Care outside the clinic

[wheezey]

So I was diagnosed with cystic fibrosis in December at 28, primarily because my brother was diagnosed. I have very minor symptoms (cough up a little goo daily, but no persistent cough, etc). To get diagnosed, I had to go to a pulmonologist outside of my city's only adult CF clinic, as they will only see you after diagnosis. Since then, I've been going to the clinic, but it seems designed for people with higher needs than I currently have. My FEV is around 110-115%, I only culture staph, and I am pancreatic sufficient and don't have CFRD. Despite all this, the clinic visits I have had were 4-6 hours, the clinic is difficult to deal with, and their only hours (Mondays 8 am - noon) are very inconvenient for me. I asked the diagnosing pulmonologist if he would see me quarterly for PFTs, sputum tests and routine care until/unless I need more care from the clinic. He said he would be happy to. He actually thought that the clinic might not take me as a patient since I don't have a lot going on (I've since realized that the CF Foundation requires them to take on anyone with the diagnosis regardless of health). I live in Seattle, and the next closest clinics are in Portland and Spokane (2.5-5 hours away), so another clinic isn't a realistic option.

Has anyone sought primary CF care outside an accredited clinic? Are there any issues with this plan? It seems like my doctor has a pretty good relationship with the clinic doctors, if he needed to consult with them, and I would have no problem calling them up for a second opinion if I was concerned. I'm just nervous because it seems like everyone always goes to the clinics, so maybe I am not considering some of the risks.

 

[Ratatosk]

Our situation is a little different as we choose to travel 3.5-4 hours to a clinic. DS spent the first 6 weeks in a NICU at a children's hospital in the City 250 miles away that also had an accredited cf clinic. We were introduced to a doctor who'd treated cf patients for a number of years and had privileges at the hospital, but the clinic he practiced at wasn't an accredited cf clinic. When we got home, we made an appointment with the local CF accredited cf clinic; however, due to the vast differences in treatment practices, we decided to continue going to the doctor in the City who isn't part of an accredited program. DS sees the head of the local cf clinic during regular office hours just as his pediatrician and for his annual bloodwork. We go to the City at least twice a year, have lung scans and pft tests there as well.

 

[peter]

Hi Wheezey. I think in your particular circumstance, seeing your local pulmonologist, a remote system for PFT checking might be sufficient. MIR make some quality varieties but so do other companies. That way you can update your doctor daily but still keep quarterly visits according to his protocol. You'll need a prescription for the remote monitoring system. It seems safer because if your PFT's begin to fall, you'll know right away, and treatment can begin sooner. John's Hopkins did a study showing PFT decline begins 2 weeks before other physical symptoms appear. Wonderful you are "symptom free" but aging may take some of the edge off your "immunity" to infection. Further more the less often you visit or have to visit a clinic the lower your average exposure will be to other bacterial strains than staphylococcus aureus or even resistant mutant strains of the staph you culture already. I don't have the John's Hopkins study link but someone here will put it up for you. You are the right age to embrace remote monitoring devices for everything. Obamacare is trying to ensure doctor payment for "internet doctor visits". Your attempt to do that will help push insurance internet reimbursement policies in the remote monitoring direction. Good luck. I'm so glad you started this conversation because 10-20 other people probably have the same question at this same moment but may not be inclined to address.

 

[JakeS]

Hi, my name is JakeS. I am 38, with CF. My recommendation is that you keep going to cf clinic, but only once a year, as long as your lung function, and pancreas levels stay the same. A local pulmonologist should be able to monitor you just fine. I agree with Peter, on all his thoughts. I currently only visit my clinic once a year, as it is a 7 hr drive(through Co. mountains), and my lung functions are stable. If you try remote, or internet visits, please post as i to am curious.

 

[MichaelL]

I was diagnosed at 34 and at the time was pretty healthy. Although I am still pancreatic sufficient, my health has declined quite a bit in the last 13 years. If you pick up infections (e.g., pseudomonas, steno, etc.), it can greatly affect your health. These infections should be treated as soon as possible to maintain your lung function. As long as you're seeing your pulmonologist regularly and he is checking the right things -- spirometry and proper sputum tests, these things will be monitored. I agree with the comment above that it would be worth visiting the CF clinic once a year. They understand subtle CF issues that a general pulmonologist wouldn't be familar with.

 

[Oboe]

After I turned 21, I was without a CF specialist for a while. Despite living in Tallahassee, a capital city, there's no certified CF doctor or clinic within 200 miles of here. I was seeing a regular pulmonologist for a while, but when I actually needed a hospitalization he told me "let's wait until you're sick" and then told me about how he has a friend on a transplant board at a hospital a few cities over, like he was content to wait until I got to that point. To be fair, I was in the lower 30s for FEV1 percentages, but that still stuck in my craw and prompted me to find a proper CF clinic. Now I travel that 200 miles to Jacksonville every other month for appointments.

I guess the point of my rambling is that it's best to at least have a proper CF clinic keeping tabs on you.

