Sunday, January 12, 2020

The Initial Trikafta Changes

I haven't been blogging in a while and after my transplant evaluation I felt like I needed a break. CF felt like it was taking over my whole life and I just wanted to step away from writing about it in my blog for a little. It has been almost 9 months since my evaluation and my lung function and my quality of life was remaining the same. I was getting sick more often and IVs were still not overly effective, but I didn't have a significant drop or anything that affected my transplant status. I was driving to Stanford every 2-3 months to be monitored, but I was still too healthy to list.

But this update is about Trikafta and not transplant so moving on... When I heard rumors about Vertex providing Trikafta for compassionate use I tried so hard to get on it! Unfortunately, for my mutation getting compassionate use was tricky. I fell into the guidelines and applied, but then the FDA changed the criteria and made everyone reapply. My CF nurse reapplied and said I still fell within their guidelines and we really thought I would be granted compassionate use. The wait felt like it was forever and I kept worrying I would get sick beyond repair while I waited. And then I got the news the FDA approved Trikafta. I was thrilled, of course, but part of me was disappointed that it happened before my compassionate use was granted. Now all compassionate use applications were useless and I was now depending on my insurance. I called and emailed my insurance and pharmacy every day. I was going to make sure I didn't get lost in a stack or pushed to the end of the pile.

I was approved by my insurance and started Trikafta the day after Thanksgiving. What a great reason to be thankful!! So how has it been going?

One of my best friends was visiting from out of town when I took my first dose. I literally popped the first dose and left to spend the day out and about with her. I was distracted and not paying attention to how my body felt except I knew I was coughing a LOT and my coughing felt different. More wet and being productive was so much easier.

I have always been very productive and it caused a lot of issues because I produced so much, it was virtually impossible to feel clear even after treatments. Treatments always felt like a huge workout. I had so much mucus and it was so hard to clear I would often be sweating and red in the face and exhausted after treatments! Within a few days I noticed my production went way down and by day four I was not coughing ANYTHING up at treatments. My cough just disappeared!!

This lack of mucus and coughing both thrilled and terrified me. I have a huge inflammatory response and normally when I get less productive it means my inflammation is out of control and I start getting a lot of plugging which is a nasty cycle that feels impossible to break from. I had a hard time mentally accepting that my lungs were responding to the meds and I didn't need to cough so much. What!? MY heavy producing, never clear, highly sensitive lungs didn't need to cough? It felt unreal and a bit terrifying.

Another massive change I noticed on Trikafta was my inflammation improved so much I can hardly believe it! I usually carry my combivent inhaler everywhere I go. It sits next to my bed at night and it comes in my purse everywhere. If I go for a walk it is in my pocket! I use it several times a day when well and I use it a million times a day when unwell. I now take it before treatments (and not the second I open my eyes) and that is it! I have noticed with my home pft monitor that I still have inflammation because my pfts jump pretty significantly after my combivent, but my lungs are doing so much better I don't feel a difference. Prior to trikafta I would often feel like I was suffocating if I went to long without a bronchodilator.

Within a few days of starting Trikafta I went to cut down a Christmas tree with my family. We drive to a much higher elevation and in the past I really struggled with the elevation and hills. In fact, I wondered if I should go at all this year because I was worried I wouldn't be able to handle it. I did go, but immediately saw the hills and felt dread! We were standing at the top of a steep hill and all the kids decided to run down to look for a tree. I stood at the top and was assessing if I could make it back up the hill if I followed the kids down. I decided I probably couldn't and decided to stay put. But somehow I got distracted and ended up at the bottom of the hill. And before I knew it I was in my car on the way home before it dawned on me, I just spent the entire morning walking up and down hills without realizing it!! No huffing and puffing, no headache from lack of oxygen, not struggle. In fact, I didn't even notice. How AMAZING is that?

The last major improvement I noticed within the first few weeks was that my energy level was INSANE! Within a few weeks of starting I was helping put on a school event. I warned the others on the PTA that I would help if I could, but depending on how I was feeling I may not be able to help at all. When the day arrived, I started working at the school around 11:30am to set up, worked the entire event, and was one of the last to leave when the principal locked up the school on our way out at 9:30pm. When I got home and realized I still had energy I was astounded. Usually, my energy level is so low and if I push myself I pay for it the next several days. But I was acting like a real 35 year old!

