Pseudomonas and Balancing Work & Treatment

[lauryn.tubes]

Good morning. My 8 month old daughter had a cough for 5+ weeks and the CF clinic asked us to bring her in for a check. She dx as CRMS due to her mutations (DF508 and F1052V) and is tracked by our local CF clinic. F1052V is very rare and causes CF in some, and not in others.

Her lungs sounded clear so the doctor told us to start CPT 2x/day to see if that helped. Her theory was that this wouldn't hurt her and COULD possibly help her. They did a chest xray which showed bronchitis of some sort but no pneumonia and the doc said her lungs sounded perfectly clear.

Well...two days later I get a phone call from the nurse. My daughter cultured Pseudomonas. I've read up on the Pseudomonas on this web site and google and have found out that she has the non-mucoid version, which is a good thing. The doc said she's not sure if she's going to dx my daughter as CF now or not, and I'm not overly anxious for any sort of classification...I'm just confused about the occurrence of Pseudomonas in non-CF people. The nurse told me it CAN occur in other people, but it's rare. I looked up F1052V on the CFTR website, and out of the 12 people with that mutation, 0 have cultured Pseudomonas. All of this information just leads me to believe that my daughter is leaning more towards to CF realm. And what are the side effects of Pseudomonas? Could this be what's causing her cough and congestion or is it simply a pro-active measure to prevent the Pseudomonas from colonizing into the mucoid type?

And lastly. How do you all balance treatment and work? The CPT and the Tobi treatments are going to be over 40 minutes each time. My husband and I both work. Any suggestions on how to balance all of this and still get to work at 730 AM would be amazing!

 

[Aboveallislove]

Dear Lauryn,
So sorry re the culture. It probably doesn't matter if they call it CRMS or CF (although it might for insurance purposes), so long as they treat her the same, but from what you wrote, I don't think they are treating her how a CF clinic would treat a CFer. It sounds like you weren't doing any CPT before, while CFers 2x a day is the normal minimum always and if having issues, it goes up to 4x. Also, you didn't mention any meds besides Tobi. Is she also on a bronchial dialator like Albuterol or Xopenex 2x a day? The pseudomonas does cough the coughing issues and the goal is to eradicate it because it can cause continued damage even if it isn't mucoid (of course the longer it is there it can also turn to mucoid I think???). So I guess I'd ask the doctor what they would do differently if they diagnosis her with CF and go from there. I think those with the "clear" diagnosis are strongly of the view you treat very aggressively and proactively to prevent damage that you can't see, as well as those you can.

Re how to do it: I have no idea how those who both work outside the home do. I barely handle it working part-time from home! But one thought: If you or your husband work for an employer with 50 or more employees (and have worked there for more than 12 hours and for more than 1250 hours in the past 12 months), you might consider taking intermittent FMLA leave so you can go to work later to allow you to be able to do the treatments and/or come home earlier. CF is a "serious health condition" and under the FMLA you can take the leave intermittently, meaning you can take 2 hours here or there as needed for treatments, doctors appointments, etc. You should also check sick leave policies as some companies pay for time taking care of sick kids, but if not they must give you FMLA leave (unpaid). Depending on your or your husband's bosses, they might be okay letting you do the hours on the weekends, etc. (If you or he took the full 12 weeks of FMLA leave for birth, then the other spouse could try to do it just for this hiccup). These are "general" legal points; obviously depends on the details, but if you want to try that as an option PM me and I can give more details.

 

[Rebjane]

Lauryn,

Alot of CF does have to do with prevention. All the questions you asked are valid and I would absolutely discuss your questions with your daughter's CF doctor. Are you going to an accredidated CF center? Has your daughter had a sweat test? At any rate, treating pseudomonas aggressively at first culture in a CF'er there is a chance to eradicate it. I am not well informed on CRMS. I can only speak from a CF point of view.

There is a protocol for trying to eradicate pseudomonas in CF. Talk with your CF doctor.

CF care is VERY time consuming. I have 2 kids and stopped working the first 4 years after my CF daughter was born. I now work very little(per diem). We have been doing this for almost 12 years and we still have trouble getting things done. My husband works a lot, travels a lot. So I am the one who does the day to day.

 

[lauryn.tubes]

Thank you aboveallislove and Rebjane for the comments. My daughter is tracked at an accredited CF center and has had 3 sweat tests. All were very negative (~16). Which is why I'm trying to understand the Pseudomonas and what they mean to my daughter. My daughter has two disease causing mutations, yet a negative sweat test and now Pseudomoas.

I do plan on asking my doctor all of these questions, all of this has happened over the phone so far. While the nurse is amazing, I feel that I need a face to face. Especially when it comes to figuring out how to administer the Tobi....

