many others will provide some personal background too (It usually helps me to relate to people, I hope it does the same for you).
My husband was diagnosed in 1982 because of failure to thrive (wasn't gaining weight despite eating every 2 hours like a little piggy) and had been in and out of the hospital with respiratory issues. After his diagnosis he was put on enzymes and immediately started eating less and finally gaining weight. His parents started manual chest PT because of his respiratory issues. He was put on antibiotics and they tested his sputum (deep spit from the lungs-have to work hard to cough it up) to see what kind of bacteria he was growing in his lungs. It is very common for CFers to "grow" Psuedomonas and Staph. Aureaus. There are a few other common ones but I cannot think of them right now, I am sure others will mention them.
Since his diagnosis he has not been hospitalized, the only reason why he was in the hospital was to participate in some CF studies and new medication studies. In spite of his CF Mark has lived a very normal life. He played soccer in elementary all the way throught highschool, and recreationally in his first few years of college. He does cardio for about 15 minutes 3 times a week (too much causes him to start loosing weight) but it is necessary to help keep his lungs clear. He lifts weights at the gym 3-5 days a week and that is what he believes help's to keep his weight up and helps to keep him healthy (along with a diet VERY high in calories).
As far as his treatments go he has done chest PT 2 times a day for most of his life, he skipped some years between 13-19 but when we met I emphasized to him that it was very important to me-and our relationship for him to keep himself healthy. I agreed not to nag him about ANY of his treatment if he agreed to sit down with his docs, and draw up a treatment plan for daily usage when he isn't sick and STICK TO IT. At that time all he needed was 1/2hour chest pt every am and every PM. He used nebulized TOBI (Tobramycin is an antibiotic, TOBI is a registered name for the nebulized form) only when his culture reflected a bacterial growth which was about 1-2 times a year. Other than that, his treatments were pretty simple for quite a while and I was only allowed to ask, "what time do you want to do...?" I wasn't allowed to ask "have you done....." as he felt that was nagging. It has worked really well for us. Just last year after his 24th birthday he was put on Pulmozyme to help keep his lungs clear because last year he had about 7 lung infections. The doctor also started him on a regimine of 1 month on tobi, one month off-which is a very common treatment for CF. Additionally, he started using the vest (www.thevest.com) instead of manual Chest PT. Instead of an hour of chest PT every AM and PM, it is 20 mimutes and Mark can do it on his own without the vest so it allows me some extra time to sleep in and go to bed earlier. Mark really likes the vest and feels it is very effective. Some people like it, some hate it and some are neutral but if you can use it and you do think it's beneficial-it is a very wonderful thing! I have heard that some kids who are three are using the vest, and some situations I have heard the parents state the doc felt they needed to wait another year. It could be worth looking into for your friends son.
Mark is on his last "summer off" (as I call it). THis fall he starts his last full year of school and if all goes according to planned, he will be graduating next year from WSU with his BA. A full, normal life is very likely for CFers born nowdays.
If you have any more questions based off of anything we post, please ask more questions. I know this is tough for you and your friend, but it will all be ok.
Julie (wife to Mark 24 w/CF)
Thank you both so much. I just wish that I lived closer to her so that I could be of more help physically. She is really freaked out about all of his medications, and it scares her to pieces that she is not going to do something right. I am just praying for her and I think I am going to send her a "food gift certificate" so she will not have to cook one night! Thanks so much!
Blessings,
amy
Blessings,
amy
Hi Amy. My friends and family all live far enough away that they can't come by for a visit. But my good friends have been my strength this year just by being there for me to talk to, vent to or simply get my mind off things at home. Your friend will count on your stability and friendship. I went through a week of serious grieving. My friend researched many things and found me books to read and has been an INCREDIBLE source of help through fundraising help with me. That gives me a place to put my CF anxiety so to speak. Keep in touch with her and email or call her to listen or change the subject!
