Never Let it be Said

This week I met with the pulmonary hypertension specialist who reviewed the exercise test I had done way back in June. Practically a distant memory, that, since the summer has been a very hot/humid one and I have had a viral infection for the past few weeks, both of which have taken quite a toll on my exercise tolerance. I'm in far worse shape now than I was when I had the testing done. Sisyphus has nothing on me; time to start rehabbing yet again and pushing that boulder back up the hill.

The test results were similar to the two previous ones in 2010 and 2011. My exercise tolerance is impaired, not just by the damage to my lungs, but also by my cardiovascualr system. Basically, my heart's ventricles don't contract properly while at exercise and the pressure in my pulmonary artery rises above normal. The combination of these things is what causes my need for supplemental oxygen when I exert myself.

My doctor asked if I'm happy with how I'm doing. I had to answer yes, but also asked if there was anything else we could try. There is an inhaled drug that would need to be nebulized three times a day which might have more effect; however, there were no guarantees. After weighing the time that would be necessary to add three additional treatments to my current 5, I decided that it wouldn't be worth going down that road until it's necessary. The oral medication is working well enough; sure, I'd like to be able to exercise without oxygen, but if the trade-off is more nebs, I'll pass.

The doctor did mention that I had put forth a great effort during the test, which was affirming.  Never let it be said that I didn't try my hardest, whether at an exercise test, being compliant with my treatments, or working hard to rehabilitate myself. At least there's that!

Patience Revisited

A busy medical week in that I had an exercise evaluation at MGH on Tuesday to assess how I'm doing after the surgery and see what modifications I can make to my exercise regimen.  The 6-minute walk test revealed that I'm actually doing better than I had thought, especially just 45 days after surgery. I walked a total of 605m in 6 minutes which put my pace at 3.7 mph. (Why the distance is measured in meters and the pace in mph rather than km, I'll never know) The bad news was that my O2 saturation went down to 86 on 3L of oxygen during the test, but still, no different than I had been 2 years ago, which was good.

The PT was quite pleased with how I'm doing, all things considered. It was suggested that I continue with the treadmill, start up at yoga again (yay!!!) and give it more time before re-incorporating strength training. My frustration had been that I was capable of doing both treadmill and weights, but then I'd be wiped out for days on end afterwards. Turns out I was rushing things a bit too much and need to give it several more weeks. I was also instructed to spot check my O2 sat more frequently when out and about (not just on the treadmill) to ensure my sats don't drop below 90, which will worsen the pulmonary hypertension. That's something I'd rather avoid.

Given the go ahead to return to yoga, I started back yesterday and was happy it didn't feel as difficult as I had anticipated. I've lost a fair amount of flexibility and strength, but know after a few weeks it will be much better. Patience! That has been my biggest challenge these past few months and something I need to work on developing much more of.

So, the weights will need to wait, but hopefully by summer I'll be  back into them and regain my strength. I'm so accustomed to recovering from a medical illness that this surgical experience has been quite a surprise in that it's so much slower-paced. Just another life lesson that things can't be rushed, they need to happen in their own time and that our minds are not the masters of our bodies.

Long Time Coming

When I look at the date of my last post, I'm shocked to see it is from the beginning of February. Nearly 2 months! It isn't that there haven't been things, both good and bad, that have been going on. There has been plenty of material about which to write, but for some unknown reason, I haven't been able to put anything down. I'll use the Reader's Digest format to update life events to the present.

Bathroom Renovation: Done!

New vanity and linen tower. Oh, the storage!

Toilet nook.

Millie enjoying the radiator heat. Shower to the left, toilet behind the door.

The bathroom is completed, exactly 5 weeks after starting the project. I'm thrilled with the results; we have so much more useable storage, a more spacious floor plan, larger shower and far prettier room with more light. Morning and evening rituals are now a pleasure to be enjoyed rather than necessities to be rushed through as quickly as possible. Hopefully this will be the last home renovation we need to do for quite some time.

