After several calls, Richard came into the room and told his Mom, "I know what you are working for." Mary was dumbstruck because Richard did not know what she was doing, nor did he know that he had cystic fibrosis. With some trepidation, Mary asked, "What am I working for, Richard?" He answered, "You are working for 65 Roses." Mary was speechless. He could not see the tears running down Mary's cheeks as she stammered, "Yes Richard, I'm working for 65 Roses."
Since I've started blogging, I haven't really vented about CF here. But after visiting a friend in the hospital today, I just feel the need to get some things out...
I met a young girl through her dad, Joe. Joe was a sweet man and adored his two daughters (H and S) and his wife (T). His youngest of daughters (S age 25) has CF and when I met her she had some mild complications but nothing severe.
Joe died a couple of years ago and if he were alive today he would be very sad. S has started having more complications within the last year and is in the hospital again. I finally went to see her. She looked tired but was in good spirits. Her mom was with her. I gave her a big hug...I can't imagine how she's feeling right now. Well, I can imagine...I guess that's why I'm venting, right?
The doctor's told S that she needs to start thinking about a lung transplant. They were also planning on sending her to stay at the University of Iowa hospital to get care from the adult CF team, since we don't have one in Des Moines.
I stayed about an hour and we talked lots. I hugged them goodbye and went to pick Andrea up. I knew I had to tell her about S and it just made me sad. You see, we really work hard at staying upbeat. At not letting the beast that CF is get to us. But alas the beast wins sometimes and today is one of those days of defeat.
Andrea took the news so-so. She asked some questions but she had that look like she didn't really want to hear what I was telling her. You see, last week during her CF clinic she learned more about the cirrhosis in her liver. The GI doctor had to increase the dosage of her liver medication since she's bigger and weighs more. Andrea waited a few days, then asked what all that meant and if it will get worse. It was hard explaining it to her, but it had to be done. She understands better but I know it must be difficult at her young age to absorb all of this. And now our friend S is possibly going to need a lung tx? It's a lot to take in in such a short time.
Lately there have been more hospitalizations of kids' that I know because I'm friends with their moms. Andrea knows most of them and has even friended one of them on Facebook. She asks about these kids often, so I know she thinks a lot about how CF affects them, and I'm sure she worries if what happens to them could happen to her. Oh how I hate that she has to worry about things like that!
My profile picture on Facebook is FUCF because that's what I keep repeating lately. I rarely say Fuck You CF...it's usually just Eff U CF. But I can honestly say that right now and probably for the next few days it will be...
As most of you know, part of the routine of a CFers life is to attend a CF clinic every 3 months. At the accredited CF clinic, Andrea meets with specialists to discuss how things are going with her and all her CF needs. This includes (for the most part) meeting with a pulmonologist, gastroenterologist, chest phyisotherapist, dietician, social worker and a pharmacist. We also always visit with a respiratory specialist and Andrea does what is called a PFT (pulmonary function test).
The purpose of a PFT is to get a reading of how her lungs are doing. They read the numbers of the FEV and FEV1 (forced expiratory volume). The FEV1 seems to be the one they really look at most (forced expiratory volume in the first second). Andrea started doing PFTs when she was 5 and we've been lucky that her FEV1 has always been high, usually above 100! Which means her lungs are doing better than most people that don't even have CF.
Here's a quick video of Andrea doing her PFT. This was shot in 2009...
Today (8/18/11) was an annual clinic visit. The annual visit consists of what I mentioned above plus lab and other tests: blood work to check vitamin levels, rule out infection, assess iron levels, check for liver disease, nutritional status, and CFRD (cf related diabetes). Andrea also has to have a chest xray and ultrasound of her liver, stomach, gall bladder, intestines, plus they do a glucose tolerance test as well.
We started our morning checking in at 7:00 a.m. to get paperwork ready for all the tests. We then went to start the first of a few vials of blood to be drawn. This time it was only 7. She must be fasting during this portion. She thens drinks a high glucose concentrated drink and must come back for more blood to be drawn 2 hours later. We left there at 7:30.
We headed to radiology and didn't have to wait long. First was the ultrasound, which takes about 20 minutes, then the chest xray, that took about 10 minutes. We were ready to head to get PFTs done.
The PFT room has changed a bit and they have a new computer system to run the tests. I waited in the larger room, just outside from where Andrea does her PFT. I couldn't believe what I was hearing. When she was doing the second of three standard breathing techniques, I turned to look because she sounded like a power horse. Her breathing sounded so great and when we got the numbers they proved it to be so. Her FEV1 was at her all-time highest of 119!
By the time we finished with PFTs and got to the CF clinic it was 8:30. Our appointment wasn't until 9:30 but they got is in within minutes. She got weighted again, and measured AGAIN, and had her blood pressure checked. We couldn't wait to tell everyone how PFTs had gone.
