How do you know when you need IV antibiotics?

[triples15]

I don't have any input, but did want to second Gammaws about the pseudo,.. that that is usually treated immediately and hard to eradicate. I'm thinking they might though think given the late diagnosis that he's had for a long time, but even then...

I did though have a question for Autumn and Gammaw as this is something I don't have experience with and it might also be important for mom to know: Is the reason to head to ER as oppose to the CF clinic arranging for an ADMIT, is because the ER can tell if it is pneumonia or something? And couldn't the on call CF doctor just order the admit even on the weekend and even without knowing, as oppose to going to the ER?

Hi love!

Yep, as Gammaw said, my CF clinic is not staffed on weekends or holidays. The only person you can get a hold of is the pulmonary fellow on-call. If you are very sick and feel like you need to be seen they will advise you to go the ER. They wouldn't arrange admit or anything. I've only gone to the ER at my center (well, now it's my previous center) once late at night and all they did was page the fellow and then kept me basically on "observation" status until the CF team rounded in the morning and decided what to do with me. So by the time they ordered my meds, got me a room, and got everything started it was late the next afternoon (basically evening) before I really got much treatment. Ugh. It's SO much easier when it's pre-arranged by clinic. They have a room ready, meds ordered, your treatment plan in place. Going in through the ER really really stinks, but on weekends or holidays it is the only option if you're concerningly ill.

Hope that helps!

Autumn

 

[triples15]

Autumn:

I stand corrected, I should have just said 14 days of IV's. As someone on Medicare, it is 14 days inpatient.

My feeling is that these two young men are still seeing local Doctor's and not having any connection with a CF Specialist.

I was dx at age 47, when there was only one CF Specialist at Massachusetts General Hospital and his work load was to great to add me as a patient. I know what it is like to be treated for CF by a "local Doctor" and this looks like that to me.

Bill

Hi again Bill!

Oh yes, Medicare doesn't cover home IV's, so 14 days (or longer) inpatient would be the case. I really think Medicare should cover home IVs, but that's a topic for another day.

I completely understand your concerns about whether or not they are being seen at an accredited clinic. I share your feelings regarding "local doctors", and believe if CFers are being seen by a general practitioner they should run, not walk, to an accredited center. It does sound to me though like these 2 boys are being seen at an accredited CF clinic, unless I've completely missed something.

Autumn

 

[triples15]

Thanks for all your caring replies. I'll try to answer all the questions in turn.

First -- he felt somewhat improved today so I did not take him to the ER. He is taking Musinex and Cipro, and of course Pulmicort. The head CF doc at his center has seen him since he was 18 (prior to that he was at the child center). The doc prescribed the Cipro, and left it at our discretion whether he was sick enough he had to go to the ER. That was the motivation for my post -- I wasn't sure what "sick enough" looked like. I have CFRM and when I was his age I would get sick frequently and go to the ER. They would always just give me IV fluids and send me home. Now I have a *serious* phobia to IVs after all those incidents. I was also on antibiotics for a lot of my childhood and on prednisone frequently in my 20's. Furthermore both my children have CF so I just don't know what normal sick looks like. My older son had a cough for years and I took him to every type of doctor, only to be told in the end that the cough was "behavioral". (He has a history of autism as well as the CF).

Thank you all for your concern about the Pseudomonas. i am very concerned also, and shocked that we were not told about it. I can't help but think that there may be some downplaying of his CF because only one gene has been found despite sweat chloride levels in the 60's-70's.. I have not been going to his CF appointments but will go to the next one to advocate for better care. He can have trouble navigating some social situations, and I think this shows he needs me there.

Aboveall -- The doctor did not give him the choice of going into the center to admit through the center. That is because it is a holiday. Last time DS was this sick they saw him at the CF center and they gave him the Cipro. Then I had him move back into my house for a month so I could monitor. Now he is back living on his own.

Windex -- he can't take Bactrim. Last year he developed a serious adverse reaction to Sulfa (face and mouth puffed up like a balloon.)

Hey there again Fel,

I'm so glad he is feeling a bit better, hoping it continues! Hey this post brought up a big question for me...... am I understanding correctly that the only med he is nebbing is Pulmicort?? If so, I would REALLY question the reason behind that given the diagnosis, and especially with this cough he's got. If he's not already, I feel like he should really really be on hypertonic saline, and maybe even considering Pulmozyme. Can I ask what his baseline is? Does he always cough? I think at this point if he's not on anything other than pulmicort it's time to push for hyper-sal, or even 3% saline at the least. Something else to moisten up his lungs and help him cough stuff out while he's vesting. I think that could help A LOT. I don't mean to be pushy, but I'd ask the doc if he could start saline ASAP! Again, all of this is if he's not already using it. Hyper-sal, and normal saline are both VERY safe (salt water), and pretty inexpensive. So I can't think of a reason a doctor would not be on board with this.

