The Scleroderma Chronicles: Lung Biopsy Story

Last Monday, May 2nd, was the date of my lung biopsy procedure. My pulmonologist had diagnosed me with interstitial lung disease a couple of months ago and the biopsy was required to definitively diagnose the type of ILD I had. This is kind of complicated, but the simple reason for the biopsy is that I don’t really fit the profile of the usual scleroderma ILD patient, and the treatment is expensive and somewhat risky. Biopsy time.

At 4:30am Monday morning my son drove me through the rainy dark to the hospital where I was going to be admitted for the surgery. I had on my new raspberry clogs for good luck, and I was exhausted after being up most of the night completing pre-op tasks at home. I was fighting off a sense of impending disaster…

Image retrieved online from Wikipedia Commons at https://en.wikipedia.org/wiki/Respiratory_failure

The day I met with the surgeon he drew a funky little drawing on his white board to explain what he was going to do. Check out the diagram above: your right lung is actually different from the left with more tissue and three lobes. My surgeon planned to use special instruments guided by a tiny video camera (VATS) to go through my chest wall and get the tissue samples. This is a minimally invasive procedure that will only take a few minutes. The catch: the right lung will be deflated before he takes the samples.

Atelectasis is the medical term for a collapsed lung. Both of my lungs were experiencing incomplete atelectasis at the time of my last CT scan, and I’d been getting steadily worse all April. I was a little concerned, but I trusted that all would work out fine in the end. I was prepped, bundled up, and rolled off to surgery. There was the most fantastical robotic surgery machine in there, but before I even got a great look at it, I was on the table, a mask was put over my face and I was gone…

… and I emerged from unconsciousness at the bottom of a rugby scrum with all these faces looking down at me. I was in pain, a lot of pain, and I couldn’t breathe at all. The muscles on the right side of my back were seized up and cramped in what felt like Charlie horses, and I was thrashing around as I tried to get someone to rub on my back muscles. The rugby players in masks (I was later told that there were 8 of them) were attempting to hold me down as they put hot packs under my back, removed my oxygen cannula, and placed a larger oxygen mask over my face. “Do you remember the Xray?” one of them asked. Oh. That’s what set off the muscle cramps. My oxygen was below 75% even on highest oxygen flow available in the recovery room. I got transferred to the ICU.

That right lung refused to reinflate. I couldn’t be given any pain medications or fluids until my oxygen levels came up. New doctors began to arrive and talked to me. To be clear, talking set off coughing and was very painful. I just wanted someone to help me, not talk to me! A pulmonologist arrived to doctorsplain my disease to me and informed me that he was changing some of the drugs used to treat my lung and heart conditions. I told him that I didn’t know him, he couldn’t make any changes to my treatment plan without talking to my doctors, and that I needed him to do something right away to handle my immediate situation (I was in acute respiratory failure). He argued about contacting my doctors. I insisted.

I think that I deserve a huge gold star for standing up for myself while in extremis.

I was placed onto a high flow oxygen therapy machine within minutes and my oxygen levels came up.

I kind of look like I was underneath a rugby scrum, huh. That machine delivers heated water vapor and oxygen at the unbelievable rate of 60 liters/minutes. My oxygen came up into the low 90s on the machine and I finally received oxycodone. Yay!!! The last oxygen number on the monitor before I fell asleep was 94%.

The next day they got me up into a chair (more oxycodone!) and I started respiratory therapy to get the lung working again. On Wednesday my chest tube came out and during the day the flow rate on the machine was reduced until I could come off it.

Day three in the ICU. I am off the high flow machine and finally using a normal cannula. I’m still on 15 liters/minute of oxygen at this point.

That annoying pulmonologist came by every single day that I was in the ICU; my doctors had called him back! After talking to them he made some med changes (and told me that it a shared decision) and his manner completely transformed. The physician’s assistant who removed my chest tube told me that she had also read the care notes with all the data, email traffic, and decision-making by my rheumatologist/pulmonologist/cardiologist team. She was struck by the interdisciplinary care that I was receiving and seemed a little wistful and envious.

Another gold star for the team!!

Over the next two days I was slowly titrated down on my oxygen while doing my lung expanding exercises without fail. Late in the morning on Friday I finally escaped.

I put on my raspberry clogs, black leggings, a little black top, and my raspberry-colored down vest. “How cute you are!” exclaimed the nurse. My son drove me home through the late morning light along streets lined with newly leafed trees. While I was in the ICU the world had turned green.

No matter how lifeless and barren things seem over the winter, spring always comes with the promise of fresh starts and new life. I still trust that all will work out fine in the end.

Hannah is so glad to have me home again.

Now we just have to wait for the biopsy results.

Those Crazy Days of April, 2022

April went by in a blur for me. I entered the month feeling pretty sick. I had just had an appointment with my pulmonologist, and he had referred me to a surgeon for a lung biopsy. Well, not what I was looking for, but necessary to definitively diagnose what type of interstitial lung disease I had. There are several types, and the treatment plans for each are different. The surgery was set for May 2nd, an entire month away. Annoyed, upset by the wait, pretty darn sick, it was hard to wait all through April.

April rose to the challenge. Never was there such an ill-behaved month. For some bizarre reason my Amazon account reset to Spanish. Do you know how hard it is track orders and make changes to your account settings in a language that you don’t read well? Then email in Italian began arriving. Someone tried to hack into my PayPal account, setting off a flurry of new password and security settings on my part. Then my Facebook feed was flooded with pictures of… owls.