NoDayButToday
New member
You've come to a great place, and maybe, once she is ready enough, your friend could come here too. I recently posted something about my life with CF on a post with something about mutations in the topic, and I imagine it would be a positive thing for you and your friend to read. Good luck<img src="i/expressions/face-icon-small-smile.gif" border="0">
I have to tell you that I think you are an awesome friend for coming to this site and caring so much to do so! I have a child with CF and only a few select friends have done anything to try to help me...other than write checks! Not saying that I don't appreciate this because I most certainly do, but I am talking about getting involved like you are. Some of my closest friends even had a mutual friend, who passed away from this disease, and they don't even send money for our letter writing campaign....sorry, just had to vent a little! Anyway, get as much info as you can and stay in contact with your friend...that will make a big difference. Knowing that someone cares means the WORLD!!! <img src="i/expressions/face-icon-small-happy.gif" border="0">
<img src="i/expressions/face-icon-small-blush.gif" border="0">ne of my oldest friends called me two hours ago to tell me that her 18 month old son has just had two positive sweat tests and that they are 99% certain that he has cf. I came looking for something unsure what really, so that when I go to the hospital, they've taken him in tonight for a week is that normal? and be able to help offer some comfort or be yelled at whatever she needs and I came across this site and saw your email and found lots of answers so thank youQ
NoDayButToday
New member
As of this week, the median life expectancy was upped to 34.5 years or something close to that. So yes, the doctor was making a guess, but an educated one. Come back with any questions<img src="i/expressions/face-icon-small-smile.gif" border="0">
I may have interpreted this wrong (very possible since I can't even spell interpreted correctly), but when they sent that notice out that the life expectancy is now 35.1 years, I believe it was for TODAY'S lives. The median age of everyone with CF who passed away this year was 35, so that would mean that for people born in 1970, the life expectancy was 35 years.
I took that as good news, because even though they can't say what the life expectancy will be for babies born today, those are statistics for people born when a lot of the advances such as TOBI hadn't been made. It's possible that the expectancy won't change, but doesn't it seem more likely that it will continue increasing?
Again, I may be way off mark, that was just how I read it.
-- Jenica
I took that as good news, because even though they can't say what the life expectancy will be for babies born today, those are statistics for people born when a lot of the advances such as TOBI hadn't been made. It's possible that the expectancy won't change, but doesn't it seem more likely that it will continue increasing?
Again, I may be way off mark, that was just how I read it.
-- Jenica
Jenica, I believe your explanation is correct, that those born in the 70's....
There are many people with CF who live a lot longer than those 35 years so I know it is difficult but please don't get wraped around a number. Everybody's CF is different, and this child may live to be 70-nobody really knows.
Take care and please come back with more questions,
Julie
There are many people with CF who live a lot longer than those 35 years so I know it is difficult but please don't get wraped around a number. Everybody's CF is different, and this child may live to be 70-nobody really knows.
Take care and please come back with more questions,
Julie
My friend is really not handling this well at ALL. She doesn't want to be around her son right now, and I understand her... not many do, but I do, and I just don't know what to say to her to help her through this. I think that she is just under so much shock and pressure right now that she is not really thinking properly. I hate this so much for her.
<img src="i/expressions/face-icon-small-sad.gif" border="0"> Amy
<img src="i/expressions/face-icon-small-sad.gif" border="0"> Amy
Amy, are you the initial poster? A few other's posted after you so I just want to make sure. Just be there for her right now, since you live so far away call her and talk to her on the phone. Can you take a few days off or a weekend or something to go and visit her and provide some one on one support right now? What do you mean by " she doesn't want to e around her son right now"? Is it because she is upset or because she is still in shock, or both? I know it's difficult but she needs to make sure that she also takes care of her son as much as possible. It may be beneficial for her to contact www.cff.org and get some some contacts for the area. It may help her to talk to other parents of children with CF and hear their experiences. Is she interested in coming here at all (if you've even mentioned it yet? I am just kind of thinking outloud right now.
Julie (wife to Mark 24 w/CF)
Julie (wife to Mark 24 w/CF)
Amy, I agree with Julie's last post in her suggestion to contact your friend's local CFF chapter. When our daughter was diagnosed, someone on this board gave me the same advice, and it was so helpful to my husband and I to actually put names and stories to this scary disease. I was contacted by three local families, two of which were moms of children with CF, and the third was a 44 year old woman who has CF herself.
It is such a difficult thing hearing this diagnosis, but I found talking to people who were living with it was very comforting. I heard about their treatments, their routines, and just as importantly, all of the "normal" day-to-day activities that went on in their houses. Their kids did physical therapy in the morning and at night, but they also played soccer and swam.
If you can help your friend see that she is not alone in this, I can imagine it would be hugely beneficial to her. It was a lot less scary for me when it stopped being this faceless disease that I had only read about on the internet.
Best wishes for you and your friend, and if she has questions, this board is a great place to go for answers.
-- Jenica
It is such a difficult thing hearing this diagnosis, but I found talking to people who were living with it was very comforting. I heard about their treatments, their routines, and just as importantly, all of the "normal" day-to-day activities that went on in their houses. Their kids did physical therapy in the morning and at night, but they also played soccer and swam.
If you can help your friend see that she is not alone in this, I can imagine it would be hugely beneficial to her. It was a lot less scary for me when it stopped being this faceless disease that I had only read about on the internet.
Best wishes for you and your friend, and if she has questions, this board is a great place to go for answers.
