My 12 year old son had a sweat test, and last Monday I received his result and it was 102. He has always had some respiratory issues. Before he was 3 he had pneumonia twice. He has never been hospitalized. The Dr just gave us antibiotics to give him at home and he recovered. He had colds more frequently than my two younger sons, and the cough would last longer. Really he has had a cough on and off most of his life. He does seem to have allergies also. As sometimes he will get watery eyes, and nasal congestion with cough and allergy medicine seems to help. At one point a Dr said he had asthma. When he was younger he would occasionally vomit when he was coughing and it'll now he does it sometimes in the mornings when he isn't coughing and it's mostly been clear mucus. He gets out of breath pretty easily, but I assumed it was from the "asthma." When I was pregnant with my second son I was told I was a carrier for cystic fibrosis. I didn't really think much of it at the time. I think I just assumed he has been screened for it also, but I can't remember for sure. The last couple of years he has had really huge bowel movements. I only know this because he would clog up or overflow the toilet at times. It was not diarrhea they were formed just very big. A couple of years ago he said he had some orange stuff in his poop, and I thought maybe it was something he ate. About a month ago he showed my husband his poop, because he thought he had blood in it. My husband took a picture and showed me and there was orange/reddish greasy looking substance in the toilet along with light colored bm. I was concerned so I made an appt with the doctor. I also did some research and one of the conditions that came up was cystic fibrosis. Two years ago my sister had a son and he came up abnormal on the newborn screen. He had a sweat test and turned out to be just a carrier. When I was doing research recently I realized that in 2004 Indiana was not doing cystic fibrosis screening as part of the newborn screen. They didn't start until 2007. I realized then that he should have a test to rule it out. We went to the doctor and the doctor was going to just give him a laxative for the gi problems, but I requested that they do a test for cf. He scheduled a sweat test so now here we are. We have an appt with an educator at a cf clinic on Wednesday and another appt with the specialist the week after. I feel horrible. I'm a nurse and I feel like I should have put this all together sooner. I'm so worried about what damage he may have from being diagnosed so late and not having treatments all his life. I'm not sure if these issues with his bowel movements mean that he is pancreatic insufficient. He has been pretty thin the last few years, but he has gained weight and seems to be normal height for his age. Although probably on the lower end of normal for weight. It's interesting when reading about the diet that he has mostly gravitated to a high salt and fat diet on his own, and he has never really liked sweets. He loves pretzels, beef jerky, and Gatorade. I've felt pretty devastated about all this, and I'm so worried about what this means for his life and his future. His symptoms seems pretty typical, although maybe more mild than what some people with this condition have in childhood. I did tell him about it, and I've told him that we will do whatever the Drs say he needs to do to stay healthy. He's very smart and I knew he would do research, so I told him to come to me if he sees anything that worries him. He did see soemthing about a man with cf having a lung transplant, and asked me if that happens a lot. I told him that they have come a long way with treatments just in the last few years, and that when the man was born 30 or so years ago treatments weren't as advanced. I also told him he may have a milder form than what some people have. I don't want him to be scared, but I don't want to lie to him about anything either. Anyways I'm sorry this is so long. I'm still trying to wrap my brain around all of this.
12 year old just diagnosed
- Thread starter Sunny9432
- Start date
I don't know if this is unusual or not, but as an infant and a toddler he was always in the 90 some percentile for height and weight. It wasn't until the last few years that he seems to eat like a horse, but stay very thin. I kept thinking maybe it's because I have always been pretty thin, and because he kept having growth spurts in height and his weight just hadn't caught up. I guess the pancreatic insufficiency can develop over time?
DS was diagnosed at birth due to meconium illeus. If he hadn't had the obstruction, I doubt he would have been diagnosed until later. Newborn screening wasn't until 2005 in our state. His symptoms were mainly digestive and sinus, plus his sweat test at 2 weeks was a normal 32. Without enzymes his stools are orange, very loose, bulky and leaves a greasy residue on the water line. He struggled maintaining 50% for weight, prefers salty snacks, eating salt... Sweats out a lot of salt. I have a friend whose husband was diagnosed at 14, he was having difficulty gaining weight. He has the same mutation as ds and in his mid 40s. DS takes enzymes, does vest and neb treatments to keep his lungs healthy, extra vitamins....
I was diagnosed late too at age 7 and that was considered late in 1992. I also had phantom symptoms that many doctors thought were just allergies and stomach issues. I'm sorry for this challenge your son has to face now. However, the treatments and medications coming out for CF patients are exciting and life changing. So it's a good time to be alive with CF. I can tell you that this forum is one of the best and everyone here is extremely supportive and can help with any of your questions. We're all in the fight together and we're all figuring things out together. Don't beat yourself up. Whatever questions you or your son has we will do the best to help you figure it out or get you the information you need.
Sunny:
Don't beat yourself, most Doctors can't spell CF much less diagnose or treat it. Unless you were a nurse on a CF floor you would not have enough knowledge to suspect it in your son.
There are almost 2000 known mutations, any 2 will cause CF. 2000 X 2000= 4,000,000 different "strains" of CF. This is not a class in Nursing School.
I am 77. Had a very active childhood. I played college basketball (not well but I played). I was a Lifeguard for the Commonwealth of Massachusetts on an Atlantic Ocean beach, for five summers. I have 2 college degrees and I have been married for 54 years. Most of this was without CF treatment.
Given all of the current drugs and treatments, your son will live a long and full filling life.
I am not the oldest CF patient in my clinic nor am I close to the oldest living CF patient now living.
God Bless you and your son.
