Today was a big clinic day! This counted as his annual visit. Today was a big day. FIVE hours at clinic! Here's his update: We arrived early for a fasting OGTT test to check for CFRD (cystic fibrosis related diabetes). Cf patients are at a high risk for CFRD , which is kind of like a combination of type 1 and type 2 diabetes. This trooper got stuck 5 times today! (New girl that stuck him three times, blowing his vein before asking for help). The results are in and he appears very normal! Thank God. This is a yearly test. His liver values from this morning also are still high but coming down. This is good. His weight was down 5# since June , which is a little concerning. We will work on that. He did gain a tiny amount in height. His pfts were okay. He started out at 13% below baseline but pushed up to 5% below baseline. 2% decrease from last month. You can see from the pictures he was trying hard! His amazing RT came in and did his yearly assessment of all of his equipment and his efficacy and ability to do his treatments . His Dr gave us the go ahead to increase his symdeko to the full dose (he's been on a half dose since starting in March).
June 19, 2019
Good clinic visit! He gained a tiny bit in height and about half of a pound. The good news is his PFTs went up to 128 from 125 in May. Still not his baseline or the goal he wanted, but it's the first time his PFTs have gone up like that without a hospitalization. He had blood drawn to make sure his liver is handling the Symdeko well. We will see what his throat culture says next week and probably decide in two months whether we need to do another bronch.
*update- his liver values have tripled, so we are starting Ursodial twice a day and will recheck in one month
May 8, 2019
Pretty good clinic visit . Lung function down 2% from last month and 8% from baseline but still good. He gained 2.5#. His doc gave him the green light to skip cayston next month at Disney ( we believe he eradicated pseudomonas). We will return next month after his trip to recheck his pfts and liver enzymes. We can decide from there if we need to admit for bronchoscopy.
Apr 22, 2019
Today Philip went in for a sweat test and to recheck PFTs. We are redoing the sweat test to see if his results have changed at all since being on the corrector drugs. We should know this afternoon. During PFTs his doc poked in and told him to get to 127%. He didn't quite make that, but did reach 125%. So, he is still down 6% from baseline, but he is doing well.
Apr 3, 2019
We just did Philip's follow up appointment for his new drug Symdeko. It has only been 2 1/2 weeks. His weight is up 1.5# (about 5# in the last couple of months ), but his pfts only went up 1%. He's still 11% below his baseline of 131%. His doctor and I had hoped to see more improvement, but it's still early. The plan is to return in two weeks for a sweat test and pfts and then another visit in 2 months to check his kidneys and liver enzymes.
Mar 15, 2019
Pretty decent follow up visit! His pfts were 120% (up from 119 a month ago, but still down from 131) He grew a half an inch (which also skews the pfts, making them seem a little lower), and he gained 3.5# in a month!!! Symdeko will arrive Sunday and we will monitor him throughout the day to be sure he has no reactions . He did liver enzyme labs today and we will monitor that, along with his eyes annually , assuming the Symdeko is well tolerated.
Feb 21, 2019
Awesome ENT visit. No problems at all! (Sinus issues are extremely common in CF patients). He doesn't have to come back for a year!!!! Six years ago we were here talking about chronic sinus infections and surgery was eminent for tonsils, adenoids, ear tubes, and open sinus surgery. We saved and scrapped and built him a salt cabin at the house and that was the end of his issues. It was life changing and I'm so glad I went with my gut and pushed for it.
Feb 13, 2019
Today was Philip's follow up visit. He is only down slightly in weight. His lung function is down 15%. The Dr tried an Albuterol treatment and redid PFTs but it only changed by 3%. So, we are going to treat the aspergillis (mold) in his lungs with an antifungal for a month and see if that helps. In good news, the info that insurance requested on Symdeko was sent last week, so hopefully we see an approval soon!
Jan 2, 2019
Today was Philip's follow-up. He feels good, but has a slight cough. PFTs are down 7% but still great. Weight is stable, BMI is good, appetite could be better. Dr believes we may have gotten rid of the pseudomonas. The plan is to recheck in 6 weeks (or earlier if cough worsens) so we don't lose more lung function. I personally don't like the way his eyes look, the cough, etc, but I'm hopeful he can get rid of this with his current oral and inhaled antibiotics. We are submitting a request for Symdeko, which Cigna is almost guaranteed to refuse. They already have a plan in place for compassionate release program through Vertex as Philip is the perfect candidate (Orkambi didn't work for him, new cultures, two hospitalizations in 3 months). So, hopefully we will get him on this soon.