Tuesday, August 30, 2011

Finally Got the Shakes

Nutritional Shakes I mean.

Insurance approved the nutritional shakes so I don't have to pay out of pocket. They aren't Scandishakes and don't taste as good but it is better than nothing.

I don't understand insurances sometimes. Mine wouldn't approve Scandishake because it is too expensive. I understand, it is a pricey drink (over $100 per month). The other stuff is cheaper by the dose, but to get the same amount of calories as what I would get from the Scandishakes I have to drink twice as much. Which means 4 cans a day. That comes to 50% more in price compared to 2 a day of the Scandishakes. If I did math that badly in school, I would have never made it into Algebra much less Calculus. I'm not sure how these people get to keep their jobs.

Anyways, I'm taking what the insurance will pay for. It doesn't have that weird metal taste that most shakes seem to have (which I hate). It has this weird heaviness though. I'm going to have to experiment with the stuff to make it more palatable. I mean if I'm supposed to consume between 3-4 cans of the stuff, I need to come up with ideas as to how to do that. I wonder if I can use it to make cookies. At least it doesn't upset my stomach like some other shakes do.

I am still looking up high calorie recipes. It's hard these days since so many people are trying to lose weight. I want to gain weight but don't want to consume unhealthy calories. I need some avocado trees. Then this weight thing would be no problem. I have found that if you look on the body building sites they do have some good ideas for nutritional high calorie foods. Some just lack in creativity. Darn me for inheriting my grandfather's palate.

On an upside. We are starting the fundraising journey for my transplant. All of the details from the transplant and fundraising aspect will be posted here

Thursday, August 25, 2011


The Glossary is a work in progress. This just gives a general definition of commonly used terms in the CF world. By no means is this to be considered medical advice. I have tried to include links that give a more in depth explanation than I can for each term. Please let me know if I am missing anything.

65 Roses: Another name for Cystic Fibrosis.


Allergic Bronchopulmonary Aspergillosis: ABPA. Asthma like response to aspergillus colonization in the lungs.

Aspergillus: Fungus that can attack CF lungs.

Beating: See CPT

Breathing Machine: See Pulmo-Aide


CFer: Patient with Cystic Fibrosis. Usually can be identified by their "smoker's" cough, purple fingernails and blue lips (natural Gothic look) :o)

Chest Vibration: See Vest.

CPT: Chest Percussive Therapy. Therapy designed to loosen mucous from the lungs so that the patient my expel it.

Creon: Brand of pancreatic enzyme. Aides in the digestion of fats, sugars, and proteins.

Cystic Fibrosis: A genetic condition that mostly affects the lungs and digestive system. Other organs are affected over time.

Cystic Fibrosis Foundation: A place to get up to date information on Cystic Fibrosis, medications, treatments, trials. Anything related to CF.

DNase: See Pulmozyme

Dornase Alpha: See Pulmozyme

Enzymes: Catalysts in the body that speed up the breakdown of molecules. When used on this site, it refers to the capsules that most CFers take to aid in the digestion of food. There are a few brands on the market, but they usually all contain a combination of protease, amylase, and lipase.


Lock Up: Term referring to hospitalizations.

Lung Oyster: Mucous expelled from the lungs.

Miralax: A laxative that is mixed in liquid. Helps rehydrate the intestines. Certain brands of enzymes dehydrate the intestines. Miralax reverses this and reduces constipation.



Nebulizer: Cup like device that connects to a pulmo-aide by a long plastic flexible tube. Designed to aerosol liquids so they can be inhaled during treatments. Several designs out there, each corresponds to specific types of medication. Allowing optimal delivery and the least medicine waste.


PFT: Pulmonary Function Test. A serious of breathing tests that measure how much air the lungs are able to take in and expel in a certain amount of time. Gives doctor an idea of how well the lungs are doing.

Plug: Thick wad of mucous blocking airways. Some are like small pebbles others are lettuce-like in shape. Hard to bring out. Can be painful.

Postural Drainage: Patient lies at a slant to the ground. Head towards the the ground and feet up towards the ceiling. Gravity pulls mucus from small to large airways where it can be coughed up.

Pounding: See CPT

Pseudomonas Aeruginosa: Bacteria known to colonize lungs of CFers.

Pulmo-Aide: A medical grade air compressor. They're designed to provide a certain amount of air pressure needed for nebulized treatments (usually 8 liters/minute). There are several brands out there.

