Living With CF for 51 Years!

anonymous

New member
<img src="i/expressions/face-icon-small-smile.gif" border="0">I am new to this chat line business, so be patient with me. I turned 51 years old last month. I was not diagnosed as having cystic fibrosis until I was 35 years old. Just happened to get a doctor who really looked at my past medical history, both written and oral, and put two and two together. She had me tested for CF. It was positive. Had a second test done, and it was also positive. I was sent to a specialist at Tucson, Arizona's University Medical Center. He doubted I had CF because I was overweight. I explained that I was underweight all my life until giving birth to my only child years earlier, at which time I gained 80 pounds! This doctor tested me again, and again, it came out positive. He said just to be thankful that I obviously had a recessive form of CF or I'd have already been dead of it by then. I went through all the reactions of being told I have an incurable disease, got some literature to find out more about it, and began receiving treatment for it, which at that time was a daily dose of tetracycline antibiotic to help control respiratory infections.I now reside in a very small town in the White Mountains of Arizona. We have a small hospital and two very small clinics. We also have a high turnover in physicians. Within the last year, we have had our two pulmonologists leave town so they could make more money elsewhere. I have been battling chronic pneumonias for the last year and also lost almost 90 pounds since one pneumonia episode April of last year. I have no appetite. I went to my regular physician, but he specializes in gastroenterology and says he can't be of much help to me since lungs are not his speciality. This is very frustrating, and also very frightening, since I have this nagging suspicion that I am perhaps entering the end stages of this disease. That may just be my fear speaking to my mind, but I can't help but feel that this is perhaps the last best year I'm going to have.I am interested in talking to other adults who have CF, and am curious about the types of treatments you are receiving. I am on oxygen 24 hours a day, and I do nebulizer Atrovent/Albuterol breathing treatments 4 times a day. Every other 28 days, I also twice a day do a Tobramycin antibiotic breathing treatment. Tobramycin is actually meant for injection, but the pulmonologist I saw last April, who moved away, told me we needed to take more aggressive measures in treating the pneumonias I was having one right after the other, so he prescribed this liquid (injection) antibiotic so that I could inhale the antibiotic in, directly into my lungs where it might hopefully help control the pneumonias. That helped during the first year, but it's not helping this month, since I'm just finishing up another 28-day regimen of Tobramycin, and still have a worsening respiratory infection. I am on Social Security and cannot afford all the out-of-pocket expenses for some of the medications necessary to help me, such as Pulmozyme. Any suggestions? Have any of you had to seek "indigent" aid directly from the pharmaceutical companies so you could get the medication for free?Anyway, I would like to hear from other adults living with cystic fibrosis, although I'm open to communicating with anyone else who is curious about a CF-er of such longevity. Thanks.
 

Liza

New member
Wow what an amazing story! I have heard of people not being diagnosed until they were adults, having very few if any "symptoms" of CF. I am not an adult with CF just a parent of two with CF. We lived in Phoenix out in the west valley for almost 5 yrs courtesy of the USAF. Both of our girls, elementary age then, did wonderfully out there. Our oldest, now 16, insists that she needs to go back to AZ so that she can do better again. But to your problem... Have you checked into the CF foundation pharmacy? I heard back when our oldest started on Pulmozyme that Genetech (makers of Pulmozyme) has something set up for people who can't afford it so they can still get it. Did they ever do genetic studies on you to find out what mutation you have? Back when our daughters were diagnosed in 90' they weren't able to find their mutation but just this year they redid the studies on our oldest and came back with two mutations. They do not have the D508, that much we have always known. But it seems that the one they have has been known to respond well to Gentimiacin(sp) an inhaled antibiotic. So they are looking into starting that up. She (16yr.old) had been on that before when we were in AZ, and we wonder now if that is why she did so well there. Who knows, but we hope to find out. What I'm trying to say is I guess some mutations respond "better" to some inhaled antibiotics than others. I don't know what the expense is involved with the genetic studies. And I can't help but think about the vaccines and tests that my kids had to have while we were in AZ. Have you had the pneumonia vaccine (you only get that once). My kids both had to have annual TB tests as well as Valley Fever (I dont know if that one is just for the Phoenix area). OH but your in the mountains, I forgot. Have you explored the possibility of being seen in a CF center in Tucson? I am assuming they have one, if not I know Phoenix does. But I would start with the CF foundation, www.cff.org They have always been great in helping us out. I hope this helps in some way.Liza
 

anonymous

New member
Wow! Realy amazing. I don't have CF but my daughter does. My daughter does the inhaled tobi every other month too. She does bactum the other month. Are you associated with a cf clinic? or is it the cf clinic that is having all the turn over? why don't you check out this site http://groups.msn.com/cysticfibrosis2chat There are chats daily and a message board. Lots of people with CF there as well as parents and relatives of cfers. Lots of advice too!Have you considered lung transplant? There are alot of options. Do you use enzymes? gosh I have a lot of questions dont I? Andrea
 