Umm… the universe wants me to learn a new language? Maybe the next BioGeek post should be about owls? This is really strange. April was getting off to a rocky start…

I kept struggling with my health as the month advanced, taking care of essential items in preparation for months of difficulty ahead if I was moved into a 6-month course of chemo. I filed my taxes. I took Matao in for his vaccinations. I filed the paperwork to be excused from jury duty. I got the last vaccine I needed (shingles) before the surgery. A neighbor installed the new outdoor lighting that I had purchased before Christmas, and another neighbor will arrange with her grandkids to take care of my lawn. I ordered more oxygen cannulas, and in keeping with April craziness, 4 cases of tubing arrived instead. I made arrangements to be tested for a portable oxygen unit… first appointment is in June. April, you are killing me here! One of the side windows of the car shattered without warning. I continued to get sicker. I was sick and tired of April, too!

My wild bunny moved into the front yard where I could see it each time I went out of the garage. Very used to me now, it never runs away when I go outside. Bunny support on sad days.

As the month wore on, I began to sleep a lot. Like 10-12 hours a night. I had to use oxygen during the daytime. I was losing weight. I wrote my doctors an email towards the middle of the month telling them that I felt that I was declining, and that I was worried it was taking too long to begin treatment. I was scheduled for a series of additional tests and had appointments with the pulmonologist and the cardiologist. Oh, good, April. Now I have to drive all over town without portable oxygen.

How to respond to a worsening situation that I have no control over? I bought happy new shoes in an outrageous color.

These are the shoes that are going to walk me into the hospital!!

And then I cast on new socks that would do the shoes proud.

This yarn’s name is “Squad Goals” and dyed by Hue Loco.

The results from the heart, lung, and kidney testing came back and they all showed that… I was getting worse. My doctors conferenced back and forth, debated different treatment modifications, and came to the consensus opinion that I should have the biopsy done May 2nd. I got that email late on Friday before the surgery. Checking the physician notes attached to my last appointment I found the full text of all the test results and the email chain of discussion between my doctors. I felt very lucky: what a great team! How good I feel that the decisions about drugs and treatment is being arrived at through this interdisciplinary collaboration by my doctors, who have chosen to make this all visible to me. Knowing that, however, did not change the fact that the upcoming surgery is now a little risky.

So, I cleaned the house, did laundry, paid the bills, and got in groceries. I planted flowers in the front tubs and along the walk. I also cast on some baby booties for my next-door neighbor who just brought home a new little one. This pattern, extremely versatile, is our family recipe passed down through three generations over almost three quarters of a century. It is the same as this one posted on Ravelry.

And just like that, the month was done. I had made 2 hats, 4 PICC line covers, 2 pairs of socks, and used up 3.38 skeins of yarn. I read 6 books. I did work on a sweater, but since I’m now in the colorwork portion of the knitting I put it on hiatus to await better days.

Goodbye April, you crazy, bad-boy of a month! I want you to stand in the corner and think about what you have done!! I expect a whole new attitude when I see you next year.

The Scleroderma Chronicles: It’s okay if you cry…

My fatigue lately has been off the charts. I struggle to get the simplest of tasks done, and to be honest I just don’t feel like getting out of bed for days on end. I have been slowly, slowly sewing on a quilt top over the last few weeks. It is soooo exhausting to pin two fabrics together, guide the fabric through the sewing machine, and then to stand up to iron the seam. I handled all of this by 1) sewing only for an hour a day, and 2) lowering the ironing board so I didn’t have to stand while ironing. Take that, you nasty, exhausting fatigue!!

The quilt has a panel in the middle with really cute pictures like this one. Super cute, right?
Here’s the finished quilt top. This baby has really simple quilting in one border and then simple, simple borders around the central panel. Yeah. This took over two weeks to complete. Thanks, fatigue.

When I noticed some strange terminology on my last heart imaging test report, I contacted my pulmonologist about it, and he ordered a CT scan of my lungs. (You can read about that adventure here.) I knew that something was up when I got a call from his office telling me the date and time of the earliest possible appointment with this doctor. The nurse had intervened and made the appointment for me ahead of time. Then there was a call from the cardiologist’s nurse that was the same; an echocardiogram and appointment with that doctor had also been scheduled for me in order to secure the earliest possible appointment. Kind of the harbinger of a tough appointment, right?

Today I had a pulmonary function test and met with the pulmonologist soon afterwards. We joked about the horrible year we had both had. (He is a pulmonary critical care specialist who has been on the front lines of Covid care for two years now; for me lockdown never ended and the BLZ was running wild.) We laughed at my summation of the year: Crushed by Covid. We decided that “Crushed by Covid” could be the name of a really sad band. Then he whipped out his laptop and had me move over to look at it with him.

He had prepared for my appointment with a spreadsheet of my lung function tests over time and my latest lung scan along with that of a normal person. The spreadsheet showed that I was losing volume in my lungs. The scan of a normal lung was really interesting (old biology teacher here…) and then we looked at mine.

Um… my lungs were really cloudy. Like frosted glass. Like… “Hey. Is that what they call ground glass lung?” I asked.

“Yes. That is exactly what we’re looking at. That’s why I wanted you to come in. This isn’t the type of conversation that you have over the phone,” he replied.

Ground glass is not good, folks. Ground glass is the type of lung imaging that Covid patients with pneumonia have. Covid presents like systemic sclerosis because there is an extreme immune response going on in the lungs; both are aggressively treated with drugs that target the immune system. I also have some honeycombing that is the beginning of fibrosis; first the inflammation (which creates the ground glass appearance), then the fibrosis follows. My ground glass lungs are, in his opinion, absolutely not Covid. It is not likely to be just pulmonary edema. It’s systemic sclerosis at its worst. This is interstitial lung disease.

