Where to start

[Greta Cherry]

I am a foster parent and we have recently taken in a child with CF. He has only been with us for a just over a week. We are planning on adopting him. I knew nothing about CF and I am feeling a bit overwhelmed with all the information out there. When he came to us he was kinda dropped off with his equipment and meds and no real education on CF. I want to educate myself and I guess I am looking for the best place to start. We have an appointment with his doctor next month but I am not even sure what to ask. You know I don't know what I don't know. We finally got his treatments down (he came to us not on all of his treatments but his nurse got me on track). His nurse sent me a book Beginning CF care which did give me some of the basics however I found it geared more towards an infant. My child is 9. I also have other kids in the house is there anything I should be concerned with? I know kids get sick but again I feel so naive when it comes to CF. Any recommendations? Thanks.

 

[Aboveallislove]

First, congratuations on the new addition to your family! What a blessing for both of you!

What are the current treatments he is on? If you can list those and when/how you do them and the current care, it will easier to see if there is something of concern.

And have you had one CF appointment yet with him so you know what that is like?

Re the things to be aware of: If the other kids are sick, keep them away as much as possible. There are certain things that are more riskier for CFers, like playing in mud, puddles, jumping in piles of leaves, swimming in lakes which might have higher bacteria, coming in contact with stagnant water. Basically there are bacteria which are not dangerous to others but are for CFers. Those are the biggies where they grow.

 

[Greta Cherry]

No we have not had a CF appointment yet. It is scheduled for next month. He does 4 neb treatments in the am atbuterol, pulmicort, hyper tonic saline, pulmozyme and his vest. He also takes a multi vitamin and loratadine. In the pm he does albuterol, hypertonic saline and then his vest. He also takes Zenpep with food. And thanks we are real happy he has joined our family.

 

[Aboveallislove]

two other quick questions and then I'll try to respond more after I get our DS done.
1) Do you know what mutations he has?
2) Do you know what bacteria he is culturing?

 

[Aboveallislove]

Here are my thoughts/insights, for what they are worth:
1) The meds/treatments all sound perfectly like he is being treated at an appropriately aggresive center, meaning giving the "standard of care" for maintenance. The pulmocart I am not familiar with but googled and it looks like it is for asthma, so I guess if you aren't aware, I'd ask if there is an asthma component. Re the treatments and vest: Important that they are done daily, no matter how he feels, etc. The compressor for the nebulizer and the vest machine both have filters which need changing every 3 - 6 months. You might consider asking for new filters to change now since you probably have no idea when they were changed last. The nebulizer cups need to be washed after every use and sterilized every other use. Have they gone over that with you? If not, I'd call the nurse and ask for info and/or check the "instructions" that came with it. Make sure you are using "durable" nebulizer cups and not "disposable ones" the later of which cannot be steralized. Pari LC Plus is a very typical one that is durable. We order/pay for extras so we can sterilize and have ready several at a time.
2) The typical CF schedule is quarterly CF appointments and certain things done annually. I'd ask at the quarterly where he is at for annual tests and whether they should be run now. annually they do blood draws to make sure there are no vitamin deficienices. And also lung xrays to check for structure changes. I'd also ask when they start testing for CF related diabetes (CFRD). I think it is 12- 13, but just so you know when that testing starts. They also might do more intensive lung function on an annual basis--our son is too young for that so not sure. Re the typical quarterly appointment. A tech will take vitals and do height and weight. They want a BMI of 50%, but don't start pushing food or it could backfire! A tech or nurse will review all the medicines and see if you need refills. A social worker and/or psychologist will likely come in check on you. The nurse will do a throat culture to see what bacteria he is growing. Some are fairly harmless (staph, hemophilo-influenze? sp??),others can be pretty serious. The "harmless" ones they usually don't treat until he gets sick/has an excerbation. Others like pseudomonas they try to erradicate. You'll get the results in 3 - 5 days after the throat culture. You'll see a dietitian as well who will likely discuss meals/food, vitamins and enzyme. The doctor will then come in for an exam. If he has GI issues, he may also see a GI specialist. (But given that he doesn't take Miralax, Zantac, or Prilosac, he might not have any. Which leads me to remember to let you know that "constipation" for CFers can be a series condition and not constipation, so I'd find out if he has any history of issues and also just that you are in the loop with his "stools." They ask about "stools" at the CF appointment so that might be a time to ask your foster child how he wants to keep you updated on those or ask the nurse how children typical do it at the age of 9. They do lung function I believe at the quarterly but our son is too young so I can't help with that.
3) I would think there would be some issues that are "normal" for foster kids to face, which take on an added dimension with CF. I LOVE the book parenting chilren with chronic health conditions by Lisa Greene. I'd strongly recommend you get a copy of that, but also to schedule a separate appointment with the psychologist if there are concerns, etc.
4) Finally, when he gets sick, any time you are unsure, call the CF clinic. That is what they are for and they can help you assess what is going on.
5) Oh, I guess the final final is that CF is caused by having 2 CF mutations and there are more than 1000. I'd find out what mutations he has. The most common is d508f. If he has two copies of that, that is good to just know in the back of your head. IF he has another other mutation, then please post it and ask for insight because there is a drug called Kalydeco which can help "fix" (truly a miracle drug for some) those other mutations. Hugs and prayers momma!