Health

After limping through most of the fall and winter not feeling 100%, I was finally unwell enough to realize I needed a hospitalization. Well, actually, it took my docs to point that out to me, as usual. Even after being admitted, I told Joe: "I really don't think I need to be here".

Surprise! I did. It was actually a good thing. Lots of diagnostics, specialists and time to rest sorted a few things out. The IV antibiotics did their job and my lungs are now in better shape than they were prior to going in. The new medication, Revatio, the pulmonary hypertension specialist started me on back in January seems to have kicked in; I'm less short of breath with activity. A cardiologist ordered an echo and 24 hour heart monitor (both results still pending) but was able to explain the seemingly unexplainable test I had last August. Short version: it seems that I have some new abnormality with my vasculature in which the vessels don't dilate as they should when I exercise, causing my blood pressure to skyrocket. This, in turn, puts pressure on my heart, which is pumping against abnormally high resistance and causes the left ventricular dysfunction (cardiomyopathy). In addition, the vessels between the heart and lungs have a much higher than normal pressure when I exercise, also known as exercise induced pulmonary hypertension - EIPH for short. Now it all makes sense and I feel that there is a good team of docs in place who are able to treat these interrelated issues. Unfortunately, no one specialist covers all 3 things so there is a pulmonologist to treat the lung disease, one to treat the pulmonary hypertension and then a cardiologist to treat the systemic hypertension and cardiomyopathy. Bring them on, the more the merrier, I say.

The good news is that 2 weeks after discharge, I'm able to exercise closer to my previous baseline than I had been in the past 6 months. There has been an enormous amount of deconditioning which will take months to recover, but at least I have the energy and ability to do it now. Weights 4 days a week, yoga once a week and treadmill/walk at least 5 times a week- without fail. Time to get in touch with my inner drill sargent!

Garden

While I was in MGH all the snow melted, something I wouldn't have believed could happen in a 2 week period. Miracles never cease to amaze me. We were treated to several 50-70 degree days in which I was able to clean up about 1/2 the yard's leaves that had been buried all winter. The temps have turned colder this week and we've had flurries several days so no yard work this week; fortunately these things will keep.

I had planted a tray of seeds prior to being admitted and Joe dutifully watered them while I was away; I have broccoli, peppers, eggplant, tomatoes and lettuce to look forward to planting out in a few weeks! He also treated me to a tumbling composter as a welcome home gift- which is now loaded up with the leaves and kitchen scraps from the past 2 weeks. More black gold for the garden, there's never too much of a good thing.

The crocus are very late in blooming this year due to the late departure of all the snow. We actually have snowdrops out now- at least a month later than usual- but no less welcome. I'll take any sign of spring no matter how delayed at this point. Next week is supposed to be a tad warmer (40s) so I'll be sure to get out and photograph what is going on in the gardens. It's good to be back.

Circular

Life is made up of circles, cycles; some big, some small. Every so often we end up back where we started from. Each day is a starts and ends the same way, there are always 24 hours, but naturally there are never 2 that are exactly alike.

Some cycles are reassuring. It's nice to know that spring always follows winter and fall comes after summer. Others are frustrating- for example the never ending supply of laundry.

The worst one for me is the health cycle. Yes, everyone experiences ups and downs in their health but it's the intense effort required on my part to keep myself stable and healthy only to be undone by a little cold virus that frustrates me. Several months of hard work set back in a matter of a few days.

Buddhists have a saying that when you get a new cup you should picture it already broken, so that when it happens, as it eventually will, you will be prepared for it. Everything, in the end, must fall apart, and so too must my health. I can't change that fact but I can try to alter the way I look at it. Instead of being frustrated by the loss of function I should try to view it as part of the circle; something that is expected. Not welcome, certainly, but not a surprise.

Therein lies the trick!

Priorities

Sometimes you have to hit rock bottom in order to make a change and get yourself together again. It may be a life event, a situation, or simply the time of year that will illicit the change. Life consists of many paths and fortunately we are never stuck on one; it's always possible to alter our course or reverse direction depending upon our need.

Putting things in writing always helps me. Sometimes it's a list, a memo or just a bunch of free-floating ideas. I thought it might help keep me focused to put my priorities into writing, just to keep me reminded of the things that are important to me, the things I want to focus on right now.