So, it is not good. On the other hand, this is good. I’m in trouble, but the problem has been identified early on and that means aggressive treatment now may stave off the worst of the fibrosis. I am so glad (and lucky) that I googled those crazy medical terms and then followed through with an email to my pulmonologist.

I’ve been referred to a surgeon for a lung biopsy procedure. Evidently that will land me in the hospital for a few days. Following that, if things go to plan, I will be started on more aggressive immunosuppressive drugs. He is going to talk to my rheumatologist about starting a course of chemo and an anti-fibrotic drug. One of the last things that my pulmonologist said to me was, “It’s okay to cry about this, but we have a plan.” That’s when it hit me that this might be really bad; lockdown will continue, and the fatigue is probably going to get worse. Oxygen 24/7 is right around the corner.

Crushed by Covid plays on. What a sad little band it is.

Luckily for me and the cats it is squirrel season. They will have lots of entertainment while I’m in the hospital and laying around like a slug.

The Scleroderma Chronicles: The shunt hunt takes a left turn…

I’ve been continuing my adventures in cardiology over the last several weeks. If you have been following along on my scleroderma adventures you know that I had a trip to the Cath lab that led to the discovery of a cardiac shunt: a hole in my heart. I also was eventually diagnosed with exercise-induced pulmonary hypertension and started on drugs to treat it, which is a lengthy process as I was slowly titered up on two different drugs while monitoring for side effects. I’ve been mostly living in bed for the last 6 weeks except for short trips out for more testing and blood work. The cats have been loving this, by the way. I’m kind of their captive right now.

This is edema in my arm. I’ve been dealing with headaches, muscle pain, edema, low blood pressure, and extreme fatigue. I cough a lot. Every new weather system is a nightmare. Ugh!

While the whole process has been pretty difficult, I am breathing much, much better and that blue lipped thing has mostly faded away. No more panting!! I haven’t had to put my head down because I felt faint for weeks. This is huge, people!!

I may have to retire the whole BLZ logo the way things are going!

My cardiologist is still hunting for the shunt that was detected in the Cath lab. I have one that they can see (a patent foramen ovale, which is pretty common), but for the really significant disruption of circulation that was detected in the Cath lab the feeling is that I have something much bigger somewhere. I’ve gone through 3 rounds of testing looking for the dang thing, and so far, no joy.

When the test results come in, I always read the entire text and google terms that I don’t recognize. The last imaging of my heart did not find the shunt, and my cardiologist sent an email letting me know that my heart looked pretty good. Umm… okay, but where is that infernal shunt?!!! This is getting a little frustrating, but I am doing better, so I guess I should just roll with it. I did notice this little sentence in the report about the portion of my lungs seen in the heart imaging: “There is mild subpleural reticulation and bibasilar atelectasis.” Say what? I googled and …. bibasilar atelectasis is a partially collapsed lung. I shot off a little email to my pulmonologist to ask if this was something new.

This is why I decided to write this post. As it turns out, this is new. Both of the things that were noted in that test result were significant (subpleural reticulation is evidence of scarring in my lung), and I was immediately sent to get a specialized lung scan that shows I have sustained moderate advancing lung damage over the last 10 months. Oh. No wonder I’m so exhausted. At least they didn’t use the word “severe” in the report. I seem to have developed pulmonary edema and my lungs took a big hit during the last few months; scleroderma is now attacking my lungs. If I hadn’t read that report and then contacted my doctor, no one would have picked up on this. The BLZ may be on hiatus, but the lessons she learned during that drive for the pulmonary hypertension diagnosis really paid off now.

What do you do when you get a sad little lung report? Why, you put on your Catzilla shirt and go start a load of laundry, of course!!

Tuesday I go for a pulmonary function test and then immediately afterwards I will meet with my pulmonologist. I’m kind of thinking that there might be more drugs in my future. Anyway, there is a lesson here that I decided I should share with you all.

Be proactive! Read your test results and ask questions of your doctors. Google is your friend, and those online portals that let you shoot your doctor an email are priceless! Use them!

And now readers, back to the shunt hunt…

Mateo: and now readers, back to my nap! After that I’m going to go swat some more helicopters!

The Scleroderma Chronicles: June is Scleroderma Awareness Month

Five years ago the course of my life altered forever when some blood test results ordered by my doctor arrived: the results showed that I definitely had two autoimmune diseases. Specifically, I was producing antibodies that were diagnostic for scleroderma and Sjogren’s disease. I was expecting lupus, so this was kind of a surprise shock. After a quick trip to Google to establish what type of scleroderma was associated with my positive test results I burst into tears. It was worse, much worse, then I expected.

The scleroderma diagnosis was the problem. Through an unbelievable sequence of serendipitous life events I was more knowledgeable then the average newly diagnosed patient, and I knew that scleroderma was a disease that impacted connective tissue, that it was progressive, disabling, and that there was no cure. This was a life-altering diagnosis, and I was in for a long fight that would last the rest of my life.

You see, I used to work in a rheumatology research lab, and I did research in scleroderma. I was a member of the research team that found the first identified antigen associated with scleroderma. I had visited scleroderma patients in the hospital. Later in my life I taught AP Biology and spent years trying to explain connective tissue to students.  It’s a type of tissue that we just never think of, but it is critical in organizing and operating our bodies. Connective tissue makes your skin elastic and strong. It organizes your muscles and makes up your tendons and ligaments. It is a critical layer in your blood vessels, and is part of the essential structure of all of your organs.  In scleroderma all or parts of this connective tissue is under attack by your immune system.