We met with the nurse first and she handed us a sheet of all her meds, to go over them and make sure all was accurate. I noticed it didn't include the calcium we started giving Andrea about a year ago. There are CF standards that is followed regarding meds one must take depending on symptoms one is having: miralax to help with bowel obstruction, pancreatic enzymes to assist with food fat absorbtion, azithromycin antibiotic to help avoid lung inflammation, singular and nazonex for sinus allergies, ABDEK vitamins to help maintain levels at a norm, ursodial to assist with her liver cirrhosis (inflammation and scarring), advair and xopenex for asthma symptoms, pulmozyme to keep mucus thin so it's easier to cough up and spit out AND on top of all of these--on our own--we give her omega 3/6 pills plus the calcium.
The best part of going to CF clinic is the team role we each play. Of all the meds mentioned above, the doctors did not prescribe the omega and calcium. There is no study saying this helps, but with research I've done I believe it helps overall and decided to start her on those...so the team makes note of it because it plays a huge role in her CF treatment. Whether they like what I do with Andrea or not, together we keep each other informed of her overall healthcare needs and proceed, keeping in mind that things may need to change depending on what transpires as she grows older.
We met with the social worker next. They're starting a new program with the CF teenagers to help them stay compliant with their CF protocol. It's available through Novartis, the makers of TOBI (inhaled antibiotic). Lucky for us, Andrea is very compliant...but through the questionnaire she filled out she did notice she needs to work on eating a healthier breakfast.
By the time we were done with the social worker it was 9:30 and time for the second of blood to be drawn. Normally it would have only been 2 vials more, but because Andrea volunteered to be part HOLA-CF they took a total of 5 vials more. This is a big deal, because Andrea does not like needles and she used to faint when having her blood drawn. But she was a trooper and did fantastic!
HOLA-CF is a study of Health of Latin Americans with CF and they are investigating potential genetic modifiers among Latinos with CF. Andrea gets $20 to participate in this study. BONUS!
Back we went to CF clinic. We met with Dr. Bacon our pulmo doc, then with Elaine our dietician, Dr. Sloven the GI doc and lastly with the nurse so Andrea could have her throat swabbed. This is to check for any bacteria growth inside the lungs. We left clinic with everyone elated of Andrea's PFT numbers and her overall excellent health. And just when we thought we were done...we had to go to the diabetes clinic and meet with our endocrinologist.
CF affects not only the lungs, but the digestive system, liver and pancreas. Because of that many CFers will get CF related diabetes. Thus the reason behind the glucose test. We met with Dan and Andrea's fasting numbers were great, under 100, but her non-fasting numbers were above the 140 range. It proves that Andrea's body has a hard time handling too much sugar and that we just need to avoid an overload as much as possible. Thankfully she does not need insulin and thankfully she's not a sweet tooth or a simple/carb junkie!
By the time we left the hospital it was almost noon. Five hours of meeting with docs, having lab work and tests done, talking and repeating ourselves over and over again. We went out for lunch, ran one errand, got home and we both hit the sack. Andrea napped for almost 4 hours and I for almost 5.
So there you have it. A very long-ass blog about our long-ass day during an annual CF clinic visit. I promise not to blog about it again, but I get asked a lot about how our clinic visits are, especially the annual ones. Now I have it in writing and can direct people to come read this. And if you've read this far...thank you! Thank you for your interest in Andrea's life and taking time to learn more about it.
I haven't posted anything since our 65 Roses countdown project ended on May 20th. After Great Strides I got busy with lots of work and getting ready for our trip to Peru.
Yes, I took the kids to Peru for a full month. Jeff joined us for 10 days. Going away for 30 days does take a lot of planning, making sure there's enough money to cover bills and to have during our travels; packing, scheduling tours, etc. are what everyone does when they travel. Then you add CF into the mix and boy is there a lot more planning needed.
Because we'd be gone for a full month, I had to make sure I'd have enough CF medications while we were there. Our refills are due around the 3rd week of the month, so I had to call our insurance company to make sure they would okay a 2 month refill. Luckily they did. I spoke with the pharmacy as well and all was set. Two months worth of miralax, advair, zithro, singular, albuterol, urso, abdeks, and pulmozyme were in check. I had one carry on strictly for meds and vitamins and one carry on for her vest and compressor.
I was prepared to be pulled aside during the security check and sure enough I was. I prepared the kids for this too, so they wouldn't be scared why we were asked to step aside and go with the TSA officer to look through our bags. I had all my paperwork in order...prior to leaving I had the hospital write a note on their letterhead, stating why Andrea needed all the things we were bringing to Peru. They also made a list of all her prescribed and over the counter meds she's on. I was prepared for everything except for the personal question the TSA officer asked..."bless your heart, how do you handle all of this? I have a hard enough time packing my babies diaper bag." I don't know why that caught me off guard. I guess it was the anxiety of traveling and I hadn't slept a lot...but I could barely answer her because I got choked up. I actually thought to myself "ya, how the hell do I do all of this?" I composed myself and gave her the "you gotta do what you gotta do" answer. I was glad to just be done with the bag check and get going to our gate.
