A Difficult Patient

As I prepare for the Linda Lee Wells Memorial Annual Education Day (what a mouthful!), I have spent more time reading the comments and questions on "the scleroderma sites," particularly on www.inspire.com. Here, the Scleroderma Foundation has joined with Team Inspire to create a safe place for patients to communicate with one another, to share their stories of success and failure, and to question their doctors' advice.

I find that last area is, in fact, where a lot of posts focus. Perhaps it's just the discussions that I am drawn to opening and reading... but a lot of patients are wondering why their doctors have so much difficulty relating to them as people, listening to their concerns, considering them as partner, and approaching them with compassion. I admit it is hard for me to see the medical community lambasted at times, but my perspective has definitely shifted over these past 4 years.

When I first became a scleroderma blog voyeur, I was offended by the vitriol often blasted at the medical field. But, having now lived in the world of "patient" for some time, I admit I could blast a few doctors, myself. I have become, quite probably, a difficult patient. I refuse to return to doctors who offend me, who don't listen, who don't believe, or who patronize rather than work with me as a partner. It's hard--especially as a woman, and someone who loves to be liked and hates conflict--to stand up for myself. So, mostly I don't. I just don't schedule the return visit. But, driving home I have the best comments and most brilliant comebacks for all the injustices of my latest medical experience.

Becoming a patient is never a choice. I certainly don't relish the "sick role." But, if I have to be a person with scleroderma, I'm going to fight the disease and fight for the right kind of care. I'm a difficult patient. I think that we all need to be difficult patients. Not angry patients, not abusive patients--but patients who challenge our doctors' advice, who read the medical literature, who ask the right questions and demand the answers. Patients who understand the disease, want to know the options, accept the limitations of science, and can partner with their doctors. And, I can also assure you, doctors are out there who welcome these partnerships, who don't label this behavior as difficult, and who thrive on the challenge. Those are my fabulous doctors. I owe them a debt of gratitude I can never repay.

The philosophy of my health care team, whether they know it or not, is called "Patient-centered care." In this context it means "treating patients as partners, involving them in planning their health care and encouraging them to take responsibility for their own health." (AAFP 1998) Research also shows that patients who help to shape their treatment plans are far more likely to adhere to the plan. Difficult? I think not!

I read a brilliant essay on being a difficult patient which inspired my post today. Written by a scientist and a mother who died a few years back from scleroderma, I highly recommend it if you or a family member are struggling with any kind of disease.

Article on Being a Difficult Patient

How to Save a Life

Success! I am home from Chicago and feeling quite well, despite a, ah, pneumomediastinum as a rather unusual complication of my heart catheterization. Seems to be healing up fine, though!

The best news continues to be the dramatic drop in my pulmonary artery pressure. In addition, however, the CT scan of my lungs also showed substantial improvement. It would appear that the stem cell transplant was a resounding success!

I am quite hopeful that the damage already done to my body by scleroderma can be controlled. My heart testing, in particular my echocardiogram, showed continued decline in the function of my heart. My TAPSE (tricuspid annular plane systolic excursion) has dropped from above 3.0 cm less than a year ago to 1.6 cm on this study (with a level of 2.1 cm in August 2010). This value can estimate right ventricular ejection fraction (RVEF), and a level of less that 1.96 cm correlates with a RVEF of less than 40%. This, in turn, predicts big problems. Also, my right ventricle continues to become more hypokinetic--it doesn't move as well or pump as strongly.

Dr. Burt is eager to publish the findings of his study, and showed me several graphs of his results. The bottom line is that Cytoxan does not seem to work. The stem cell transplant works quite well, but is far more effective done earlier, before Cytoxan, than when it is delayed until after Cytoxan as was my situation. He intends to begin a new trial comparing two variations of stem cell transplant and will no longer use a control arm with Cytoxan based on these results.

How to Save a Life: if you have aggressive, early scleroderma, please talk to experts about getting a stem cell transplant early.