B

B

Guest
No diagnosis till age 35! That is amazing! I am 26 f/with CF. As far as treatments go, I do the Tobi/abuterol one month and then coleymicin (? spelling)/abuterol the next. and then the plumozyne. However, I do not chest PT-I exersize instead. As far as assistance with med. The CF pharmacy has several diffrent programs they adminsture for the drug companies. I also hear they are good about working out payment options if you have to pay for it or with co pays (copays each month can add up fast when your on so many meds!). From my experiance regular plumonoligst may know lungs but they are not to great when it comes to CF. I had one mess mine up bad because he wanted to do a broncio scope for curiousty while I was out for sinus surgery. I had perfect lung functions at the time and they have never been the same since--It took me forever to recover too. With all of your weight loss, have you been tested for Diabetes? I went through a dramatic weight loss a year and half ago from various infections along with a high paced job. I never could seem to get over the infections and I was (for the first ime in my life) terribly under weight. My Dr tested me and found out I had diabetes. After we got that under control, I started gaining weight, infections decreased, and my lung functions greatly improved.Another thing, with the name of your town I assume you live in the mountains. With my previous job i traveled all over the country (seen around 20 diffrent places in one year). I was very tuned in to how diffrent climates affected my lungs. I found I had more problems breathing when I was in the mountains. My coworkers would have problems for the first day or two then adapt; however, during my two weeks in each location I never could adapt. OOHH Just thought of something else--Have you tried taking Zithromax. I have just started that and noticed a great improvemnet with the lungs! Patients of all ages are singing its prasies. It is the same thing as a Z Pack that Dr prescribe when normal people think they are dieing from the "normal" sinus and lung "crud", However, instead of just staying on it a week you stay on it contiously! Also, there is an oral antibotic that works wonders for me--Its Tequin-its a cousin to cipro, so you can't stay on it long at a time or you become resistant. The normal treamtn is 10-15 days. However, I can tell a dramatic difference my second day on it! Others have sang the same prasies as well!Hope my insite helped!
 

anonymous

New member
That definitely is an amazing story. It concerns me however that you seemed to be fairly healthy until you were 35, and now are having such problems. I feel as though a lack of adequate care or advice is the problem. I know from experience that every CF-er is completely different, and just because one med might work wonders for some patients, it might be the worst thing for someone else. The biggest mistake doctors can make with CF, i have found, is simply "throw" meds at them, all in conjuntion with one another. I for one DO NOT respond well to inhaled meds at all--i wish i did, but in the past, more than once, they have simply irritated my airways, worsening the infection, and not reaching the deep areas of my lungs. In fact, I am surprised that your doctor would refer to inhaling TOBI as a more aggressive treatment than IV tobramycin. IV antibiotics (not necessarily Tobramycin), are usually considered the most aggresive forms of treatment. Also, do you know which bugs you are culturing? You could be growing bacteria that the medicines you are taking do not even treat. I agree with the other woman about living in the mountains also. I have always found it difficult when I have visited places at high altitude, or gone hiking in the mountains.It seems to me that you definitely need someone who can look at your disease with a more specific aproach, and a less "by the book" attitude. Something as simple as a change of meds could change you dramatically, i feel terrible that after 35 years, you now find yourself at 51 and on 24 hour oxygen. I also wish that someone had explained the disease to you further...being diagnosed as a baby does not mean you are dead by 35, and there are no specific "end stages" or "last good days" of the disease that just start to "happen" at a certain time. Take control of your life and do what you can to insist you see someone who can give you some answers, and walk away from any doctor who is foolish enough to talk to you about a death sentence. feel free to email me at any time zoe7777@aol.com.
 

anonymous

New member
I would really look into a CF center somewhere else like Tuscon (mentioned earlier). Even if it is too much of a pain to go every month or three depending on the docs advice, you could at least go one or twice a year to make sure your local docs are doing a good enough job. A big turn around rate worries me because then no doc gets to know you and how your body responds to meds very well. Also I didn't hear you mention your therapy. Do you do the vest, flutter, pep, or acapella? If not I think it would help you sooooo much. About the appetite, when I was going through a loss of appitite because of a med I was on I found it easier to drink rather than eat. So I blended shakes as much as I could so i wouldn't lose weight. I used yogurt, scandishakes, ensure plus, bananas, and anything else I could find around. I also used a straw...for some reason it was easier to get it down that way. I actually gained weight on this all liquid diet. I know its not the healthiest diet, but for a weak I could not handle food and I figure its better than nothing.
 

