I was diagnosed by an accredited CF clinic and am still undergoing testing to determine my treatment. I have had recurrent sinus infections, bronchitis and breathing issues my whole life. Recently, this summer everything just seemed to increase in severity. In the past two months, I lost 15 pounds, have been put on an corticosteroid inhaler (I don't respond to albuterol), enzymes and they are testing me for CFRD due to spikes and drops in my blood sugar. Is there any information out there on disease progression? Has anyone else who has been diagnosed in their 30's noticed a sudden decline in health?
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Dx last wk at 32; any information on disease progression?
- Thread starter Smirizzle
- Start date
I agree you may find you have health improvement now that you are diagnosed with CF. You should be seeing an adult CF doctor at a CF clinic. There they can treat your specific CF needs for example CF specific Bacteria causing the sinus infections, and Bronchitis. Do you do any airway clearance or VEST therapy? Seeing a specialist to treat your CFRD(ie endocrinologist familiar with CF patients)
Are you on Pancreatic enzymes? CF is a very individual disease but if you are able to get your weight up(treating the CFRD and addressing pancreatic issues should help) this tends to help with lung function. It does take time to see what regime works for you.
I am sure there are some CF adults here who can offer you advice(I am a mom who has a child with CF, I do not have CF)
Are you on Pancreatic enzymes? CF is a very individual disease but if you are able to get your weight up(treating the CFRD and addressing pancreatic issues should help) this tends to help with lung function. It does take time to see what regime works for you.
I am sure there are some CF adults here who can offer you advice(I am a mom who has a child with CF, I do not have CF)
This disease is hard to recommend therapies for because there are so many different mutations. With that said, i think in general things do gey harder as you age. Finding the right therapies through work with cf docs/clinic is key. Also informed trial and error. Ie. Trying therapies, that help other cf patients with your mutation. Finally we have to live a very healthy lifestyle. Exercise and nutrition being key. It is kind of like living with a cancer that will never go away. You have to constantly fight it. Good luck!
I was diagnosed in 2001 at age 34. I had a nasty lung infection for months that wouldn't go away. After a referral to a specialist, I was diagnosed by a genetic test. Once they knew I had CF and confirmed which infections I had, they put me on the right treatment and I was feeling better in a week or two.
I started the recommended ongoing treatments -- for me this was lung clearance with a flutter device and inhaled TOBI -- and my health was pretty stable until I picked up a nasty bug in 2008.
It's important to learn more about your genetic mutations. They can indicate the severity and symptoms of your case. Usually, people diagnosed as adults have more mild forms of CF. You should also find out if you're pancreatic sufficient or insufficient.
I started the recommended ongoing treatments -- for me this was lung clearance with a flutter device and inhaled TOBI -- and my health was pretty stable until I picked up a nasty bug in 2008.
It's important to learn more about your genetic mutations. They can indicate the severity and symptoms of your case. Usually, people diagnosed as adults have more mild forms of CF. You should also find out if you're pancreatic sufficient or insufficient.
Thank you all for your input. I have started daily lung clearance and have noticed some improvements. My sputum culture came back positive but since it is the holidays I have to wait to see the doctor to know exactly what I grew. It's all new to me and I am doing pretty well so far in keeping myself from getting overwhelmed. It's nice to know there are things I can do to make myself feel better.
W
welshwitch
Guest
I just turned 35 and have I remembered asking my doctor that very question. And he couldn't give me a straight answer. I basically wanted to know how many years I have left (this was when I was going through an existential crisis at age 27).
First off, sorry to hear about your diagnosis. It's a scary thing, and really difficult.
CF is a spectrum. We all have different speeds of progression, different outcomes, and are at different stages.
Me, I'm 35 with 100% lung function and I've never been in the hospital. Yes, I work my butt off to maintain that. But, I've realized that my disease is progressing slower than probably the average person with CF.
We have children who are at end-stage lung disease, and we have older adults in their 60's and 70's who are still holding up pretty well.
I think there are some ways to measure how things are going for you:
*Your lung function
*Frequency of hospital stays
*Overall "quality of life": are you able to work full time? How much is your disease preventing you from doing the things you want to do?
However, it sounds like recently you've experienced a decline in health. Is this before or after your diagnosis? Let us know how you are doing with treatment and God bless!
First off, sorry to hear about your diagnosis. It's a scary thing, and really difficult.
CF is a spectrum. We all have different speeds of progression, different outcomes, and are at different stages.
Me, I'm 35 with 100% lung function and I've never been in the hospital. Yes, I work my butt off to maintain that. But, I've realized that my disease is progressing slower than probably the average person with CF.
We have children who are at end-stage lung disease, and we have older adults in their 60's and 70's who are still holding up pretty well.
I think there are some ways to measure how things are going for you:
*Your lung function
*Frequency of hospital stays
*Overall "quality of life": are you able to work full time? How much is your disease preventing you from doing the things you want to do?
However, it sounds like recently you've experienced a decline in health. Is this before or after your diagnosis? Let us know how you are doing with treatment and God bless!
