kayray.org

Yesterday I felt tired all day, and today I feel tired and headachy. Yuck. I remembered http://www.framerater.co.uk/home and have been having a nice time checking movies off the lists, and adding ones I haven’t seen to my Netflix queue. And I watched a few movies yesterday, too. Finished “Crimes and Misdemeanors”, which didn’t do anything for me. Also watched “The Lady Vanishes”. Not that great. But “Witness for the Prosecution” was fun! Charles Laughton FTW.

Today is my 15th day on Tyvaso, the inhaled medication I’m taking to treat my pulmonary hypertension (see previous post).

The first thing I’ll say is: if you’re starting Tyvaso and it seems daunting and miserable and horrible and impossible, be brave and stick with it. It gets better! I’ll try to write down as much detail as possible in case it might help someone who’s going through the same thing. Let me know if you have any questions.

On Friday July 8th a big box arrived from my specialty pharmacy containing the actual medication plus two nebulizers (one to use and one spare) and all their parts and accessories, and three or four instruction books that all say the same thing, and a little carrying case, and some other random stuff… Don’t worry, you will be able to pack most of it away in the closet in case of emergency. The stuff you need every day will only take up a little room in a kitchen cupboard or somewhere like that.

You get three power sources for the nebulizer — regular wall-wart, rechargeable battery pack, and a car adapter, plus every kind of international plug adaptor. I leave the wall-wart plugged in in the kitchen (which is where I use the nebulizer) and keep the car adaptor in the carrying case.

Stephanie, a nurse from the specialty pharmacy, showed up that afternoon to teach me how to use the thing. Actually, TWO nurses showed up… the second was there because Stephanie travels all over the west coast, and they wanted someone local to be familiar with my case. Anyway nurse #2 wasn’t important.

Stephanie showed me how to assemble, use, and clean the nebulizer. It’s quite small. Looks like this when it’s ready to use:

The base contains a little bit of distilled water. A little conical cup sits on top of the water, and the medication goes in the cup. The machine vibrates the water a few million times a second, which vaporizes the medication. The circular parts contain filters and you breathe through the nozzle thing.

In the morning, you put water in the base, put in the Tyvaso, put fresh filters in, and put the whole thing together. After each treatment (four per day), you take off most of the bits and put some plugs in the holes:

This keeps the water and Tyvaso contained, and you can even knock the thing over and it’s ok. At the end of the day you take it all apart, dump out the base, wash all the plastic bits (just a swish with soapy water, nothing fancy), and let it air-dry till morning.

The machine has a timer and tracker so you know how many breaths you’ve taken. It seems really confusing and time-consuming and intricate at first, but dealing with the little machine becomes habit really fast and it’s nothing to worry about. I can do each treatment in under 2 minutes, probably.

The HORRIBLE HORRIBLE HORRIBLE part is actually breathing the stuff. I don’t know if it makes everyone cough they way it did me, but wow. After my very first breath I was coughing so badly I could hardly breathe and I had to pause a long time between breaths. After the three-breath treatment (they start you at three breaths, four times a day and work you up to a higher dose slowly), I coughed violently non-stop for the full four hours until the next treatment. My mouth and throat were miserably dry, and my throat was raw from coughing. It was AWFUL. There are several things that can help the coughing and dry mouth for some people — lozenges, hot tea with honey, yogurt, etc., but none of them helped me even the slightest bit. This was extremely depressing. I couldn’t do any recording at all and it felt as if I never would be able to again, and all the coughing left me so exhausted I couldn’t do anything.

It was almost unbearable for about, oh, three days. I just lay on the couch, coughing, and waiting for four hours to pass so I could start it all over again. Ugh. After that, it got slightly better each day. Still bad, but slightly better. Then, after seven days, I had to increase to four breaths at a time and the misery started all over again, almost as bad as the very first day.

However — on the 9th day I noticed that, despite the coughing, I actually felt a little better for a few hours. I felt like I didn’t need my oxygen, I had a little bit of energy, and I felt, you know, like maybe the Tyvaso was actually helping a bit!

Ever since then, the coughing has been decreasing day by day and I would say that, on the whole, I definitely feel better. I’ve been running errands without taking my oxygen along, and not missing it. I don’t always feel like I need to spend several hours lying down in the afternoon. Some afternoons I do, but not every day. One evening I even had enough ambition to go to the store for ingredients and baked a pie! A very simple recipe, but still something that would have seemed pretty near impossible, or at least a very bad idea, just a few weeks ago. My poor family has been living on frozen pizza, bagels, and take-out for far too long because cooking proper meals has been just too exhausting for me. I have the energy to cook more often now. In fact, today I’m planning to make a big thing of scalloped potatoes, with grilled chicken and asparagus.

