Old folks: how did you do it?

[welshwitch]

Hi all,

The recent birthday posts (esp. for people in their 50's and 60's) have gotten me thinking.

Is there a way to predict how long you'll live with CF? I know this is an age-old question. I am at the sprightly young age of 33, but when I see these posts, I think, "Why not me?"

So my question is: old folks: how did you do it? What were some indicators that you were going to make it to the ripe old age that you are now? How was your CF progressing at, say, age 33? What were your PFTs? How often were you in the hospital? What tips would you give us? I don't want to get to your age and regret not doing some of the things I always wanted to do.

I welcome any advice from the wise sages on this site.

 

[mebergerson]

Hi, I am 31 yr old with df508 and have wondered the very same thing! I am going to be hospitalized again next week, 2nd time this year. My lung tests were perfect until I caught h1n1 flu the summer of 09, now I can't seem to get out of the 50's... I feel like I'm missing something. Thanks for your post!

 

[Aspiemom]

I am one of the "old folks". Funny, I don't think of myself as old, but as middle-aged, but I can see why CFers in their teens and 20's would think of me as old.

I think it depends on the type of CF and the genes we have. Those of us who live older have one regular CF gene and one mutated CF gene. Our symptoms tend to manifest as we age. For instance, my CF symptoms started with coughing during grade school, then by high school I started getting what they thought was bronchitis constantly. By my 20's I was coughing up blood and constant sinus infections. Late 20's I changed drs. and they diagnosed Bronchiectasis at age 27. But I didn't respond to treatment so they sent me to Johns Hopkins since they know of more diseases than traditional drs. That is where I was diagnosed at age 43!!! By then I was getting pretty bad and my FEV1 was 45%. Moved to GA where allergies are pretty bad and it dropped to the 30's, so I'm now 52 and FEV1 is 37% and I'm on oxygen.

It varies with every person, anyway, but those that live longer have A-Typical CF like I do.

 

[JustDucky]

I too am considered "a-typical CF"...diagnosed at 33 but had symptoms all of my life, just not so striking that CF was even considered. I am now 41, lung functions in the mid to high 20's the last time they were checked and am O2/vent (unrelated to CF). I did know a classic CF'er who did make it to her 50's, her sister died at 27 with CF. It varies across the board. If you are in your 30's and have high lung functions, I'd say (barring any catastrophic illnesses) you have a bit of time left in life. But, even so, I don't dare predict because CF is such a unpredictable illness.

Jenn 41 w/CF

 

[welshwitch]

Thanks for your responses! Yes I have great PFTs, never hospitalized, had a couple sinus surgeries here and there but nothing serious. Never on IVs. I guess part of this is that I'm thinking about having kids. If I somehow had a chart or a map about where I will be when I am at age 40, 50, and 60, it would really help w. my decision But it is true, with CF you really don't know.

 

[Printer]

I think that am the oldest member here so I will try to answer your question. Many people here say that I must have a "mild case". I am still alive so there is some valididy in those comments. My CF Doctor says I am lucky because of my mutations.

I was born in 1940 and the Doctors at that time were all trained prior to 1915. The 1930's were said to be the period of discovery for CF. The earliest paper written on CF was made by Swiss pediatrician Dr. Franconi who called the disease "celiac syndrome", which he defined as "changes in the pancrease as observed in children".

The name Cystic Fibrosis was coined by Dr. Dorothy Andersen who also stated that CF was caused by a deficiency in Vitamin A.

In the 1940's, Doctors Sidney Farber and Harry Shwachman connected abnormal mucus to the disease and challenged the Vitamin theory.

The sweat test was discovered in 1950. In 1955 Dr Shwachman laid out the foundation of modern treatment.

I point this out to show how difficult, even impossible, it was to be diagnosed prior to 1955. In 1955 I was 15 "and way too old to have CF". I am the most mis-diagnosed person to ever walk the face of the earth.

