Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Body

Wise Counsel

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Sunday night I started my new steroid inhaler, Breyna. As I wrote a couple of weeks ago, my pulmonologist, Dr. A, who follows me for interstitial lung disease (ILD), felt this was necessary, given a slight drop in my PFT that was concerning. I held off until this week because I needed to discuss the whole situation with my lead rheumatologist at Boston Medical, Dr. T.

Getting the appointment was not easy. At first it seemed the nearest date was the end of July for an in-person visit and in August for telemedicine. But fortunately, Dr. T directed his assistant to get me in sooner, and so I was able to have a telemed appointment last Friday. We spoke for about a half-hour, and I was able to ask all of my questions about whether this PFT result was due to Sjögren’s disease, rather than scleroderma, and if so, what were the implications. I also needed to understand more about the steroid’s impact, how much more vulnerable I’d be to respiratory infections while taking a steroid, and if I needed to mask in public.

Fortunately, the answer to that the latter questions was no. The inhaled steroid, he said, acts like a topical dressing on an infection. It doesn’t get absorbed systemically. I should be fine with normal precautions.

But what about Sjögren’s? Does the fact that my dry eyes have gotten significantly worse this past year, and the data that my diffusion rate is a bit worse, does that mean I’m having some kind of flair-up? He explained that he has always viewed Sjögren’s as secondary to my scleroderma, but the only way to know if it has become a more significant factor is to do a biopsy, either of a salivary gland or a tear duct.

Usually, he said, those biopsies are negative, because you have to reach a certain evidence threshold of lymphocites in glandular tissue for a positive diagnosis. Other than knowing what we’re dealing with, the only real benefit of that information, if positive, is it would enable me to be eligible for some new Sjögren’s-specific medication that is going to be available in about a year. Up until now, there has been nothing to treat Sjögren’s other than to manage symptoms. I’ve taken Evoxac for decades to make more saliva, and I use several eye-drops to help with dry eyes. But it would be great to have something that actually treats the disease.

So, I need to find out more about the tear duct biopsy, if it risks more damage than it’s worth. If so, I’ll do the salivary gland biopsy. As Dr. A requested and Dr. T agreed, I’ll get an updated CT scan of my lungs and another PFT in the fall, to see if there’s any change due to the inhaler.

The most reassuring information he shared: in 17 years of practice, he has never seen a Sjögren’s flare-up. I may have hit some kind of tipping point, but there is no reason to suspect that all of a sudden my Sjögren’s is doing anything other than what it has done for decades, just plodding along and making my body age a bit faster.

I’ll take that as a win.

Over the weekend, I was in New York City with my younger daughter to see some wonderful art, and I noticed that when I walked on congested streets, the car exhaust made it a bit harder to breathe. Also, when I walked to my train platform at Grand Central, the heat and stale air affected my breathing. So, between that experience and Dr. T’s advice, I decided to start the inhaler when I got home. You have to be careful to brush teeth and rinse your mouth well after using the inhaler so you don’t develop thrush. Not a big deal, just one more part of my daily care ritual.

It’s too early to tell if it is making any difference, but I think I may be inhaling a bit more easily. Sure hope so.

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com. Please view Privacy Policy here.

Image: Roger Bradshaw

And the Winner Is . . .

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And now, a detour from scleroderma and related complexities . . .

As I shared back in March, my World War I historical novel, Line of Flight, at long last has found a publisher, Köehler Books. Over the past couple of months, I’ve been working with their excellent team, preparing for publication on November 17. We went through two rounds of manuscript edits, which turned out to be a productive, painless process. In fact, the text remains largely as written. Kudos to my editor, Joe Cocarro, who caught some important issues with foreshadowing and other details that needed fixing. I was grateful for that and for the vote of confidence in my work (and for learning that when the word ‘then’ is used to denote order of actions, it is not preceded by a comma.)

Recently I have been reviewing text layout and cover designs by Catherine Herold. It’s wonderful to see my words shaping up into book format, and she has been very skilled at creating covers that realize my vision.

Now, here comes the fun part: You get to vote on your favorite cover design! We’ve narrowed it down to two. You’ll see a summary of the novel as well as the two choices in this link.

Your input matters! I really appreciate your participation.

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com. Please view Privacy Policy here.

Image: Hkyu Wu

Best Vacation Ever

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As a child, I used to watch a public TV program about Japanese brush painting, and I learned how to paint the images that the artist demonstrated. It has been a dream for decades to travel to Japan. And so, for two weeks in June, Al and I made that dream come true. Back home since Thursday night, after a phenomenal trip—strenuous, but worth every minute.

We flew from Boston to Montreal, stayed overnight, then made the 12-hour flight to Tokyo, where we stayed for a couple of nights in a ryokan, a traditional Japanese inn with tatami mats as flooring and a futon bed on the floor. Then it was on to Kyoto by the Shinkansen bullet train. Our accommodation there was a rented home in a family neighborhood, lovingly preserved and decorated. The bedrooms were up a narrow staircase hidden behind a sliding bookcase.

