Today I had a long day of appointments but I was spoiled because I had my mom with me to drive and help out today! I admit I was a bit cranky starting the day early because I have been having what I thought was a cross between my sinusitis acting up and bad migraines for the past few days and lots of pressure in my head… so waking up early has been extremely hard. Even my sinus rinses and decongestant haven’t been touching my constant head pain.
Upon arrival, we realize that AGAIN , the front desk has oxygen but nothing to wheel them except the extra large wheelchair. And AGAIN the second the man brings me the wheelchair an elderly woman comes up and tries to take it… so of course I just give it to her and stubbornly insist on carrying it myself… which in turn just ended up being another thing my mother took over and carried when I got tired (sorry mom ;P) Anyway.. first appointment was the ENT… I have grown to have a love/hate relationship with my ENT appointments. Like most people with CF who often have several surgeries, have now had two endoscopic sinus surgeries in the past few years. People with cystic fibrosis tend to have underdeveloped sinus cavities and often experience issues with sinusitis, polyps and frequent sinus infections due to the same thick mucus that gets clogged in our airways. Frequently a cold can often turn into a sinus infection which can then drop down into the lungs so it is important to check and treat sinuses if infected to prevent further damage to my lungs. At each appointment my Doc goes up my sinuses with some barbaric looking tools (after spraying me with some numbing meds) and makes sure that my sinuses aren’t filled with pockets of infection. If anything is detected, he suctions it out with what I can only describe as a tiny metal suction thingy that fits up your nose. Obviously that is not the technical term but that's what it is… a vacuum for your boogers. It is not a pleasant feeling, and I can often feel suction behind my eyeballs… it’s hard to describe but it feels absolutely terrible!! For a few long seconds I buckle down, grind my teeth and my eyes automatically tear… but the relief after is indescribable and 100% worth it. Anyway… to my surprise, my sinuses are “terrific”. Doc says he can do a CT scan if I’m absolutely convinced my sinuses are causing the headaches but I’m not because migraines have always been an issue with me as well so I’m going to just continue with my sinus washes and be happy I don't have to be suctioned today! I also discovered that my right frontal sinus is famous… hehe.. my doctor (with my permission) used my post-surgery scans and pictures in a lecture about standard surgery outcomes with cystic fibrosis sinuses and it was presented in a few different locations around the world. I offered my autograph and we moved on to the next appointment J
Next appointment was at my CF center for a checkup. I recently got the lab results back which stated that I am still fighting the fungal infection (trichtosporon) and now I have also cultured Pseudomonas again… which explains why I am feeling exhausted and run down again. It has been 2 months since I’ve had IVS and this is usually the exact time when I go back on them for a 3 week “tune-up”. But because we are hoping for a call for lungs within the next year, my doctor has been trying to keep me off IV treatment as long as possible to avoid resistance to the drugs that I will need for post-transplant. For this reason we talked about adjusting my oral antifungals, continuing my inhaled antibiotics and adding a second oral antibiotic into the mix daily. I did a Pulmonary function test, which revealed that my function went down from 27% a few months ago to 25% and my Oxygen need has increased from 2 L to 3Liters. This is no big deal for the concentrator I have at home, which goes up to 10… but my portable concentrator which my niece calls “my robot” only goes up to 2. Soo I will have to retire my robot and switch it for the refillable oxygen tanks… I don't mind this, except it’s just another fun dance with insurance and paperwork with the oxygen company. FUN.
Anyway after discussing oxygen and the plan for the next two weeks of med adjustments I got a healthy dose of reality. I am very thankful for my CF doctor because she doesn’t sugar coat things and is always very honest about the reality of my health and this disease. The topic of the waiting time for lungs at Columbia came up and I expressed my concern with how my health trends downward every winter and how the last few years it has been harder to recover. As a general statement, the lung transplant center has given me an average wait time of 6-7 months… however, my CF doctor expressed that it isn’t always the case with her experience with her previous patients and they end up waiting longer. We agreed that double listing is a good plan because it allows me to have a backup if my health continues to decline or begins to decline faster than we expect. She expressed her confidence in my ability to stay on top of my medical regimen and to stay diligent with being active and keeping my muscles strong… but we still can’t predict the unknown so it’s better to have a back-up plan. UPenn was where we thought best because of the reputation for being a top facility and its close proximity. It doesn’t hurt that I am familiar with Philadelphia and would be close to many people that I LOVE for all my checkups and surgery if I end up in the city of brotherly love ;). I am not thrilled about having to go through another whole pre-transplant evaluation but my doc has assured me that many centers allow you to transfer at least some of your records and paperwork from your original center. We will have to see.
The next appointment was a meet with the lung surgeon. He made the appointment to ask if I would be willing to participate in a study that will look at biomarkers of genetics and outcomes of surgery post-transplant. They will take a tiny snip off the bottom of my new lungs (most new lungs need to be shaved down anyway so it will not affect the performance of them) and a tiny piece of my old lungs and they will be used for research purposes so that they can continue to do research to understand rejection and why some patience accept the new lungs and have better outcomes than others. I am all about medical research so I accepted and signed some paperwork. That was the end of a long day of appointments!