pulmonary hypertension – Yarn, Books & Roses

The Scleroderma Chronicles: Unforeseen Circumstances and Unintended Consequences

I guess almost everyone in the world knows by now that the United States Supreme Court issued a ruling last month (June 24th) that overturned the constitutional right for a woman in the United States to seek an abortion at any time during the first two trimesters of pregnancy.

This post isn’t really about abortions, okay. This is about the unbelievable unintended consequences of that decision for women who are dealing with unforeseen circumstances. Women who are dealing with medical conditions that they never saw coming, and that they absolutely did not bring upon themselves.

Like autoimmune disease. Like cancer. Like any one of a number of medical conditions that require serious, high-risk medical interventions by the medical professionals who are treating that woman.

My first inkling that there might be a problem with the Supreme Court ruling that was much larger than what was being reported in the press happened the evening of June 29. A woman in one of my autoimmune online support groups posted that she saw a message from a doctor who had a patient reporting that she couldn’t get her methotrexate prescription renewed. I literally couldn’t sleep that night I was so upset. In the days that followed there were more reports, and this is really happening to some women. They are being denied refilling of their methotrexate prescriptions. This drug is a common one used to treat autoimmune diseases like rheumatoid arthritis, lupus, and scleroderma. It is a chemo drug, it can harm an unborn child, and it can also be used to induce an abortion. Here is an article on Health.com, another at msn Everyday Health, and this one from Time.com.

There are some serious drugs there in my hand. The green pills, Myfortic, can harm an unborn child by causing birth defects or a miscarriage. I take 6 of those every day. The white capsule, omeprazole, should only be used “only if the benefit outweighs the risk to the fetus”. I have to take two of those each day. That little pink pill towards the upper left is the really big problem in my hand. That drug, ambrisentan, required a rigorous enrollment process with paperwork from my cardiologist and myself along with two interviews by pharmacists. I had to prove that I could not get pregnant to avoid taking a pregnancy test prior to each month’s supply of the drug being shipped to me. Even with all of that, I am screened each month before the next month’s supply is overnight express shipped to me. This drug poses a serious risk to a fetus. In my support groups for pulmonary hypertension there are women who are reporting that they are getting dropped from the programs that allow them to get the drug.

I’m okay because I’m beyond childbearing age. Many other women who are dealing with autoimmune diseases that require drugs such as these are of childbearing age. Why? Because the people in the population who are at the greatest risk of developing an autoimmune disease are women of childbearing age, that’s why. Because of the recent decision about access to abortion these women are also now at possible risk of being denied treatment for their autoimmune disease, or in the worse-case scenario, access to an abortion in consultation with their medical professionals.

I literally couldn’t sleep when I read the first reports of the problems with access to methotrexate in my online forums. This is absolutely unbelievable. This is unbearable. How could this be happening to a population of desperate woman, through no fault of their own, who are in this horrible situation? Right now, methotrexate is being targeted, but there are all these other drugs that are used for so many patients with so many conditions. Women, absolutely, are at risk of receiving inferior health care in the US.

This is awful. Have a rose. Right now, I need a rose. And a hug.

This is hitting me hard this evening because I have been dealing with autoimmune pneumonia since last December. My doctors are trying to get it under control with high dose immunosuppressants, but I am coming off one of them right now and I am struggling with shortness of breath and chest pain once again. I’m back on daytime oxygen and I’m feeling a little low. The lung biopsy was the first engagement with my interstitial lung disease, this current drug strategy the second battle, and the third battle is looming on the horizon. This isn’t a disease; it is a war. I never asked for this, and yet, here I am trapped in this unforeseen circumstance, engaged with an uncurable foe. Without the drugs in my hand above, I would already be gone. Yay, science!!

I think that there is another CT scan in my near future, and then there is a possibility that my doctors will move to the Plan B (see what I did there) that they have already discussed with me. Cyclophosphamide (a chemo drug) and OFEV (an anti-fibrotic drug) are on deck if my doctors decide to escalate my care. Both of these drugs can harm an unborn child. I will get access to these drugs. If I was in my 30s, as many other members of my support groups are, it might be a different story. What about women in other support groups that I don’t belong to who just got diagnosed with cancer or an equally serious disease who also need medical treatment with high-risk drugs such as these? Women of childbearing age. Women who may already have children who they need to think of and care for in their health care journey.

