Need help and ideas for my toddler - having a rough time and on 4th hospital stay

[Helenlight]

I'm looking for families and adults who might be able to relate to our situation and offer anything that might help us along.

Our girl is 19 months old, DDF508. We have had real trouble keeping her lungs clear and each hospital stay she gets better for a few days and then gets clogged up again. The doctors seem a bit mystified and can't give me any solid answers, they just keep us in for weeks trying things that don't seem to do much while she picks up hospital bugs. It can be so frustrating!

What I'm wondering is- is this happening to other people? Did this happen to you or your child and they improved as they got older and got into exercise and other treatments? Or did it stay the same or get worse?

Our girl has some allergies and signs of asthma- could some of the mucus production be because of this and not necessarily due to an infection?

Currently at home we do:

Physio x2 daily
Creon with meals and breast feeds
orange puffer x2 daily
blue puffer x2-x3 daily
salt in food and with enzymes (in the puree)
fat soluble vitamins
some physical games (running around etc) and tickling etc

She has been trialled with hypertonic saline but it made her too wheezy
Pulmozyme is too difficult to get unless we are staying in the largest hospital in the country (Auckland- we live in Wellington NZ) she should be eligible for this when she turns 6
She generally cultures Staph A which gets treated with augmentin, cefalexin, or ceftazidime (she reacts to fluclox)
Pancreatic insufficient. Bowels seem fine, usually gains weight when her lungs are doing ok, but struggles to gain much otherwise. This time around she is being given Elecare via nasal gastric tube at night with minimal effect because it seems to have put her off daytime feeding. She's 9.5 kg (20.9 lbs)
She had an early lung infection at 5 weeks old and one specialist thought this maybe a reason why she has struggled since.
Prone to eczema and intermittent wheezing. Allergic to eggs and sensitive to other foods and substances but they are difficult to pinpoint.

That's all I can think of for now. Any help would be much appreciated because I don't know what to expect or what other clues/answers I could be looking for.

Thank you !!

Oh- one other note: her dad is a US citizen so she will be eligible for a passport and probably citizenship as well, so we will consider moving if it would be of considerable benefit to her. Probably to NY since that's where her dad is from.

 

[jshet]

Have you considered coming to the US to have her evaluated at a cf accredited center. It might not be a bad idea to have her evaluated to see if there are any better treatment options for her. It sounds like she has had a rough road so far and it may be in her best interest. I will pray for her and your family. Good luck.

 

[Murphysmama]

I'm so so sorry to hear about the rough times. My ds is 2.5 and has been on pulmozyne since he was 1. I think it really helps keep his lungs clear. His doc off label prescribed it to him and all the insurance wanted was a preauthorization. We also do a daily probiotic and run hepa air filters. We also really encourage the outside play. I think the cardio also helps a lot with clear lungs. None of these are silver bullets but just a couple ideas. I know it depends on insurance and doc but our pulmozyne shows up delivered every month and I really think its a big part of his good health so far. I'm not sure how common pulmozyne is for the little ones across the board in the us but the option is at least available. Best wishes for a speedy recovery! PS - Have they done a blood test to isolate allergies?

 

[Ratatosk]

When DS has been sick, we've usually increased CPT (physio) from 3 to 4 times a day. We have a vest; however, when he has issues, we also do mini-cpt treatments while he's sitting on our laps to focus on the areas of concern.

 

[Kaethe108]

We had to increase therapy recently and it did wonders for our daughter's lungs.
We do 4ml of HTS (6%) morning and evening. Pulmozyme at midday. Lots of exercise on top (blowing games, supporting breathing etc. ).
Did you try to increase the percentage of HTS slowly?
we started with mixing 0,9% with 3%, then switched to 3% and always slowly higher.
If she reacts getting tight in the chest, you need to combine with Sultanol.
Until 3% HTS you can mix it together, but you should not mix it with 6%.

 

[MotherofCFBoy11yrs]

Hi Helen, if I were you I'd organize a visit to the Auckland hospital and see if they have better ideas. You could explain you are worried and that you'd like to see if your daughter could get more help if you lived there. In this way you could get their opinion and consider your options.

If I've understood correctly your daughter has been on IVs repeatedly without any nebulized antibiotics? If that's the case I think it is pretty backward. For example ceftazidime can be inhaled. Nebulizing is safer than IVs and more effective with infections in the upper airways. And it can be done at home - I think you're absolutely right about the infection risk in hospitals.

 

[briarrose]

I would definitely increase CPT to 3-4 times a day. (We give our son 2 puffs if albuterol before each treatment.) Also, are you pounding hard enough when you are doing the CPT? I have always pounded really hard on my son and he has stayed mostly clear. (He will be 2 on Sunday.) I know some parents have bought their own stethoscope to listen to hear where the lungs sound bad and pound harder and longer in those specific spots. Good luck!