Bill
Don't beat yourself, most Doctors can't spell CF much less diagnose or treat it. Unless you were a nurse on a CF floor you would not have enough knowledge to suspect it in your son.
There are almost 2000 known mutations, any 2 will cause CF. 2000 X 2000= 4,000,000 different "strains" of CF. This is not a class in Nursing School.
I am 77. Had a very active childhood. I played college basketball (not well but I played). I was a Lifeguard for the Commonwealth of Massachusetts on an Atlantic Ocean beach, for five summers. I have 2 college degrees and I have been married for 54 years. Most of this was without CF treatment.
Given all of the current drugs and treatments, your son will live a long and full filling life.
I am not the oldest CF patient in my clinic nor am I close to the oldest living CF patient now living.
God Bless you and your son.
Bill
LittleLab4CF
Super Moderator
Sunny9432,
Forgive yourself for taking 12 years to arrive at a diagnosis. As Printer noted, you will discover people on this site who weren't diagnosed until 30, 40, 50 even 74, and not just a few anomalies. Hindsight is great if you want to catalog all of the CF symptoms in retrospect, but I am plenty smart, I have smart doctors and as a nurse, you should know better than most that the story of the blind men and the elephant is endemic in medicine.
I was diagnosed by sweat test in 2001 at age 51. After that I had genetic testing and was declared Atypical CF because my lungs still worked and they found just one CFTR (Cystic Fibrosis Transmembrane ((electrolyte conductance)) Regulator) gene mutation. My brand new CF specialist looked over my genetic assay and said "so, you're just a carrier? It wasn't a happy moment, I didn't want CF, but I had been battling "The worst case of whatever you have,... that we've seen" diagnosis since I could remember. I assumed that I had inherited a number of bad genes for most of my life.
Taking the approach of seeing the appropriate specialist for each individual problem works for the most part. Big things fell through the cracks like malnutrition, malabsorption and the involvement of my pancreas and gut, common in CF but uncommon in the general population. Discovering that I was suffering the diseases of malnutrition/malabsorption for fifty years, osteoporosis, arthritis and such, was a shock. My CF diagnosis is still atypical because I do not have penetration of CF into my lungs. Just a guess, but your son may be the same. I have had enough post nasal drip to choke on, and a common occurrence is aspirating that thick, infected goo. I aspirate food as well and a night time aspiration gives me pneumonia too frequently. Still, this is an inconvenience, I don't require daily nebulizing, use a vibratory vest or anything except when I am kicking a round of pneumonia.
My old GI doctor always noted a mess of mucus in my stomach whenever I had an upper endoscopy. The average person swallows a liter of mucus daily. CFers make at least as much but the viscosity and potential infection swallowed is enough to make one sick. The stomach can't easily pass the thick mucus so it's always mushing it around. Vomiting is more common with CF, sometimes by a lot. Just keep a tighter schedule of having him scoped from both ends. With kids, that is likely zero, but esophegeal cancer, pancreatic and liver cancer are more common. I am not wanting to alarm you, if any kid is vomiting weekly or more, it's important to watch it. These are rare cancers for younger CFers, not much more than a healthy child, depending on the severity of the CF. It is a good thing for him to remember as he takes charge of his own medical care.
My poor mother, actually both parents, but my mother was playing medical detective from 1950 on. I had all the usual head and chest infections up until about the age of your son. The fact that your son feared blood in the stool probably means that he too has become a close observer of his BM's. This will serve him well because bowel nirvana is a moving target with CF. His lungs are of paramount importance as is getting established with a good CF Center. This usually means contacting the CF Foundation (CFF.org) to find the closest accredited CF clinic if you aren't happy with your clinic. Being a juvenile, he has a better chance of finding a clinic. Adult diagnosed CFers used to be rare. As a geneticist, I am fairly certain that the 35,000 or so CF patients in the U.S. is probably off by 200,000 or so. Time will tell.
To compare life for a sick kid in the 1950's or before those darling ear tubes they put in kids prone to ear infection, they had to wait until you either blew an ear drum or. God help me, they lanced the infected ear drums. I endured this exactly twice. The first time, at about age 6, the doctor put most of his upper body into holding me down and then BOOM!!! I was up in the exam room cabinets, and nothing or nobody was getting me down. My doctor was a huge 6'4" man but it took him, my mother and two other people to pry me out of the furniture and lay me down for a repeat performance on the other ear.
By your son's age, it was apparent that I was becoming the medical miniature of my father, probably a CF patient himself. He had ulcers, I had gastritis, he was seriously constipated, I was constipated and so on. I was even taking the same medicines as my father, in lower dosages. If my life was no more complicated than constipation and the habit of catching every respiratory bug going around, I might complain, but it would be livable.
Food makes me sick. It made me sick as a child and it makes me sick today. I believe one of my attractions to the "Lost in Space" science fiction world was the idea of swallowing a few pills and nutrition be satisfied was quite compelling. By my teens, like most boys I was devouring anything food. We had built a calorimeter in science class large enough to place a meal in. I was devouring 3000-5000 Calories daily, fairly healthy food for the most part. From as young as I can remember I won the skinniest kid in the class award. Not really aware of the mark on my back for budding bullies, I amazed my fellow kids with a strong flashlight, glowing through the flesh in my hand, arm or whatever, revealing moving bones just like a fluorescope. The fact that your son has a particular stool pattern, my guess is he is going to present GI dominance in his CF. I hope. People underestimate how debilitating a dysfunctional gut can be. As a nurse, you know already that the gut is the center of our immune system, and that gut feeling is more than an analogy. Our emotional health and psyche can create calm or chaos in the gut. It has the same effect the other way, IMHO. As he builds a CF team, a counselor might become and important asset, for now he has you, and you might need to offload your fears and worries, since your normal support group is usually involved family.