Family

Cultivating and nurturing existing friendships: the good, bad and ugly

Taking care of myself (physically, emotionally) and not putting the needs of others ahead of my own, unless it's emergent

Prioritizing what is truly important, what can wait and what isn't worth worrying about.

Taking care of my responsibilities (pets, houseplants)

Maintaining a home that I feel comfortable in and that welcomes those I care about

Living within my values

In order to maintain these priorities I will engage in:

meditation daily

yoga

exercise

neb/vest treatments twice a day

volunteer once a week

continue to do typing for Yoshi

set aside time to maintain the house and yard

schedule time with friends, but allow spontaneity

enjoying every moment.

Staying Put

Prior to and during my consultation at Children's hospital I was quite sure I was going to make the switch back. I liked the doctor, she seemed to listen to and understand everything I was saying and what I was looking for in a center, and a doctor. The staff was all attentive, the clinic clean. I spoke to the research nurse who listed several studies they are doing that I would be eligible for. It all seemed good.

Yet as I left, for all the familiarity, I still had an unsettled feeling. Nothing I could put my finger on, but something in my gut that didn't quite sit right. I decided not to make a decision until I felt strongly one way or the other, I'd sit with the feeling and see where it led me.

The more I thought about it the more I felt that the pediatric setting wasn't right for me. The care would be good, no doubt. But there is a different philosophy and attitude at MGH and, for me, it feels more comfortable. The one comment I kept going back to that the doc at Children's had made was that they didn't emphasize exercise there as part of the treatment plan because it wasn't really necessary with children. They're naturally more active than adults. (Though, as an aside, I'm not convinced of the truth of that statement in this day and age of X Box and Nintendo) Regardless of the validity, if they are treating adults, I don't think they can apply the same rules as they do for kids.

The comment in itself wasn't a big deal. And in all honesty, I don't need a doctor or PT to tell me I should be exercising and to devise a plan for me. I already have my routine and it works well, so that part doesn't really make any difference. I think it was the attitude that they didn't do something because it didn't apply to children - which brought me back to the main reason I left in the first place - the fact that they didn't want to let go of their adult patients, but they aren't able to completely shift their approach to that of adult care.

I didn't spend much time agonizing over the decision this time. I was able to trust that eventually I'd know what was going to be right for me, one way or the other. I feel very comfortable with my decision, and also know that nothing is set in stone; just because the time isn't right at this moment, doesn't mean that it won't come up again in a few years. All I know is that for now, MGH is the place for me.

Get Back to Where You Once Belonged

Yesterday I made an appointment for a consultation at the CF clinic where I used to be a patient. When I was diagnosed in 1984, the average age for a patient was 19, which happened to be my exact age at that time. Because the majority of people didn't live into adulthood patients were treated at children's hospitals. I thought nothing of this: CF was, after all, considered to be a pediatric disease.

I received top quality care from doctors who were pediatricians, and since many had quite a bit of adult experience as their CF patients got older this really wasn't a huge issue. Sure, it was a bit uncomfortable being treated at a pediatric institution. I had to explain that I, not my son, was the patient. There were times that the doctors were pretty much out to lunch on some of the adult issues such as employment, families, the social implications of the disease and what it was like as a "grown-up" to live with this chronic disease.

Fast forward 20 years. With the many advances made in research, drug development, and prevention the average age is now 37. As the age of patients started increasing there became a trend to transition the adults into adult clinics or to adult hospitals affiliated with the pediatric institutions. My hospital, Children's Hospital in Boston, was one such place.

Any transition is difficult, but in particular it is difficult for patients to leave a place where they have been treated their entire lives. With a disease such as CF patients form very tight bonds with their care providers. They are seen in clinic every 3 months and often treated inpatient for weeks at a time with IV antibiotics to keep the infections at bay. For many, the hospital is a home away from home.

