When to call?

[lauryn.tubes]

Good morning. My daughter has been diagnosed with CRMS. She has df508 and a mutation with varying consequences f1052v. Apparently f1052v causes CF in some, but not all it's patients. So my daughter (8 months old) is tracked at a CF accredited center and has been doing well with no symptoms to date. She got a scary high fever last month and our pediatrician instructed us to take her to Children's Urgent Care. This center is affiliated with her CF doctor's and communicated with them to ensure she was being tested for everything she should be. The doctor said she had a diminished lung (meaning it didn't sound as healthy) but her xray showed no pneumonia etc. They snot sucked her and her lungs sounded better. All her CF cultures came back clean and green.

Well...now a month later and my daughter is still sick. No fever anymore, but shes still coughing. A lot. I actually think the coughing has gotten worse. I don't want to constantly be taking her to the doctor if she just has a cold like any 8 month old, but I'm wondering if there could be more going on. Just looking for advice as to when to take my daughter to the CF doctor. Her breathing also sounds very...different. I'm not sure if it's wheezing but I hear a high pitched..whistle sound? I feel like I could be blowing everything out of proportion because I'm just so worried about her all the time.

Just looking for some advice from people more experienced in CF symptoms. Does this sound like it's doctor worthy? Or something we should wait out a little longer.

Thanks for all the advice upfront. I really appreciate the support of this forum.

 

[Ratatosk]

Did they test her for RSV? When DS was that age and RSV was going around, despite getting the synagis shots for RSV, he did develop a wheezy cough. Our CF doctor told us to "beat it out of him", meaning increase CPT from 3 to 4 or more times a day. We were also supposed to call his office if he developed a cough because his baseline was no cough --- usually if he developed a more bronchial or chesty cough and based on past cultures, he'd prescribe an antibiotic. I too felt as if I was blowing things out of proportion, especially with our local pediatrician once told me "they cough, it's what they do", but then ds ended up at the cf clinic with bronchitis. As a parent, we know when something is off.

 

[lauryn.tubes]

Thanks Ratatosk. They didn't test for RSV. I think I'll just call the nurse and ask for her opinion. I'd rather be a nuisance and have nothing be wrong, than my daughter be sick and not be proactive about it. Thank you very much for the response. Makes me feel better to know I'm not the only one who struggles with this!

 

[Aboveallislove]

I'd call and see if you can get In to cf center doctor. When ds was a couple months old he coughed and being new to it I called and they had us in in a day. In retrospect it was acid reflux, but th cf doctor started hm on pulmozyme which also I retrospect I'm glad fr. You didn't mention treatments but they also had us up to four times a day his bronchial dilator xopenex and his percussion chest therapy and frankly it was nothing like you describe. Good luck.

 

[TreasureGoddess]

Any time something just doesn't feel right, you have every right to call in. I'd suggest setting up an appointment with the CF team and letting them know you're uncertain if it's a cold or not, but if it's something more severe, you'd like to have an action plan. If you don't currently have a regimen of nebulized medicines (pulmozyme is fantastic for helping lungs function well) and/or airway clearance cupped hands pounding her chest, etc, you may want to talk to them about setting something up. Whether or not your kiddo is officially classified as having CF or not, if she's having issues breathing or wheezing or coughing more, you absolutely want a way to help her feel better and to get those lungs clear. I spent a lot of years letting doctors and nurses make me feel silly for calling in or coming in for multiple appointments, and now that I have a GREAT CF care team, I'm comfortable saying "something doesn't seem right" and they see him. I wish I'd have been more proactive when my son was younger. Don't doubt yourself, you're doing a great job being proactive for your child's health. HUGS!

 

[Printer]

Even at my age, my Doctor yells at me for not calling him when "something" is doing on.

If you think that you should call, then call.

Bill

 

[Beccamom]

I got yelled at by my CF for sending a non urgent email verses calling about coughing up blood. My wonderful CF nurse put it well. Yes coughing up blood is a CF thing but CF things need treated. Do not hesitate to call and just he honest and say you are worried and not sure when to call but this is what is going on.

 

[LittleLab4CF]

We used to have our doctor's home number and on occasion, he came to our house. This was also a time when doctors were exalted as gods and nobody wants to interrupt god's golf game for trivial matters. The fact of the matter is, medicine is a 24/7 business and though I was too young to remember my being bundled up in the middle of the night to meet our doctor at the hospital, I remember my sister's croup and the dozens of times she got the late night ride.

CRMS or Cystic fibrosis Related Metabolic Syndrome is a wait and watch group for developing CF issues. In other words, a CRMS diagnosis could become a CF diagnosis depending on what health issues develop and their severity. Anybody in the position of raising a CRMS child is looking for developing issues, by definition. By starting with the more conservative CRMS diagnosis, a lot of assumed problems like pancreatic insufficiency aren't blindly treated. But, CRMS doesn't have a timer attached to it. If a child is showing CF issues, they are treated promptly and aggressively.