This is getting long so this is the last thing I will say. My whole adult life my CF has been a huge rollercoaster ride. So many times I felt like my health turned around and I was so optimistic for the future, but then as soon as it improved it would all crash down. My health history taught me that the highs were temporary and the lows were always right around the corner. Years ago I would celebrate my improvements, but as my health got more complicated I learned to enjoy the highs in my CF, but to brace for the low. It was always right around the corner. And in the past few years my highs lasted a week or two before I would feel the downward slide back to poor health. My brain was so trained that for weeks I couldn't feel overly excited about Trikafta. I felt like I would enjoy better health, but like always, it would be ripped away from me before long. I have been on modifiers before and the results seemed temporary and faded just like everything else health wise. So I talk about these improvements and I noticed them and felt hopeful, but it was clouded with doubt and caution. But I felt hope, cautious hope, but real hope for the first time in years.

Monday, October 21, 2019


The FDA approved Vertex's triple!!!

I realize I have been mia recently, but I am still alive and well!

Friday, May 10, 2019

Transplant Evaluation Day 4

I have been putting this entry off for a while. Partly because I have been busier with the nicer weather, but also because I just wanted to move on. When I first got back from my evaluation I was wanting to process what I had gone through (we actually went on a little getaway right after which was why my blog posts were delayed). I wrote the 2 entries right away, but then I felt my feelings switch and I just wanted to tuck it away in a quiet spot in my mind and move on. But I know how much I wanted to hear about the evaluation process when I was waiting and so here I am writing about my final day even though so much of me wants to shut the computer off and forget that piece of my life. I clearly, have bad coping skills!

Thursday was the day I had scheduled for my heart Cath. We had to leave our air BnB by 11 and my appointment was for 12:30. We packed up all our food (bringing food and staying in an air bnb was such a money saver btw) our clothing and then went to find gas knowing it would be a long day and we had a long drive when it was over. We got to Stanford early and checked in around noon. The heart cath area was so busy. They had screens with case numbers (which we were given when we signed in) and everything was color coded so while family waited in the waiting room they could watch the progress of the procedure via the colors on the screen. If your loved one's case number was glowing green you were getting prepped, if you were orange you were in the procedure, etc. It reminded me of an airport! It also made me feel a little less nervous seeing how many people were undergoing some variation of the heart cath. They clearly do a ton of them at Stanford!!

I needed to be fasting and I was surprisingly hungry. The various fasting requirements and the schedule made me feel like I was in a calorie deficit and I was starting to feel it by the 4th day. The average age in the waiting room was probably 70+ so I felt my husband and I looked very much out of place. There was an elderly couple that were sitting across from me and I couldn't help, but hope that my husband and I can still be alive and well when we get to their age. Unfortunately, the heart cath was very behind. I was finally called back after waiting for what felt like forever. I had to go alone and they told my husband that after I was prepped they would come get him. They then asked a million questions before the procedure. She asked a few questions about my husband and I and then commented that we must have married very young. I got married at 26 which is relatively young in todays standards (and even more so for our area). The nurse acted surprised and then asked how old I was. She was shocked I was 35. CF patients are notorious for looking young and I did during my whole malnourished underdeveloped stage. But being fair skinned in a sunny climate hasn't been kind to me and I think I look my age so I instantly loved this nurse. Of course, like I said the average age in the heart cath was 70+ so I doubt my nurse had a good gauge on how old 35 year olds are supposed to look. She also told me about a friend of hers whose daughter had CF and had a lung transplant.

During my intake a nurse came to access my port. This was the first time at Stanford that I felt like the nurse was unsure of how to access a port. My intake nurse was trying to tell me a story about how they are using something from a squid to do CF research. I am sure (like many nurses over the week) she was trying to distract me from the port access. However, I can NOT be distracted during a port access because I am super controlling about it. I felt rude, but basically ignored the intake nurse and was focusing and giving advice to the nurse working on my port. She explained she used to access them all the time, but it October was her last access after changing departments and she was having trouble feeling the 3 dots on my power port. The second she poked me I told her she missed (I was right), but she got it the second time and it was fine. After they brought my husband back and Ii figured the procedure would begin soon after, but we had no idea just how long our wait would end up.