I always say that if my Mother knew that I had CF, she would have tied me to a chair and I would have never moved. Instead I persisted, played with my friends and while I had pnuemonia and other problems, I did what was necessary and went back to play. I was a competitive swimmer, a lifeguard on an Atlantic Ocean Beach and I played College Varsity Basketball (poorly). I adjusted, I could never swim freestyle so I became a breaststroker, where breathing was easier.

While I had alot of lung issues, most of my problems are in my stomach and pancreatis. My FEV is in the high 80s and my FEV1 is about 75. I can't eat as much as one cookie without 5 Creon 24. I take 10 or 12 with every meal.

In short, there is no secret. Stay as active as possible and try to wake up everyday. I am here today, only by the "GRACE OF GOD".

Bill

 

[welshwitch]

Wow, 72 years old and your FEV in the high 80s!?? That's incredible! Keep on truckin', Printer! Thanks for your story. I will try to wake up every day

 

[azdesertrat]

While I'm not in my 50's (yet) I guess I could be categorized as a 'CF Old Fart'.
I was born right in the middle of family of 7 kids. I think the fact that Dad was in the Army when I was born was a good thing. I received all the best care available at that time.
My Mom was the primary care-giver because Dad was 'Itinerant Helicopter Trash'. He was a helicopter pilot & spent lots of time away from home so Mom pretty much did the parenting. I always thought she did a fantastic job.
Anyway, as a kid, I was expected to do my chores & help out. We lived on a small farm; we raised our own milk, eggs, etc. and grew most of the food for the livestock in addtition to a large vegetable garden to feed us humans.
Mom never coddled me. I worked as hard as I was able. When I was forced to take a breather, I did.
I credit this 'normal' treatment with building a solid base of health that served me well.
Mom always told me I could do whatever I wanted to.
Well, that wsn't necessarily true. I wanted to join the Army & fly helicopters for Uncle Sam just like Dad did. (Vietanm Combat Veteran, Purple Heart, Silver Star, 21 Air Medals along with several with 'V' Device)
Well, I tried to join & failed the physical miserably.
Driving a semi for a living was my second dream. I was very successful at that; I drove for 23 years & have over 2 million safe miles having seen every state in the US & driven in every major city.
Funny, my CF Pulmonologist said that choice of work probably lengthened my life by quite a bit. The constant vibration of the truck kept all that crap in my lungs loose & easy to bring up. I kept a spittoon in the console of my Peterbilt & spit that crap all day long.
I had my first hospitalization when I was around 35 years old. I started going downhill quickly after that first round.
It got to the point I needed a lung transplant when I was 40. I recieved my personal miracle in 2005 at the tender age of 41.
I guess my advice has always been just do what you want. No matter what it is, you can achieve your dream.
Well, I guess there are some limitations, but for the most part its true.
Don't act like you have a disease & don't go through life thinking you can't do that because you have CF.
Try it first. If you can do it, so be it. If not, try something else!
Just don't lie down I guess...
Best of luck in the coming year.

 

[welshwitch]

Great story, thanks for sharing! I especially like the "spittoon" in the truck. I should get one for my car! (only half-kidding he he.)

 

[Jet]

I just turned 50 after Thanksgiving. I couldn't agree more with azdesertrat. I to had a large family and was never treated differently. My parents and both grandmothers placed high expectations on all of us. They demanded excellent grades in school. Early on they talked about college as a given for me even though none of them attended college. I was expected to participate in as many extra curricular activities as possible, band, sports etc. My grandmother in particular never let me use CF as an excuse. She was great for finding stories in the newspaper or on TV about people or families with far worse situations and challenges. I think one other key was that early on they made me own my disease and the responsibility for my own health. My mother was certainly protective and wouldn't allow me to do anything to foolish. However she never kept anything from me, even all the tough and scary stuff. I was always present for any discussion with the care teams. I think it helped prepare me for the transition to an adult patient and going away for college. I feel like I have had meaningful connections in my life at every stage. Post College it was work. Now it’s been my own family. My wife and kids are a big motivator for me. They're extremely supportive and know when I need a kick too. I think the more people and things you can find to keep you engaged and passionate about life the better. Of course also be as compliant as possible and don't do anything to stupid, or at least not more than once.