 

[GlennyB]

Hi Helen

we live in the Waikato, we always are prescribed pulmozyme during every hospital stay, it was my understanding all cf'ers were automatically put on it during hospital stays, regardless of age. I would push the point with your team. They can do it, pulmozyme is easy to access as an in patient. The other option we tried was azithromycin 3x weekly to reduce the inflammation in the lungs, which allowed the physio and hts and pulmozyme to work. kaiya had 9 weeks iv's at one stage, and was only getting worse, and we did go to starship and they did a ct, bronch etc, what they saw was highly reactive inflamed airways, that no amount of iv antibiotics was going to change. We plugged away with exercise, azithromycin, pulmozyme, hts and eventually she was clear. She looked like she had asthma at the start, but it wasn't, I hope our experience helps. Good luck! As Kaiya got older, her airways got bigger, and she was more able to clear things for herself, so colds end up being simple colds, rather than two weeks iv's. It sounds as though Kaiya is an old lady now. She is 5. Thinking of you!

 

[Helenlight]

Thank you everyone for your replies, they are so, so helpful!

We have been trying hypertonic saline progressively over a period of a few days and it seems to be working really well. Even though there was quite a lot of wheezing at first, that seems to be improving and Leyla's lungs are much clearer, virtually no coughing at all. Since this is the first thing that seems to have made a significant difference, we are going to be doing it at home and see how she goes. I'm really pleased with the results so far.

I will try to answer some questions:

I'm confident that the specialists in Auckland are up with the play with international standards of CF care. Leyla has had bronchoscopes up there before and cared for with standard CF practice. In Wellington we have had a recent specialist change from someone who had been caring for CF paeds for 20 years to someone new. I think Leyla has been a bit of a challenge for him, but he is in close contact with the specialists there about her case. If she began to really struggle with no answers, then we would look at getting checked out in NY.

In New Zealand the medical system works quite differently to the US, so in terms of funding for pulmozyme we can only get it in hospital until our daughter turns 6 (at which point it is fully funded if it is proven helpful), which doesn't really help much at this point. Insurance is not really a suitable option for someone with a pre-existing condition like CF in NZ -they either wont cover you, or it will be outrageously expensive. General care is usually free otherwise- Creon, supplements, antibiotics, hospital stays etc are all covered by the public health system.

I will ask about nebulising antibiotics.

We are quite firm with CPT so I'm confident we are getting the most out of it that we can. Good idea about getting a stethoscope, I will look into this.

Hi GlennyB, thanks for your input. That's what I thought too, so I will push the issue and find out what the deal is. You guys did 9 weeks IVs! Tough going, huh? I really think some patients are just not as simple as they'd like. High dose IV antibiotics have their own disadvantages that need to be taken into consideration, especially when it comes to removing good bacteria which can result in inflammation, more wheezing, and mucus production. Not to mention the gut issues and fungal overgrowth. Glad Kaiya improved over time, is she on hypertonic now? Thanks so much, I'd love to keep in contact if you're interested?

 

[kosdancer]

It sounds like allergies/asthma could be a large component here, so maybe getting her checked out by an asthma and allergy doc would help. Also, when she's ill doing more than 2 CPT rounds a day is really important. 3 might be enough, but you may even need to go up to 4. It doesn't matter how well you're doing it, when you're sick it "wears off" faster because mucus is just constantly accumulating and the more often you can try to clear it out, the better! If there's any way you can push for Pulmozyme I would try to do that, and definitely look into inhaled antibiotics.

 

[MotherofCFBoy11yrs]

Hi Helen, I think your doctor can get Pulmozyme for your daughter if you insist. My son was born in Sydney and I managed to convince his doctor to get it for him before he was 6 months - he had to get it approved by the Ethics Board so it was a lot of work. I also got my son on inhaled tobramycin (we got the IV formulation from the hospital pharmacy because Tobi was not available then) at the same time. Pulmozyme is proven to reduce staph infections, tobramycin prevents PA.

I got the advice to start Pulmozyme asap from the Copenhagen CF center in Denmark, everyone there starts it on diagnosis. Continuous daily preventative tobramycin was recommended to us by Jeff Wine who is the director of the Stanford CF lab and whose daughter has CF. He gave a plenary about this approach in the 2008 North American CF conference - there is a video of it online - and I later discovered that there is a CF center in Belgium where they have used tobramycin prophylaxis for two decades with excellent results: http://www.ncbi.nlm.nih.gov/pubmed/16790367

We haven't bothered with physiotherapy because in Copenhagen they don't worry about it, but my son is physcially active. He has never needed IVs, his lung function is good, he never coughs. He had his first CT scan a few monts ago and his doctor said it was remarkable, his lungs are absolutely clear.

 

[GlennyB]

Hi Helen,

love to keep in contact all for supporting each other. Hang in there with the hypertonic, it is worth it in the end. I am on FB too, this winter seems to be particularly nasty. Keep building that rapport with your team, it's so much information, all the time with cf, such an individual process. We spend a lot of time in hospital, and don't fit any picture apart from being highly complex, which is what they call us. Azithromycin and pulmozyme changed things for us. I wish you luck x