The nurses seemed to switch around and it seemed like person after person was being called back and we were left to wait. We tried to ask what was going on, but it seemed everyone we asked said they weren't on my case anymore. I am still not sure which nurse was mine towards the end (I had so many switch to different patients). The woman across the way (we were all lined up in a big room separated by curtains) was someone that was in my lung transplant class. We exchanged thumbs up as she was wheeled to her procedure . I tried to nap and watch videos on my phone but the hunger and thirst (the worst!) and the nerves were getting to me a bit. I guess while I was napping the elderly man from the waiting room (his wife was having the procedure) popped in to check in on our progress. It was so very kind for him to show concern for us when he clearly  had big concerns of his own. Later, when he was leaving he walked by our waiting area and waved and wished us the best of luck. Writing this is bring tears to my eyes. It was such an emotional week and this particular day was hard because we waited so very long and I was so nervous. To have a stranger reach out and show some support when he had his own concerns and struggles reminded me how kind hearted people can be and it meant so much to the both of us.

A little before 5 (!!!) the surgeon finally came too talk to me! This was 5 hours after getting to Stanford and after fasting for way too long. The first thing the surgeon said was, "where is your IV?" When I told him my  port was accessed he said the port was great for anything they will give me during the procedure, but he needed an IV as a guide for the catheter. I seriously wanted to scream. I was so dehydrated I felt like I was a raisin after fasting (both liquids and food) for so many hours and they wanted to start an IV. That should have been done at 12:30 when I had only been fasting 6 hours or so. Of course, the first IV failed and the nurse called someone else. They started getting stressed because the nurse failed in the spot that needed an IV. They ended up putting it in more to the right and thank goodness the second one worked (although this ended up backfiring later).

Finally, a nurse came out and started to get me ready for the procedure. I told her the intake nurse told me I would get a valium, but since I was delayed I never got it. This nurse acted like a valium was overkill, but agreed to give me one since I requested. I have never had a valium before so I am not sure what it is supposed to feel like, but I think they gave me a sugar pill or such a low dose it was useless because it had zero effect on me. As I was being wheeled out of the heart cath a family was sitting in the last curtain waiting for their own procedure. As I was being wheeled by they all gave me a thumbs up and wished me luck. There are so many kind people in this world!!

When I got the procedure room they prepped my arm, hooked me up to heart monitors and put oxygen on my nose (although they said it was a precaution and there was no oxygen in it at the time). They used lidocaine to numb my arm. The whole thing reminded me a bit of getting a PICC line in radiology with a bit more set up and many more nurses and respiratory therapists around.

When I am having medical procedures done I have this control issue that I have to know what is going on. I think having people doing things TO YOU can make you feel out of control and the only way I don't stress is if I know step by step what is happening. So I kept asking a bunch of annoying questions. Did the catheter go in? Is it stringing okay? I bet they wish I was put to sleep. And my stress level/anxiety clearly wasn't soothed with the valium they gave me. And then I knew that something wasn't going well because with PICC lines the line is put in until it hits right above your heart. With a heart cath it goes into your heart. In distance, this isn't a big difference, but I could tell it was taking too long. So I started questioning again. The nurse was kind and turned the screen so I could watch the wire. He actually didn't need to show me because I knew what was happening. It was the same thing happened so many times in the past with PICC lines. The wire was curling back on itself because something was blocking its path. They said it may have been my port among other things. I knew it wasn't my port because this was an awful dejavu that I had been through a dozen times before. It was some weird anatomy of my veins. The next thing I knew some woman came out of a back room and said that my arm wasn't working so they were going to try through my neck. I groaned. They decided to try one more time on my arm. I protested a bit because of my history with PICC lines. Considering my last PICC line took 9 attempts I was more concerned about getting it right than going through my arm.

It is interesting because I haven't had a PICC line in over 6 years. It was amazing how quickly I was thrown back into all the feelings and stress that came with all the failed attempts. The heart cath procedure wasn't bad, but it brought back a lot of old feelings that I had forgotten and somehow felt so much heavier and uglier when they resurfaced.

So they tried the second time. I asked them to tell me when the catheter was placed, but heard the distinctive POP that comes when a vein is accessed so I told them before they even had a chance to tell me that it worked. I have a feeling they hated me and my nonstop commentary. When I asked if the wire was able to thread they assured me it had. I felt so much relief until the nurses started rushing around my bed and the mood of the room changed. I looked around for someone to tell me what was going on, but everyone was clearly very occupied. I finally said, "Is everything alright? Am I alright?" Apparently, my heart monitors stopped showing clear readings. They assured me that it was nothing to do with ME, but the monitors were acting funny. At this point I was really wishing the valium had helped, but instead I watched the clock on the wall trying to pretend it was 10 minutes later when the whole thing would be finished.

