Today was considered Philip's annual appointment. He did well. His weight was up to 19.1 kg/42.2 lbs. His height was up a few cm. He did well on PFTs but was making a huffing sound at the end, which made the result inaccurate. This made it look like his PFT had dropped, but the dr thinks it did not. He is doing very well at this time on Miralax half cap every other day so we will plan to cancel his GI appt for August.
May 30, 2014
We went to clinic today for Philip still acting "off" . He has a random cough (rare), but when he does cough, it sounds deep. Dr Schmidt in not quite sure what is going on. We did PFTs. They were good. There was a slight drop, that does show a decline over time. We did radiographs as well. The lungs look great. His stomac did have a lot of gas, but no obvious obstruction. Dr S feels like we are most likely dealing with a sinus issue, even though it is not obvious. He is culturing his normal (staph, h.parainfluenzae). DrSchmidt is starting him on Cepalexin and Rifammpin for 21 days. Rifampin is typically prescribed for TB and is pretty powerful. Hopefully this will get him straightened out.
April 28, 2014
On April 18, Philip suffered a partial bowel obstruction. We have been able to clear it at home, however he has not eaten any solid food since then (with the exception of a few bites here and there). On 4/24, we started cyproheptadine to boost his appetite. He missed almost an entire week of school. The one day that he did go, he was tired, had red cheeks (no fever), spacey, and grumpy. Today we went to the cf clinic and he complained of headache,eyes hurt, mouth hurt, and ears hurt. His PFTs dropped 8% to 117%. It appears he may have a sinus infection. He had a slight amount of fluid in his ears, but no infection. We will start 21 days of augmentin to heal his sinuses and combat the h. parainfluenza he is culturing.
April 23, 2014
Today was his ENT follow up visit. Excellent visit...no news. She was ecstatic with his healthy winter and will see him again in 6 months.
April 9, 2014
This clinic was a long one again, but it was very good. Quite possibly his best visit ever. His weight was 41.7#, his height was 42" and bmi was 16.6 (81st %tile). The pulmonologist was very pleased with the increase. His lungs sounded clear and his PFTs were higher than ever! He achieved an Fev1 of 125% and his FVC was 133% ! He also completed the PFTs with the most ease ever. They are certain that his efforts met criteria and they were accurate. The one thing that was concerning was the slight concave area on the PFT chart. That can indicate underlying lung disease or asthma/allergies. They gave him albuterol and repeated but they were exactly the same, so it does not appear to be due to an irritant. Dr Schmidt says he has CF and he has staph in his lungs...so we can pretty much expect lung involvement. Overall though, this was a great!
January 8, 2014
Today was a three hour visit, but it was a good one for the most part. His height was up a little and his weight as down 2 pounds to 39.6, but he is still 73rd percentile, so they are not concerned. He did PFTs and he got one that was 117%, however they stuck with the final number of 115% FEV1. Still very good. The only thing that was slightly concerning was that the RT said based on the shape of the curve (the readout on the computer for his expiratory volume), it could indicate early lung function loss/ involvement. He also said it could be due to the noise he makes with his glottis when he blows. Time will tell, so I am not going to get upset about the possibility. He told me I am doing everything right and more educated/invested than most moms. I got lots of compliments today on his care by the team that knows me very well and that is always comforting. His highest lung function was 123% back in October and I would love to see that number again!
I asked our RT about huff coughing and if we could start to teach that. This helps clear the alveolar and deep areas that normal cpt don't reach. He said most kids start this at around 8 years old but we could try. He taught him to do controlled breathing through his diaphragm and he immediately picked it up. The huff coughing was pretty good. Now we just need to add force. I was quite surprised to see him learn the basics so quickly. We will be practicing this at home and incorporating this into his vest therapy (which will add a little bit of time to each therapy). I also asked about vibratory PEP, and they recommended the Acapella for now and then on the next visit we will change it to something else that is better. I will buy this tonight and hopefully start soon. He will blow into it periodically throughout the day and the vibrations loosen secretions in his lungs.
I realized when I got home that they did not do his throat culture, but in looking through his paperwork he has not cultured staph since APRIL!