At The End Of My Rope & Need Some Advice Please

[blonde1]

I am at the end of my rope, friends. A typical day looks like this for me: using my nebulizer 4x a day, popping tons and tons of pills, trying to eat but coughing so hard it comes up, feeling too exhausted to function. The only time my cough doesn't feel miserably suffocating is the few minutes right after a neb when it's a little more lose. I cough so hard that my ribs, chest, back muscles feel completely fried- stabs of pain from pulling muscles daily. I also cough so hard that I gag and cough up mucus, so my doc put me on Zofran to hopefully help me stop throwing up as much, especially since I think that impacts my absorption of antibiotics and meds. I of course have trouble keeping on weight, and have lost some. I'm on enough steroids to choke a horse... almost constantly on varying rounds of 'biotics. My nails are beginning to club (my thumbs a lot worse than the others), and my fingers turn blue periodically- normally right before I wind up in the ER. This week I passed out going up the stairs- stabbing pain in my lung, wasn't getting enough air... and down I went

I have gotten pneumonia about every 2 months since I was a kid, and only worsened with age. My doc told me I likely wouldn't be able to have kids when I was a teenager because of various issues- but I've had two miracle babies... I am now a single mom and supporting/raising them myself. I definitely know having another would be too much for my body- the last pregnancy all but killed me, and my last baby was born with pneumonia herself and a collapsed lung. But within a week of being out of me (not so talented an incubator apparently), she is healthy as can be and zero lung issues now. I went gluten-free about 5 years ago to help aid some of my GI issues, and it helped a great deal- but of course didn't solve all of my problems by any means

So, this year has been a big decline. I can't go upstairs without gasping for breathe and pain. I can't pick up my youngest without pain. Basically, anything that requires actual breathing is not so fun. I keep getting infection after infection (which is nothing new but seems to take more and more out of me lately). I'm 26. Doc found lots of bacteria in my lungs on recent bronchoscopy, completely raw esophagus and trachea, and I always have pneumonia/bronchitis, as well as poor breathing on all ER visits and doc appointments. Of course my veins are a wreck- most are blown out from frequent IVs and treatments, but most just don't respond

Sometimes I forget how normal people function. My boyfriend bounces up in the morning and moves happily about his day. I wake up feeling like I never slept, like I'm starved/desperate for more, and like the most impossible thing ever will be standing up, breathing and functioning throughout the day (which of course I do anyways) ; )

But guess what? CF has never been firmly diagnosed. I have a lung specialist, and am on constant meds, in and out of the hospital, infections, the cough.

Has anyone had this hard and long of a diagnosis process? Not that it is (it's been discussed), but it certainly is something that is very very parallel. Any other ideas of what this could be? Any thoughts or tips in general? I'm at my wits end : (

 

[lilmac7]

Are you pancreatic sufficient? All I can give in way of advice is you have got to push yourself to get moving. I assume you are not working, correct? If so then even better for you as you have more time than I do to exercise, but I cannot stress enough how much exercise has made a difference for me and again I proved it recently. I started college for the fall semester and between that and working full time, finding the time and energy to workout eventually dwindled to me just not going and sure enough I got hit recently with THE worse chest infection I've had in years. Basically all the symptoms you described above except I have not passed out but have had my vision close in on me a few times going up stairs. I got myself in and on a course of IV antibiotics which I'm still on at the moment and have now started back slowly with working out, with a PICC line in my arm, and already feel a difference. My first day back at work on Monday was rough, I got breathless quite a few times with all the up and down, Tuesday was better and today even better still. Apart from this, I've been below the point of transplant consideration and with the aid of exercise was able to get myself out of that bracket and gt myself to feeling much better and being able to function. It is not easy to do while you are feeling so exhausted and the pain and all but it will make a world of difference.

Exercise not only will strengthen your lungs but also help make you cough out more of that crap in there causing the problem and infections. Just go at it slowly and work your way up, also do it responsibly like if you are prone to passing out make sure you have a partner with you that knows what to do and take your breaks in between. Choose a form of exercise that you will be more likely to enjoy, like for me I don't like running but prefer weight lifting so that's what I took up. Weight lifting will also do more for you than just your lungs, exercise will also raise your appetite and help with you to put on some weight.

Lastly, never give up. Yes it will be hard at times but it's times that is the hardest that we have to knuckle down and fight even harder to beat this. Good luck and I hope you get some relief and a sense of a "normal" life soon.

