A
Allansarmy
Guest
I really wasn't sure how to do this. I am the father of Allan. He is a bit shy to post his own thread and definitely not a YouTube video. So here I come. Hope its Ok to start my own thread. You may post in here and ask questions if you wish, if you have CF, a parent of a child with CF or just curious about CF. I am an open book. I can only put in this thread of what my son tells me. He knows this is being done and I do have his consent.
So just a tad bit of background on Allan. He is 19 y/o and he was diagnosed at 4 months old by a Doctor here in Texas.
His Normal FEV1 is usually around 85% on a good day. Had a bout of infection a few weeks ago sending his FEV1 to 64% and went in hospital for 5 days, sent home with PICC. Is blowing 81% last week and they are doing 1 more week of IVs almost done Wed he goes back for follow up. Feels better than he did about 3 weeks ago.
DAY 1 - Started his Orkambi this morning at 8:15am 2 pills in the AM/PM (Allan does not eat breakfast. Its rare, he is always nauseated) so this is gonna be harder than it looks. Orkambi must be taken with fat containing foods. Spoke with doctor and pharmacist. A glass of chocolate milk it is. This evening where it will be much easier. Allan is different than most with CF. There is no other words for it than his appetite sucks! He has been a picky eater since around 2 years old, the doctor said he will grow out of it..... ya right, more like grow into it. He eats about 500 cal a day if he is lucky, then gets 1500 cal a day through his G-button. So thats around 2,000 cal a day. The dietician wants him about 4,000 cal a day. We have tried everything including certain meds and it does not increase his appetite. So this will be interesting to see if Allan can gain more weight. He is 5'7" and weighs 122 ibs. So will keep everyone posted on his appetite and weight and his lungs, and bowels I guess.
About 10:00am he is starting to say he has a bit of shortness of breathe (probably psychosomatic to be honest). The doctor, YouTube, Orkambi site, the nurse and yes Mom and Dad have all been telling him that he 'may' and probably 'will' at some point get some shortness of breath, cough etc. They wanted him to start Monday today August 24th, 2015 so they can call in Prednisone if needed encase he has worst exacerbation. Will post more of an update later this evening.
EDIT: Night time dose was uneventful.
Day 2 on Orkambi - So far uneventful. No side effects as of yet. However, Allan is stating he is bringing up old phlegm. He says it looks old to him. The doctor is expecting a lot more to come out. Now one thing that I want to point out and maybe others can share light on this. Orkambi must be taken with fatty food. Is it possible that he is not getting enough fat with the Orkambi for it not to work as well? I fear this as Allan has hardly any appetite. He rarely eats, I might have to start giving him the Orkambi later in the evening when he does his feedings. I know that the Nutren 2.0 that he does through his G-button has fat as well as other calories, so I might try and give it to him right before he does a feeding. Allan does not like to do a 6 hour feeding pump. He was not eating hardly at all so the dietitian talked him into doing a 1 hour bolus through the Kangaroo Joey pump to get into 3 cans. I would love to say though he does this every night but he doesn't. He is 19 and is a bit stubborn.
Day 3 on Orkambi - I am having a difficult time with him even getting him to drink whole milk with this drug. It needs to be taken with fat and you would think this would be a cake walk, but when a person has zero appetite.... So far, he has a bit more cough and that is it. He states that he does not notice a difference.
Day 4 on Orkambi - A bite more of a cough, especially in the evening. I have not noticed and he has not noticed a significant cough production.
Day 5 on Orkambi - Cough is off and on, nothing drastic, still having problem getting him to take this in the morning mostly. He just is getting tired of drinking whole milk over and over. He has no appetite and will not eat ANYTHING else. Had a great call from Vertex the maker of Orkambi, and they are going to help lower his copay down to $15 as we have commercial insurance. Best news I could ever hope for!
Day 6 on Orkambi - Only slight cough with slight more phlegm production. Starting to complain of fatigue especially in the evening now. Stools are a bit more solid and has some constipation. He was in a slight better mood and actually cooked some eggs and milk to take his Meds. No shortness of breath or excessive coughing. Kinda worried though that some of his doses may not be adequately absorbed because how little fat he is consuming. I did however weigh him and he has gained 2 pounds!