Thanks for all the information on CF Care. Right now, staying home is just not an option....i guess we'll have to re-evaluate after her next culture. Hopefully we can get rid of the Pseudomonas and go back to just CPTs 2x/day

 

[Beccamom]

I agree with eradicate the PA and I also share the concern your child is not being treated the same as she would with a CF diagnosis. I was diagnosed with CF as an adult and although I always had CF symptoms my lung issues became much more significant after I cultured PA. My 15 year old is genetically a CF carrier, but do to no diagnosis her lung function hit a low of FEV1 50% before you received appropriate CF treatment being a mucus clearance vest, Hypersal nebs, and antibiotics based on sputum culture. She has permanent lung damage from her mistreatment and damage to her adrenal glands from inappropriate use of steroids at 4 times the FDA approved amount for her age. I now question every doctor, discuss all options with my pediatrician, and then make my own decision about any medical care my daughter receives. As for how to handle treatments and working. I took FMLA during my daughter's sickest year and it saved my job. I caution against intermittent leave unless you are in danger of loosing your job. Each day of intermittent leave is deducted from the total 12 weeks you can take in a 12 month period of time. Who cares for your child when you work? Can this person or can you change to a day care provider that can do the morning treatment for you. While your child has a cough you should do the CPT 3 to 4 times a day. Maybe you can find a provider that would go to CF clinic and properly learn to do CPT. My mom helped with my daughter at her sickest and she has multiple Schlerosis, so she was not able to do Manuel chest PT and that helped us get our insurance to pay for her vest. A vest would be easier for a day care provider. I have tried all forms of daycare from center, to in home daycare, to neighbor, to family, to working part time. It can all work.

 

[lauryn.tubes]

Thank you everyone for the kind responses. I've been obsessively researching pseudomonas and it seems to me that an 8 month old is pretty young to culture this. Does that seem to jive with that you all know? And if so, does that suggest anything about my daughter's condition? Also, does any one know what the odds are for eradicating this bug on our first try?

 

[Aboveallislove]

My understanding is that by age 6 50% of CFers have cultured pseudo. It varies by child (and mutation). But my understanding is that with the proper aggressive treatment it can be eradicated. Also, I'm virtually positive FMLA intermittent leave can be in hour increments, and not eating up whole days. You could call the cff.org attorney to confirm if you are hitting the point where you just can't physically get it done and to work. Of course, it also all depends on your job and your boss as to whether it is worth it to try to do that. Good luck!

 

[Ratatosk]

With DS we started a routine from day one. CPT for 20 minutes before work, after work and before bed. We figured out what time we needed to be to work and what all needed to be done and worked from there. When he cultured pseudo at 3 months and got put on Tobi, we added 20 minutes to the morning and after work routine. While throwing another drug into the mix was a bit overwhelming at first, it was just one of those things we did to keep ds' lungs happy and healthy.

Most people do vesting or cpt twice a day when their kids are healthy and bump it up to 3-4 if their kids are sick. We've always done 3 a day -- vesting for 30 minutes each time. I get up at 6 and strap ds' vest on him and run take a shower. change out his nebs when I'm done. Then he gets breakfast and he's out the door for school. We get home a little after 5 and he straps on his vest and nebs. Supper, homework, bath and playtime. Then around 9 he does his last vest and neb for the evening. It is what it is.

 

[lauryn.tubes]

Ratatosk thanks for the response. AJ got her first neb treatment last night and this morning went off pretty well! She stopped struggling, squirming after 10 minutes, and laid there just staring at me for the last 10 minutes. I'm praying she'll stop struggling as she gets used to this routine

 

[Ratatosk]

DS struggled at first and we didn't start doing 20 minutes. We learned the different positions. Held ds on our laps/knees. He got used to it and it actually calmed him. He'd fall asleep or sleep during it. We gradually increased the times. Our CF clinic in the City was very proactive and stressed keeping the lungs clear/healthy. That even though ds didn't have a cough we should do CPT at a minimum of twice a day. The times he was a bit fussy and cried, we powered through -- sang songs, figured with those deep breaths he took to yell at us, he'd get the tobi deeper into his lungs. Was just something we knew we had to do. Sure I resented it that other parents got to take their babies home from the hospital and only deal with feedings and diapers, while we had a whole list of things to get done, but.... We got to take our baby home.

 

[lauryn.tubes]

AJ is doing very well with the routine. She doesn't even fight the nebulizer anymore! But the Tobi is really messing with her system, unfortunately. She's getting a nasty diaper rash and has constant diarrhea (I'm talking 4-6 times a day). She just had her normal 9 month check up, and has lost weight since her 7 month weigh in they also said she had croup. Sigh...it's one thing after another.

My daughter is dx with CRMS and I feel like it has impacted her immune system and made her more vulnerable to upper respiratory viruses (we're on number #3 in two months). I cannot imagine how hard this season is on the CF babies. Kudos to you parents for being amazing at what you do!

 

[Ratatosk]

It's weird that Tobi could be causing loose stools... DS has been on it since he was 3 months old and never had a reaction like that. Did with augmentin and cipro though. Could she be teething? DS would also get really loose stools right before popping out a tooth. As for the rash -- we used a triple paste specifically for people with incontinence issues. Was called criticaid. Formed a nice barrier that didn't really come off until bath time.

 

[lauryn.tubes]

That concerns me a little that loose stools aren't the norm for Tobi. However, a week ago, her pediatrician put her on amoxacylin and her reaction was much worse than the doctors expected to see with such a mild antibiotic. At least that's what they told me. I've been less paranoid about everything today, since the steroid the doctor gave her for her croup has made a mountain of a difference. She's only had diarrhea once today (instead of the usual 4-6 times) and her cough is nearly non-existant. Unless, the Tobi is just started to help with the pseudomonas infection. Could pseudomonas cause diarrhea?