 

[bethylove]

I don't have much in the way of advice but I just wanted to say that I am so sorry that you are struggling. It sounds like you are feeling like absolute rubbish, and I can only imagine how frustrating it is especially when no diagnoses is being made. All I can offer is that you keep pushing, fighting and advocating for yourself. Hang in there.

 

[dmac]

I'm so sorry to hear you have been having such a tough time. I'm 62 and been through a lot with CF over the years. A couple questions, if I may? Has your pulmologist discussed the possibility of you having a portacath implanted? When my veins became unusuable, they did that for me. The first one lasted 10 years before it started leaking. I have had the current one in me for 7 years and it's still working fabulous. It's a help with the IVs. And I also wondered if a gene test was ever done to determine a definitive diagnosis of CF for you? The sweat tests on me were always unreadable, so they said. Finally my pulmonologist sent me to a CF specialist at MassGeneral hospital and the doctor did a gene test and determined, yes, all this suffering was in fact CF, something my doctor had actually been treating me for with the meds, but still he had doubted I had it. Very strange. But having the diagnosis for some reason made me feel better. This was actually when I was 40. Up til then they called it all sorts of things, pleurisy, bronchitis, bronchiectasis and so on. I'd been having hemoptysis since I was a child. And coughed up gunk constantly. At least I finally knew what it was. It was a huge help to then be seeing the CF specialist at the CF center. I no longer saw my pulmonologist, the CF doctor took over.

One other point I'd like to bring up. When I was at my worst, my lung function had dipped into the lower 20s and I was on the transplant list, I did something I never thought I would try. I started walking a little bit each day. Some days were so bad it would only be a minute or two. When I got up to 6 minutes, I decided to go into the pulmonary rehab program offered by the hospital. It made a huge difference. It was a hard thing to do but paid off immensely. Then a few years ago I wanted to learn tai chi. But I kept missing classes due to my health and couldn't get caught up. However, in one class the teacher taught us something called, the "brocade" from Qi gong. It's 8 simple exercises, the breathing is important in them, and I do these when I'm able. They make a big difference. And it isn't hard. And doesn't take too much time. If you are interested there probably is a book in the library you could find. If not, let me know I'll give you more info.
My heart goes out to you. Please keep on trying. Some days will be better than others. 62 years later I'm still saying that. The other thing I wondered is if you are receiving enough chest PT. I didn't remember seeing that in your post. That is VERY important. Hope you find something in this to help.

 

[blonde1]

Wow, I cannot thank you all enough for answering me back. Your advice.. all of those suggestions.. even just hearing your similar stories and feelings makes me overwhelmed with emotion and gratitude. THANK YOU

They are actually running the CF genetic blood tests again, which actually raises more questions on my part: 1) I think it's only for the 35+ genetic span... should I push for the really broad spectrum test? Were most of you diagnosed with the more basic one? I am utterly clueless. 2) how long does it normally take to come back?

Otherwise, your suggestions were amazing. So far I have the same treatments as CF. I am going to start taking NAC supplements too... I DO need to be on top of chest therapy, but right now tend to just cough and cough until I get some of the "gunk" up (but sometimes I feel desperate- like I'm suffocating).... and I want to find a mini or portable neb so if I'm traveling or away from home, I don't keep missing opportunities. Any suggestions there?

Sending so much love to all of you! xoxo

 

[Kristan]

I am 47 and being treating like I have CF. I tested negative for CF with a sweat test in childhood and negative on a basic blood test 4 years ago. The CF doctor told me diagnosis or not, the treatment would be the same. Do you have PA and are you inhaling antibiotics regularly? Also do you have a vest? Nebulizing HTS is also helpful with keeping the lungs clear. These would probably help with the coughing a great deal and should be available to you. You may also want to check for GERD and possible blood sugar problems from the steroids.
Keep pushing for treatment. I have three children (6,11,13) and it gets harder in many ways, so make yourself a priority! Good luck.

 

[lilmac7]

Hi Blonde1, I have the Pari Trek S portable compressor with battery pack and comes with the Pari LC Sprint nebulizer, car and wall plug/charger. Works a treat, I take it to work with me in the car everyday in order to do my treatments. I work full time so it's very handy and I've taken it when I travel as well. I buy it on ebay with no need for prescription and the lot but unless you have insurance and want to go through that route for compensation then that's a different story and I can't advise on how to get it from that way as I'm not a US citizen. The cost out of pocket for me is far worth me wasting time fighting to try and get it through mu insurance so I just suck up the cost instead of dealing with delays and all the drama just to save a little.