His love of salt is good. Although the usual salt is important, CFers really need the entire electrolyte series to be properly balanced. Super hydration for a normal kid is what should be his norm. I hate drinking water, I don't know if this is my own aversion but beware, most of my life I was chronically dehydrated, if it is a CF thing, and it could be, kidney stones will eventually challenge that assumption, and habit. I had more than I can count, seriously. It would be like forgetting how many kids you gave birth to, but I used to produce "gravel" and I still couldn't get past proper hydration. They were so frequent, when gallstone attacks were starting to happen, my doctor kept looking for the wrong kind of stone. Why wouldn't he, I am not FFF rather male, thin and in my thirties. You need better advice concerning his gallbladder, just be aware, it may be sluggish and could be contributing to his odd stools. Mine was simply removed. I think things are different now for kids.
A recent scientific article, possibly in "GUT", found a correlation between very high sweat test numbers and GI dominance in CF. Your son rung the bell at 105. I imagine your son's new doctor has said something about supplemental enzymes. I can't emphasize enough, become an expert on enzymes. The process is dose titration and in my limited experience, it is extremely difficult for a doctor or nutritionist to wrap their brains around adequate enzymes without personally having CF. There is a fecal elastase test that can determine approximately how good or bad digestion is. It is based on the patient consuming at least 100g of fat/day for an adult, there may be a pediatric chart, but when I had my most important test, they failed to ask if I consumed anything close to 4 ounces of fat daily. I didn't.
Big numbers are scary and Creon 12, for example has 12,000IU of Lipase and similar numbers on the other included enzymes. Don't be put off by the numbers, these are enzymes digesting meals. The fact that his stools are toilet stoppers can be superficially handled by having him flush frequently, assuming he isn't depositing a softball sized stool in one move. You mention hard and voluminous formed stools, not diarrhea. It sounds like you are implying they are at least pliant stools with the reference to diarrhea. As the doctor adjusts his enzyme routine, get your son comfortable about discussing the quality of his BM. Enzymes at first are like manna from heaven, but it might not last at the initial level. His stool volume, the odor and embarrassing overflows almost certainly will be helped with supplemental enzymes.
To understand the potential problems with maldigestion, which of course means malabsorption, the fecal volume will be reduced by supplemental enzymes digesting the meal much better. Nutrients are absorbed and used by your son rather than surrendering them to probiotics in the gut, which are little gas factories making his stools larger. From this point, I am going to refer you to another current post under Adults on this site titled DIOS. I wrote more than you need to know about enzymes in my piece of that thread. I don't mean to be lazy, but if you want information, there's plenty on this site.
As for your child's life, Printer's life seems to be more common than not. Maybe it is all the stress hormones fighting infections and inefficiencies called to overwork our immune system and what's left spins in the brain, or the phase of the moon when we were all born, but something makes CFers fighters, and often exceeding their wildest dreams. I certainly have.
LL
Forgive yourself for taking 12 years to arrive at a diagnosis. As Printer noted, you will discover people on this site who weren't diagnosed until 30, 40, 50 even 74, and not just a few anomalies. Hindsight is great if you want to catalog all of the CF symptoms in retrospect, but I am plenty smart, I have smart doctors and as a nurse, you should know better than most that the story of the blind men and the elephant is endemic in medicine.
I was diagnosed by sweat test in 2001 at age 51. After that I had genetic testing and was declared Atypical CF because my lungs still worked and they found just one CFTR (Cystic Fibrosis Transmembrane ((electrolyte conductance)) Regulator) gene mutation. My brand new CF specialist looked over my genetic assay and said "so, you're just a carrier? It wasn't a happy moment, I didn't want CF, but I had been battling "The worst case of whatever you have,... that we've seen" diagnosis since I could remember. I assumed that I had inherited a number of bad genes for most of my life.
Taking the approach of seeing the appropriate specialist for each individual problem works for the most part. Big things fell through the cracks like malnutrition, malabsorption and the involvement of my pancreas and gut, common in CF but uncommon in the general population. Discovering that I was suffering the diseases of malnutrition/malabsorption for fifty years, osteoporosis, arthritis and such, was a shock. My CF diagnosis is still atypical because I do not have penetration of CF into my lungs. Just a guess, but your son may be the same. I have had enough post nasal drip to choke on, and a common occurrence is aspirating that thick, infected goo. I aspirate food as well and a night time aspiration gives me pneumonia too frequently. Still, this is an inconvenience, I don't require daily nebulizing, use a vibratory vest or anything except when I am kicking a round of pneumonia.
My old GI doctor always noted a mess of mucus in my stomach whenever I had an upper endoscopy. The average person swallows a liter of mucus daily. CFers make at least as much but the viscosity and potential infection swallowed is enough to make one sick. The stomach can't easily pass the thick mucus so it's always mushing it around. Vomiting is more common with CF, sometimes by a lot. Just keep a tighter schedule of having him scoped from both ends. With kids, that is likely zero, but esophegeal cancer, pancreatic and liver cancer are more common. I am not wanting to alarm you, if any kid is vomiting weekly or more, it's important to watch it. These are rare cancers for younger CFers, not much more than a healthy child, depending on the severity of the CF. It is a good thing for him to remember as he takes charge of his own medical care.
