Hello, My wife is 31 with CF D508 I believe. We have a beautiful 5 year old daughter. We have been together for almost 10 years and married for almost 7.
My wife was diagnosed with CF when she was 18 months I believe. She has always taken very good care of herself and mostly just has lung issues so far. She has exaserbations on average once every year to year and a half and has to go on IV's. She has the typical culprits with Psudomoneous and MRSA. Since I first met her in 2002 she has only been in the hospital once which was back in 2002. Her baseline from 2002 till now has decreased a little from about high 60's to more recently 55-60 give or take.
From 2002 up to about a two months ago whenever my wife has had and exaserbation and gone on IV's to get a clean out and feel better and get PFT's up she has always responded to the IV drugs she was put on and they are usually the same ones each time with a little variation. She usually stays on the IV's for 2-3 weeks. Typically for her when she gets sick and feeling worse her pft's go down to the mid to high 30's and after her IV's she get back up to her baseline in about 2-3 weeks.
2 months ago my wife went into clinic as she was not feeling well and knew it was time for IV's and had been about a year since the previous round. Her pft's were about mid 30's and they recommended for the first time in about 7 year to go into the hospital for treatment. None of us were very excited about the hospital and was a little scary because she had avoided it for so long. She did not want to be away from home and our daughter and normal life. Anyway, she ended up being in for two weeks and really focusing on herself and breathing treatments and IV's. This time her pft's went up and down and did not really increase very fast or much during the hospital visit. She got up to about the low 40's over two weeks and went up to 47 after the end of the 3rd week at home on IV's and after she was off IV's for about a week was able to only reach 50. While she was in the hospital and not responding like she normally had they considered doing a Bronchoscopy to see if anything else was in her lungs that we could treat but since she got up to 50 finally, they decided it was not necessary at that time. My wife was not happy with that number as it was below her baseline and she had spent 2 weeks in the hospital focusing only on her and her treatments. She felt pretty good after but was scared she was getting worse overall.
A month later, which was about two weeks ago, she could feel that her lungs were filling up again and she was not feeling as good. She went into clinick a week ago tuesday and she was back down to the mid 30's again. She had her first bronchoscopy done to see if there is something else in her lungs that was treatable and causing such a decline so fast. They put her back in the hospital on IV's again for 3 days and is back home still on IV's with about the same results and response to the IV's she was on 2 months ago. We are just waiting for cultures to grow and can take up to 14 days for some and 6 weeks for one other.
So to say the least this is not a normal response for her and she is scared she is going to die and is just declining at this point. Everyone else has more optomisom and the clinical trial for her mutation should be available to start at the end of April. We know that she might get the placebo and she first has to qualify by maintaining a 40 pft for at least 30 days off of IV's and oral antibiotics and we are not sure if she is going to be able to do that. We are hopeful they will find something in the cultures that are treatable to get her back up to around her baseline again and we know that just a month ago she got back up to 50 which gives us hope as we know the capacity is there, but she is worried she will not be able to maintain and is just declining.
Has anyone had a similar situation and be willing to share their story? Also, any insight experience would be much appreciated.
Thank You
Worried Husband
My wife was diagnosed with CF when she was 18 months I believe. She has always taken very good care of herself and mostly just has lung issues so far. She has exaserbations on average once every year to year and a half and has to go on IV's. She has the typical culprits with Psudomoneous and MRSA. Since I first met her in 2002 she has only been in the hospital once which was back in 2002. Her baseline from 2002 till now has decreased a little from about high 60's to more recently 55-60 give or take.
From 2002 up to about a two months ago whenever my wife has had and exaserbation and gone on IV's to get a clean out and feel better and get PFT's up she has always responded to the IV drugs she was put on and they are usually the same ones each time with a little variation. She usually stays on the IV's for 2-3 weeks. Typically for her when she gets sick and feeling worse her pft's go down to the mid to high 30's and after her IV's she get back up to her baseline in about 2-3 weeks.
2 months ago my wife went into clinic as she was not feeling well and knew it was time for IV's and had been about a year since the previous round. Her pft's were about mid 30's and they recommended for the first time in about 7 year to go into the hospital for treatment. None of us were very excited about the hospital and was a little scary because she had avoided it for so long. She did not want to be away from home and our daughter and normal life. Anyway, she ended up being in for two weeks and really focusing on herself and breathing treatments and IV's. This time her pft's went up and down and did not really increase very fast or much during the hospital visit. She got up to about the low 40's over two weeks and went up to 47 after the end of the 3rd week at home on IV's and after she was off IV's for about a week was able to only reach 50. While she was in the hospital and not responding like she normally had they considered doing a Bronchoscopy to see if anything else was in her lungs that we could treat but since she got up to 50 finally, they decided it was not necessary at that time. My wife was not happy with that number as it was below her baseline and she had spent 2 weeks in the hospital focusing only on her and her treatments. She felt pretty good after but was scared she was getting worse overall.
A month later, which was about two weeks ago, she could feel that her lungs were filling up again and she was not feeling as good. She went into clinick a week ago tuesday and she was back down to the mid 30's again. She had her first bronchoscopy done to see if there is something else in her lungs that was treatable and causing such a decline so fast. They put her back in the hospital on IV's again for 3 days and is back home still on IV's with about the same results and response to the IV's she was on 2 months ago. We are just waiting for cultures to grow and can take up to 14 days for some and 6 weeks for one other.
So to say the least this is not a normal response for her and she is scared she is going to die and is just declining at this point. Everyone else has more optomisom and the clinical trial for her mutation should be available to start at the end of April. We know that she might get the placebo and she first has to qualify by maintaining a 40 pft for at least 30 days off of IV's and oral antibiotics and we are not sure if she is going to be able to do that. We are hopeful they will find something in the cultures that are treatable to get her back up to around her baseline again and we know that just a month ago she got back up to 50 which gives us hope as we know the capacity is there, but she is worried she will not be able to maintain and is just declining.
Has anyone had a similar situation and be willing to share their story? Also, any insight experience would be much appreciated.
Thank You
Worried Husband