-- Jenica
Hi Amy - although I wanted to be around my daughter every second of every day when she was disanosed (obviously different than you friend who doesn't want to be with her son), I was afraid to look directly at her because I couldn't imagine what her life would be like. Every time I looked at her I saw a very healthy looking girl that I thought would end up being very sick...it was so hard for me! Again, I think that you are awesome (I posted a while ago saying that). PLEASE stick by her and just show her love! If you think she needs someone to talk to, please put your email address on here and I will get in touch with you and then eventually her! Hang in there <img src="i/expressions/face-icon-small-smile.gif" border="0">
My son was diagnosed 7 months ago (he was 4 months old at the time) and we still have days where we feel we are awakening from a nightmare. However, I can say that many days it is because he is doing so well that I still can't believe he has the disease. Once we digested the label of CF it took a while to find our way back to our son who had been through so many terrible things his first few months on earth. The "not-knowing" was agonizing, but knowing was just as challenging. My husband and I have felt every emotion there is since that day in the hospital -- guilt that we gave this to him, anger at God, relief for knowing we could help him get on track, hope for his future and how far the treatments and alternative therapies have come, resentment that we had to go through all of this (always the toughest one to admit), concern for our daughter and how her life was about to change, how are lives were about to change, overwhelmed by the doctors and the language they use, overwhelmed by the additional care he has required at times, scared to trust he is well and growing -- so many things to absorb.
I found sitting quietly before going to bed a great way to absorb my worries and ask for some help from within. I don't know how to explain it but it was usually the only quiet I had during the day and it seemed to help me sleep and wake up with a greater calmness. I think each of us has our way of getting through the stress of such a earth shattering event. It takes time, patience and tenderness with oneself. It takes asking for help with our homes and meals some days. I was totally unable to research information for my son because it would consume me with worry and fret. My husband on the otherhand felt very comforted by researching the internet and joining groups like this one. I just this month felt a need to connect to other families and women who have CF kids. I also just picked a private spot in the park to weep for all I feel we lost in his first year. I just sat there and allowed all the feelings that couldn't surface when I had to be brave for him come out. I was surprised with how deep and intense they were but I needed to let go. Your friend is going through alot. She will need space sometimes, she will need to draw close sometimes, other times she won't know what she needs. She will find her way back to her son and she will create a network of health care providers and helpers. SHe will also realize there is something greater than she ever imagined there for her, helping her find her way.
Everything is very fresh for us and I still have some bad days. But we are very hopeful for our sons future and we try very hard not to let the lable "CF" define his future too much. Please let her know she is welcome to email me or if she needs to talk on the phone I'd be happy to do that at well. We are all here for each other, she is not alone.
Jody
jody@meta4tech.com<img src="i/expressions/heart.gif" border="0">
I found sitting quietly before going to bed a great way to absorb my worries and ask for some help from within. I don't know how to explain it but it was usually the only quiet I had during the day and it seemed to help me sleep and wake up with a greater calmness. I think each of us has our way of getting through the stress of such a earth shattering event. It takes time, patience and tenderness with oneself. It takes asking for help with our homes and meals some days. I was totally unable to research information for my son because it would consume me with worry and fret. My husband on the otherhand felt very comforted by researching the internet and joining groups like this one. I just this month felt a need to connect to other families and women who have CF kids. I also just picked a private spot in the park to weep for all I feel we lost in his first year. I just sat there and allowed all the feelings that couldn't surface when I had to be brave for him come out. I was surprised with how deep and intense they were but I needed to let go. Your friend is going through alot. She will need space sometimes, she will need to draw close sometimes, other times she won't know what she needs. She will find her way back to her son and she will create a network of health care providers and helpers. SHe will also realize there is something greater than she ever imagined there for her, helping her find her way.
Everything is very fresh for us and I still have some bad days. But we are very hopeful for our sons future and we try very hard not to let the lable "CF" define his future too much. Please let her know she is welcome to email me or if she needs to talk on the phone I'd be happy to do that at well. We are all here for each other, she is not alone.
Jody
jody@meta4tech.com<img src="i/expressions/heart.gif" border="0">
CFHockeyMom
New member
"<b>She doesn't want to be around her son right now</b>". I'm sorry but I cannot for the life of me understand this attitude nor can I come up with any rationalization for your friend to feel this way. When my son was diagnosed (and I'm talking within seconds of the Dr. leaving the room), I was crying and my husband said to me, "It's going to be alright. It's not like we'll love him any less, right?". My response was (through the tears), "No, I'll love him more." Sure, it was tough. For the longest time (almost a full year after he was diagnosed), all I thought of when I saw him was CF. I couldn't see past it but those were rough times and he was pretty sick. Once his health turned around, CF took a back seat and we moved on.
She needs to pull her head out and grow up! Her son needs her now more than ever and if she thinks he can't sense her feelings toward him she's wrong!
She needs to pull her head out and grow up! Her son needs her now more than ever and if she thinks he can't sense her feelings toward him she's wrong!