As white blood cells invade my tissues and attack this connective tissue it produces too much collagen in response. The built up collagen produces thick layers of tissue and scarring. My fingers look really swollen, but it is actually very thick hard skin. My skin is also getting really shiny which means that the collagen is hardening up and losing flexibility. Must knit faster!!

You can perhaps see that same thickness on my face, especially on my cheeks. It means that I don’t have wrinkles, but it is also hard to open my mouth, my smile is mostly gone, and I can’t turn my neck well. Crazy, huh.

That is the most ironic aspect of scleroderma: you look pretty darn good, especially if you are a senior citizen like myself, but you actually struggle daily with your illness. For many scleroderma patients their disease just  involves the skin, but for others, the disease is more than skin deep.

As it turns out, my skin is the least of my worries. The rare type of scleroderma that I have, systemic sclerosis, also causes scarring of internal organs. The muscles of my stomach and esophagus have lost function. My kidneys are damaged and I have chronic kidney disease (stage 3). My lungs are scarred and my diaphragm isn’t exactly happy any more. Part of my stomach herniated up into my chest this year… whatever was it thinking of?! My tendons are getting calcified due to inflammation and at least one has partially ruptured. My muscles are sore to the touch and I have bruises everywhere. I have nerve damage and trouble controlling my body temperature. It’s hard to walk. Blood vessel damage affects circulation to my hands and feet and I’m starting to develop open sores (ulcers)… There is a long list of diagnosed conditions linked to my scleroderma, but you get the idea. Pretty much I’m a walking limping train wreck. Well, a knitting train wreck for sure!

There is no cure for systemic sclerosis, but there are treatments that really help a lot. I am taking four different drugs to crush my immune system into submission; it’s a balancing act as I need my white blood cell count to stay high enough to protect me, but low enough to control my symptoms and prevent more damage. I take a drug to shut off the acid produced in my stomach so I won’t accidently inhale it in my sleep since the muscle barrier that usually keeps it in my stomach is now gone. I take a couple more drugs that help control inflammation, and some supplements that help with nerve damage. I’m on oxygen at night. All of these drugs/supplements have made a huge difference for me: my last lung scan showed improvement and my high heart pressure, the most concerning complication that I had, has returned to normal ranges. My kidney damage continues, but it has slowed way down. There is something funky going on with my red blood cell count, but you can’t win them all, right? The main point is that I continue to manage and live independently.

MacKenzie and I last year when I posted this online as part of the “Face of Scleroderma” campaign.

In short, I am a mess. And yet, to the joy of my doctors, I continue to do really well. Okay, I have blue lips, am short of breath, and struggle with tissue damage, but I also continue to thrive compared to other scleroderma patients that they treat. I have had to make many changes to my life, but I have found work-arounds and I still do things that I love. Attitude is all!

Well, knitting, the cat, and the garden are pretty darn essential, too!

So, there it is. What an annoying disease, right? How dare it make you look younger while shortening your life? How dare it do all of this invisible internal damage that makes people think that you are lazy or an attention-seeking hypochondriac when actually you view each day that you are able to leave the house as a personal victory? I’m in several online support groups and there are people dealing with crushing negativity like that. I can see how it can happen; it is so hard to understand something beyond your own experience that is hidden from view.

That’s why there is Scleroderma Awareness Month. It is hard to have a rare disease, especially when it is one that is hard to pronounce (Sclero… what?!). It’s harder still to have one that has no cure and a pretty high fatality rate (hey, with all of the drugs that I’m on my 10 year survival rate is now up to 80%!!). It makes you learn to laugh in the face of terminal complications while forcing you to take every possible precaution to avoid contracting Covid-19. It messes with your head; it gives you power, but it’s also strange and a little lonely.

That’s why we scleroderma patients share our journey with all of you every year so you can get a glimpse of our lives.

 

If you look harder you will see the signs of my scleroderma on my face. The small red spots are called telangiectasia and are symptomatic of my form of systemic sclerosis. The skin of my forehead is tight and shiny, my hair is falling out,  and my dimples are now buried under my thick skin. My upper lip is trying to decide if it wants to turn blue… 

I am the Face of Scleroderma.

Footnote: In addition to scleroderma I also have Sjogren’s Disease and fibromyalgia. The symptoms from these three diagnosed conditions overlap and always make things interesting in sorting out my treatment plan. You can learn more about any of these autoimmune disease by checking out the links in my post.

The Scleroderma Chronicles: The Blue-Lipped Zebra Goes On Oxygen

This week I finished up the pulmonary tests to see what was up with my lungs. This was pretty darn stressful and the poor BLZ was just besides itself with the trauma of walking into a major hospital to get admitted for outpatient testing.

This is my son’s kitten Jonesy, named after the cat on the Nostromo, the ship that accidently picked up a deadly Alien while answering a distress beacon on a strange planet… Jonesy is utterly fearless, more than capable of facing down a scary monster alien. This Jonesy is also pretty darn fearless. Be brave, face this down, I told myself while petting Jonesy.