My poor mother, actually both parents, but my mother was playing medical detective from 1950 on. I had all the usual head and chest infections up until about the age of your son. The fact that your son feared blood in the stool probably means that he too has become a close observer of his BM's. This will serve him well because bowel nirvana is a moving target with CF. His lungs are of paramount importance as is getting established with a good CF Center. This usually means contacting the CF Foundation (CFF.org) to find the closest accredited CF clinic if you aren't happy with your clinic. Being a juvenile, he has a better chance of finding a clinic. Adult diagnosed CFers used to be rare. As a geneticist, I am fairly certain that the 35,000 or so CF patients in the U.S. is probably off by 200,000 or so. Time will tell.
To compare life for a sick kid in the 1950's or before those darling ear tubes they put in kids prone to ear infection, they had to wait until you either blew an ear drum or. God help me, they lanced the infected ear drums. I endured this exactly twice. The first time, at about age 6, the doctor put most of his upper body into holding me down and then BOOM!!! I was up in the exam room cabinets, and nothing or nobody was getting me down. My doctor was a huge 6'4" man but it took him, my mother and two other people to pry me out of the furniture and lay me down for a repeat performance on the other ear.
By your son's age, it was apparent that I was becoming the medical miniature of my father, probably a CF patient himself. He had ulcers, I had gastritis, he was seriously constipated, I was constipated and so on. I was even taking the same medicines as my father, in lower dosages. If my life was no more complicated than constipation and the habit of catching every respiratory bug going around, I might complain, but it would be livable.
Food makes me sick. It made me sick as a child and it makes me sick today. I believe one of my attractions to the "Lost in Space" science fiction world was the idea of swallowing a few pills and nutrition be satisfied was quite compelling. By my teens, like most boys I was devouring anything food. We had built a calorimeter in science class large enough to place a meal in. I was devouring 3000-5000 Calories daily, fairly healthy food for the most part. From as young as I can remember I won the skinniest kid in the class award. Not really aware of the mark on my back for budding bullies, I amazed my fellow kids with a strong flashlight, glowing through the flesh in my hand, arm or whatever, revealing moving bones just like a fluorescope. The fact that your son has a particular stool pattern, my guess is he is going to present GI dominance in his CF. I hope. People underestimate how debilitating a dysfunctional gut can be. As a nurse, you know already that the gut is the center of our immune system, and that gut feeling is more than an analogy. Our emotional health and psyche can create calm or chaos in the gut. It has the same effect the other way, IMHO. As he builds a CF team, a counselor might become and important asset, for now he has you, and you might need to offload your fears and worries, since your normal support group is usually involved family.
His love of salt is good. Although the usual salt is important, CFers really need the entire electrolyte series to be properly balanced. Super hydration for a normal kid is what should be his norm. I hate drinking water, I don't know if this is my own aversion but beware, most of my life I was chronically dehydrated, if it is a CF thing, and it could be, kidney stones will eventually challenge that assumption, and habit. I had more than I can count, seriously. It would be like forgetting how many kids you gave birth to, but I used to produce "gravel" and I still couldn't get past proper hydration. They were so frequent, when gallstone attacks were starting to happen, my doctor kept looking for the wrong kind of stone. Why wouldn't he, I am not FFF rather male, thin and in my thirties. You need better advice concerning his gallbladder, just be aware, it may be sluggish and could be contributing to his odd stools. Mine was simply removed. I think things are different now for kids.
A recent scientific article, possibly in "GUT", found a correlation between very high sweat test numbers and GI dominance in CF. Your son rung the bell at 105. I imagine your son's new doctor has said something about supplemental enzymes. I can't emphasize enough, become an expert on enzymes. The process is dose titration and in my limited experience, it is extremely difficult for a doctor or nutritionist to wrap their brains around adequate enzymes without personally having CF. There is a fecal elastase test that can determine approximately how good or bad digestion is. It is based on the patient consuming at least 100g of fat/day for an adult, there may be a pediatric chart, but when I had my most important test, they failed to ask if I consumed anything close to 4 ounces of fat daily. I didn't.
Big numbers are scary and Creon 12, for example has 12,000IU of Lipase and similar numbers on the other included enzymes. Don't be put off by the numbers, these are enzymes digesting meals. The fact that his stools are toilet stoppers can be superficially handled by having him flush frequently, assuming he isn't depositing a softball sized stool in one move. You mention hard and voluminous formed stools, not diarrhea. It sounds like you are implying they are at least pliant stools with the reference to diarrhea. As the doctor adjusts his enzyme routine, get your son comfortable about discussing the quality of his BM. Enzymes at first are like manna from heaven, but it might not last at the initial level. His stool volume, the odor and embarrassing overflows almost certainly will be helped with supplemental enzymes.
To understand the potential problems with maldigestion, which of course means malabsorption, the fecal volume will be reduced by supplemental enzymes digesting the meal much better. Nutrients are absorbed and used by your son rather than surrendering them to probiotics in the gut, which are little gas factories making his stools larger. From this point, I am going to refer you to another current post under Adults on this site titled DIOS. I wrote more than you need to know about enzymes in my piece of that thread. I don't mean to be lazy, but if you want information, there's plenty on this site.
As for your child's life, Printer's life seems to be more common than not. Maybe it is all the stress hormones fighting infections and inefficiencies called to overwork our immune system and what's left spins in the brain, or the phase of the moon when we were all born, but something makes CFers fighters, and often exceeding their wildest dreams. I certainly have.