Soon enough the doctor assured me that my results were good and that my CF had not negatively effected my heart and I was off to recovery. Unfortunately, because I had two catheters in I had to go to a step down recovery where my husband could not join me. They needed a nurse to remove my catheters and hold pressure on them for 15 minutes and so I needed too be in a step down where a nurse could give me the needed attention. The nurse that did remove my catheters was actually my favorite nurse of my stay in Stanford.

Finally, I was moved to a regular recovery area and I was finally able to see my husband. And thank goodness for him because one of the first things he said was, "when can she eat?" The nurse got me a snack kit with brie, crackers and grapes. It was one of the best tasting things I have ever eaten. I was so happy to have food and water again! At this point it was just about 7:00 and I was so ready to say goodbye to Stanford and get back home to my kiddo. Unfortunately, (I am using that word a lot), the worst was yet to come...

Friday, May 3, 2019

Transplant Evaluation Day 3

To start with Day 1 go here.

I woke up Wednesday and turned to my husband and said, "I am over it. It was fun, but now I want to go home." I had a lot of adrenalin the first day and some of that carried into Tuesday, but by Wednesday it had warn off and I was ready to go home. But we were half way done and had no choice, but to continue on! So I put on my big girl panties and went to my first test.

Echocardiogram with Bubble Study: I checked into the echo lab around 8:45. I went back into the medical room where they had me change into a gown with the opening in the front (opposite of the norm). The nurse came to start an IV. I asked if she knew how to access ports and she said she was more than happy to use my port instead of a peripheral line. I was so happy! My port was coming in so handy! She left to get port supplies while the tech (Dr? Sorry, I had so many tests I wasn't sure who was a Dr and who was a tech) started the sonogram. It was very reminiscent of when I was pregnant, but instead of looking at a beautiful baby growing inside of me they were looking at my beautiful heart. Again, I was watching the screen, but it was a lot less fun than looking at baby feet and baby hands. All the pictures looked the same so I was only half paying attention. The tech was so warm and kind though which seemed to be a trend at Stanford!

When the nurse came back she accessed my port and we started the bubble portion of the test. They push saline with bubbles in through your IV and watch how they pass through the heart. Apparently, they are looking to see if any bubbles find their way to the left side of your heart. If they do, it means you have a small hole in your heart which apparently is somewhat common. When the nurse pushed the bubble saline into my port it traveled through so quickly they actually missed it! The combination of using a port which is much closer to the heart than a peripheral in the arm and my very fast heart beat made the bubbles travel faster than they anticipated. They repeated the test and then did it one more time while I clenched my stomach (like I was going to the bathroom) which apparently puts more pressure on the right side of your heart. I am 99% sure I failed this test, meaning I have a small hole in my heart. Nobody ever told me what this actually means as far as transplant or life in general. Hopefully, it isn't a huge deal since nobody mentioned it, but I will be asking my CF doctor about it next clinic.

During the test the nurse in the room started asking about my schedule for the day. When I mentioned I had blood work she told me that I should get the labs taken from my port. I wasn't sure the regular outpatient lab would do that so she started making calls to see if someone could take blood from my port. Finally, she told me she would walk me down to the lab, wait with me and then ask the phlebotomist if she could do the draw instead. You guys, that was so insanely nice and was the overall feel at Stanford! Everyone went so above and beyond what was expected of them and they just all genuinely wanted to make my life as easy as possible. So we went to the lab together and she drew 8(?) more vials of blood and then took out my port needle.

Lung Transplant Clinic:

After my labs were done we went over to transplant clinic. I was surprised to find other people with CF (pre-transplant) in the waiting area. In recent years my current clinic usually brings us back pretty quickly (where we still wait forever) to avoid having too many CF patients in the waiting room at the same time. Just like my current clinic we waited forever to be seen. Also, like my current clinic we saw a team of people. I had to give an overview of my medical history and any family history of diseases. I was glad my dad was there because outside of my immediate family I wasn't sure on family history as far as health issues. The social worker stopped in and said I was a good candidate from his end. I was able to skip the nutritionist because my weight is fine which saved us a bit of time.