Day 7 on Orkambi - Definitely more coughing now. Bringing up old phlegm - Dark green. Its important to note that Allan has finished 3 weeks of IV antibiotics as well through his PICC line. The PICC line is out and the CF clinic has put him on oral Augmentin as well. He tends to grow some fairly bad bugs as in MRSA to name a few, but this last one I would have to look up the proper spelling. His appetite was better last last night wanting to eat 15 pieces of bacon. I am going to take some advice given here by a person in this thread. They have been where my son has been (being told to take showers, do treatment) not feeling like they have control of their own body. I know this is what my son is going through. He knows this medication Orkambi is the medication my wife and I have been raving about and praying for years now. I just wanted my son excited about it too. But like the other person in this thread had mentioned that they would sometimes get angry at seeing how well others are doing on treatments etc. Maybe his is what he is doing. Perhaps I should take a step back and not ride him so much. I love him so much and I just don't want to see him end up like my wife's sister (double lung transplant) and passing by the age of 27. I want him to live a long fruitful life and have a family. Its hard to let go. Sorry, I know this journal is suppose to just be about my son's
symptoms but I have turned it into a sounding board. For that I am sorry. I will try and stick to just Allan and not be selfish here.
Day 8 on Orkambi - Allan is feeling a bit more tired. Cough has lessened except for in the morning. He seems to be bringing stuff up. BUT the tissue in his trash can has greatly been reduced. Morning dose went well today. Was able to eat and is not getting nauseated which is great!
Day 9 on Orkambi - Allan is starting to 'feel better'. He can take a deeper breathe he states. Coughing still in the morning mainly. I can hear him as I get ready for work around 7am. Tissue beside the bed is going down. His appetite is coming back YAY! Eating 2-3 times a day now. Really good for him. Ok my wife and I are thrilled on Day 9. Allan has gained 4 pounds just in the last 2 days. He was 124 on Sunday and now 128 and he is actually hungry. This is odd just typing this. I am convinced that this medication is helping him. I will keep you posted. This is great news!
Day 10 on Orkambi - Allan is feeling a little more tired, but he actually got up at a decent time again. He is starting to become more motivated. Cough has been greatly reduced. I hear him cough, but no more coughing fits to the point of vomitting. His appetite is still good, not great but just good. He has been mixing his own nebs since he was around age 10. Hard part is getting him to sterilize them properly. He tends to do the treatments and just lay them near his computer for hours at a time. Trying to get him to take them to the sink and rinse with hot water, soap etc. We occasionally boil some of the Pulmozyme Nebs for a few minutes but have to keep a close eye. They are a more steardy pastic but even they can melt. I have heard of devices that can go into a dishwasher. Does anyone else do this? If so, can you let me know what you use to do this? I have seen videos of other CF patients just putting them in a strainer type device and sealing it into a tupperware, adding hot water with soap and just shaking it for about 5 minutes, then rinsing. Have also heard about using vinegar. How is everyone else doing their neb cleaning? -
Day 11 on Orkambi - Today is a good day. He is coughing some morstly in the morning. I think the mucus just sits in his lungs due to gravity and non movement, and after he gets up and starts moving around he gets the urge to cough. His appetite is still good which we are encouraged. Coughing very little
Day 12 on Orkambi - Allan's words "I AM ALWAYS HUNGRY!! WHAT IS THIS!!!" I told him, welcome to being a normal teenager. I think most Teens, especially young men have a big hunger. He has never experienced this before. He eats, and 30 min later he feels more hungry. He has gained 5 pounds in just under 14 days now. His breathing is beyond excellent. I rarely ever hear him cough anymore. If he does it is very occasionally and it is only in the morning. His appetite picks up in the afternoon, so morning meals are still a bit of a challenge. However, this morning he wanted pancakes with bacon. Definately better than just a glass of milk to absorb the Orkambi with.