I was brave. On the day my state opened up the first drive-through testing station for the Covid virus, I presented myself at outpatient admissions. I wore my fleece gloves the whole time I was there and followed all of the safety guidelines. I combined two appointments into one so I could pick up the equipment for overnight oxygen level monitoring while I was in the building for pulmonary function testing. The hospital was almost deserted and had bottles of hand sanitizer out for use at every stop on my route, so it wasn’t as bad as it could have been. I literally swerved and walked around anyone in my way, especially if they were wearing a face mask…

In case you all need a reminder, my red blood cell count (rbc) is way too high, and I have blue lips and shortness of breath. My internist diagnosed polycythemia, and once I googled it I discovered that I have every single one of the listed symptoms. The purpose of all of this testing is to uncover the underlying condition causing my rbc to rise. So, let’s just jump to the chase. Here are the results:

    • My lungs are scarred and I have interstitial lung disease, a type of restrictive lung disease. I am stable and haven’t gotten worse when compared to the last testing two years ago.
    • It’s not pulmonary hypertension. Yay! That would have been really bad news. My echocardiogram showed that I am at the upper limits of heart wall thickening, and my pulmonary artery is at the upper limit of normal, but I’m hanging in there.
    • My oxygen level fell below 89% for almost an hour while I was sleeping the night I wore the monitoring equipment. Yay!! Houston, we have found a problem!!

Once the results were in the pulmonologist’s office called for a phone appointment and I got the good news. Well, the sort of good news. I have to go on oxygen overnight to keep my levels up, and the hope is that my rbc count will start to drop. If overnight oxygen doesn’t do the trick I may have to go to oxygen 24/7, but I’m not there yet. (The BLZ is happy but a little sad too. The BLZ hates to wear oxygen, and really doesn’t want to wear it when it goes out shopping… ) Seriously, I don’t appear handicapped until I have to walk with a cane and wear oxygen… then there is no hiding it any more.

Thinking about future appointments and the procedure with the hip specialist (I need an injection of steroids into my bad-boy hip), I asked what my risk from Covid was… (The BLZ was trying to not cry) and my pulmonologist told me straight out that she thought I would survive an infection but that I should put off any more trips to medical clinics for a few more weeks so that hospitals could finish gearing up for severe cases. Well, shoot. (The BFZ is now sobbing…)

Then I throttled the BFZ into silence and headed out to buy the last of the supplies that I needed to get through a few weeks alone. I’m immunocompromised; the medications that I take to control my autoimmune diseases have helped me to become stable, but they also make me high risk for any infection. Nothing has changed, I reminded the BLZ, but now the risk of a serious infection is coming at a time when the medical system may be overwhelmed and unable to take care of me. I’ve been using good judgement, social distancing, and hand washing for a few years now; I will continue for myself and everyone else sharing this scary time.

The truck carrying the oxygen equipment was at my house waiting for me when I returned so I am set up. Hopefully the BLZ will now slowly fade away and I will stop panting for air every time I move.

If this was a race to get new test results into my chart, the underlying cause of my polycythemia identified, and remedial measures into action, I have made it. I have a phone appointment with my new rheumatologist next week and I will ask her about the hip procedure and referral to the foot specialist, but I think that in the current unfolding Covid crisis there isn’t any hurry.

As for the inner voice that worries and feels panicky, my inner BFZ voice, I am going to smooth it into submission with the peaceful zen of knitting. Be brave, be brave, I purr to myself. Just like my son’s kitten Jonesy.

You all be safe out there!!

The Scleroderma Chronicles: The Blue-Lipped Zebra Report!

I’m thinking of myself as the “Blue-Lipped Zebra” these days. Let’s just call me the BLZ for short. You know, a rare breed of difficult patient who is ornery, persistent, and stubbornly insistent on getting straight answers. I’m trying to not use that zebra voice, but if pushed I may whip it out. I’m going after all my doctors to get to some explanations about my panting, blue-lipped, exhausted current state of being. I made myself promise to keep my internal dialogue under control and to not get pulled off topic. Sounds like a good plan, right?

Whew. It is only Wednesday and I’m pooped! I have talked with or met with three different doctors and scheduled 5 different tests. I also made repeat appointments to get back to these doctors after the testing is done. I went into this determined to do a better job coordinating with my doctors and to shift the conductor role to my new rheumatologist. I’m reflecting on what’s happened so far and what my next steps are, and I’ve decided to share with all of you.

Before I tell you about my medical adventures this week I want to show off the monster orchid. It now has 5 blooms open and it looks great!! Isn’t that an amazing color?

Prologue: I have been struggling with shortness of breath, extreme fatigue, and feeling dizzy/faint. My lips frequently look blue. The itching is insane, and what is up with these rashes? I have more recently developed abdominal pain in the area of my spleen. After initial testing my internist has diagnosed secondary polycythemia.

Act 1: The Internist

This was my first phone appointment. My internist is the doctor who diagnosed me with secondary polycythemia, which is a condition with too many red blood cells. The high cell count, in my case, is now presumed to be due to an underlying problem involving my lungs or heart; since lung and heart damage is common with systemic sclerosis that makes a lot of sense. We talked about my gene test results (I do not have the genetic mutation that would have caused the more serious form of polycythemia, which is good!) and planned my call with the pulmonologist. That’s right: I got some coaching from my internist! I agreed to send her an email about what happened with the pulmonologist and rheumatologist after I met with them, and she assured me that she would order any testing that the other two doctors did not. That’s right – I asked her what testing I should ask for. I love this doctor!!