 

[Simba15]

I don't go to clinic

Yes I do not goto a clinic. Two reasons: they know little to nothing about my mutation and are using me as a guinea pig to learn about. Also I have received sub standard care there (rude employees, doctors literally guessing at treatment and then stating "we solved that" - um no I still have the problem). All in all arrogance with little to no care. My specialists are more concerned, see me as a person and if they don't know, they state that instead of guessing. I probably won't go to a clinic unless I get very sick (need hospitalization). Also most clinics know nothing about GI problems in adult CFers.

So I was diagnosed with cystic fibrosis in December at 28, primarily because my brother was diagnosed. I have very minor symptoms (cough up a little goo daily, but no persistent cough, etc). To get diagnosed, I had to go to a pulmonologist outside of my city's only adult CF clinic, as they will only see you after diagnosis. Since then, I've been going to the clinic, but it seems designed for people with higher needs than I currently have. My FEV is around 110-115%, I only culture staph, and I am pancreatic sufficient and don't have CFRD. Despite all this, the clinic visits I have had were 4-6 hours, the clinic is difficult to deal with, and their only hours (Mondays 8 am - noon) are very inconvenient for me. I asked the diagnosing pulmonologist if he would see me quarterly for PFTs, sputum tests and routine care until/unless I need more care from the clinic. He said he would be happy to. He actually thought that the clinic might not take me as a patient since I don't have a lot going on (I've since realized that the CF Foundation requires them to take on anyone with the diagnosis regardless of health). I live in Seattle, and the next closest clinics are in Portland and Spokane (2.5-5 hours away), so another clinic isn't a realistic option.

Has anyone sought primary CF care outside an accredited clinic? Are there any issues with this plan? It seems like my doctor has a pretty good relationship with the clinic doctors, if he needed to consult with them, and I would have no problem calling them up for a second opinion if I was concerned. I'm just nervous because it seems like everyone always goes to the clinics, so maybe I am not considering some of the risks.

 

[Aboveallislove]

My thoughts are that at a minimum you go to the CF clinic annually and have them review all the annual results (blood, xray) and have them do pft. And make sure they will admit you if needed. You might have a great local doctor who is trained/able to handle, but if you get admitted to a hospital, you want one with a CF clinic.
Also, totally off topic, but do you know you mutations? Because given your situation, I'd think you have to have one that has some residual function and can benefit from Kalydeco. If you know what I'm talking about, great. If not, hopefully you can post your mutations for more info.

 

[wheezey]

Hi, thanks everyone for your thoughts so far. It sounds like maybe I should plan to go to the clinic once a year, and I guess I probably grit my teeth and bear that much. I do plan to have my pulmonologist do quarterly sputum cultures for the full range of CF bugs as well as spirometry, and I know that he has a good rapport with clinic doctors if he had any concerns.

I think in your particular circumstance, seeing your local pulmonologist, a remote system for PFT checking might be sufficient. MIR make some quality varieties but so do other companies. That way you can update your doctor daily but still keep quarterly visits according to his protocol. You'll need a prescription for the remote monitoring system.

I've been thinking about buying one of these on my own, which I know is not as good as a prescription, high function one. But I think it would be sufficient to track trends. I also run about 15-20 miles a week, which is a good gauge of changes in my lung function. With those two methods, I feel like I could be on top of calling my pulmonologist and/or the clinic if I were concerned about a change. This is what I'm thinking about buying: http://www.amazon.com/Microlife-PF-100-Meter-Spirometry/dp/B000BH8TUA/

Further more the less often you visit or have to visit a clinic the lower your average exposure will be to other bacterial strains than staphylococcus aureus or even resistant mutant strains of the staph you culture already.

I agree, this is one of my concerns about going to the clinic while in good health, if I can avoid exposure to CF bugs, that would be a big upside.

And make sure they will admit you if needed. You might have a great local doctor who is trained/able to handle, but if you get admitted to a hospital, you want one with a CF clinic.

I may have misunderstood my clinic, but I believe the doctor told me on our first meeting that I need to keep my own pulmonologist on my care team for this reason. They say they do not admit except under really extraordinary circumstances, and that if I wanted to be admitted, I would have to do so under the care of my own separate pulmonologist. I know that sounds crazy and backwards, but it's what they told me (and one of the reasons I feel uncomfortable relying on them).

Also, totally off topic, but do you know you mutations? Because given your situation, I'd think you have to have one that has some residual function and can benefit from Kalydeco. If you know what I'm talking about, great. If not, hopefully you can post your mutations for more info.

Yes, I know my mutations, and one of the is R117H, which I understand is in the FDA queue for Kalydeco.

On a separate note, I did a search on old topics for others in my position and it seems like I'm not the only person who feels frustrated by my particular clinic:
http://forum.cysticfibrosis.com/threads/15104-does-anybody-seek-CF-care-outside-a-CF-Center?

 

[Aboveallislove]

I'd suggest you get Kalydeco off-label now. The evidence is there to support and insurance company policies generally specify scientific proof it works which is there. Might as well start getting the benefit than waiting FDA approval. Good luck!

 

[jaimers]

having a little pft/spirometry machine at home would probably be a great tool for you. My clinic gave me one and would have me do PFTs in clinic and then do a couple measurements on my home spirometer to see how the two compared and so that i could have a better understanding of what the results would look like at home for me and when to be concerned if numbers dipped, etc.
very helpful.