In early child development, the point when the lungs mature could coincide with the development of CF pulmonary issues. Strictly from a genetic standpoint, lauryn.tube's daughter easily could have been diagnosed with CF instead. It ultimately comes down to the health issues of your child. Her genetics may or may not present CF but it sounds like issues are surfacing. I don't know exactly why your doctor chose CRMS over CF. It could be adherence to classic CF genetics where two identical mutations known to cause CF are required for a diagnosis. Or your child didn't immediately develop classic CF issues, or they were "mild".

It may sound like punishing your doctor for her/his diagnosis but CRMS/CF in an infant is going to be a high maintanence endeavor and neither your doctor or you can predict the future. A CRMS diagnosis could change to a CF diagnosis at anytime or never. And infants develop so fast that you can almost watch changes going on before you.

This accelerated stage is easy to overreact and react too slowly to problems. Trying to second guess your child's doctor just introduces a worry that isn't needed, by anybody. I may be part of a very small minority having studied CF prior to being diagnosed with it. It is difficult enough to observe and diagnose gas verses colic in a baby who hasn't learned to talk. Issues as simple as snot become the subject of considerable and protracted discussion, for good reason.

When do you call your child's doctor? My best answer starts with acquiring some home medical instruments. A thermometer is vital and usually needed to answer the doctor's first question. Always measure the left ear if an ear thermometer is used. Measure both ears at first to make certain your baby is typical. The left side is warmer above the heart and usually measures a fever sooner. An ear infection can be indicated if you know what's typical for each ear and you're not getting typical values.

In all cases, measure of temperature will give you and the doctor important data and a sense of a good reason to call if she's running a fever. Her doctor will suggest a minimum fever for concern. A Pulse-Ox, the thing that the nurse puts on her finger, is essential (for CF or CRMS) for measuring her oxygenation and pulse rate. Oxygen levels again can be worked out with the doctor. Generally speaking, 92% and lower is worthy of concern for a healthy person. Pulse rate is a good indicator of how much stress your little patient is in. Pulse rates can be high with the stress of fighting infection. It can drop if the pain level shoots up. In this case the slower pulse is heart pound, a strong beat. Low or high pulse that's thready and soft or weak is urgent.

This segues into a sphygmomanometer or a BP cuff. To be honest I've never seen a cuff for infants outside of a doctors office. At 64 and having Parkinson's disease on top of CF, a cuff is important and useful. I suggest it for the long term record keeping. If she needed monitoring, her doctor already would have prescribed a cuff. The bouncing bar graph on her Pulse-Ox can tell almost as much.

By studying the bar graph, it goes up quickly and drops to a certain level before bouncing up again. The highest bar reached is analogous to the systolic blood pressure and the lower bar reached is diastolic pressure or the 70 in 120/70. I have a harmless "murmer" that I can see in the rhythm of the Pulse-ox bar graph. On occasion I have a double beat or extra systole. When it occurs, the bar graph peaks and begins to drop, peaks again and drops to the normal diastolic level. What I am saying is a thermometer and a Pulse-ox are primary diagnostic tools for your doctor and for you.

Being prepared with your daughter's vitals will validate your reason for calling and inform the doctor with essential data. Put on your doctor hat and write down the observations that have you concerned. The reason for calling a doctor is concern. Sometimes it will be gas or it could be a life threatening blockage, in either case you need to call. If you're organized and concise, the doctor can't fault you and most likely will appreciate it.

You may need to sit down with your daughter's doctor and define bright lines like at what temperature of fever is a point of concern. Hydration, dehydration and growth all fall into CRMS and each one is a measurable symptom. Maybe discussing the topic of when to call will help. Just asking the question will esuage most doctors fearing you're going to hound them. It's not the end of the world for a doctor being called for something that wasn't an emergency. Fairly quickly certain emergencies will reappear and the solutions repeated.

Welcome!

LL

 

[lauryn.tubes]

Wow. Thank you everyone, so very much for such supportive and informative answers. I did call the CF nurse who tried to get us in, but could not. She referred us to our pediatrician. Our pediatrician prescribed an antibiotic and stated that my daughter's lungs sounded clear. Unfortunately, the antibiotic has caused severe diaper rash/chemical burn on my daughter. We've gone back to the doctor and gotten a prescription diaper ointment for her.

The CF nurse called us back and asked for us to come in on Monday. However, my daughter did sleep last night. The first time in a month she hasn't been up all night coughing or sneezing up insane amounts of mucous. So, I think the antibiotics are working, which settle my worries that CF could be showing itself.

The CRMS diagnosis is a hard one to manage. I am hyper-sensitive to all coughs/mucous symptoms in my daughter and I sort of feel like my mama intuition is a little flawed because of this. However, I think I've decided that I'd rather call the doctor/go to the appointment and have nothing be wrong...than to NOT call the doctor and cause further damage to her lungs/health.

And just to answer the question as to why my daughter was dx w/ CRMS instead of CF: she has two mutations, but one of the mutation is "of varying consequences". Apparently, it causes CF in some, and not others. That coupled with the fact that her sweat tests have been very low (14-16) gave the doctor comfort in dx CRMS. From my understanding, there's only 12 known people with f1052v and only 4 with her exact mutations (df508 and f1-52v) in the database.

Thanks for entertaining a worried, overwhelmed mother! Can't tell you all how much I appreciate it!