The other thing is that I definitely used to get sick more at back to school time, and almost every October during season change... I think the back to school stuff is just catching the usual bugs that float around in the germ infested school environment. Most other kids just shake it off in a matter of days, but for us it can lead to full blown exacerbations. I agree with others who said to maybe try to time Tobi for those months, or stay on top of it and ask for an oral antibiotic at the onset of symptoms, even if it seems to "just be a cold", because for us it so often turns into something more.

Hang in there mom! Keep advocating for and taking good care of your son. Please keep us posted and let us know of more questions.

Autumn

PS. I agree with everyone that it sounds like someone dropped the ball on treating pseudo, and hitting it hard and aggressively would be the ideal approach. I'm sorry that the system failed you on that. I hope you are able to get Tobi asap.

 

[fel]

Thanks again this has all been very helpful. I will pass along the information about Active Cycle Breathing at night, although I am not sure what that is so I will need to look it up. See -- we missed the education on what to do in all these circumstances. My son doesn't even know really how to clean his nebulizer properly -- no one every showed him, and I keep reading different recommendations on the internet. It is all very confusing.

Bill -- Both my sons are seen at well-known, accredited CF centers. But I think their care has been somewhat confounded by their mixed profile -- both have sweat chloride > 60, but are pancreatic sufficient and they both have only one allele that has been found in a full genetic work up. I think the docs may not take their CF that seriously. With my younger son, the doctor told him he did not need to do airway clearance because he only had sinus issues and mild asthma, then 6 months later his lung function went down over 15% and the doctor asked him why he wasn't do the Accapella twice a day. Yet if you look at the medical records it is clear he wasn't ordered to use the accapella. Very confusing.

This is why I worry about all the people on this list who get the "in between" diagnosis. I think it is fine for someone my age (late 40's) to be in between because I can figure out things for myself, and because I truly don't make the full criteria. But when the docs tell a teenager that some treatment is optional or a condition is "not that serious" it is very very difficult for parents to then convince them to do hours of daily treatment for a life-threatening condition.

 

[Aboveallislove]

Did they definitely order and run the full gene sequencing? Sorry...if I'm asking a duh, of course question.
i too share that kinda diagnosis dread...
im wondering also what treatments he's on? I complete concur re the hyper sal and pulmozyme and of course xopenex or another bronchodilator which he'd need before hyper sal...but I'd think he's on that already...but if not...a ugh. This has to be hard on someone who didn't grow up with it being their normal and must be so hard on you too. Also if they are pancreatic sufficient then Kalydeco should work so I'm wondering if you can try to get the doctor to try off label given his current problems?

 

[fel]

So as an update he was admitted to the hospital today. I think he was getting worse because he refused to leave bed even to get a drink, and was rather dehydrated. Then he fell down his stairs because he was so tired and thirsty. As soon as he was on an IV and getting fluids at the hospital he felt a lot better. (He has a history of fainting from dehydration). The x-ray was clear, but they are now *** finally**** treating him for the PA (which it turns out he cultured in early June!!!).

Yes Love my whole family has been sequenced. We are part of a genomic study of unexplained (atypical) CF at JHU because the scientists can't figure us out. So far 1.5 years after sequencing the full genome they still can't figure it out. The short story is both my sons (full bio siblings) have sweat chloride above 60 but they don't share any CFTR mutations (they have different ones).

 

[Aboveallislove]

Thanks for the update. Hopefully the treatment can eradicate the PA and he'll be back feeling 100% soon. What a horribly stressful weekend for you all. I am so sorry.
And my goodness you do have a complex case with the genome.
Please know of my prayers for all. Hugs,
Love

 

[Gammaw]

I'm glad your son's getting more intensive treatment at last. I suppose knowing how to treat unusual genetics is difficult. But I would think culturing pseudemonas is .....well, still culturing pseudemonas. Treat it. So it's hard to understand why it was not done. Since it didn't even show up on your portal, I wonder if the CF clinic even saw it? The point is he's getting treatment of course. I would still want to uncover the glitch to ensure it doesn't happen again.