Thank-you for the very organized information, Welshwitch.
The progression began this summer. I began losing weight and was having constant loose, bulky, greasy stools. I kept telling doctors it was like no matter what I ate, I wasn't absorbing any of it. They ran me through the battery of usual tests starting with the gallbladder. On of those GI docs is still convinced I have celiac's disease despite testing negative twice while symptomatic. About a month after not relief, I started becoming short of breath with simple tasks. I couldn't tolerate any level of exercise and I am an avid exerciser. (Mountain biking, BMX racing...I have a rowing machine and a stationary bike in my house!) I couldn't do five minutes of yoga without feeling like I would pass-out.
So, my husband and I ordered a genetic test through 23andme. It showed my delta F508 mutation and that I have Haemochromatosis, hetero for H63D and C282Y. (My iron has been moderately elevated for the past year, but the doctors didn't think it was enough to be HH.)
With these genetic results I found a new primary care doctor with specialties in genetic diseases. He did routine blood work, a complete genetic test of CFTR genome and ordered PFTs. I have about 10% loss of lung function with mild scarring on my lungs. This was probably the most surprising finding out of them all because I have always been so active. He gave me enzymes, an inhaler, and a referral to the UF CF Clinic.
My husband remembers in 2002 I had a really rough time for a couple of months with constant diarrhea. He says I would sleep more than usual, eat more than usual and still be tired and hungry. I was constantly sick for those two months. During that time I was earning a dance degree and working for a modern dance company, so I was dancing between 10-14 hours per day. The doctors think my activity levels when I was young may have helped stave off the progression some.
To address your bullets at the end:
*70% FEV1
*1 hospital stay since this progression started
*Quality of life: I am just returning back to 1 day a week of working and building from there. I get tired so easily that I get cranky and frustrated. I have not been able to exercise at my normal levels in almost 4 months. I have gained 5 of the 20 pounds I lost back but only by constantly eating. My doctors are confident I can get back though and so am I. I have to just be constantly reminded that it takes time.
I know that was a lot but that was my experience this year. I still get stubborn and try to do too much. Then I spend the next day or two recovering. I know it could be a lot worse and that's what keeps me going. I just wish there were a way I could help people who are going through this disease. It's pretty cruel that we can't be around each other.
The progression began this summer. I began losing weight and was having constant loose, bulky, greasy stools. I kept telling doctors it was like no matter what I ate, I wasn't absorbing any of it. They ran me through the battery of usual tests starting with the gallbladder. On of those GI docs is still convinced I have celiac's disease despite testing negative twice while symptomatic. About a month after not relief, I started becoming short of breath with simple tasks. I couldn't tolerate any level of exercise and I am an avid exerciser. (Mountain biking, BMX racing...I have a rowing machine and a stationary bike in my house!) I couldn't do five minutes of yoga without feeling like I would pass-out.
So, my husband and I ordered a genetic test through 23andme. It showed my delta F508 mutation and that I have Haemochromatosis, hetero for H63D and C282Y. (My iron has been moderately elevated for the past year, but the doctors didn't think it was enough to be HH.)
With these genetic results I found a new primary care doctor with specialties in genetic diseases. He did routine blood work, a complete genetic test of CFTR genome and ordered PFTs. I have about 10% loss of lung function with mild scarring on my lungs. This was probably the most surprising finding out of them all because I have always been so active. He gave me enzymes, an inhaler, and a referral to the UF CF Clinic.
My husband remembers in 2002 I had a really rough time for a couple of months with constant diarrhea. He says I would sleep more than usual, eat more than usual and still be tired and hungry. I was constantly sick for those two months. During that time I was earning a dance degree and working for a modern dance company, so I was dancing between 10-14 hours per day. The doctors think my activity levels when I was young may have helped stave off the progression some.
To address your bullets at the end:
*70% FEV1
*1 hospital stay since this progression started
*Quality of life: I am just returning back to 1 day a week of working and building from there. I get tired so easily that I get cranky and frustrated. I have not been able to exercise at my normal levels in almost 4 months. I have gained 5 of the 20 pounds I lost back but only by constantly eating. My doctors are confident I can get back though and so am I. I have to just be constantly reminded that it takes time.
I know that was a lot but that was my experience this year. I still get stubborn and try to do too much. Then I spend the next day or two recovering. I know it could be a lot worse and that's what keeps me going. I just wish there were a way I could help people who are going through this disease. It's pretty cruel that we can't be around each other.
I was dx at age at the age of 44. I had suffered all of my life with sinus, lung inflections, and pancreatitis. I am now 58 years old and and have been receiving care at my local adult CF center for the last 14 years. My FEV1 is 39%. I feel that my quality of life is better after being diagnosed and receiving the proper treatment.
W
welshwitch
Guest
Sounds like you're going through a tough time, Smirizzle. I hope that with treatment you start feeling better -- putting weight back on, raising your PFTs and having the energy to work as much as you wish. Hang in there!