We spent five days in Kyoto, which included a day trip to Nara, the ancient capital of Japan where deer are sacred and roam free. Then we took a Shinkansen to Hiroshima, where we stayed in another traditionally decorated apartment in a residential neighborhood. Our time there included a powerful visit to the Hiroshima Peace Memorial Museum, which commemorates the victims of the atomic bomb attack on August 6, 1945. We also took a day trip to the nearby island of Miyajima, home to UNESCO World Heritage temples and shrines. Then we moved on by train and ferry to spend three days on the island of Naoshima, known for its art museums and galleries. On our way back to Tokyo, we caught a glimpse of Mount Fuji.

Yes, it was another of my super-charged itineraries. We saw exquisite gardens, ancient temples and shrines, museums, shops, street life, and navigated the food scene. Google Translate was a huge help, both for speaking with people and translating signs and other text.

I will be thinking about this trip for years to come. It was inspiring, transformative, and, yes, challenged my physical endurance. But I am so, so glad we went, as is Al. Here are some pictures. Enjoy.

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com. Please view Privacy Policy here.

Gut Feeling

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For months, now, my gut has been unhappy. Al and I both got the flu over the winter, and ever since, I’ve been having diarrhea most days, often several times a day. This is not a new issue, and I’ve discussed it at length with my Boston Medical rheumatologist, who has explained how scleroderma affects the intestine, slowing it down so that you get bacterial overgrowth. I have gained relief for decent stretches of time from antibiotics, particularly Cipro, which seems to solve the problem.

However, I recently saw my new GI specialist at Boston Medical—an appointment that took months to get—who ordered a battery of tests to get to the bottom of it all (no pun intended). He is very reluctant to prescribe Cipro, because he doesn’t want me to develop resistance to it. He prescribed a different antibiotic that is specifically for bacterial overgrowth in the intestine, but even with my insurance, it cost several thousand dollars for a few weeks’ treatment. I couldn’t afford it, and so I didn’t fill the prescription, and told him so. He understood.

Now I have to go through all the tests and get results before our next appointment. So far, a blood test ruled out Celiac. I have a breath test for bacterial overgrowth later this summer, which I was supposed to do in April, but I had to take antibiotics for an infected ulcer a couple of weeks before the appointment, and that violated the 30-day period prior to the test of no antibiotics. In August, I have an upper endoscopy. And last week, I did a barium swallow, a test I haven’t done in decades.

I figured that there might be some changes in the process or equipment since the last time, and there were. The barium drink wasn’t delicious, but it wasn’t as vile as I remembered. Which made drinking three-and-a-half cups of it tolerable. The most interesting part of the test was that I could actually see the progress of the barium through my digestive tract on the black-and-white fluoroscope and discuss it with the radiologist in real time.

To everyone’s surprise but mine, my first sip of barium got stuck in my esophagus, hanging there on the screen like a black, sagging water balloon. It matched the familiar sensation of swallowing bread and having it just sit there. I always drink a lot of fluids when I eat, and, of course, drinking water finally helped the barium to begin to trickle into my stomach.

The team began discussing various strategies to finish the test, because the plan was to see how barium traveled through my intestines, as well. Given my slow start, they were anticipating many hours. But fortunately, once I drank more barium (three cups!), everything started to move. When they checked the fluoroscope a half-hour later, the solution had moved well into my stomach and intestines. I was free to go home.

The test results were pretty positive. Apart from my sluggish esophagus, a known scleroderma issue for decades, everything else was fine. And once the barium passed out of my system (it took about two days), my gut settled a little. But not a lot.

I’ve lost about eight pounds since I had the flu—one way to get rid of some age-related weight gain that I’d just as soon as do without. But I don’t recommend this way of losing it. I’m hoping things will stabilize sooner than later. And that the rest of these diagnostics go well and provide some direction.

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com. Please view Privacy Policy here.

Image: Mika Baumeister

Pharmacy Hack

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My extremely dry eyes, due to Sjogrens Disease, have been giving me a lot of trouble of late. No matter how carefully I manage my set of eye drops to keep them moist and clear, inevitably they begin to blur within a few hours, especially when I’m writing at the computer. It is incredibly frustrating, and I have yet to find the right combination and timing, despite excellent support from my dry eye specialist.

Managing all those eye drop prescriptions is another challenge. However, I am pleased to report that I’ve succeeded in finding a good Canadian pharmacy for one of my drops, which I’ve only been able to get when I or family or friends travel to Europe. EvoTears create a barrier to reduce moisture evaporation. They cost about €25 abroad and are available over the counter. Here in the U.S., they are marketed as Miebo® and require a prescription. The latest price I got via an online pharmacy with an insurance “discount” was $225.

I’ve been wary of ordering from a Canadian pharmacy for several years, ever since another medication I need that is very expensive got impounded by U.S. Customs. That experience led me to discover Mark Cuban Cost Plus Drugs, a domestic supplier that is really excellent. But they don’t carry EvoTears.

So I did some more research recently, and I discovered Northwest Pharmacy in Canada. And I just received my EvoTears in only a few weeks. Total cost: $60 plus shipping. The return address on the box was a New Zealand pharmacy. Apparently they use distributers around the world. It had a customs label, so it was properly packaged and identifiable by U.S. authorities. No issues.

A friend is traveling to Germany this summer who will pick up some more bottles for me, so I’ll be covered for the year.

It’s ridiculous that we have to go to such lengths to get affordable medications so necessary to our health and well being. Thank goodness for the internet. For all its flaws and risks, it is a mighty tool for making worldwide connections and purchases possible.

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com. Please view Privacy Policy here.

Image: Shraga Kopstein