 

[kcarlson90]

My story is very similar to aspiemom! I started reading it and thought maybe I had already chimed in!

I am 56 and started declining a few years ago. After being put in the hospital for my first IV antibiotics a year ago March, my pulmonary doc had my genes mapped. DF508 and Arg347. That certainly answered a lot of questions. He put me on CF meds and O2. My PFT's were low enough to be evaluated for lung transplant and I was officially listed in November.

Looking back, I don't think I would have done anything differently. My old pulmonary doc, who originally diagnosed Bronchiectisis, never thought to test for CF since I am pancreatic sufficient. I used to bike hundreds of miles each week, ski with the best of them every winter, and considered myself fairly healthy. I was not very good about using the inhalers he prescribed. I wonder if I had been more diligent, I would be in better shape today? But I don't dwell on that. I am fortunate to have lived so long with this nasty disease and did not let it slow me down. I still work full time (much of which is out of my home, thank goodness). My greatest achievement is my daughter. We had a tough time conceiving and we were blessed with her 19 years ago, when I was 37 years old. I was not able to conceive again so she is our miracle baby. She does not have CF.

My advice to all young people is to stay faithful to your medication regimen and do some form of exercise (I purchased a walking treadmill and walk every day, along with a selection of exercises with weights for upper body). Also, be your own advocate. No one is going to care as much about your health as you. Do research, ask questions and do not take no for an answer. Spread the word about CF. I found so many people had either never heard of it or had misinformation. I stay positive and have surrounded myself with a wonderful support network of family and friends. And finally, a sense of humor goes a long way...nothing feels better than a belly deep laugh!
All the best to you!

Karen
56 yrs old, diagnosed at age 55, DF508 and Arg347, married 22 years, 19 year old daughter-sophomore in college. No other family members with CF. Waiting for new lungs!

 

[bcl0328]

I am one of the "old folks". Funny, I don't think of myself as old, but as middle-aged, but I can see why CFers in their teens and 20's would think of me as old.

I think it depends on the type of CF and the genes we have. Those of us who live older have one regular CF gene and one mutated CF gene. Our symptoms tend to manifest as we age. For instance, my CF symptoms started with coughing during grade school, then by high school I started getting what they thought was bronchitis constantly. By my 20's I was coughing up blood and constant sinus infections. Late 20's I changed drs. and they diagnosed Bronchiectasis at age 27. But I didn't respond to treatment so they sent me to Johns Hopkins since they know of more diseases than traditional drs. That is where I was diagnosed at age 43!!! By then I was getting pretty bad and my FEV1 was 45%. Moved to GA where allergies are pretty bad and it dropped to the 30's, so I'm now 52 and FEV1 is 37% and I'm on oxygen.

It varies with every person, anyway, but those that live longer have A-Typical CF like I do.

you sound a lot like me right now. i'm 25. hopefully i can make it in my 50s before i need a transplant.



all the older males here with kids, i assume you didn't have kids the natural way?

 

[LittleLab4CF]

Hi. At the risk of feeling baited, I can't resist encapsulating my life story. Printer laid out a killer timeline to provide perspective on the state of medical insight punctuated by sporadic breakthroughs. CF remains baffling to the average doctor and is not abundantly understood by any one specialist.

Born in 1950 I will turn 63 soon enough. I was born in Wyoming which in 1950 didn't have a private hospital. The location of a person in 1950 had considerable bearing on medical access. From my birth to age 20 I had three doctors in total. Two were partners and whomever delivered you became your pediatrition. I was cared by one until 13 and saw the other until I turned 20. My adult doctor saw my father, diagnosed from his post mortem to have CF.

Our family dynamics played a strong part in my dealing with undiagnosed CF. My earliest memories of food are unpleasant. Food made me feel sick. In a way I wonder how I made it to 62 with my hearing intact. Those cool ear tubes that eliminates the occasional burst or lanced eardrum hadn't been invented. Lancing my eardrums was done all too often. There's an indescribable pain! The county health department could use me as an early warning agent for any upper respiratory infection I had them so often. Pneumonia was like a cold. My parents cobbled an oxygen tent for me until I was a teen. I didn't use it constantly but it saved 4 or 5 hospitalizations a year.