LL
Last edited:
Thank you all for your encouragement! I know I shouldn't beat myself up, but guilt and worry seem to come with parenthood for me at least even without throwing in a chronic genetic disease. We had our first appointment at the CF clinic on Wednesday. I had already looked up accredited clinics on the Cystic Fibrosis Foundation website and found one, and that is the one we had been referred to. Lucky for us it is less than 15 minutes from our house, and they see children and adults. They ordered a fecal elastase test and should have the results in a couple days, but they gave us enzymes to start after the sample was obtained. I'm pretty sure he is pancreatic insufficient. He was 25 percentile for weight and 50 for height. And when he was younger was always 90 some percentile for both. They want him to gain 7 lbs as his bmi is 16 right now, so hopefully enzymes will help with that. I found out my mutation is f508del. He will be having testing soon to find out what mutation he got from his father. They took sputum for a culture so we are waiting on that also. He has an appointment next week with the doctor. The one this week was more of a meet and greet and information session. They are going to do pft I think and start pulmozyne and hypertonic saline at his appt next week, and probably a vest soon. So after all that I guess we just need to get into a routine. Luckily for me he is a very good and smart boy and is ready to follow his treatments to stay healthy. Apparently they have problems with compliance in teenagers but I don't think if will be an issue with him. Now if it was my middle son it my be a different story. So again thanks for the encouragement and the information.
My son started coughing up blood today. He's done a good bit of coughing in his life, and he's never coughed up blood before today. It about gave me a heart attack. It wasn't just pink mucus either. It was almost completely bright red blood. I took him to the ER and they all seemed surprised that he had just been diagnosed. The nurse even asked if he had a sweat test like she didn't believe it. None of them seemed all that concerned about the coughing up blood either. The doctor said his lungs sounded clear, but ordered a chest X-ray and came back saying he had early pneumonia. He started him on two antibiotics. He hasn't had pneumonia since he was two, so this has me really worried that he's getting worse all of the sudden or that the disease is progressing. The doctor said his lungs looks grainy on the X-ray and that that is typical for cf, but I'm not sure what that means. He started taking his enzymes and it's a little confusing how often he should take them. He takes 3 with meals and 1 with snacks. I think if he's eating more than an hour apart he's supposed to take them again. I just don't want him to take more than he needs. I really wish there was a cure for this or at least something to make him healthy and not need all these treatments and pills. He's suddenly going from no pills a day to like 16 with the antibiotics.
logansmom09
New member
Your son's story is identical to my son's story, so I'll give you run down of how things will go for you both (I say with tongue in cheek, because as parents, we want to KNOW what will happen, but every child is different and encounters different things). Logan was diagnosed at 12 also. Always had a cough, doctors said asthma, asthma, asthma...bowel movements were always huge. Large at birth (almost 10 pounds), high percentile in height and weight which declined steadily over time. For the most part, he was really healthy. In elementary school one year, he even got a perfect attendance award. When updating his "asthma" medication, we were walking out the door when the doctor noticed his large fingers- the clubbing, and sent us for a test. Eureka! An answer (with 10,000 additional questions)! He was never hospitalized until he was 16, but had a few rounds of heavy antibiotics each year. Fast forward....he is 20 now, in college making great grades, having a blast in a fraternity, just got back from Spring Break where he was buff and tan and taking care of himself like he should. The trick? He learned he could control his health to a certain extent: he FEELS it when he doesn't do his treatments, so he DOES THEM (yeah, all teenagers skip because there are things they'd rather be doing. But eventually they realize that feeling good allows them to do more). Second tip- he exercises ALOT. Like, he's almost a gym rat. If it's basketball, track, soccer, Ultimate Frisbee, whatever- cardio is king, and keeps them healthy. So if your story falls in line with ours, then yes, I would take it as a good sign that he hasn't been SO sick that he needed more intense treatment until now. I think you're probably safe believing his condition isn't as bad as some others. I know- things can always change for anyone at any time, including my own son, but I just want to give you another mother's stamp of approval that it's okay not to assume the worst. Treatments have improved so much just since Logan was diagnosed (8 years). They are continuing to improve. My problem now is convincing Logan that he's going to live long enough that it wouldn't be a waste to start a retirement fund. Best wishes to you- let him know he has a LOT of control over this, but he has to choose it.
AussieinTexas
New member
First thing I suggest is lose the pill paranoia. CF is ALL pills and treatments. I was of the same thinking as you, as soon as I accepted that my grandson NEEDED all the pills and treatments to stay healthy I was able to be more supportive. You sound panicked and that only leads to us not taking everything in. It's a one step at a time thing, you'll find shortcuts (for lack of a better word) to suit your lifestyle as you go. Right now you need to breathe, start writing it all down and get a routine in order that suits you both. It's going to be ok I promise. You just do it, every day, so it's a new lifestyle. My grandson was diagnosed at birth but I remember how I felt when everything became real ... we do our best to keep the kids healthy. You are going to be very proud of your son when he starts owning his care routine, but he needs your help now. My grandson is 7, he knows more about CF than most of the nurses we meet and that's not that they are bad nurses, many just don't come across CF often, if ever. It's going to be ok, don't panic, don't let it all overwhelm you. It's not a pill a day or antibiotic dose passing bug, it's a constantly changing, no two alike, day to day routine. You've got this!
Katherine H.
New member
The amount of enzyme is based on each patient. My daughter takes 36,000 creon. She takes 3 with meals depending on the amount of fat and 2 with snack. IF very fatty the meal she takes 4. My daughter has Severe Pancreatic insufficiency.
My daughter was diagnosed at 18. Due to nonclassical presentation. The doctors would dismiss symptoms instead of accept it. They spent more time disproving it. She has to take pills for deficiency in fat soluble vitamins. She also takes stool softener. I know there are many questions. It just takes time to learn. There are many that can help.