Pulmozyme:  An inhaled enzyme designed to break up mucous. Makes it easier for CF to expel the mucous and helps reduce infections (and plugs).

Resort: Term referring to the hospital.

Scandishake: Hi Calorie nutritional shake. Usually about 600 calories per serving. Comes in vanilla, chocolate, and strawberry.

Spa: Term referring to the hospital.

TOBI: Inhaled tobramycin. Antibiotic designed to weaken the bugs that tend to colonize CF lungs. Doesn't taste too good when inhaled. Can cause sore throats. Recommend milk or butterscotch candy to coat throat. Chew lots of peppermint gum to get rid of the medicine taste after treatment.

Ultrase: Former brand of enzyme. See Enzymes.

Vest: An inflatable vest is attached by flexible tubes to a machine that vibrates it at high frequency. The vest vibrates the chest to loosen mucus. Kind of makes you look like an alien in a space suit.

Back From Camping and a New Toy

The camping trip was great. Breathing in the salty coastal air makes me feel much better than when I inhale Hyper-Sal. It could all be in my mind or it could be a real difference. I just know that if I could, I would live on the coast full-time rather than these short visits. I'm hoping to make another trip in October for the whale migration. That should be fun to watch.

The coast was freezing though. Usually, there's a break of two hours during the day where it's warm enough to wear shorts and brave a dip in the ocean. This time it was cold all the way through. I kept myself pretty well bundled. I was thankful for the sleeping bag and cot my dad gave me. I stayed warm and dry.

Packing for a camping trip can be tricky, but doable. In order to maintain good health I can't skip out on treatments. And with no electricity, it takes a bit of planning. That's where a battery operated pulmo-aide comes in. There's a few out there so you have to find one that fits your needs. I like mine because it's light-weight and I can recharge the battery in the car. When I was younger and there wasn't a battery operated pulmo-aide, my family brought a generator so I could do my treatments. Generators are quieter now than the one I used, but it served it's purpose. I got to go camping. They still don't have a battery operated Vest machine so my mom does manual CPT. So both of us have to take time out to do my treatments. When it comes to packing my meds, I always pack a couple of days extra. It's better to be prepared than run out of meds and have an "Oh crap" moment. I have a small cooler specifically for meds. Keeps me from having to dig in the food cooler for little vials of pulmozyme or TOBI. I also let my doctor know I'm going camping so he can give me a few "just in case meds". Usually it's Bactrim and more Prednisone. Those are in case I get sick and need something to tide me over till I can get to a CF clinic. I also pack a ton of Purell. I'm out in the dirt and don't want to give germs an invitation.

So a camping trip is doable. And it's worth all the planning. I had a blast watching my dog chase the waves. And my niece learned to collect shells.

While I was camping, I received a phone call from Hill-Rom. I had been calling them and asking for a new vest machine for the last two years. They kept having reasons as to why I couldn't have one. It was getting to a point where I was going to just save the money and buy one myself (I'd be in my forties, but hey). See my Vest is one of the earlier models (103) weighs half as much as me and has no wheels for me to move it around. My first one I had used so much it finally gave out, and I was on my second one. I had already twice as many hours on the second one than I did on the first. It seemed I was going to be stuck with the same model for the rest of my life. Then I got the phone call. Seems I finally qualified for a free upgrade. I was ecstatic. They sent me the latest model. It weighs about 17 lbs (which is less than my dog). It has its own carrying case with wheels. And it is so easy to use. I sent back my old model. The new model should be able to travel with me. And I put it on a wheeled table so I can push it from room to room.

On top of camping and a new Vest, I am moving. For someone who enjoys permanence I sure move a lot. I'll be closer to family (right behind my grandparents) and out in the country. The sad part is I have to say goodbye to mountain life. I love having my own winter wonderland when it snows. I will dearly miss that part. Upside, there's a lot of trees at the new house so I'll get to be outside more (no trees here, so it gets like a desert in the summer).

The big move day is in September. I still have a couple of weeks.

Friday, August 5, 2011

Monday's Appointment

I had an appointment with my regular CF team on Monday. Since I was feeling so good, I figured it would be a short appointment. Boy, was I wrong. I might have been feeling good but the other CFers at clinic weren't. The team had to deal with them before getting to me. My PFTs are up. Not where I want them (I prefer at least 60%, I'm at about 29%).