From the hospital's perspective, I'm sure it was incredibly difficult to write the policy and move patients to another, albeit nearby, institution. A relationship was being formed between Children's and Brigham and Women's hospital in which patients would be treated up until they were 18 at the pediatric institution, then move over to the adult care setting for their adult care.

At the time of the change over of adults to The Brigham (as it is referred to in Boston) there were many different and conflicting stories, the new hospital wasn't up to speed on cystic fibrosis and it was, in my opinion, quite chaotic. I didn't feel good about moving over to the Brigham.

For this reason, along with several others, I decided to move my care to Mass General Hospital, the biggest hospital in the city, which had both pediatric and adult care. There was no need for their younger patients to leave the hospital once they became adults. I loved the adult care providers, the physician and nurse practitioner, and they seemed to be building a solid clinic in which the patients would receive excellent care. There was also a lung transplant program at the hospital (something Children's didn't offer at the time, the adults were transplanted at The Brigham) which was appealing as an option for me should I decide to take that route further down the road.

After a year at MGH the CF physician decided to leave to pursue her career elsewhere. The NP stayed behind and was the main care provider for over a year as they searched for a replacement physician. Once one was hired, the NP also left. So much for stable, consistent care!

Since then, there have been several more staff changes. The team that is currently in place is good. I particularly like the NP who has many years of clinical experience and is great at prioritizing. She knows her stuff. The doctor is good, I've been impressed after some appointments and disappointed at others. I think the bottom line is that he is very overwhelmed and the hospital is reluctant to pay for a second CF physician, which is crucial. There are over 150 adult CF patients for one doctor, who also covers in the ICU and has regular pulmonary patients. On top of that, the transplant program at MGH appears to be sputtering.

The clinic has grown in my time there with the addition of a nutritionist, an excellent physical therapist and a social worker. The thing that I find to be lacking is a passionate physician, one who is up to date on the latest developments, drugs and research, who will guide the clinic to be one that people seek out for excellent care. The care is adequate, but not outstanding. After a conversation with a dear friend, whose husband has had experience with both MGH and the Children's/Brigham clinic, I started thinking about giving the latter another look. Her description of the care providers at the Brigham as being "passionate about CF" really struck me. That's the main thing I find lacking at my current hospital: passion! I'm going to consult with a physician at the Children's/Brigham CF clinic and see how I feel about switching back.

I don't consider my time at MGH to have been a failure or a waste. My PFTs haven't slipped more than a couple percentage points in the 6 years I've been there, which is better than the 2% average that CF patients lose per year. I can't afford to lose much more without a drastic decrease in quality of life. For the past 12 years I've been super-compliant and willing to stick to a tough regimen of daily care; I want my providers to be the best they can be. It doesn't make sense to be doing hours worth of treatments per day and not be using the latest data/treatments to optimize my time and the results.

I'm not agonizing over the decision as I did last time when I moved over to MGH. I'm going in with an open mind: I want to check the clinic out, see how it is run, how the relationship with the adult hospital works, what research is being conducted, what the adult doc is like. If I do decide to leave MGH, it's nothing personal, just that I'm looking for a clinic that will best fit my needs and give me the best care possible. I won't have any problem explaining that to my care providers. Boy, do I love being in my 40s!

Back at Square One

After 10 days of feeling pretty rough due to the flu (not the swine flu, thankfully!) I can finally say I'm starting to feel better. If I had a dollar for each person who asked me if I did have the swine flu, I'd probably be able to pay my medical bills. And that's sayin' something!

A week ago Saturday I developed a fever and fatigue, not unheard of in the world of CF. When I was still feeling lousy on Monday I called my doc who wanted me to come in. Tuesday I was seen, Xrays done, blood tests, sputum cultures and viral swabs taken. The worst was the viral swab, up the nose with what looked like a mascara brush, and it went so far up I swear I felt it tickling the back of my head! It was all I could do not to sneeze in the poor nurse's face. Not even a day later I got the call: the viral culture showed h. influenza. Ok, at least I knew what I was dealing with. Thankfully, the Xray looked unchanged.