LittleLab4CF
Super Moderator
The large amount of blood he's been coughing up are very likely hemoptysis. My first incident of hemoptysis was a surprise. I can't remember if I caught the first one or maybe the next couple of blood clots, but I was in my fifties and not easily terrified. They are huge in terms of blood clots, enormous for coughing it up. Mine were 4cc and 11cc respectively the first time. If it's not your blood, especially when it is your son's blood, that's a lot!
Your son may have bronchiectasis, common in CF and often the source of big bright red blood clots. Another source is pleural effusion. It seems similar to pleurisy, it's an infection between the pleural membranes, but they are often painless themselves. There's a correlation between bronchiectasis and pleural effusion when it comes to hemoptysis. The fact that the ED doctor didn't get excited about the huge blood clot, possibly with fresh arterial blood spraying for a while afterwards, almost guarantees it was a hemoptysis. I'm not saying that they can't be serious, but serious would be a bleed that isn't stopping. Excepting CF athletes who are accustomed to a blood mist during exercise, a bleed that doesn't stop soon is reason to call your CF specialist NOW, or get to the ER/ED.
His grainy Xray was likely reference to "ground glass" scarring. The term has nothing to do with ground glass, it's a description of how the lungs look on Xray. Because of the many lung infections at a young age, we often develop a grainy or sort of a grey patina image of the lungs.
You know that your son didn't just suddenly show symptoms of CF. The disbelieving nurse is one you should be able to relate to. Many years ago I was at the Milwaukee Circus Parade. It was a bright sunny July day, I was wearing my SPF 90 sunscreen and sunglasses with red tinted Polaroid lenses. I prefer the color and it gives me the super power of seeing skin capillaries and the early onset of sunburn. A lady next to me had a newborn baby without any sun protection. I was alarmed by the extent of the infant's sunburn, lowered my glasses so I could have an unbiased look at the baby and her sunburn, she was obviously sunburned, I could see the difference between her skin directly in the sun and the areas protected by some shade. I told her that she needed to apply some sunscreen, cover the baby or something like that. She retorted "oh, she doesn't burn, she tans!"
My expression must have spoken volumes because she moved back from the curb, prime viewing real estate and a few minutes later the newborn, who's tan must have washed away at birth, was slathered in sunscreen. I unwittingly embarrassed her. "Bad mom" fear is invisible to all except other moms. I'm not a neonatal expert, by a long shot but I knew her baby's first sun tan/burn was probably just happening. The nurse may have not believed you and was offering you another run at it. I wouldn't take offense, people who have been dealing with CF for a long time know that most medical professionals have very little knowledge of CF, the general public knows even less. The fact you knew what a sweat chloride test was, and he had already tested positive is frankly very impressive.
Please, do whatever you need to balance the guilt associated with the hereditary nature of your son's CF. He has no clue what life would be like without CF and you can only imagine what he's going through. A good balance of guilt would be zero or no guilt, IMHO. At the same time, my best friend is the mother of four children and the two boys have autism, one with an added dose of Asperger's. Chris is still suffering personal recriminations over 'something she didn't do, did do or should have done'. Her grief is hard to reconcile and occasionally it breaks through her shell. It angers me. Not because she is being overly dramatic or irrational but because my knowledge of genetics eliminates any chance that something you or even Chris did, didn't, or should have done would make an iota of difference. See a counselor, someone who should be part of your CF team for you and when it's appropriate, for him. Your seeing a counselor will help you help him and he will accept counseling easier if he sees that you are benefiting from it.
I mentioned a post I made under the title DIOS. I explained enzyme titration. If the doctor has him on say Creon 12's. The number of capsules taken with a meal is already approximated in the directions, hopefully. Actually, if you don't mind, post the prescription directions, enzyme supplement name and strength. Typically doctors try to make it such that 3-5 capsules are taken with a meal and 1-2 with a snack. I try to take mine right before I begin eating, so I don't forget and Creon takes 2 hours to become effective. They're enteric coated. From the initial dose, follow his poop. It's probably hard for lack of enzymes prior to diagnosis. From here, refer to my write up on DIOS to adjust his enzymes. Generally more fat, nut, milk, or meat fat, you might want to do 5 capsules assuming you are suggested to give him 3-5 with a meal.
I hope this helps answer your questions, soon enough you'll be an accidental authority on CF.
LL
P.S. Like KatherineH's daughter, I am on Creon 36, and it is the highest single dosage made by Creon. I am on the same dose regiment.
Your son may have bronchiectasis, common in CF and often the source of big bright red blood clots. Another source is pleural effusion. It seems similar to pleurisy, it's an infection between the pleural membranes, but they are often painless themselves. There's a correlation between bronchiectasis and pleural effusion when it comes to hemoptysis. The fact that the ED doctor didn't get excited about the huge blood clot, possibly with fresh arterial blood spraying for a while afterwards, almost guarantees it was a hemoptysis. I'm not saying that they can't be serious, but serious would be a bleed that isn't stopping. Excepting CF athletes who are accustomed to a blood mist during exercise, a bleed that doesn't stop soon is reason to call your CF specialist NOW, or get to the ER/ED.
His grainy Xray was likely reference to "ground glass" scarring. The term has nothing to do with ground glass, it's a description of how the lungs look on Xray. Because of the many lung infections at a young age, we often develop a grainy or sort of a grey patina image of the lungs.