Being diagnosed with an autoimmune disease like the ones that I have (systemic sclerosis and Sjogren’s disease) is a punch to the gut. Being denied access to treatment because of your childbearing status is an unbearable second blow. Becoming pregnant while on one of these drugs would place a woman in an impossible situation. Nothing, NOTHING, about this situation is good. Where is the privacy for these women? Where are the HIPPA protections that they are entitled to? The decisions that these women face are absolutely heartbreaking, morally complicated, and ethically challenging. They deserve privacy as they make them along with their health care providers, religious advisors, and families.

This post isn’t about abortion, not really. This post is about unforeseen circumstances and unintended consequences.

I feel a lot better for having gotten this out.

Peace be with you all.

But if you decide to share this post, write a congressperson, join a march, or take some other action to ensure that women have equal access to health care, that would be great.

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: And Do You Exercise Regularly?

It has been an eventful week in big and small ways. I had been mostly in bed for most of a week as I struggled my way through two snowstorms with significant air pressure drops. Ugh. I had chest pain, coughing, heart palpitations, and more sleep than I want to admit to. Towards the middle of the week, I went off my immunosuppressant drug and the flare of my disease(s) arrived over the next two days. Ugh. So predictable, but still discouraging. I dragged myself together on Monday, double masked, and made it to the pharmacy where I had an appointment for a Covid-19 booster that afternoon.

Mateo: Do I need a booster too? Here’s my arm…

I have this really wacky sense of humor. The entire experience just kind of cracked me up. You see, I got the appointment at my local grocery store’s pharmacy. Here are some of the highlights:

I have had so many shots at this point that they had to use the back of my vaccine card. At this rate I will need an accordion-like pullout for the vaccine information in a few months.
- Why so many shots? I’m immunosuppressed. I went off my drugs this time to give my immune system a better chance of responding to the vaccine.
The staging area for the shot was at the Fritos display across from the pharmacy. Seriously, the pharmacist said, “Go stand with the Fritos and wait your turn.”
The shot was easy, peasy. I think that the syringe was spring loaded it was so fast. “Go walk around the store for 10 minutes before you leave,” I was told.
So I waved goodbye to the Fritos and walked around the store. Mostly I just looked at the empty aisles for the 10 minutes feeling sorry for myself. No milk. No Snapple. No cat food. No guacamole. NO GUACAMOLE!!!! Oh, yeah. Genius me scheduled the booster shot during a grocery store strike by the competing chain’s employees and this store was basically stripped of essentials by the descending horde of shoppers who didn’t want to cross the picket line. As they shouldn’t. But they could have left me a little guacamole, don’t you think?

I was able to get the cats a Boston fern to replace the palm that was chomped to death by… I wonder who could have done that? Hmmm…

I also got a Starbucks. Not the worst trip out of the house. I ended up with a sore arm and was so exhausted that I slept for 12 hours.

Today, 48 hours after the booster shot, I feel great!! The flare is gone. I haven’t felt this good in weeks. This happened to me the last time I got a Covid booster. I think that it must be the increase in antibodies or something; I’m so immunosuppressed that my gamma globulins are way too low (a medical condition that my doctors are just ignoring because I do have enough white blood cells). Maybe the boost in antibodies following the shot actually makes me feel better somehow. Maybe my white cell count goes up. It’s a mystery. I’ll take the win!

And in that winning mood I went to see my cardiologist for the first time since my trip to the cath lab last fall. I was a little short of breath but was walking okay when I got to the office. I received an EKG test and the nurse checked me in:

Nurse: “And do you exercise regularly?” (in a judgmental tone of voice…)

Me: “Oh, please. Let’s not even pretend that I am able to exercise!”

Nurse: “Oh. I’m so sorry that I asked you that…” We both started laughing, but I meant it! I am so over feeling defensive about being unable to exercise. In fact, trying to exercise with my condition was damaging my heart.

I also think that I had my snark on under my mask.