A full week later I finally am free of the wretched body aches, fever and only a nasty cough and generalized weakness remain. Ok, time to start rehabbing. I took a short walk with Joe last night - clearly this is going to take some time! I'll start back at yoga today which will be a nice, gentle way to ease back into the routine. I never stop being amazed at how quickly the body gets debilitated: 6 weeks of hard work gets undone in a matter of a week. Better get started!

Dashboard

This is my second attempt at this post, I wrote it out a few days ago and then when I went to publish it, the computer ate my homework! Usually Blogger is pretty good about saving the work as a draft, but no such luck this time. Ah well, maybe it happened for a reason.

The other day when I was on the treadmill, with my mp3 turned up nice and loud to distract me from the fact that I really don't like exercise, one of my favorite songs came on. It's such a good song that it makes me walk a little faster, just to keep up with the beat. I always feel lucky when a great tune comes up on the random play setting, it's like a little bonus, or it seems that way, anyhow.

As I was listening to the lyrics of the song I had an "a-ha!" moment and I heard them in a different way than I had before. Taken literally, Modest Mouse is singing about their dashboard being melted (like back in the 80s, when your dashboard would crack from being in the sun??) But, no matter.... they still had the radio. The song goes on with a similar theme... "the car was on blocks, but I was already where I want" to be etc, etc etc. The tune is so catchy I had never really thought about the lyrics. Maybe it was all the blood circulating faster than usual and therefore giving my brain a bit more juice, who knows, but all of a sudden I heard the lyrics saying that they were looking at the glass half full, rather than half empty. Sure, the dashboard might have melted, but hey, the radio is working!!! Talk about being grateful for the small things. The realization made me smile.

I'm terrible at interpreting poetry and I suppose music falls into that category as well. I tend to take things very literally so making this connection (which probably would have been obvious to most everyone else) made me happy. Sure, I hate to exercise, but I did get to listen to a great song, and be reminded to see things in a positive light. Thanks Modest Mouse!

I Really Love This Part

Finally, finally about a month after the last cold symptoms disappeared I'm seeing some progress in the right direction. I have been increasing the time I'm using the incline on the treadmill by 5 minutes a day; I was at 6 minutes per session last week and now am up to 20 minutes as of today. I hope to be up to the full 30 minutes by the middle of next week, just in time for my exercise tolerance test at MGH. Hopefully if the weather is nice this weekend we'll be taking some long walks outside, which will help with my endurance.

I was doing so well last spring and early summer, easily in the best shape I'd ever been in. I had an exercise tolerance test last March and was in a pulmonary rehab program to maximize my abilities. My lungs will unfortunately never get any better, but I can strengthen my muscles and improve my endurance, which will give me greater functional ability, allow me to exercise well and in turn help to keep my lungs as healthy as possible. I felt great- strong, vibrant and healthy. Then with the trip to Ireland, no vest or bipap due to our converter not working properly and everything sort of slipped away from me.

After many fits and starts, some IV antibiotics in January, followed by the setback of the most recent cold, I'm finally getting some endurance back. The downhill slide is quite depressing; the not knowing how long it will last and when, exactly, it will stop. The lack of control, lack of energy and lack of ability to do anything about it. You can only wait and once things have leveled off, again begin the long process of rehabbing. Starting again at square one can be so daunting and quite frankly discouraging. But once a bit of progress is being made there is a boost and it becomes easier -actually enjoyable- to exercise.

When I'm not feeling well and attempt to exercise (as the docs and PTs tell me I need to do- easy for them to say!) I feel worn out and exhausted afterwards, even when not exercising to my full capacity. So depressing. But when things are on the upswing, after about 15 minutes of exercise, I get the proverbial endorphin rush: suddenly it becomes easier. It feels like weights have been removed from my ankles and my chest releases. Breathing is smoother and less of an effort. It almost feels like I could break into a jog, or maybe even fly. Gotta love those endorphins!

I know that it's important to enjoy all parts of life, both the good and the bad. To the extent I can, I do try to. But it's so much easier to love this part, when exercise is easy and a pleasure; when it feels good; and when I can see some progress from day to day. And especially when I have enough energy to enjoy my day. Results, at last.