You know that your son didn't just suddenly show symptoms of CF. The disbelieving nurse is one you should be able to relate to. Many years ago I was at the Milwaukee Circus Parade. It was a bright sunny July day, I was wearing my SPF 90 sunscreen and sunglasses with red tinted Polaroid lenses. I prefer the color and it gives me the super power of seeing skin capillaries and the early onset of sunburn. A lady next to me had a newborn baby without any sun protection. I was alarmed by the extent of the infant's sunburn, lowered my glasses so I could have an unbiased look at the baby and her sunburn, she was obviously sunburned, I could see the difference between her skin directly in the sun and the areas protected by some shade. I told her that she needed to apply some sunscreen, cover the baby or something like that. She retorted "oh, she doesn't burn, she tans!"
My expression must have spoken volumes because she moved back from the curb, prime viewing real estate and a few minutes later the newborn, who's tan must have washed away at birth, was slathered in sunscreen. I unwittingly embarrassed her. "Bad mom" fear is invisible to all except other moms. I'm not a neonatal expert, by a long shot but I knew her baby's first sun tan/burn was probably just happening. The nurse may have not believed you and was offering you another run at it. I wouldn't take offense, people who have been dealing with CF for a long time know that most medical professionals have very little knowledge of CF, the general public knows even less. The fact you knew what a sweat chloride test was, and he had already tested positive is frankly very impressive.
Please, do whatever you need to balance the guilt associated with the hereditary nature of your son's CF. He has no clue what life would be like without CF and you can only imagine what he's going through. A good balance of guilt would be zero or no guilt, IMHO. At the same time, my best friend is the mother of four children and the two boys have autism, one with an added dose of Asperger's. Chris is still suffering personal recriminations over 'something she didn't do, did do or should have done'. Her grief is hard to reconcile and occasionally it breaks through her shell. It angers me. Not because she is being overly dramatic or irrational but because my knowledge of genetics eliminates any chance that something you or even Chris did, didn't, or should have done would make an iota of difference. See a counselor, someone who should be part of your CF team for you and when it's appropriate, for him. Your seeing a counselor will help you help him and he will accept counseling easier if he sees that you are benefiting from it.
I mentioned a post I made under the title DIOS. I explained enzyme titration. If the doctor has him on say Creon 12's. The number of capsules taken with a meal is already approximated in the directions, hopefully. Actually, if you don't mind, post the prescription directions, enzyme supplement name and strength. Typically doctors try to make it such that 3-5 capsules are taken with a meal and 1-2 with a snack. I try to take mine right before I begin eating, so I don't forget and Creon takes 2 hours to become effective. They're enteric coated. From the initial dose, follow his poop. It's probably hard for lack of enzymes prior to diagnosis. From here, refer to my write up on DIOS to adjust his enzymes. Generally more fat, nut, milk, or meat fat, you might want to do 5 capsules assuming you are suggested to give him 3-5 with a meal.
I hope this helps answer your questions, soon enough you'll be an accidental authority on CF.
LL
P.S. Like KatherineH's daughter, I am on Creon 36, and it is the highest single dosage made by Creon. I am on the same dose regiment.
Last edited:
I know he needs the treatments and the pills. I'm not being pill paranoid. I'm a nurse though, and I know that it's better to take the lowest dose of a medication that is effective. All medications come with some cost. It's all about weighing benefit vs risk. Even the pamphlets for the enzymes say to take the lowest effective dose, because higher doses can cause some serious issues. It is a big adjustment though for a 12 year old to go their whole life taking no medications except for the occasional allergy pill, Tylenol, or an antibiotic every few years to taking 16 or more pills in a day. Most of the elderly people I see don't even take that many medications. Thanks for the encouragement. It's been an overwhelming couple of weeks. I wish none of these kids or adults had to do all of this, but luckily he is being very compliant and knows that he has to do it to be healthy.
I looked it up as soon as he started coughing blood and saw that it usually means a person with cf is getting an infection. There wasn't any clots just bright red blood with a little mucus. My Mom said that's it's crazy for this to happen for the first time right after he is diagnosed, but I guess really his symptoms getting worse are why he got the diagnosis in the first place. I'm a hospice nurse, and even most of the eng stage COPD and Lung cancer patients I see don't cough up blood like that, so it was definitely concerning for me. I don't feel guilty about passing on my genes to him. I didn't even know that I was a carrier at that time or anything about CF. I've felt guilty about not putting it all together sooner so he could be diagnosed, especially after finding out that I am a carrier years ago. I don't know why I didn't, but I also don't remember the doctors office saying too much about it other than telling me that I am. Which seems strange to me. I guess at least I finally did figure it out, or he would be here just taking Miralax and probably losing more weight. Both of my younger sons should have been screened with newborn screen at birth, so I think they aren't even carriers. Although they have a different Dad than my oldest son with CF. They started him on Zenpep with 15000 Lipase. They said to take 3 with meals and 1 with snacks. It can be confusing because the last few years the kid has ate nonstop, so it's hard to know when to take the capsule again. None of us could figure out how he could possibly still be hungry or where all this food was even going. Now I know that his body was hungry, because he wasn't getting what he needed out of his food. We weighed him Saturday morning and he weighed 89 lbs. We weighed him again last night and he already weighed 92 lbs. That's just amazing 3 lbs in 3 days. He had set a goal for himself to gain 2 lbs by the end of the month, so he was happy that he already achieved that. I'm trying to learn all I can about CF. Ive always been interested and wanted to learn about anything to do with how the body works, but I want to know everything I can to help him. I think the nurse was surprised, because he doesn't look unhealthy and most people these days are diagnosed at birth. She made a comment about how he must have been doing well. Well yes and no. Probably better than some people with CF in childhood, but from what I've read its not that unusual for their symptoms to get worse as they become teenagers.