I can only guess that my parents feared I had inherited whatever was making my father sick. Most of my life he didn't appear at all sick. In fact he was strong and vital until I was 14 when he went down with a hemorrhaging ulcer. As with Bill, I was just another kid on the block. I ran and played with no sense I was any different from the other snot nosed kids. Oh yeah I was the skinniest kid which despite a couple minor scrapes with school mates working on notches, I naturally turned it to my advantage with a strong flashlight on my transparent body.

Being sick was very difficult for me. First I had nor have any desire to be sick but more so my parents gave us kids no quarter. On anything! We lived by unyielding rules with clearly defined dicipline. This included being sick. As an adult I look back and appreciate the clarity and consistancy of my parents. Summertime was no time to be sick and remember playing while very sick and collapsing. This was important as it showed me there was a difference between me and other kids. I was always sick. Just when does a sick kid play? No I didn't posess this kind of insight.

During the school year if I was too sick to attend school I was sequestered to my bed. My only escape from boredom was in the form of books. When I wanted to go to school sick we had problems as no simple set of rules applied. Strep was at least as bad when I was in grammar school and every school came with a school nurse. I had a pattern of no fever in the morning that would spike at school to add to the weekly sore throat/strep test/get sent home routine. I just carried my own thermometer and aspirin, game over.

As a teen my health was following more like my father's being abdominal in nature over lung infections. Upper respiratory infection was a constant companion. Who'd a thunk that ulcers are mostly caused by a bacterial infection? So I was treated for what they defined as a nervous stomch. This included tranquilizers and antacids. They added medicines to alternately relieve abdominal spasms and stimulate motility. It wasn't todays technology but it didn't kill me.

The typical abdominal CF issues were repeatedly treated and abdominal workups rerun every couple of years ending with the requisite visit to a psychiatrist, the last vestage when medicine fails. As I grew older the management of CF grew well past a bunch of annoying health issues.

Until I was nearly 60, nothing held me back. Being sick and in pain doesn't necessarily imply suffering. I have suffered enough, not too much nor too little. For the most part I have had so much fun, the roof could fall this instant and I haven't been denied a life of wonder. Eeeeyhaww!!!
LL

 

[Printer]

But Little Lab, you were dx late in life and you are almost 63, THEREFORE you have a "MILD CASE OF CF".

At least that is what a number of ANONYING, people in here keep telling me that I have.

Bill

 

[maryiris]

I'll be 53 this year; FEV 1 is 21% and I am NOT on oxygen I do everything I am supposed to do - wake up and do the meds, vest, acapello - START THE DAY. Then same routine at night. But I never let it stop me from doing anything. Up until 40 I would snow ski all the time - coughing, stopping to catch my breath, but keep on going. 3 things I pay attention to: 1. Meds/clearance; 2 EXERCISE and 3 SLEEP!!! I have been so sick before, IV meds, hospital stay. But you never seem to get good sleep. Then I'll sleep for 26 hours - almost straight - and my body has done some repair and I'm back on the warpath - taking on CF with gusto. I firmly believe that a good mental state is the winner. Never let it get you down - always kick it's butt.

 

[LittleLab4CF]

Ooh yeah that "mild" crap sticks in my crop as well. As a disclaimer my knowledge of CF as a patient doesn't include anymore than personal experience, I haven't died at 4 nor am I sucking air through gifted lungs. I call it long suffering. If someone can iimagine 3 years of CF quality chronic pancreatitis, then imagine 30 years of constant pain with debilitating nausea from that same chronic pancreatitis. Now work 70 hours a week, do the international business schlep 180 days a year for 20 of those years all because I had to. I wasn't enslaved by anything more than my heredity but that included CF and being wired for adventure.