I was told pretty quickly that I was not ready for transplant. My 6 minute walk was good, my lung function numbers are not good, but not at transplant level yet and I am not on oxygen. We went in anticipating this result, but I felt so much relief knowing that I could put transplant off a little bit longer. It does scare me a little bit though because I was told without hesitation that it is not time. I think about how low my energy is and how often I feel sick and I can't help, but feel a sense of dread for what things must be like when it is time. It is one of those times that it is better to just not think about the future and try to stay in the moment because no good will come from thinking about your inevitable decline.

Clinic was the last appointment of the day. I was hoping to feel relieved that I was almost done with my week at Stanford, but I was absolutely dreading my right heart catherization and finishing day 3 meant that I was one day closer to the heart cath.

Thursday, April 25, 2019

Transplant Evaluation Day 2

**Start here for Day 1**

Tuesday was much shorter (which means a much shorter blog post too...whew!

I had to start the day fasting again. I know it sound silly to complain, but I know I was at a calorie deficit from the day before so I woke up really really hungry!

10:00 Esophagram: I checked in for my esophogram at 10:00. They had me change into a gown and explained the test. Then I waited for a really long time. I guess they were finishing up with another patient and it was taking longer than expected. Finally, the doctor came in to get started. I was given a cup of barium with a straw. I was told that I would be asked to take a large mouthful of barium and not to swallow it until instructed. I would then be moved into various positions and asked to swallow the barium. They were going to watch how the barium travelled down my esophagus and into my stomach. I had heard Barium was chalky and gross, but honestly it wasn't bad. It tasted like a  flavorless Ensure. It is much easier to drink than the syrup they make you drink for diabetes testing!

So I took gulps of barium while standing up, then while laying on my stomach, and on my back. Then they had my lift my legs in the air, put them down and lift again. I believe they were doing this to look for reflux. I was trying to watch the monitor while the test was going on although it was at a weird angle so I only got quick glimpses. It was pretty fascinating watching the way food travels through the body.

I thought I would be excited to break my fast, but I had so much barium that I actually felt full. I ate a little something anyway because I knew my next appointment would be long.

Class For Transplant: This was a 2 hour class. I was in class with 2 other transplant hopefuls and all of their support people. It was a full house. I was nervous being in a class with other people with lung disease, but it was obvious none of them had CF and I am sure they didn't pose a threat to me, but I wore my mask anyway.

None of the information was new to me and I had heard it all before through research or being part of the CF community. But I surprisingly started to get a bit teary when they talked about donors. It is hard to think that your only chance at life is if someone else's ends tragically. It also made me think about if I ever lost my loved ones in a sudden tragedy. There is no getting around the weight that all transplants represent earth shattering loss mixed with life saving hope.

We were done by 3, but for some reason I left feeling a bit shell shocked and exhausted. Like I said the class didn't cover anything new, but maybe talking about transplant in regards to yourself feels very very heavy! I was glad that this day was shorter and we were able to go back to our Air BnB to relax and get ready for the next day.

Tuesday, April 23, 2019

Transplant Evaluation Day 1

My evaluation started on a Monday, which is standard for Stanford. It was the day I was the most stressed about because the schedule looked so full! It ended up being a very long day. We got there at 6:30am and didn't get to leave until about 7pm! I had appointment scheduled for 7:30, 9:45, 12:30, 3:30, 6:00 and 6:20. I was also given 3 tests, blood draw, EKG and Chest X-ray, to do "at my convenience." This is a detailed breakdown of my day. I wanted to include a lot of details so maybe someone in the future that has an evaluation at Stanford can get an idea as to what they are in for:

6:30 Labs: My paperwork said I needed to get my blood drawn first and foremost when we arrived at 7am and that it needed to be completed before my first appointment at 7:30. I needed to be fasting for the blood draw. We were told the lab opened at 7am, but since we had never visited the hospital before we wanted to leave early and get there with plenty of time to find the lab. We took a Lyft and arrived at 6:30. We were so excited to find that the lab was actually open. I was told by the phlebotomist that one of the blood tests ordered for TB couldn't be done until 8am (due to it needing to be incubated and that wasn't available until 8??) and that I would have to come back to get that test done. They said I could either do all the other tests first and come back just for the TB which meant I could break my fast, but I would get poked twice. Or I could just wait and do it all at once with only one stick, but I would have to fast until I came back. Food trumps needles in my life so I decided to do all the tests minus the TB with the plan to return after 8. I gave about 15-20 vials of blood and was a bit worried I may feel a bit dizzy from fasting and giving a lot of blood, but I felt fine. I also gave a urine sample. I had extra time so I decided to get a few other unscheduled tests done.