My cardiologist is freaking awesome. He asked lots of questions about how I was doing. (Face now mostly not blue. Yay! Some panting and chest pain, but so much better. I went up a couple of flights of stairs with no problem.) We discussed the fact that I don’t fit the usual diagnostic model for pulmonary hypertension but based on physiological changes consistent with PH and my dramatic response to treatment with a PH drug, he made the call and entered the diagnosis. The matter is now settled until new data comes along.

Exercise-induced pulmonary hypertension. As in, you look perfectly normal when you are on the table getting your lung/heart tests, but the minute you exercise all hell breaks loose in the blood vessels of your lungs. Fabulous. There is a really invasive testing protocol that I could be subjected to, but there is enough evidence now to establish the diagnosis without it. The diagnosis became part of my medical record today. At last.

It has been almost exactly 5 years since the BLZ began her journey to find help and answers. This has been really, really hard, but I made it.

We discussed the pros/cons of more testing. We talked about the risks and benefits of adding a second drug to the one I’m already on. We talked about who will take over management of my PH (he will) and how he will integrate with my rheumatologist. We talked about how important it is to be comfortable with “out of the box” thinking when dealing with a patient who is basically at the far end of the bell curve… in other words, a zebra. Oh, I like this guy!!

I agreed to start the additional medication which will be added to the one that I’m already taking. There will be more side effects as this second drug kicks in and I will be getting several phone calls to check on me as I start it. The plan is to try to slow down my progression before I develop full blown PH.

Next up: more testing to hunt for that dang hole in my heart. It’s like a snipe hunt, but so much less fun. As in, heart surgery anyone?

Hannah: Don’t worry mom: you’re got this!

The Scleroderma Chronicles: The Blue-Lipped Zebra Gets Her Diagnosis!

At last. I mean, this has been going on for more than 5 years and had reached the point of utter absurdity. If you haven’t been keeping up on all of this, I have been struggling with shortness of breath and sporting blue lips for way too long. I also have pretty significant fatigue, chest pain, and major muscle and joint pain. I’m a mess.

I have a rare autoimmune disease called limited system sclerosis(scleroderma) which makes me high risk for lung and heart issues. People with rare diseases are called “zebras” in the medical community; since I’m sporting blue lips I’m the Blue-Lipped Zebra (BLZ). Got that?

My doctors do routine testing to monitor me for heart and lung conditions associated with systemic sclerosis; each time I had an echocardiogram and a high resolution CT scan the results were that I was… fine. No indications of a major problem.

But I was absolutely, positively not fine. I began to refer to the reassurances that all was okay as medical gaslighting. I transferred to new doctors. I got copies of all my test results, did lots of google searches, read research papers and articles in medical journals, and began to have evidence-based discussions with my doctors. They ordered up more aggressive testing of my heart and lungs. I posted about the my right heart catherization and CPET here if you want to catch up.

Last Monday my cardiologist called and gave me the final diagnosis. I have a type of pulmonary hypertension that is exercise-induced that is being complicated by a cardiac shunt in my heart. At rest, for all those previous echocardiograms and CT scans, everything was fine. When I’m in motion it is another story.

So, what exactly is pulmonary hypertension and why am I, as a systemic sclerosis patient, at high risk for it? In the most simple terms, the interiors of my lung arteries are narrowing due to scleroderma scarring and tissue growth; as the openings get smaller, the pressure of blood flowing through the arteries gets higher.

When we exercise the body needs more oxygen delivered to tissues; arteries constrict to raise blood pressure, your heart speeds up and your respiration rate increases. In my case, that constriction of arteries in my lungs makes the blood pressure in the lungs increase too much; blood struggles to get through the pulmonary arteries, and the downstream pressure in my right heart forces blood to flow from the right side, through the shunt, and into the left. My body’s blood pressure zooms up as the left side of the heart fights to push blood out of the heart past the jet of blood coming in from the right side through that dang hole. It’s a catastrophic cascade that happens in seconds, and the entire phenomenon is being driven by my systemic sclerosis created pulmonary hypertension. “You’re a challenging patient,” my cardiologist told me as we talked about my future treatment. Yep. That’s me. A challenge. I excel at challenges.

