PFT's are lower than they have ever been. Antibiotics (second round, second type) are not working. Doc wonders if he would respond to IVs. The problem is, 1. I don't want him unnecessarily exposed to MRSA, 2. *** IF we hospitalize him he will automatically be ineligible for the Orkambi trials , which means up to two years before he can get the drug that potentially can bring these pfts back up. So, we ( myself and his doctor) have decided to not hospitalize. If he declines in the next two weeks, we may have no choice. We feel that the participation in the trial at this point outweighs the risk of waiting. If he needs to be hospitalized once the trial starts, that is also a valid option and he could stay in the trial. Making these decisions is extremely frustrating especially since if insurance had just approved the prescription months ago, we wouldn't be here at all. Please keep Philip in your prayers that he can remain stable to participate in the trials and that shortly after, his pfts come back up and this is NOT permanent disease progression.
October 14, 2015
An emotional day for Philip at clinic! We were there for three hours. RT went over all of Philip's home therapies and he changed from his acapella device to the Aerobika for PEP. He is still suffering from an exacerbation in spite of a round of antibiotics, so we are changing to a stronger, more frequent antibiotic. His pfts did increase by 6% from two weeks ago, but he is still far from his baseline. Through a few tears ( always a sign of illness), he was a trooper for the whole visit. We recheck in two weeks and luckily the Orkambi study has been delayed, so he's still eligible to participate! Please say an extra prayer for Philip so he's not miserable on vacation (minimal side effects from meds) and heals before the trial.
September 25, 2015
Philip has just been acting slightly off, so I decided to take him in. His cultures have not shown anything new, but his pfts were down drastically.. Doc came in after Pfts and said, " you are the best mom ever!". I asked why and he said because I noticed a problem brewing under such subtle signs. Philip looks fine, his lungs sound fine, his sp02 was 100%, BUT his pfts were the lowest they've been in 2.5 years and they dropped 13% since July!!! Thank God we caught this early. He said another week and we would have been excluded from the trials due to illness. Waiting on throat culture results, but starting antibiotics and returning in a couple of weeks hopefully healthy and ready to start the trial!
July 1, 2015
This was a long visit. It was his yearly visit, so he had pfts, rads, and bloodwork too. It was a great visit! The dietitian came in to review. His weight was 47.1 pounds, so that was great. She questioned him being on enzymes at all, since he is on such a low dose. She said it is not enough to even do anything, but doc S decided to keep him on them since it seems to be working for him. He blew 118% on his pfts and the RT said it was the biggest volume inspiration that he has ever had! He also blew the best quality pfts he has ever blown on his first try. We met with the research dept as well. They initially told us that when Orkambi is approved Philip will not be eligible for the trial because his PFTs are too high. (*Edited- we obtained a prescription for Orkambi on the day the FDA Approved it , July 2. We are still waiting for insurance, but in the mean time, Dr S informed us that Philip will be eligible for the 6-12 year old trials since they lifted the PFT limit. He should be the first of only two chosen from our clinic for the trials). Philip was a champ for the radiographs and bloodwork as usual. A great appointment overall!
March 11, 2015
This was an excellent visit! Possibly, one of his best! He has gained almost two pounds. His BMI is 70th %tile. He did PFTs well and we did not need to meet with the nutritionist at all. His weight was 45 pounds. We go back in 3 months!