This forum only allows up to 10000 characters, sorry guys. I will post in my own thread as to how he is doing. I will probably start stretching it to every few weeks. But I will do my best to answer any questions.
So just a tad bit of background on Allan. He is 19 y/o and he was diagnosed at 4 months old by a Doctor here in Texas.
His Normal FEV1 is usually around 85% on a good day. Had a bout of infection a few weeks ago sending his FEV1 to 64% and went in hospital for 5 days, sent home with PICC. Is blowing 81% last week and they are doing 1 more week of IVs almost done Wed he goes back for follow up. Feels better than he did about 3 weeks ago.
DAY 1 - Started his Orkambi this morning at 8:15am 2 pills in the AM/PM (Allan does not eat breakfast. Its rare, he is always nauseated) so this is gonna be harder than it looks. Orkambi must be taken with fat containing foods. Spoke with doctor and pharmacist. A glass of chocolate milk it is. This evening where it will be much easier. Allan is different than most with CF. There is no other words for it than his appetite sucks! He has been a picky eater since around 2 years old, the doctor said he will grow out of it..... ya right, more like grow into it. He eats about 500 cal a day if he is lucky, then gets 1500 cal a day through his G-button. So thats around 2,000 cal a day. The dietician wants him about 4,000 cal a day. We have tried everything including certain meds and it does not increase his appetite. So this will be interesting to see if Allan can gain more weight. He is 5'7" and weighs 122 ibs. So will keep everyone posted on his appetite and weight and his lungs, and bowels I guess.
About 10:00am he is starting to say he has a bit of shortness of breathe (probably psychosomatic to be honest). The doctor, YouTube, Orkambi site, the nurse and yes Mom and Dad have all been telling him that he 'may' and probably 'will' at some point get some shortness of breath, cough etc. They wanted him to start Monday today August 24th, 2015 so they can call in Prednisone if needed encase he has worst exacerbation. Will post more of an update later this evening.
EDIT: Night time dose was uneventful.
Day 2 on Orkambi - So far uneventful. No side effects as of yet. However, Allan is stating he is bringing up old phlegm. He says it looks old to him. The doctor is expecting a lot more to come out. Now one thing that I want to point out and maybe others can share light on this. Orkambi must be taken with fatty food. Is it possible that he is not getting enough fat with the Orkambi for it not to work as well? I fear this as Allan has hardly any appetite. He rarely eats, I might have to start giving him the Orkambi later in the evening when he does his feedings. I know that the Nutren 2.0 that he does through his G-button has fat as well as other calories, so I might try and give it to him right before he does a feeding. Allan does not like to do a 6 hour feeding pump. He was not eating hardly at all so the dietitian talked him into doing a 1 hour bolus through the Kangaroo Joey pump to get into 3 cans. I would love to say though he does this every night but he doesn't. He is 19 and is a bit stubborn.
Day 3 on Orkambi - I am having a difficult time with him even getting him to drink whole milk with this drug. It needs to be taken with fat and you would think this would be a cake walk, but when a person has zero appetite.... So far, he has a bit more cough and that is it. He states that he does not notice a difference.
Day 4 on Orkambi - A bite more of a cough, especially in the evening. I have not noticed and he has not noticed a significant cough production.
Day 5 on Orkambi - Cough is off and on, nothing drastic, still having problem getting him to take this in the morning mostly. He just is getting tired of drinking whole milk over and over. He has no appetite and will not eat ANYTHING else. Had a great call from Vertex the maker of Orkambi, and they are going to help lower his copay down to $15 as we have commercial insurance. Best news I could ever hope for!
Day 6 on Orkambi - Only slight cough with slight more phlegm production. Starting to complain of fatigue especially in the evening now. Stools are a bit more solid and has some constipation. He was in a slight better mood and actually cooked some eggs and milk to take his Meds. No shortness of breath or excessive coughing. Kinda worried though that some of his doses may not be adequately absorbed because how little fat he is consuming. I did however weigh him and he has gained 2 pounds!