7:00 X-Ray: I tried to get my EKG done, but it wasn't open yet. So I went to get my x-rays done. It was a basic chest X-ray. One facing the screen and one standing sideways. It was the same as every other X-ray I have ever had for CF. It was super quick and easy.

7:30 Bone Density Scan: My husband and I packed a backpack cooler full of food, water and snacks the night before. I also packed my Symdeko and morning pills because the morning was so busy and we just weren't sure if we would make it to the cafeteria in time. I don't love fasting or going hungry so we wanted to make sure we had food with us. I got to break my fast after checking in for my dexa scan. Whew! Soon after they called me back for my scan. This was my first scan and I wasn't sure what to expect. It was quick and painless. They took a scan of my lower back and each hip. I had to sit still for 2 minutes for each body part they scanned. It was similar to getting an x-ray. Simple!

8:00 Blood Draw #2: We ran back to the lab to get my TB blood draw. The amazing tech left my file open so when I got to the lab I did not have to wait in the line again and was brought back immediately. One vial and I was done. Super fast!

8:15ish EKG: We were ahead of schedule and I was so happy to be cruising though my "extra" tests because I did not want them hanging over my head. Since we had time we went over to the EKG lab. We tried to check in, but they said that walk ins (which is what we were) couldn't be taken until 9:00. Since my next scheduled appointment wasn't until 10 we decided to wait it out. At 9:00 they called me back and I got an EKG. It was super simple and fast. They put some monitors on my ankles and chest and took a reading for a few minutes. The tech gave me a print out of my results to bring home. I had no idea how to read it, but it looked cool so I took it. Simple and painless.

9:45 SNIFF test: We checked in at 9:45 for a 10:00 appointment. This test was easy! The brought me back to a room with a huge bed that was standing upright (as in the foot of the bed was on the ground). I stood against the bed while they brought a machine (essentially an x-ray) in front of my chest. The tech was taking scans while asking me to breath normal. Then he took a scan(s?) while I took a deep breath. Finally, he told me to sniff 3 times while he did one last scan. Apparently, this test checks to make sure your diaphragm is working in unison and properly. I passed. I have no idea what it means if you fail or what problems it can cause in terms of transplant, but since I passed I didn't bother to ask.

12:30 Social Worker: You guys, this was a hot mess. Going into it I assumed they would ask the basic social worker questions. But the social worker came in with a packet of questions and I knew he was judging if I would make a good transplant candidate. The problem was the more (random) questions he asked the more I started to wonder if I would pass. For example, one of the first questions he asked was if I had gone to public or private school. Internally, I kept thinking, "wait, what does this have to do with transplant...does your elementary education really impact transplant??" Obviously, I was over thinking these questions, but I was starting to wonder what the relevance was. After taking a step back I think they were just getting a profile on me, but at the time the questions made me nervous as if every step of my life was being  judged. The other part of the social worker meeting that made me panic a bit was when he asked about finances. We are a single family income. After we told him our income he did mention that the cost of living near Stanford (which is required 3 months post transplant) is much higher than where we live (and basically completely unaffordable for us). For reference, the average home value in Stanford is $3 million which shockingly is not affordable for us. And even though we wouldn't be buying a home in Stanford post transplant, clearly rent would probably be significantly higher than out mortgage. Considering we hope to keep paying our mortgage while we rent in Stanford I was wondering how we would possible pull all the finances off. So I was internally freaking out now thinking they are going to reject us for sure! After it was done he explained that they do not disqualify people based on income. I wish I knew that upfront! This is getting long, but the last part that I will mention is that I hate mental math. So when he asked me to count down from 100 by 7s I got nervous all over again and lets just say I will never trick anyone into thinking I am a math genius. It wasn't pretty!  

3:30 Pulmonary Function: Next I had an appointment with a respiratory therapist (who was insanely nice and encouraging). We started with an atrial blood gas test which is when they take blood from an artery in your wrist vs a vein in your arm. After hearing it was painful from various people (including nurses) I was really nervous! Luckily, they gave me some lidocaine which made the test way less scary. She missed the first time and had to do the test twice to get a sample. I only felt a bit of pain on one attempt, but the other was painless. After, we had PFTs. I had to do the first one without any inhalers (I had to stop inhalers 4 hours before meeting the pulmonologist). I was having a good lung day and lucked out because my asthma can get out of control. Not taking my inhaler before PFTs was actually one of my biggest worries about the day. We did several lung functions tests which can be exhausting, but having CF, these tests were nothing out of the ordinary.  