 

[Greta Cherry]

Thank you so much for all of the information. You have definitely given me some things to think about and how to start formulating my questions for the cf clinic. I will take a look at the book you suggested. Again I can not thank you enough for pointing me in the right direction.

 

[Aboveallislove]

Please let me know if you have any other questions. Trying to push this up too for others to give their insights!

 

[Printer]

Great job on this one love.

Bill

 

[Aboveallislove]

Oh, meant to add: The pulmoyzme MUST be in a new nebulizer cup--you cannot use one that you just used for the other medicines.

 

[4hats]

I think this link will be very helpful to you http://www.cff.org/UploadedFiles/tr...roduction-to-CF-for-Patients-and-Families.pdf

 

[JENNYC]

I wanted to add my congratulations!!! I think Aboveallislove covered everything beautifully!! I think knowing what I know now, I would want to know all I can about his previous medical history (any surgeries, hospitalizations, cultures) Basically what your new CFer has problems with as every one is different. And it can help you assess illness in the future. For instance my little one has sinus issues, where others have tummy issues. I worry when mine gets sniffles, and other worry when theirs has stomach pain. You need to know what to look out for I guess is what I am trying to say. And always remember if you have questions always feel free to post and more than likely someone can give you some insight. I love this group, they are very helpful!!! Or if you just need to vent. Welcome to the group!!!

 

[albino15]

Just wanted to say that that info was awesomely specific, described my CF appointments almost to a T. He's 9 right? So he should be doing Pulmonary Function Tests (PFTs). At least I know I was doing them at every appointment before that age.

Oh, and to quote Printer: "Make sure he is being seen at an accredited CF care center." If he isn't already. LOL

​

 

[Aboveallislove]

Just want to RESOUNDINGLY second Jenny's point above. I hadn't even thought of that, but it really is imperative you get a full history--they can easily print out everything (I'd suggest you have that done and faxed/mailed to you know before the appointment so you can review and ask better questions.) We had DS's entire weight/height and culture results all printed and faxed b/f our "normal" ped. 3 year old check-up so we could go over that. I'd see if they will print and send the file if possible, and if not, at a minimum: 1) height/weight; 2) medicines; 3) cultures; 4) hosptilizations/ER/sick visit to CF clinic. As Jenny noted, every CFer is different, but every CFer typically then repeats the problems. Jenny and I "chat" often and DS has horrible tummy issues and as she noted, her DD has sinus issues. In a way we're talking a foreign language to each other b/c while both our kids have CF, the issues they have differ so greatly and as she said, she wouldn't think twice about a stomach ache, whereas, every little stomach ping has me worried about another ER trip. So having this history will really help.