I was asking if you had digestion issues and have to take enzymes with your food but you never answered that. Maybe I worded it in clinical terms hat you never understood? Reason I ask that is if you are fine with digestion and tests for CF have been negative, then I will ask if you have been tested for PCD - Primary Ciliary Dyskinesia aka Immotile Ciliary Syndrome? It is a disease in which the ciliary does not function as it should which leads to hindered clearing of mucus from the lungs and over time congestion and infections that are basically similar to CF minus the stomach digestion issues. Many times people with PCD are thought to have CF but obviously don't test as having CF and this condition is overlooked as it's a more rare condition. Either way though treatments for PCD are based off of CF as far as lung health management is concerned and very often PCD patients actually are seen/managed at CF clinics.

 

[blonde1]

Oops- missed that question. I do have the digestive issues. The specialists settled on celiac disease about 6 - 7 years ago, but it was never a concrete diagnosis (more of a 'we don't know what's wrong with you so we'll go with this' kind of a deal). Eating gluten-free does help- I've been GF for about 5 years - but only made a tiny dent in helping my issues overall, so I've always wondered if it was really the lung/CF issue being misdiagnosed, or if one influenced the other, or if there was a connection somehow.

Have enzymes helped you guys?

I had problem with [and I know this is gross] upset stomach- still do on and off... throwing up (which now I think was just from the coughing and infections)...and not absorbing. I am not dangerously underweight at all, but I am a skinny person who gets tons of the "I wish I was as thin as you" comments from people (while I'm thinking, "I wish eating and breathing at the same time wasn't so tiring"). Right now I'm just on Zofran to try to keep my weight stable (I've lost maybe 10 pounds this year, but for me- that's a LOT because I'm petite to begin with. I feel like even 5 - 7 lbs is a lot for me, you know what I mean?)

Anyways, I have never heard of that other condition so that's fascinating and really good to know! Thank you SO much for the advice

Also, now I'm just waiting on the "in house" (in hospital lab) CF testing they've done. In everyone's experience, should I request the "ship away" larger spectrum test, or do you think I'll be fine with this one as far as detection goes? I'm highly symptomatic since of course, but just no label as of yet. I am so drained from all of this : ( Your conversation and insight means everything!

 

[Melissa75]

(((Hugs)))

Like others who have responded, I think you need to explore a CF diagnosis as deeply as possible, and also PCD and immunodeficiency. In my case, I've been to a CF Center and National Jewish, and I don't have CF or PCD or immune deficiencies. I have bronchiectasis, allergies and "digestive issues" (fructose and sorbitol malabsorbtion --edited to delete "intolerance," I always get that confused -- are all I can pin down). I am so sorry you are plagued by nausea. That is awful. My issues are just gas and no solid poops, but I'm rarely queasy.

I was tested for CF as a baby, but I had years of being basically fine before having problems again in my twenties. I'm 37 and have organized care and I learn as much as I can here. My health has improved since starting to exercise and being consistent with treatments/airway clearance. It has also improved since my kids got out of toddler age. When they were younger, I remember wondering how I would ever "keep on keeping on."

I hope you get some answers from drs and experience an improvement in your situation.

 

[rmotion]

dairy free too has helped me along with gluten free.
this f-n pseudomonas drags me down too, i hate it it robs us of a normal life.
i too deal with the constant cf struggles
you cant try to compare you to your boyfriend we all have our own struggles to deal with and all learn at different pace. if he is supportive that is a good step.
it is best to focus on your health and having a baby is a tough choice how can you care for you and him/her both optimally.

 

[dmac]

Someone mentioned the Pari Trek S. That's a good one. I now use the DeVilbiss Traveler. You can get these online at a couple different sites. For instance, currently at Justnebulizers.com the Pari Trek S with battery is $163, the Devilbiss Traveler with battery is $139.95. Both include the adapter for the car and the charger. Another place I sometimes get supplies, particularly my parineb mouthpiece sets is Vitalitymedical.com. Last time I paid 11.95 a piece for them (they last 6 months) and they are much more money elsewhere. Prices change at all these sites so you just have to check from time to time to catch what you're looking for at a good deal. As for the blood tests for CF they did the big one on me at first but both the genes were common ones it turned out and probably could have been caught with the smaller test. In your case if it doesn't show on the first one, have them try the larger test and everyone on here had good suggestions about other possibilites to check for if this doesn't come up definitively. Keep trying. Don't give up. The answers will come. Anytime you need an e-hug, we're all here for you.