Not much change in my weight so I'm going to have to start drinking shakes. Unfortunately, my insurance won't cover the one my doctor wants (Scandishakes). The shakes my insurance will approve makes me sick, so we're going to see if my insurance will approve a different shake that's not as pricey as Scandishake. If I could I would just pay for the Scandishakes myself. I've had them before without any problems. The state doesn't provide me with enough funds to be paying out of pocket for them. So I'm waiting to see if I'll get the other stuff.

We still have no clue as to why I feel exhausted by midday. I sleep eight to nine hours at night and still need an hour nap in the afternoon. The doctor wants me to get a CT scan. Basically, trying to see what else we can do since I'm "too healthy" to be put on the list. He also said that my sugars plummeting is probably a sign of my pancreas giving out. The doctor thinks that my pancreas isn't making glucagon anymore. So now I have to do more snacking to keep my sugars level. This CF business is hard work.

A good note. My sinuses had been swelling up pretty badly and causing me some severe pain. So my doctor recommended I try taking Ibuprofen each day to reduce the swelling. Well, it worked (woot, woot) and I noticed that I was in a lot less pain in general. My back isn't spazzing, my knees don't hurt, and my hands aren't locking up. So I asked him about taking some ibuprofen every day and my doctor said I could as long as it didn't upset my stomach. He gave me a maximum to not surpass. So now I'm taking a small amount of ibuprofen each morning and I am able to be more active throughout the day.  I'll see in a couple of months how I'm feeling.

So an appointment that should have lasted an hour, lasted for three hours. I was starving by the time I left. I think I should start packing a lunch for these appointments.

Wednesday, August 3, 2011

August Update

I think my last entry was a little hurried. I was trying to cram a lot of stuff into it and didn't adequately cover my appointment at the transplant clinic.

I can say that after a year of making the four hour drive (one way), my mother and I have decided to drive to Stanford the night before and stay at a hotel. That way I'm more rested for my tests.

The clinic is going through some changes. Some I don't like. Before, my appointments were in the afternoon. My afternoon PFTs are always the best. I've had time to clear crap out and a chance to eat a couple of times. Now appointments are being scheduled in the early morning. Not so good since I still have crap to clear out of my lungs and I can't eat right before a PFT (all that squeezing the air out can cause vomiting). The other thing I don't understand is that during PFTs I'm doing a long of coughing, but they don't ask for a sputum sample until I get to an examination room. By then I have nothing left to get out. This last time I had to jump up and down and then have my mom beat my side, just so I could bring up a sample.

The part that annoys me the most about the changes is that they are combining the Stanford Adult CF Center with the Transplant Center. I don't see eye to eye with the CF doctors there. And they have different ideas about my care. I constantly have to tell them that I will bring their ideas to my CF team at home. I see no point in switching care teams, when the one I have works so well for me. You don't find a doctor you like that often and I've had one for the last 10 years. Not switching teams if I don't have to.

One of the more positive changes is that the lab for all the blood drawls is right next to the new clinic location. Before I had to hop in the elevator, go down a floor and then walk all the way to the other side of the hospital. For someone with limited lung capacity, that was a long and breathless walk. Wish they had food next to the lab though. I always have a ton of blood taken out that I feel funny.

What I don't like about the lab is that all the spots for drawling blood are right next to each other with no dividers. I have trouble with having my blood drawn and have trouble with the site of blood. I had to keep my eyes shut the whole time, because they had a guy in the chair next too me having a massive amount of blood taken out as well. Sometimes people just don't think everything through.

The appointment ended with the doctor saying I still wasn't sick enough to be listed. I am starting to wonder what they consider sick enough. I can't work, I can't swim (which I loved), I can't hike, I can't run, I can't ride my bike, I can't chase my niece around. I can sit at home and watch TV all day. It's just exhausting dealing with these people.

Maybe some miracle will happen and my lungs will find a way to heal on their own. I tend to baffle doctors so maybe I'll do it again.

Mid-July brought my 30th birthday. Every CFer knows that is a huge milestone. My family threw an Over the Hill party for me. There was tons of food. And I had a blast. That night we camped out in my grandfather's tipi (my niece calls it a TV). Turning 30 was great. I guess it helps to be 30 and look like you're barely 20.

I think the issue with the Christian group has been resolved. We shall see. The people involved gave an apology and just said that they were trying to protect me. I let them know that I didn't make it to 30 by taking unnecessary risks. Also, if they wanted people to fly they need to let them fall on their butts a few times.

In a week I'll be camping on the California coast. Nothing better than salty air and hearing the ocean as you fall asleep. This should be fun.