There is no evdence that a long life of illness has more quality than a short one. I am old enough to feel the self loathing of deterioration. I have had Parkinson's disease almost as long as I have known I have CF. I wouldn't change a thing in my life although I spent 45 years attempting to cure CF. It appears I have failed. As a consolation prize I have given the science of genetics many critical tools to continue. So the next person bemoaning false statistics brought on by late diagnosis for CF or whatever bee buzzes in your bonnet maybe that late living CFer is going to end all CF. Believe me, we old CFers bow our heads periodically to remember and celebrate the lives of those younger CFers who have and will pass before us.

The population of CFers is like any family or tribe. A child with CF needs all the attention their parents can give. That CF came from parents of which could also have CF or "mild" CF so that makes an extended family important. An extension of that family are old CFers who can relate in ways a grandparent w/CF could only do. We are educators of and advocates for CFer's rights and CF research.

I can't deny that to date CF complications haven't killed me. I knew men who walked over the piled bodies on Omaha Beach, fought bloody battles for years and came home. They never can explain why they lived when men next to them perished. These weren't clean up troops but front line soldiers. Like DDF508 these frontline CFers are fated to die. Some die in battle young some die after many battles.
LL

 

[Julie Desch]

I'm 52 and have "typical" (read: double delta 508) cystic fibrosis. I'm fairly healthy, with lung function in the high 60's. I am active, and raising two teenage boys (adopted). I had a brief career in medicine before I retired to spend the time I needed to take care of myself properly. I have had two siblings who have died before me, one at age 58 and one at 31. I say this to encourage even those who are double delta 508's, because there is SO much more at play that just the CFTR mutations that you are born with. There are ongoing environmental effects (nutritional status, exercise, exposure to cigarette smoke, etc) as well as epigenetic effects that nobody really understands yet. NOT TO MENTION that the disease as we know it is on the verge of turning into one that can be controlled just as diabetes or hypothyroidism are controlled with medication. The bottom line is that there is no timeline. "Life expectancy" is a moving target, and fortunately it is shifting to the right very quickly. Live as best as you can (meaning do everything you can to stay healthy now as we wait for medical progress) and live your life as though you are going to live to be an old man/woman rocking your great grandchild on your lap!

 

[rtorres25]

I'm 47 years old. I had pulmonary problems all my life, but wasn't officially diagnosed until age 38. I have a "mild" case, but my official diagnosis was a miracle that I got once I started praying for healing for myself!

The CF meds have changed my life. I've had IV's at least once a year for the most part, but have had a few years with none. 2012 was a great year. My FEV1 is around 65%. I feel great most of the time. I'm a wife and mother of 2 teenaged daughters. I walk at least 4 miles 3 days a week with my friends. We talk as much as we walk I have ridden horses most of my life and beleive this is great therapy.

My secret is to put it all in the hands of God. He has a purpose for my life and this dx is part of my testimony! (also, I never miss a treatment!).

 

[azdesertrat]

One more thing I'd like to add;
NO MATTER HOW BAD YOU THINK YOU HAVE IT, ALL YOU HAVE TO DO IS LOOK AROUND. YOU CAN ALWAYS FIND SOMEONE WHO HAS IT WORSE'.
That has sustained me through quite a bit.

 

[windex125]

All of the above stories were great to read. I turned 58 in Dec. Was diagnosed as a infant then undiagnosed at age 8 and re diagnosed as a adult. Confused so was I as Printer mentioned there was not alot of information in the 50's. We were quite poor as a family and I remember going to clinic once a month, and having to take a nasty liquid med. But I went though life as though I was not sick, I was never treated like a sick child either. I took on more household chores as a child then my siblings. I hated always having that nasty cough with the green junk.First hosp stay was in 70's got my first pick line in the 80's had my port put in in the 90's. M y Pft's are 49-50 but also have one colasped lung it does not even show up on xray anymore. MY body has adjusted. I am PS which is helpful, but have other digestive issues. I say live yr. life as best you can, don'tthink I can'y do this as I not live to that day. Bad thinking. I never thought of dying and really was quite sickly in the 90's but feel I do my share and the rest is out of my hands. As we all know ever case is different. What is that old saying it is not written in stone. Be Well, Take Care, Go out dancing when you can. I always loved that. female 58-CF amd alot of other issues...