Day 7 on Orkambi - Definitely more coughing now. Bringing up old phlegm - Dark green. Its important to note that Allan has finished 3 weeks of IV antibiotics as well through his PICC line. The PICC line is out and the CF clinic has put him on oral Augmentin as well. He tends to grow some fairly bad bugs as in MRSA to name a few, but this last one I would have to look up the proper spelling. His appetite was better last last night wanting to eat 15 pieces of bacon. I am going to take some advice given here by a person in this thread. They have been where my son has been (being told to take showers, do treatment) not feeling like they have control of their own body. I know this is what my son is going through. He knows this medication Orkambi is the medication my wife and I have been raving about and praying for years now. I just wanted my son excited about it too. But like the other person in this thread had mentioned that they would sometimes get angry at seeing how well others are doing on treatments etc. Maybe his is what he is doing. Perhaps I should take a step back and not ride him so much. I love him so much and I just don't want to see him end up like my wife's sister (double lung transplant) and passing by the age of 27. I want him to live a long fruitful life and have a family. Its hard to let go. Sorry, I know this journal is suppose to just be about my son's
symptoms but I have turned it into a sounding board. For that I am sorry. I will try and stick to just Allan and not be selfish here.
Day 8 on Orkambi - Allan is feeling a bit more tired. Cough has lessened except for in the morning. He seems to be bringing stuff up. BUT the tissue in his trash can has greatly been reduced. Morning dose went well today. Was able to eat and is not getting nauseated which is great!
Day 9 on Orkambi - Allan is starting to 'feel better'. He can take a deeper breathe he states. Coughing still in the morning mainly. I can hear him as I get ready for work around 7am. Tissue beside the bed is going down. His appetite is coming back YAY! Eating 2-3 times a day now. Really good for him. Ok my wife and I are thrilled on Day 9. Allan has gained 4 pounds just in the last 2 days. He was 124 on Sunday and now 128 and he is actually hungry. This is odd just typing this. I am convinced that this medication is helping him. I will keep you posted. This is great news!
Day 10 on Orkambi - Allan is feeling a little more tired, but he actually got up at a decent time again. He is starting to become more motivated. Cough has been greatly reduced. I hear him cough, but no more coughing fits to the point of vomitting. His appetite is still good, not great but just good. He has been mixing his own nebs since he was around age 10. Hard part is getting him to sterilize them properly. He tends to do the treatments and just lay them near his computer for hours at a time. Trying to get him to take them to the sink and rinse with hot water, soap etc. We occasionally boil some of the Pulmozyme Nebs for a few minutes but have to keep a close eye. They are a more steardy pastic but even they can melt. I have heard of devices that can go into a dishwasher. Does anyone else do this? If so, can you let me know what you use to do this? I have seen videos of other CF patients just putting them in a strainer type device and sealing it into a tupperware, adding hot water with soap and just shaking it for about 5 minutes, then rinsing. Have also heard about using vinegar. How is everyone else doing their neb cleaning? -
Day 11 on Orkambi - Today is a good day. He is coughing some morstly in the morning. I think the mucus just sits in his lungs due to gravity and non movement, and after he gets up and starts moving around he gets the urge to cough. His appetite is still good which we are encouraged. Coughing very little
Day 12 on Orkambi - Allan's words "I AM ALWAYS HUNGRY!! WHAT IS THIS!!!" I told him, welcome to being a normal teenager. I think most Teens, especially young men have a big hunger. He has never experienced this before. He eats, and 30 min later he feels more hungry. He has gained 5 pounds in just under 14 days now. His breathing is beyond excellent. I rarely ever hear him cough anymore. If he does it is very occasionally and it is only in the morning. His appetite picks up in the afternoon, so morning meals are still a bit of a challenge. However, this morning he wanted pancakes with bacon. Definately better than just a glass of milk to absorb the Orkambi with.
This forum only allows up to 10000 characters, sorry guys. I will post in my own thread as to how he is doing. I will probably start stretching it to every few weeks. But I will do my best to answer any questions.