Act 2: The Pulmonologist

Well, this didn’t go the way I thought this would… good thing I got some coaching. It started out with this doctor saying that she didn’t know what I expected her to do over the phone when she hadn’t seen me in her office for over a year. (She fussed at me last time for coming earlier than a year. I tried to make an appointment, but the nurse insisted it would be better for me to make a phone appointment before I saw the rheumatologist… ugh!!) I explained the situation to her, and she immediately said that she didn’t agree with the diagnosis, and that she didn’t think that my rbc count was all that high. <The BLZ was pretty disgusted by this and wanted to blurt out that the diagnosis wasn’t open for debate, but I throttled it into silence…> After redirecting the conversation to my symptoms (you know, ending up on the floor panting for air after a little vacuuming…) she did agree that there was probably an underlying problem driving the elevated rbc count and it would be good to order up some tests before I came to see her in her office.

Sigh. Why is this so hard?! I wondered if we had just been talking at cross purposes about the same thing. <The BLZ wondered why I had to keep battling for routine testing when my diagnoses required it. Whatever.> The tests were ordered and I agreed to make an appointment to see her in her office a few days after the testing was completed.

Act 3: The Rheumatologist

Finally, finally I have landed in the rheumatologist office that the BLZ has longed for. This doctor felt that the lung testing was absolutely warranted, and that if nothing came up she would go right for a cardiologist referral and heart testing, and a hematology referral also if needed. She kind of thinks that this is my heart, but it is good to get the lung testing done first. <The BFZ is now bucking around… happy for action, but a little scared, too.> She reassured me that the tests that were already ordered were exactly the ones that she would have ordered, but they were just the opening round. She mentioned a test that the pulmonologist has refused to order, saying that it is the only way to get accurate information. Yay! Then she did the exam and reviewed the notes on my orthopedic referral since I still can’t walk and I’ve developed more tendon issues in my foot. “Why hasn’t there been a follow-up on this?” she asked. She ordered two MRIs to look at my hip and foot, and gave me the paperwork to get a handicapped parking pass. She also ordered blood work, told me to call her after the testing was done, and that I should be in her office again in 8 weeks. <The BFZ was stunned. Usually I’m told to come back in 6 months.> I have the scleroderma director that I’ve been looking for, people!!

Epilogue

I spent 5 hours over the last two days making phone calls, appointments, reading all of the medical notes attached this week’s appointments, and writing emails. Whew. Through the constraints of scheduling calendars I will be talking about my test results with the rheumatologist before I interact again with my slightly hostile pulmonologist. When I read the pulmonologist’s notes on our phone appointment it kind of smelled of “cover your ass” and the BLZ’s nostrils crinkled in disgust. After begging for inhaled steroids for a couple years, when I saw her last time she offered me a few months worth. Yay! I said. I wanted to check with the rheumatologist before starting them because I’m already pretty immunosuppressed, which I did, and the inhaled steroids were prescribed soon after by my internist and I’m using them right now. In the notes she wrote that she had recommended these steroids and I that I had refused. <The BLZ immediately noticed the nasty trickery with the verbs there…she offered and I deferred!> She also described my landing on the floor while vacuuming as “needing to rest while doing moderate tasks”. The BFZ is beyond disgusted at that. <Get a new pulmonologist, the BFZ barks!>

In between all of this I also got my newest pair of Snowshoe socks done! How cute are these? I knit them holding a wildly bright variegated yarn with a dark, inky blue single ply yarn. My yarn information is on my project page.

So, it was a pretty darn good start of the week. Tomorrow I head off to get an echocardiogram of my heart, and over the next week or so the rest of the testing will happen.

Coronavirus, be good and stay away from me!! I’m going to be in a lot of medical facilities for the next few days.

You all be safe out there!

The Scleroderma Chronicles: Upsetting vs. Concerning

I haven’t been blogging all that much about my chronic conditions lately… the truth is, I hardly know what to say. I’m sick. I still struggle every day. I’ve been making a lot of adjustments over the last couple of years as I tackle never-ending obstacles, but I really don’t dwell on things too much and I don’t make too many demands of my doctors. I’m still happy, busy, and I manage work-arounds for lots of things that I want to do. Then suddenly this winter I realized that I wasn’t doing all that well after all.

Background: For several years I have been consistently complaining to my doctors that I am short of breath, fatigued, itching, rocking blue lips, and not quite right. My doctors are all like… hmm… that is interesting. Well, it isn’t (insert specific issue that this doctor treats me for), so I’m not sure what to do. We’ll keep monitoring… Maybe you should ask this other doctor about it…

This last December I really hit the wall. I was so exhausted that I was afraid to go shopping by myself. I ended up on the floor panting for air more than once. I collapsed in the yarn store. My lips were blue. The itching drove me crazy. Something in my side hurt and strange bruising kept popping up. Fainting suddenly became an issue again.

I spent most of December in bed reading books and knitting gnomes!

I went to my internist and told her what was up and she immediately ordered several tests. Some scary things like lymphoma were ruled out, but others suddenly reared their ugly heads. I have way too many red blood cells and too much hemoglobin. My cell count spiked to its highest last December when my symptoms were at their worst. Suddenly a new diagnosis was placed on my chart: polycythemia.

Now the fun really begins. I have a lot of things written on my chart. I am diagnosed with several autoimmune conditions: limited systemic sclerosis  (scleroderma), Sjogren’s Disease, and fibromyalgia. These diseases come with a boat load of complications as they tend to cause trouble everywhere they go: I have stage 3 kidney disease, lung disease, hypertension, Raynaud’s Phenomenon, gastroparesis, tendon damage, bursitis, several crazy eye issues, fluid around my heart, and a stomach that has herniated partly into my chest. Seriously, who knew that was a thing? Actually, my whole GI tract is in trouble. My muscles hurt and the joints are swollen. Do you see how confusing treating me can be when a new medical problem emerges? No one wants to rock the boat! My diagnosed conditions actually make it harder for me to get good health care at this point. I know that I am a high risk patient, but this is ridiculous, doctor people!