6 Minute Walk: Next was the 6 minute walk. We were able to go to a hallway where there were not any other patients which meant I could take off my mask. Whew! The took my vitals then had me walk the hall for 6 minutes as quickly as I could. I then had my vitals taken again before being dismissed. Pretty easy and painless test!

Fasting Again: I could not eat 2 hours prior to my CT scan so at 4 I started my second fast of the day.

CT Scan: The last test(s) of the day was a CT scan of my chest, abdomen, and sinuses. They were able to use my Port so I didn't need an IV which was a huge relief. This was my first time with contrast. The tech explained I may feel a bit of a headrush and then I may have a sensation like I am peeing my pants. He told me a story while he was pushing the contrast into my port which in hindsight I am sure was a ploy to distract me from any sensations I was feeling (and maybe to monitor if I was having an allergic reaction which is very rare, but can happen). I was pretty distracted, but did feel a bit odd in the head and chest followed by a very warm sensation as if I was peeing myself. I was glad for the warning. The test was pretty easy and besides  the port placement it was painless.

The End Of Day 1
That was a super long post for a super long day! Needless to say we were pretty tired by the end of the day!

Friday, April 12, 2019

Pre-Transplant Evaluation Concerns

So I spent the past 4 days at Stanford getting a lung transplant evaluation. For me, the hardest part about going to the evaluation is that every aspect of the process was a complete unknown. Despite trying though a few avenues to learn about the evaluation process (via people who have been through it) I was going in pretty blind. I am a planner. I do not do spontaneous, unknown, unprepared well. So this week was a challenge to say the least. I had eight million questions and nobody to answer them.

I did however, find a blog from someone who was transplanted in 2012 and she wrote a detailed explanation of the evaluation process. Despite her transplant evaluation taking place several years ago her schedule looked almost identical to mine. I read through her post several times as a way to prepare. And even so I had so many more questions. So I am going to go through each day (through multiple posts), but today I wanted to clear up some of my biggest concerns that I could not find addressed anywhere else.

The schedule they give you is pretty intense especially for Monday! I had 8 appointments in that single day. 5 were scheduled and 3 were to be done "at my convenience" throughout the day. My first concern was things getting behind schedule. I was really worried I may end up late to appointments or even completely miss them. I was reassured that if a test was running late we only had to talk to the front desk and they would make the appropriate calls to keep us on track. Also, every time I had a test the tech (or whoever was preforming my test) was well aware from my chart that I was booked for tests all day and they kept things running smoothly. Actually, my first blood draw couldn't be completed because I was there at 6:30am to get my blood drawn and the TB test couldn't be taken until after 8. The lab technician left my case open so when I returned for my TB I wouldn't have to wait in the long line that had formed. Every test up until my clinic visit was right on time. Of course, after clinic was a whole different story, but I will get to that later.

Some of my appointments were scheduled very close together and had different wings for checking in. I have been at the same hospital for my entire adult life and I often find myself lost in the hospital walls because everything looks the same and feels a bit disorganized. I pictured myself wandering lost and late for appointments. Stanford is the most organized and easy to navigate hospital I have ever been to. It is almost impossible to get lost. And if you are confused there are so many people more than willing to help. A janitor walked us completely across the hospital to show us where the heart cath lab was located.

Another really nice aspect of Stanford is that they have valet parking for only $2 more than regular parking. It took so much stress off of trying to find parking and walking to the hospital. It also meant my husband and I weren't separated like we would have been if he dropped me off and then left to park. The parking attendants were so nice and recognized us everyday. Side story about the parking attendant, at one point during my evaluation I stepped outside the side door to cough into my sputum sample cup because I was trying to cough in privacy. The parking attendant just happened to walk by just as I spit a huge loogie in my sample cup. At first I was horrified because...gross, but he just gave me a thumbs up and a smile. I swear everyone at Stanford was so so nice.

Overall, the evaluation went much smoother than  I expected. The last day was rough, but I think that was because we ran into a few issues that I assume are not typical. If it wasn't for that last day I would say the experience wasn't nearly as bad as I had imagined. Even the tests, for the most part, were pretty easy and pain free.