Oh, and try to have some fun in the meantime. Is this horse the cutest thing you have EVER seen? It was so much fun to ride him the other day! And enjoy your friends and your family, and travel often, and kiss your mother with that mouth, after all. :)

One of Us

Someday, the light will shine like a sun through my skin & they will say, What have you done with your life? & though there are many moments I think I will remember, in the end, I will be proud to say, I was one of us.
-Brian Andreas

I attended my first scleroderma support group this past Saturday. It is a strange and comforting and disturbing experience: I see around the room the old me, the present me, and the future me. Kind of "A Christmas Carol" moment. :) But, more importantly, I enjoyed just meeting new friends and laughing about some of the unusual shared issues faced by those with this disease.

I am also a week away from the answer to the question that seems to be most concerning to me of late: what is my pulmonary artery pressure? I will celebrate Valentine's Day with my cardiology team in the cath room and hope for good news there!

Just hoping the weather's not too ugly in Chicago next week... we have enjoyed, as my friend Marcy says, Chamber of Commerce weather the past few days in San Diego. Gorgeous, sunny, crisp and clear. I had a wonderful ride on Xander this morning and was even able to sneak in a little trip to Disneyland with my children, husband and parents last week!

I feel my arthritis slowly but steadily creeping back up on me. I didn't miss it while it gave me a brief respite. Just hoping the big news is good news. Wish me luck!

The (Kick)Boxer

In the clearing stands a boxer
And a fighter by his trade
And he carries a reminder
Of every glove that laid him down
Or cut him 'till he cried out
In his anger and his shame
"I am leaving, I am leaving"
But the fighter still remains

-Paul Simon

As 2011 begins, I am reminded of the importance of the fight. I know that everyone has causes near and dear to their hearts, so this isn't just about scleroderma. But, today I found myself thinking about my friends who have lost their fight against this disease, and I just wanted to acknowledge their fight and their impact, in the hope that the blows they threw will collectively result in a knockout in the not too distant future.

My new year's goal is to become a better fighter. I am going to become more active with the Scleroderma Foundation now that the bulk of my treatment is behind me. I have Bonnie as my muse. :) Today is her birthday, so she's on my mind, too.

Unfortunately, my main fight will be delayed by the warm up act: a (hopefully) brief third round against C. Diff. I knew this was coming, so it's no surprise, and quick action from my doctor has hopefully staved off the worst of it this time.

And my fight will enjoy many interruptions of the best kind, if 2011 goes my way. We head to the desert to watch the boys play tennis again this weekend, and hope to hit plenty of horse shows in the year ahead, too! But, I am looking forward to focusing much of my attention and time on the fight... that is after I find my brain and my ambition, both of which seemed to have taken a much needed (?) hiatus in 2010. For the record, it was my brain that made me type "much needed" in the prior sentence. When it acts, it acts on its own these days.

Merry Christmas, My Friends!

It's the most wonderful time of the year!

I hope everyone is having a wonderful holiday season. I feel a great sense of renewal this year, celebrating my first Christmas with my new immune system. :)

Steve arranged a little birthday celebration for me (see pic), reserving a private room at my favorite restaurant so that I could be with my close friends and family. It was such a fun evening, and we could just relax and chat for hours.

I am planning a fun gathering for Christmas, as well. I admit it's hard to get it all together, but that's true for everyone, and I am so grateful that I am able to try! Can't wait! Almost ready...

Last thing to do is build a small dock out front so that arriving guests will be able to "park" despite all of this rain. :)

Going Dutch!

Steve has paid his dues with me in so many ways, but perhaps none so great as riding in the hunters.  For the past 3 years (since I was so rudely removed from tennis!), he has diligently taken riding lessons so we could once again share in sport.  Dressed in his coat and tie, he has successfully competed in the hunter classes at the horse shows, stealing only a brief yearning glance now and then at all the OTHER men riding the big jumpers (in regular shirts!) in the grass field.