Last week the new medication that my cardiologist prescribed was shipped overnight express to me by Kaiser’s National Specialty Pharmacy. Kind of unusual, right? That’s because pulmonary hypertension is rare, so there aren’t that many people taking this drug in the US. If I was a Blue-Lipped Zebra before, I am now a BLZ wearing a crown. A periwinkle crown, of course, for pulmonary hypertension.

When I started this scleroderma journey one of my doctors told me that it was good to have a diagnosis, even if it was a shame. This is true. I’ve learned a lot since my first blood tests came back hinting at an auto-immune disease that generated a referral to a rheumatologist.

I’ve learned to be patient. I’ve learned to advocate for myself. I’ve learned to take the initiative to learn about my disease and to become an active participant in my treatment plan. I’ve learned to face down the monster and to go on with my life.

Challenge accepted!

This is Pulmonary Hypertension Awareness month. About 15% of systemic sclerosis patients develop pulmonary hypertension as a consequence of their disease. You can learn more about pulmonary hypertension here.

The Scleroderma Chronicles: World Scleroderma Day, 2021

Here we are again… World Scleroderma Day.

I’ve written about scleroderma on this date for several years now. I just went back and read what I wrote last year and decided that I did a pretty good job. I talked about getting diagnosed, the complications that are being caused by my disease, and what that means in my life. Here’s that post if you would like to check out my take on things last year.

This is one crazy-ass disease and it certainly has complicated my life. In the year since I wrote that last post I have hung in there (hey, staying home for a year on oxygen slowly improving was actually good for me) and then emerged from strict lockdown to get lots of testing to try to sort out what is the cause of my blue-lipped status and the source of other little issues that I have going on. While thinking about what to write today I thought of a few things that I haven’t written about before and some things that are new since the last post.

The correct name for the type of scleroderma that I have is limited systemic sclerosis. Systemic means that every part of my body is being impacted by this disease, and the word sclerosis means that scarring is happening in tissues all over my body. What’s happening to me is not obvious to the outside viewer, but it is insidious and ongoing all the same. Somehow, by some mechanism that isn’t clear, my systemic sclerosis is being driven by antibodies that I’m producing that react with the centromeres of my cells.

What’s a centromere? I’m shocked, shocked I tell you, that you just don’t know that! Seriously, the centromere is that little place in the middle of a chromosome that can be seen when cells are getting ready to divide. This is a little tricky because the only time we can see chromosomes is when cells are getting ready to divide and they have already copied themselves: the little pinched waist in the middle of the chromosome where they are connected is where the centromere is located.

Got that?

This image belongs to the NIH; as a taxpaying citizen I hope that it is okay for me to use it here. 🙂

Somehow something happened to a protein located in the centromere area of the chromosomes in my body that made it look “different” and my immune system responded by making antibodies against that protein. The antibodies that are produced are called “anti-centromere antibodies” and their presence is highly suggestive of my form of systemic sclerosis. The alternative explanation is that my immune system just went berserk, decided to attack my own cells, and began making these antibodies on its own. I personally think that something changed and then the antibodies were made as a response. My money is on a virus.

It really doesn’t matter all that much since, once the immune system is triggered, the immune response can’t be turned off and one day you are sat down in some rheumatologist’s office and carefully told about what is happening to you and what to expect in the future. Your skin will get thick. Your blood vessels will be so damaged that they will thicken and spasm shutting off blood flow to parts of your body unexpectedly. The scarring tissue will build up in your digestive tract and damage the smooth muscles that you need to move food along. Your kidneys will lose function. Your nerves will be damaged. Your lungs need to protected since they are especially vulnerable to damage from accidently inhaled stomach acid. If your disease flares badly enough you can develop autoimmune pneumonia. Your tendons, muscles, and joints are all in trouble. You are in trouble. The damage will progress and there is no cure.

“This is really serious,” one of those early doctors told me. “This is like a diagnosis of cancer, but of course, some cancers can be cured…”

Well, shoot. Good thing there are drugs to help control symptoms and to slow the progression of the disease by dialing down the immune system.