My internist checked to see if I had the very serious and rare version of polycythemia called polycythemia vera, and since my hormone levels were normal she concluded that my polycythemia was being caused by an underlying condition, and we should just monitor my red blood cell count in ongoing routine rheumatology blood work.

What? Wait… what about my blue lips, panting and all the other symptoms? My immediate reaction was… OH, NO, WE ARE NOT DOING THAT!!! I am done with the ending up on the floor panting every time I try to do something reasonable like… say… cooking dinner! I dusted off the biological researcher part of my brain, generated a list of essential questions, and did a bunch of online searches to see what I could find. The trick to this is to have good questions and the right seach terms. As I worked, things became more productive. Wow. I learned a lot and now I had more questions for my internist. I wrote her back an email with a bulleted list of questions that… she never got. She’s on vacation. One of her partners looked at the email, became alarmed by my symptoms, and called to urge me to head to an ER immediately (!!) to be evaluated. She was pretty insistent…

Which kind of proves my point! Anyone else who ended up on the floor with blue lips, panting for air, because she tried to vacuum the living room would receive timely medical intervention!! This is not reasonable. I’m not getting the care that I should because I am too complicated and there are too many doctors involved. This has been going on for three years and now it is worse. Did one of your parents ever say to you when you were a kid misbehaving in the backseat, “Don’t make me pull this car over!” I am now pulling the car over and stopping this ride.

This is kind of intense, huh. Let’s take a break to admire my favorite orchid!

Tonight as I write this I am just hours away from a phone appointment with my internist. I have my questions ready to go. In the afternoon I have another phone appointment with my pulmonologist; I plan to tell her about the new diagnosis and to ask her about testing that I should have to help identify the underlying cause of the polycythemia. Maybe she will have some other ideas. Tuesday morning I have an appointment with my new rheumatologist and I hope to pass all of this to her. This rheumatologist specializes in complicated cases, is recommended by the Scleroderma Foundation, and prefers to personally coordinate the entire care team. Yay! Here I come! I am the girl for her!

There are many things that are happening to me that are upsetting: my hair is falling out, I have a rash on my face, and I now use a cane to get around. This is the kind of stuff that makes you want to sigh and stay in bed some mornings. You wish it wasn’t happening to you. It’s upsetting.

Then there are things that are just down right concerning. In my mind a red flag pops up and a small siren goes off: strange and unexplained bruising, double vision, pain in my left side, blue lips, panting for air while trying to open a door, tremendous itching after showers. This kind of stuff is a call to action; get out of bed and deal with it! I am now mobilizing all of my energy towards GETTING TO THE BOTTOM OF THIS and securing answers, treatment, and hopefully, an improved quality of life.

Because there is a difference between upsetting and concerning. I am not upset, but I am concerned. I am mobilized. I have questions and I want answers.

I am totally over the blue lips look!

I’ll let you know how this all works out. It should be a busy week.

The Scleroderma Chronicles: Safe House

You know, I kind of view myself as a happy camper. I have more things (ahem… knitting projects) going then I can get finished on any given day or week, books lined up to read, and a “to-do” list that I’m slowly working my way through. Hey, people, I fixed the loose tiles on my kitchen floor last week!! My cat MacKenzie is my constant sidekick throughout the day as I knit, work in the garden, read in bed, and even with me (underfoot, demanding cookies) while I’m cooking. Even on the bad days when I’m pretty much down for the count, I manage small victories. There is just one problem with this picture.

Look at these beautiful coneflowers I just added to the garden. I’m hoping to lure some butterflies into the yard. Every garden should have butterflies, don’t you think?

I just don’t fit in the world all that well anymore. In my home, living the life that I’ve created for myself, it is really easy to forget how much I have adapted to accommodate the limitations of my scleroderma, Sjogren’s, and fibromyalgia. Once I go anywhere else reality hits me hard. Every trip out of my house is going to come at a cost. Here are the worst of the offenders that will lay me low.

Air Conditioning I know that almost everyone in the world is grateful for air conditioning in the summertime, but for me it is a royal nightmare. The shock of walking into a refrigerated building on a hot summer day will trigger an immediate Raynaud’s attack. I pull on long sleeves and fingerless mitts as soon as I get into the building, but my lungs know what’s up and I have trouble breathing. The airflow makes my eyes burn; I’ve been reduced to wearing my sunglasses indoors to protect my eyes. Don’t even get me started on the refrigerated cases churning out cold air; you haven’t lived until you’ve had to pull up the hood of your sweatshirt and the sleeves down over your hands so you can score some butter and eggs.  If that wasn’t enough, there are also usually…

Scented Products Almost all buildings use scented cleaning products and sells additional items with scents. Candles. Lotions. Laundry soap. The scented bathrooms are a nightmare. If I’m not already in trouble with my breathing I will be if I have to walk down the laundry detergent aisle at the grocery store: I also start to itch and my face swells. Why do these chemicals even exist? They can’t be good for anyone!

Restaurants These are a special kind of hell for me. All the drinks come cold and with ice, and the entrees are served piping hot. There are other landmines that I need to avoid: salt, lactose, fiber.  I have to carefully select something that is very soft and that will behave itself in my gastroparesis stomach. I can’t have spicy food. I can’t eat fresh veggies. Actually, to be safe, some of this food should go through a blender… I actually once soaked a cut up sandwich in soup so I could eat it…

Walking I am trying really hard to meet my walking goals every day, but I stretch those steps out over the day. A trip to run errands can be just exhausting if I’m on my feet for a couple of hours at a time. I need to always carry water, be aware of the location of bathrooms, and have places where I can sit down if I need to.