 

[Greta Cherry]

Thanks so much.

 

[Greta Cherry]

I want to thank everyone for your thoughts and all the info. It has been an education to say the least. I took him to the doctor today because of a sore throat and the beginnings of a cough, only to find out he has a double ear infection and croup. I was worried I was over reacting taking him to the doctor since he did not have a fever. With my other kids who do not have CF I would have waited to take them. I am glad I listened to my gut. He went to the pediatrician, should I notify his CF team or not? I forgot to ask if his ped's office forwarded that info. Oh and for the person asking about his clinic he goes to the one at UVA. I finally feel like I am getting into a routine. He has been with us for just over two weeks now. Thanks again for your suggestions.

 

[Aboveallislove]

I'd call the CF team b/c my understanding is they like them to be on antibiotics that can work on any bugs they have cultured, especially with croup. (I'm not positive b/c DS hasn't had any infections yet, but I had asked early on and was told something like that. . . not that I have a good memory of the first 3 months post diagnosis! ;-). Seriously at this point do not worry about bothering anyone. . . they have "standard" I think for new CF moms which entails lots of calls!! I actually call the CF team before Ped b/c they know all his meds and whether it could be a CF issue or a reaction to the meds. For instance, brown pee a couple weeks ago, I called on call CF doctor first to make sure not a CF kidney issue and then called ped's on call to see what they wanted to do, once the doctor said it wasn't a CF kidney concern. So I'd call tomorrow and let them know status and the antibiotic and ask if they want you to switch antibiotics and also how they'd like to handle in the future . . . I'm vaguely recalling that they wanted our ped to call them re the antibiotic??? Hang in there momma!

 

[Greta Cherry]

I will call them in the morning. Thanks

 

[JENNYC]

Wanted to chime in...our pediatricians were always very sheepish with the dosing of antibiotics and our CF docs always said oh no no no...have to take a much stronger dose than that...and they were always right in our case. When Abby doesn't take the right dose it may fend it off but not get rid of it. So I would most definitely talk with your CF team. I rarely ever take Abby to a normal pediatrician just because our Peds around here do not deal with CF and so usually end up just guessing instead of knowing what to do. May not be the case with you but just wanted to give you the heads up just in case. Poor baby, can't stand it when our angels are sick.

 

[Greta Cherry]

I called this morning to our CF team and they felt the meds he was put on were appropriate. Our CF clinic is over two hours away. It sounds like hopefully the peds are on top of things. He has been going to this practice for many years and they all seem to know him well.

 

[Beccamom]

I 2nd the opinion on always call the CF team about any antibiotics. Peds tend to use a much lower antibiotic dose for the weight/age/bacterial infection then our CF teams (daughter's and mine) recommend. I also have a concern that some of our anitbiotic resistence (bacteria no longer respond to certian meds) is due to too many low doses followed by more potent meds. Also knowing what your child cultures can be key to choosing the right meds the first time. I made the mistake of not calling the CF team about an ear infection antibiotic since it was not a lung infection. This led to two ruptured ear drums for a 12 year old and 3 anitbiotics ending with an otoxic antibiotic. Had the infection been treated more aggressively 2 weeks early when we saw the ped the ruptured drums as well as otoxic antibiotic may have been avoided.

New mom please don't hesitate to call the CF clinic. That is what they are there for. As for the clinic being 2 hours away I find if I call the clinic first they will call the ped and give directions such as do a sputum culture or what anitbiotic and what dose. We do go to the ped for a listen to the chest and pulsox monitor with the CF clinic's oversight sometimes. Our teams work well together and I am really greatful for that.

I called this morning to our CF team and they felt the meds he was put on were appropriate. Our CF clinic is over two hours away. It sounds like hopefully the peds are on top of things. He has been going to this practice for many years and they all seem to know him well.