Now Steve's time has come.  We've gone Dutch!  We purchased Alexander, a 14 year old Dutch Warmblood jumper who had become a bit homeless after the death of his owner last year.  His circumstances and ours aligned just perfectly, and we are a two horse family.  :)

I have been riding Xander as well as Atlas, and having a great time.  My scleroderma does interfere with my riding, and poor Xander is now "bitted up" like my good old Atlas in a stronger port-style bit.  Because I am not able to close my fingers on the reins, I need more control with a strong bit in the horses' mouths.  I tried one day to ride Xander in the jumper field, but my endurance is not there yet.  I am still quite short of breath and need short courses and frequent breaks.

However, just when I was feeling sorry for myself and feeling like I wasn't making progress, I happened to send a video of myself on the new horse along to my doctors in Chicago.  I email with them quite a bit, as I get my labs drawn here and communicate results back to them, so I attached the video on a lark.  I was super surprised to hear back right away--they were quite impressed!  Dr. Burt is hoping to use the video in an upcoming presentation on stem cell transplants.  :)  I made a short version for him, which you can view above, and I hope it helps to inspire hope in others with this disease.  It really felt great to know that I was actually ahead of schedule.

Except on my holiday cards.  :)

Back in the Saddle

I am thrilled to report that I was able to ride my sweet Atlas last week--not once, but twice!  I rode for just a few minutes, but dang I was sore!  I have lost so much endurance and muscle since this photo was taken just a few short months ago in May.  However, that is a small price to pay for the potential benefits this stem cell transplant may bring.

One issue which is keeping me from increasing my exercise program more quickly is persistent tachycardia (fast heart rate).  I'm not sure if it is a double edged sword--my deconditioning is leading to the tachycardia which limits my exercise capacity... or if the cardiac involvement of my scleroderma is leading to the tachycardia... or if the Cytoxan-mediated weakening of my heart is more persistent than my doctors in Chicago had hoped.  My gastroenterologist discussed the issue with me at our visit this week.  She was concerned enough to suggest cardiac testing, but I admit I'm hesitant given how much testing has been done on my poor heart already this year.

So I continue with my short little exercise routines and hope for the best.

Promised updates: Jake lost in the quarterfinals of his last tournament, and Luke's been rained out.

Yes, it's true non-Southern Californians!  We've had a week of drab and rainy weather!  You can gloat away.

Baby Steps

The stem cell transplant is very tough on the body as a whole.  The transplant itself, as well as the many complications I have experienced, has left my body much weaker than before.  (see photo--just kidding)  When I saw my rheumatologist last week complaining about my level of deconditioning, she advised me to start back slow and suggested 2 minute workouts each day.

I have been trying to do a little more each day, and managed a 5 minute workout on the elliptical today at level 0.  My heart rate was between 140-151 the whole time.  I can't believe how out of shape you can get in a just a couple of months!  And, yes, it's now 2 months since the transplant.  :)

One of my neighbors had a stem cell transplant 5 months ago, and it did provide me with some perspective and reassurance to see him today and discuss our struggles with getting moving.  I also am in touch with my floormates from the hospital, who underwent the procedure the same day that I did, so I have a sense of where I should be and where I could be.

I'm glad that I have the energy to try and get going.  I know I will have continued ups and downs, but the little victories are continuing to add up!  I got to see Luke play in a tennis tournament this weekend (runner-up, as well!) and Jake also won a couple of matches (to be continued next weekend).  I watched Steve ride and have hopes that one of my 5 minute workouts this week will be on horseback.

It's been gorgeous in San Diego, and I'm just chomping on the bit to get back into the swing of things!  Thanks for all of your notes and words of support.

Bad to the Bone

I spent the last couple of days meeting with my medical team here in San Diego.  I saw my wonderful pulmonologist, cardiologist, and rheumatologist, and spoke at length with my primary internist.

I like what my cardiologist said... he, like most of my doctors, didn't want to sway my decision.  But, as we discussed my options, he determined that I had a bad option and a worse option.  "The trick," he advised, "is to pick the bad option."

I'm here to tell you honey, that I'm bad to the bone.

I have decided to proceed with the stem cell transplant, off study and fully aware of the risks of a transplant with PAH (pulmonary arterial hypertension).  I'm pretty sure that's the bad option, and I embrace it with optimism.