As for your sons' unusual genetics. Fascinating. An ongoing mutation? Evidence that the genetics are critically influenced by other factors? Wow fel. I can't imagine. But I learned a long time ago that the exception to the rule is what needs the closest examination. Ultimately it will define the rule. Your unusual genetics may one day help break the code.....Truly fascinating.

 

[Printer]

Fel:

I was dx at age 47 (1987) but only only mutation was found. It was about 2005 that they found my second mutation. It was recently discovered within 7 families in Northern Ireland. My 3rd Great grandfather left Ireland sometime in the early 1800's. The Delta F508 came from my mother and it turns out that my Father had 2 milder and rare mutations. Had my Mother had a second (mild) mutation, all of my siblings would have CF but that mutation may not be able to be discovered. In 1987, only 250 mutations were known. Today almost 2000 mutations are known and the list continues to grow.

Bill

 

[fel]

So he is still in the hospital until at least Monday. He is feeling very tired on the IVs. Is that normal? Is there something in them that makes one sleepy?

Yes Printer I think we have an undiscovered mutation or two-- perhaps I have one and my husband has a different one My husband and I each have a known mutation, but our children only received one of these each (different ones), so according to the genetics testing both should be carriers. When I first heard that 2 out of 2 of our children had CF I thought we were very unlucky, but now I think that even if we had 10 children they all would have had some form of CF. My older son with one missense mutation (and presumably one unknown mutation) is sicker than the other son with a more mild mutation. But both are pancreatic sufficient and they have their vas deferens. So these unknown mutations must be partial...

Alternatively we have some other process that impairs CFTR, and both children are carriers for CF plus have this other compromising factor.

 

[lilro]

This is not necessarily a one-size-fits-all answer, but I'll tell you what I do. I purchased my own Pulse Oximeter from amazon (they are pretty cheap, $20 or so), and whenever I'm feeling sick and pill antibiotics aren't helping, I check my o2 saturation constantly. If it drops below 90 for longer than an hour or so, that's when I decide I need to go in.

 

[Ank]

I cn feel d pain.Sorry

exacerbation means having excessive sputum,infection..Since u r taking vest 3 times a day ..That means u r having hard time.
u need to get Inter veinous antibiotic ..
Inhaled tobi will not help you if u having excessive cough..

Tobi is given as daily inhaled dose when u dont hv exacerbation

there is no hard and fast rule that when u should iv antibiotics ..i take iv antibiotic when i feel that i m nt able to breath..or carry my activities.. ..generally i try to avoid iv..and only take when situtation becomes unberable
( taking iv antibiotic (2-4) times a year is normal)

Antibiotics is given for a period of 15 to 20 days..

Iv antibiotics take 4 days to show effect..



During exacerbation a extra protein might help(my personal opinion)

Most of cfers are iron deficient..,which makes breathing more difficult...

 

[GoryLori]

My husband kisses me and he can TASTE the gram negative bugs (Pseudomonas aeruginosa) on my lips that CONFIRMS I need to go in. By that time, I am already on a decline and know I have to go in, but he backs me up. My docs laugh at it, but he's always right!! When you can't sleep through the coughing and this is NOT your normal day to day CF junk, time to go for a tune up. I usually try to do an admit through the front doors as opossed to the back ER doors, but however you get your foot in the door, DO IT!! Don't wait around.........

 

[Deb]

As far as knowing when he is sick.....trust your instincts. I can tell you from my past, whenever I was sick I was usually in denial in the beginning (those first few days) However, my husband and children quickly learned to pick up on cues that I had more than a simple cold virus. According to them: I slept more, I became irritable, and they could "see it in my eyes". They were almost always right that I had something going on and was headed for an exacerbation. You know your sons better than anyone so if you see cues don't be afraid to step in. Better early than late.

 

[occupyjapan]

For me, I can tell when I need antibiotics (which I haven't since starting Orkambi; yay!) but there's not really an "oh, I need IV's this time" thing. We do orals first. If they don't do it, I get IV's. I've only been on IV's maybe 3 times in the last decade+.

When I can tell I need antibiotics, my mucus is darker, I cough more, shortness of breath from activity that wasn't causing it before, headache, congested nose, fatigue, faster heartbeat (my oxygenation is always stable at 98-99 but when sick, my heart has to beat faster to keep it there I guess)... pretty much just feeling sick and all that comes with it.

 

[fel]

So seems like a time for an update on this thread. He got out of the hospital after 8 days and went on Tobi. Did somewhat better but not really 100%. Now it is looking good for him to get Kalydeco for an off-label trial. Wish us luck.