It is overwhelming and just take it one step at a time, one day at a time. Get into a routine and eventually enzymes, treatments will become second nature. I have a friend who just had a baby w/out CF and she and her husband panicked the first few weeks because they kept thinking they forgot to give the baby enzymes. That whole mindset of being so used to giving the other child wcf enzymes.
When DS was diagnosed, he was a baby, still in the NICU and the RT's taught us CPT (Chest physiotherapy) and how to use a nebulizer, dispense albuterol. We were taught how to give a newborn enzymes. Then we learned how to give him liquid vitamins twice a day. And the day we were released we got --- BTW, here's medication to help his liver to be given three times a day, here's a prophylactic antibiotic to be given 4 times a day, he'll need extra vitamin A & E.... And once again I was soooo overwhelmed. Any time a new medication is added, it throws me for a loop because I feel like I just got everything down, just got organized. It's not that he's necessarily progressing, but at age 2 1/2 he was put on azithromycin -- not as an abx, but it helps with lung function. Cultured a new bug --- prescribed another abx.... I wrote a lot of things down at first, have a table of medications on my computer. To figure out treatments, I calculated what time we needed to leave for school/work and how long it'll take to get ready, eat, etc. and work backwards. It's second nature, can do it in my sleep and probably have.
My MIL is a retired nursing instructor and she takes copious notes, double/triple checks what she's given DS -- but she still has issues with the amount of pills. Enzymes in particular --- the bottle says 8-10 creon with meals; however, she doesn't want to give him too many although he can easily eat a TON of calories and fat with a snack and go on to eat steak and a double order of fries for supper and hour or so later, topped off with a bucket of popcorn before bed. It's mind boggling. Her mindset is not to give him too many pills, but he's so pancreatic insufficient that he needs more enzymes depending upon what he eats.
The enzyme issue drove me nuts. Early on it was based on output and as a new parent, I didn't have a clue as to what normal poop looked like. I was told with a mysterious smile "you'll know". No, I don't know, that's why I'm asking. Throw in a predigested formula --- apparently grass green well formed poop is normal.
When DS was diagnosed, he was a baby, still in the NICU and the RT's taught us CPT (Chest physiotherapy) and how to use a nebulizer, dispense albuterol. We were taught how to give a newborn enzymes. Then we learned how to give him liquid vitamins twice a day. And the day we were released we got --- BTW, here's medication to help his liver to be given three times a day, here's a prophylactic antibiotic to be given 4 times a day, he'll need extra vitamin A & E.... And once again I was soooo overwhelmed. Any time a new medication is added, it throws me for a loop because I feel like I just got everything down, just got organized. It's not that he's necessarily progressing, but at age 2 1/2 he was put on azithromycin -- not as an abx, but it helps with lung function. Cultured a new bug --- prescribed another abx.... I wrote a lot of things down at first, have a table of medications on my computer. To figure out treatments, I calculated what time we needed to leave for school/work and how long it'll take to get ready, eat, etc. and work backwards. It's second nature, can do it in my sleep and probably have.
My MIL is a retired nursing instructor and she takes copious notes, double/triple checks what she's given DS -- but she still has issues with the amount of pills. Enzymes in particular --- the bottle says 8-10 creon with meals; however, she doesn't want to give him too many although he can easily eat a TON of calories and fat with a snack and go on to eat steak and a double order of fries for supper and hour or so later, topped off with a bucket of popcorn before bed. It's mind boggling. Her mindset is not to give him too many pills, but he's so pancreatic insufficient that he needs more enzymes depending upon what he eats.The enzyme issue drove me nuts. Early on it was based on output and as a new parent, I didn't have a clue as to what normal poop looked like. I was told with a mysterious smile "you'll know". No, I don't know, that's why I'm asking. Throw in a predigested formula --- apparently grass green well formed poop is normal.
I so wish my son had been diagnosed at birth. Mostly because he would have been getting the proper care all this time, and because all of this would have been normal for him if he had done it all his life. That's crazy that they didn't tell you what normal poop would look like. I breast fed all three of my kids when they were newborns and normal is yellow and runny for breast fed babies, and they told me that even though none of them were sick. I know that at least through baby and toddler years, until he started going to the bathroom without any help, his poop was normal. We just got the fecal elastase results back and it was 23. So he is extremely severe pancreatic insufficient. I'm not sure how he didn't starve to death already. The np said they got his sputum culture back today, and we would talk about it on Thursday at his first appt with the doctor. She said they would be starting him on inhaled abt on top of the two oral abts he's already on for pneumonia. She had already said last week they were going to start him on azithromycin probably three times a week routine. I'm really scared about how sick he is or damage to his lungs from not getting treated properly from being diagnosed so late. It seems like everything is going wrong with him all at once. He's going to run out of his enzyme samples tomorrow. They were waiting for his test result to come back, but I got it online this evening. It hadn't come back today so they sent a rx to the pharmacy. The Zenpep copay with our insurance is almost 400 dollars. I signed up for a program they told me about live2thrive that does copay assistance. The pharmacist said the copay would be 40 dollars, but they will only cover if for 330 pills or less and his rx was for 400. I'm not sure what to do, because that's how many he is supposed to take. Has anyone else had this problem? I'm thinking maybe if the np sends in a new rx and ups the dosage from 15000 capsules to 25000, and he takes 2 with meals instead of 3 then they would cover it, because it would be less than 330 capsules, but I'm not sure. I have to try to get that fixed in the morning, because he only has enough left for breakfast.
Does your insurance cover any of the other enzymes? Our BCBS will only cover creon or Zenpep. Ds was on a different enzyme and we had to switch to creon or pay 50%. We also got a copay card and only had to pay a small amount until we reached our out of pocket maximum.