Yesterday I moved up to five breaths per session and I tolerated it really well. Just a little bit of extra coughing, maybe. I think I probably won’t have to wait a full week before increasing to six, and then I’ll be two-thirds of the way to the full dose of nine breaths, four times a day.

One day last week I had to take Henry to the dentist and knew I’d be away from home at treatment time, so I took my little machine along (with its plugs in place you can just toss it in the carrying case and it’s fine), plugged it into the car, and it was all easy and quick. It made me feel less tied down to know that I could easily be gone all day if I just remember to bring my machine.

To sum up: if you’re just starting Tyvaso and feeling really miserable, don’t despair. It will probably get better for you just like it did for me, and maybe you’ll notice some benefits too!

I don’t usually talk about my health here, other than complaining about normal stuff like sore throats and colds, but Dan thought it might be helpful to me (and possibly others with the same condition) if I blog about my pulmonary hypertension, with which I was diagnosed in 2004 or so.

From wikipedia:

Pulmonary Hypertension (PH) is an increase in blood pressure in the pulmonary artery, pulmonary vein, or pulmonary capillaries, leading to shortness of breath, dizziness, fainting, and other symptoms, all of which are exacerbated by exertion.

PH makes it harder for the heart to pump blood through the lungs, much as it is harder to make water flow through a narrow pipe as opposed to a wide one. Over time, the affected blood vessels become both stiffer and thicker, in a process known as fibrosis. This further increases the blood pressure within the lungs and impairs their blood flow. In addition, the increased workload of the heart causes hypertrophy of the right ventricle (a condition known as cor pulmonale), making the heart less able to pump blood through the lungs, ultimately causing right heart failure. As the blood flowing through the lungs decreases, the left side of the heart receives less blood. This blood may also carry less oxygen than normal. Therefore it becomes harder and harder for the left side of the heart to pump to supply sufficient oxygen to the rest of the body, especially during physical activity.

I’ve also got a chronic dry cough, a rare symptom caused by my swollen heart tickling some nerves in my chest (they think).

My PH was caused by a birth defect in my heart.

I’ve been really lucky. PH is a rare disease and there is no cure and not many treatments, but mine has been kept under control all these years with simple pills — lots and lots of them (2 Tracleer and 12 Revatio per day), and they’re costing my insurance company untold thousands, but relatively easy for me to deal with.

I’ve had to take it a bit easy and cut back on activities, but up until a couple months ago I had enough energy for all my daily tasks, slow walks, the zoo, etc. Just no hiking, dancing, or running about :) We hire a cleaning crew to take care of the mopping and vacuuming.

But I started feeling much worse recently, which was frightening and frustrating. I don’t like to think about my PH, obviously, but I had to when I was suddenly getting out of breath on the shortest, slowest walks and around-the-house activities. My doctor saw me and ran a bunch of tests and it looks as if my heart is working much too hard, so it’s time for more aggressive therapy. Ugh.

The easiest and least invasive of the three possibilities is Tyvaso, a medication which I will breathe out of a small machine four times a day, so my doctor says he’ll start me on that and see how it goes. http://www.tyvaso.com/.

It’s the kind of thing, like my other fancy medications, that will be shipped to me by a specialty pharmacy every month. I’m pretty freaked out about this. I feel like I should be extremely grateful that such a medication exists at all, and that I have access to it. And I am. Of course. But it still seems scary and difficult. And I’m angry that I have this condition at ALL, and that it’s getting worse.

I also have an oxygen concentrator, a wonderful magical machine which sucks oxygen out of the air and sends it down a tube for me to breathe all night (without it I sometimes wake up in the night feeling breathless). It was hard for me to accept the fact that I need it; I don’t feel like an invalid, but breathing out of a tube seems so invalid-esque. However, I got used to it, and now I love it. Sometimes I use it in the daytime if I’m feeling worn out. It’s very soothing.

Now my doctor says I should use oxygen whenever I do anything active, so yesterday an oxygen company delivered some small oxygen cylinders. They’re about the size of a wine bottle, and I have a shoulder bag to carry one in when I go out. This is another hard thing to accept. How will I feel at the store, or the zoo, with an oxygen tube under my nose? But maybe I’ll be able to do the shopping without getting so worn out if I have enough oxygen.

Anyway. That’s what’s going on with me. I should get my Tyvaso next week, maybe. A nurse will show up and teach me how to use it. I asked if it would help me feel better, or keep me from deteriorating so rapidly, or what, and they said it might actually help me feel better, so that would be nice!