anonymous

New member
<img src="i/expressions/face-icon-small-smile.gif" border="0">I was grateful for the responses I received regarding my letter last night. I got some good ideas to take to my doctor, the one whose specialty is gastroenterology, pertaining to different anbitiotics I can take to help control the pneumonia I'm constantly battling. I wanted to let one responder know that, yes, I got the one-time-only pneumonia vaccine. Last year I had a negative TB skin test result, after being inadvertently exposed to TB from an ill relative in a hospital. (The relative was found to have active TB after our visit to him while he was in the hospital. That figures...) I always have a positive reaction to Valley Fever, and it doesn't matter where I get tested, it always comes out positive. I was told that Valley Fever is prevalent in desert areas, and I understand that a lot of people in the southwest get positive VF results.I have one child, a son named Mike. When I was first diagnosed with CF, I was told that I automatically transferred the CF gene to him. I had him tested for CF in Tucson, and it was negative. But at that time, they didn't have the test to determine who, specifically, was a carrier or which mutation of the CF gene they carried. Last year, however, Mike had a genetics test done. I was told he should have it done, even though we already knew he was a carrier, because the test would at least let us know which mutation we had. Whatever gene Mike was carrying would be the same one I had, since that would be the gene I passed on to him. Mike was found to have Delta F508, which is the most common mutation of the CF genes that exist.Regarding another letter I received, a lung transplant is simply not an option for me. I have no way to pay for that kind of surgery, and no way to get that kind of money, either. I live in Eagar, AZ, which is right next to Springerville, and between both towns, there may be 5,000 people altogether. So it's a very small town. Also, I used to use enzymes during the two years I worked where I had insurance coverage. Since losing my insurance when I quit my job back in Sept. 1998, I no longer have the money to pay for the enzymes out of pocket. The reason I even started on the enzymes was due to having chronic diarrhea, and the enzymes helped tremendously, although it didn't clear the problem up 100%. Thanks for the advice about Bactum and the other chat room.The last letter I received (as of 2:30 p.m., at least), I appreciated the advice about the Coleymicin and Zithromax. Even the Tequin, although used only short-term, sounded positive to me, if it would work for me as well as it did for you. Also, I have diabetes tests done every year, and sometimes twice, since it runs in my family, and the results always come up negative. Just had a test done in February, and there's no sign of diabetes. Thanks to all of you who cared enough to respond. Hope you have a wonderful day! Eva <a target=new class=ftalternatingbarlinklarge href="mailto:boogiefeet9@hotmail.com">Text</a><a target=new class=ftalternatingbarlinklarge href="mailto:boogiefeet9@hotmail.com">Text</a>
 

anonymous

New member
I am 31 years old, the mother of 4 beautiful girls, severly overweight (i weigh 350) and I have CF. I was not diagnosed until the age of 17. I seem to have it very mild, although my lungs are starting to not do so good because of the weight I put on. But to be honest, I smoked for 15 years, I started at 13 before my cf diagnosis and then thought I was invincible for the next 11 years after. Up until a year ago I never did any meds or anything. But that was the stupid me, because I would wind up in the hospital 3 times a year easy. I am now a non-smoker and have been for 3 years. I am on tobi one month, coli the next, I take 2 albuterol treatments a day and pulmozyme. That is all when I have the time. 4 kids is a lot of work. I feel good and haven't been in hospital for a good 3 years. However, as good as I feel, I still will not allow myself to make plans to far into the future. I know what CF can do. My sister died of the disease when she was only 20. She had a typical case of CF. Just last month at my check up my dr. was saying that they do have some CFers in their 50s and 60s, non transplant, but I told him I didn't believe it because I had never seen one at any of my visits. I guess I now owe my dr. an aplogy! I hope you are feeling good. And just know you have given me a new found sense of hope. Stay well, you are in my prayers.
 

anonymous

New member
A woman at my l;ast appointment was giving her sputum sample to the nurse as I walked by and she was well over 50. So I think your doc is right and by the time you are 50 (19yrs) they will be saying some CFers are living till they are 60 or 70! Maybe I am too optimistic, but I really think with all their advancements that even if they don't find a cure in my lifetime, they will greatly improve both the quantity and the quality of our CF lives!
 

anonymous

New member
That woman you saw giving a sputum sample could have been me. At the UW clinic? I thinak I am the oldest CFr in the state of WA.
 
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