Traveling with all the carry on bags was a lot of work. When I purchased the ticket I stated we'd have medical equipment with us and this actually helped a lot. The attendants checking our tickets knew of this and we were allowed to board before everyone else and get settled. It makes me uncomfortable to have people waiting in line on the plane while I put my carry ons overhead, so to do this calmly without a line of 20 people behind me helped a lot.
Once we arrived in Peru, it was time to unpack all the CF things and put them away, and find a place to keep the vest and compressor. My mom's place felt like home so this wasn't too bad. I had bought some transformers because the electricity voltage is a lot higher there, but what I bought could not be used for large items like the vest and compressor. Luckily my mom had one transformer we could use, but only one. So I used that one for the compressor so Andrea could do her pulmozyme inhalations, while I manually pounded her back during the 7-8 minutes it takes for her to inhale the pulmozyme. After she was done with that, I plugged the vest into the transformer and she finished her session using the vest machine. We eventually bought another transformer so I wouldn't have to pound her back by hand every night.
Traveling with pulmozyme can be tricky. It must be refrigerated and not exposed to sunlight, but it can be kept at room temperature as long as it's not more than 24 hours. I did have an ice bag I wrapped the meds in while traveling from the US to Peru, but when I traveled within Peru I would just pack it without the ice pack and as soon as we arrived at our destination I would place it in a fridge. This meant asking the hotel receptionist to place it in the kitchen of the hotel, because our hotel rooms in Peru did not come with an individual mini fridge. Cleaning the nebulizer was interesting as well. We started using the sterilization bags recommended by our CF clinic, which one places a small amount of water in the bag along with the nebs, then you just nuke it for 1-1/2 minutes and it's clean. But in Peru the water is not potable, so I had to make sure I had bottles of water to place in the bag with the neb, and get permission to use the microwave in the hotel kitchen as well. If there were no microwaves, then I'd have to get the okay to boil the neb. Luckily everywhere we stayed went above and beyond to accommodate our needs.
Jeff manually doing CPT while Andrea does her inhalation treatment of Pulmozyme
My darling husband helped me pack our things when we went to visit Cuzco. He was in charge of the vest and compressor. Our first night in Cuzco, as I went to unpack the vest and compressor, everything was there except the actual vest Andrea must wear to do the chest physiotherapy (CPT). I was fuming, but calmed down and nicely told Jeff that he would have to do the CPT manually during the entire trip in the "city of the Inkas". Five days of Andrea being manually pounded on her back and chest, Jeff was tired but did an excellent job. However, on day two of our trip in Cuzco I plugged the compressor into the wrong transformer and POOF the compressor "blew". We had just poured the pulmozyme into the neb and the tour guide was coming in two hours to pick us up for that days tour. For those of you who know pulmozyme, you know that that one dosage of liquid is worth almost $100 bucks. Off I went to the front desk and explained our problem. They called a nearby clinic and sure enough they said they had compressor we could use. Off we hurried to the clinic, after telling the rest of our traveling party the problem. We get to the clinic and there's no compressor! They had an oxygen type of tank machine and I had no idea how we could use that. The doctor on call had to evaluate Andrea (thank goodness for all the paperwork I brought with me describing her disease and medications), then he called a pulmo specialist and with the help of that doctor they were able to switch something around with the oxygen tank and it worked perfectly for the nebulization treatment. The doctor was interested in Andrea and asked a lot of questions. He was amazed at how well she looked and congratulated Jeff and I for our healthy daughter. We went back to the clinic two more times for her nightly CF routine and it ended up costing approximately $30 worth for the use of their "compressor".
At the O2 Clinica in Cuzco - Peru waiting to do an inhalation treatment
Andrea was amazed at how calm I was. She even asked me how I could stay calm and I answered, "I have to stay calm Andrea, because if I don't what would happen?" Andrea replied "you'll feel awful?" "Exactly!" I said "and I don't want to feel awful, so I choose to stay calm instead." Without knowing, this was a valuable lesson for her and for me. While maintaining calm, I was actually able to ask the right questions and provide the help my child needed to keep her going with her CF needs. I was a little concerned with my family waiting for us, our tour started over an hour late, but everyone was very understanding and our day ended up being a fabulous one.
Our trip to Peru was a trip of a lifetime. We enjoyed our full month spending time with family, visiting magical places like Machupicchu and Tarapoto, enjoying the beauty that nature gives us and eating fresh healthy food daily! But the best thing about this trip was the lesson Andrea got out of it...that despite having CF she is capable of traveling the world while keeping up with her CF protocol. That CF doesn't have to take control of her life...she's in charge and she alone has the right to live life to the fullest!
David and Andrea at one of the 7 wonders of the world: Machupicchu - Peru