Sunshine It makes me sick! Enough said.

Ready for another picture? These paper wasps are building a nest right on the edge of my deck! MacKenzie and I are not amused. Still, it kind of shows how the outside world is full of dangers…

Recently I had a tough talk with myself about pruning down my outings and being more strategic about how I expend my energy. I need fewer outings, and my destinations need to be closer to home. I need to live online. I need to in a safe environment as much as I can to manage my diseases.

My home is my safe house. I have no air conditioning and I minimize air flow. I keep the temperature in the mid 70’s in the day so that my joints and lungs will be happy. I cook all of my own food, I don’t own any salt at all, and everything that I drink is room temperature. Fruits and veggies go through the blender to become smoothies. Every product that comes into the house is scent free. I’m always close to a bathroom or a soft surface to crash onto for a quick recovery if I get dizzy. My stairs have wrought iron rails that I use effectively on bad joint days. Flourishing in my safe house I sometimes forget how sick I am because, well, I have fewer problems.

I planted this yarrow last year in a flowerbed that has killed almost everything planted into it. Not this yarrow!! The secret of gardening, and living with serious chronic illness, is to keep on trying new things, and to match your needs to your environment. Or, in my case, make your environment match your needs.

Tomorrow I have a doctor’s appointment so I listed up some symptoms and issues that I need to ask her about. It is quite a list now that I look at it, and it kind of underscores how chronic illness can trick you into thinking that things that would normally send you screaming into urgent care are “just another day of scleroderma.” Shortness of breath is an almost daily thing. When I glance into the mirror these days I sometimes notice that my face is blue. One hip keeps failing me; okay, I actually have to lift that leg to get into the car. My joints swell so much that I can’t sleep at night.

But I am good, here in my little safe house with my gardens and cat.

Tomorrow my doctor and I will attack some of these scleroderma/Sjogren’s issues. I kind of think that lung testing and a MRI of my hip are in the future, and that there may be follow-up with my pulmonologist. I’ve been gathering up my energy in preparation for these outings into a world that is dangerous for me, knowing that after each outing my garden swing, knitting and latest book will be waiting for me. With a room temperature ice tea.

And a cat!

It is good to have a safe house.

Science and the Scleroderma Girl: Hard Choices

Everything happened really fast when I was first diagnosed with scleroderma  and Sjogren’s Syndrome. I had been referred to the rheumatologist after the results of bloodwork that my PCP ordered up. The rheumatologist did an exam and some tests, told me that I had the form of scleroderma called limited systemic sclerosis, and was evasive when I asked him what this would mean for me in the long term. What would my life be like in five years, I asked. He said he needed to run some more tests and would get back to me. He also told me to stay off the internet.

Well, that was kind of ominous, don’t you think? My first clue that this might be rather serious…

I was given a prescription for a disease modifying drug (DMARD) called Plaquenil, a handful of pamphlets, and referrals for additional testing. Lots of testing. Waiting for my prescriptions to be filled I went to the lab to get blood drawn and then sat down to call to make appointment for the additional testing I needed. It was hard to not feel like the sky was crashing down on me.

Cinco de Mayo rose.
Hey, time for a rose break. Look at what is blooming in my garden this morning! 

After 6 months of the drug Plaquenil I was feeling better, so I was a little shocked when the rheumatologist told me that it was time to add additional drugs and that he was going to give me the choice of methotrexate or CellCept. He handed me information on the drugs, ordered more blood tests to determine the state of my liver, and told me to call back in a week to let him know which drug I was comfortable with.

Of course I went straight to the internet. Hello. Science girl here. How should I make a decision without more information? Time to put all that chemistry and molecular biology to work!!

Ugh. After researching both of these drugs it didn’t look too good to me. Methotrexate is actually a chemo drug, given in a lower dose to rheumatology patients to suppress the immune response.  I would need folic acid to try to minimize hair loss and other side effects, and it would knock me on my butt for a day or two each week. CellCept also suppressed the immune system by another pathway, had fewer side effects, more risk of cancer, and was really hard on the stomach. Both drugs had definite downsides. I would need to stay out of the sun to prevent DNA damage. My risk of serious infections, including  PML, a fatal brain infection, would go up. The side effects of methotrexate seemed to be worse to me, but the CellCept would be awful on my gastritis…

If I chose to remain untreated with either of these drugs my chance of developing a fatal complication from scleroderma went up. I was already in the early stages of interstitial lung disease and pulmonary arterial hypertension, both of which could be fatal, so I really did need to try to slow things down with a drug…

Ugh! I was feeling pretty helpless about making the decision. What should I do when the future was unknown? Everything was a gamble and I didn’t have any really good choices.

I called my rheumatologist and told him I wanted to try methotrexate because I was pretty sure that I wouldn’t be able to handle CellCept. It was done.

Jacob sheep
Science and the Scleroderma Girl will be taking the weekend off because tomorrow I am going to the Estes Park Wool Market with my BKB Deb. Any day in the mountains is a good day, but it will be even better with yarn, peeps, and cute sheep. Woohoo! Fiber festival time!!

This whole process was kind of awful. Just knowing how these drugs worked and their side effects wasn’t enough. I needed more info. I needed to hunt down research studies.

Science time!!