Yay for drugs!! I am on drugs to shut down my stomach acid production (my lungs are doing pretty well) and on drugs to crush my immune system into submission. I’m on anti-inflammatories to control other cellular pathways activated by those ill-behaved antibodies. I get steroid injections to help my damaged joints and inflamed tendons. I have strict dietary limitations. I do lots of physical therapy. I’m on oxygen overnight to keep my red blood cell count in a normal range. I dress in layers to help control the spasms of my circulatory system, a phenomenon called Raynaud’s. I knit almost every day to keep my fingers from stiffening up and contracting. I now have a shiny purple cane to help me walk.

Every day is a challenge, but I am fine.

So what is new this year?

Scleroderma has damaged my heart. They are still testing to clarify exactly what is going on, but so far they have established that there is scarring in the heart muscle that is making my heart “stiff” and that somehow I developed a hole in my heart. There are suggestions of pulmonary hypertension, but I need more testing for a definitive diagnosis. No wonder I turn blue in the face and pant when I walk.

Oh, yeah, there is also a pandemic going on.

One of the greatest ironies of the last year is that serious cases of Covid-19 share similarities with the clinical symptoms of scleroderma crisis. Immune system-mediated pneumonia caused by an overreacting immune system is a hallmark of both conditions. Then there is this… Remember those drugs that I take to crush my immune system into submission? I take two drugs for that purpose. One of them, hydroxychloroquine, was (irresponsibly) politicized by influential individuals early in the pandemic and therefore became short in supply; my muscles and joints immediately rebelled when my supply lapsed early in the lockdown. The other drug that I take, mycophenolate mofetil, is linked to poor response to the Covid-19 vaccine. I am vaccinated and I am making anti-Covid antibodies, but since my total antibody count is very low my doctors aren’t sure I can fight off a Covid-19 infection fast enough to stay out of the hospital. I’m advised to continue to mask and isolate because… blue face, hole in heart, stuff like that…

For me, lockdown goes on.

So, here are the takeaway messages from this post. Be kind. There are illnesses that are really debilitating that you can’t see when you look at that person walking into a store from a handicapped parking spot. Don’t be dismissive of conditions with funny names that you’ve never heard of before. I know that it is hard to understand conditions that are beyond your experience and that you can’t really see, but take a moment to let someone tell you about their illness and the daily challenges that they embrace. If you meet a person with an autoimmune disease, they deserve a hug. If they have scleroderma you should give them two hugs! Be understanding of people who are still wearing a mask in public; perhaps they are braver than you can imagine.

Today is World Scleroderma Day.

Go Team Teal!!

Note: If you would like to know about different types of scleroderma you can learn about them here.

The Saturday Update: Week 4, 2021

It was a crazy, crazy busy week with lots going on. I had medical testing, an online court appearance to give testimony in a neighbor’s custody case, and a nail in one of my car’s tires. My medical status continues unchanged (blue lips, chest pain, shortness of breath), but the machinery to get to the bottom of things is now in motion. The first couple of rounds of testing have generated a referral to cardiology and hopefully that will happen this week. My neighbor won her custody case, and the car tire is now repaired. Whew! I spent the bottom half of the week relaxing with my knitting because I was completely pooped by all of that running around.

The Kitten Mom left me all alone THREE times this week!!

Knitting

I did make some good progress this week in spite of the trips out of the house.

My Geology socks are done!! I’ve decided to try to make at least one pair of socks each month, so these are January’s pair.

I also buckled down and did the blocking and finishing work on my Secret Life of Cats (and dogz) shawl by Sharon from Security (Casapinka). This is the longer shawl version of the project; there were also options to make a cowl or a scarf. Fun color for gloomy days, huh! I’m actually thinking that I may be giving this one away to someone who loves purple and bright colors and a scarf version made with scrap yarn from the stash may be in my future.

It snowed this week I so gave in to the urge and cast on one of the sweaters that I have been dying to get going on. I have wanted to make Goldwing for a long time, and bought the yarn a couple of weeks ago with my stimulus check. Here it is, finally started:

Look at the absolute quality help that I am getting from Hannah!!

The Scleroderma Chronicles: The Pulmonary Hypertension Edition

I few weeks ago I posted about my systemic sclerosis, Covid-19, and my decision to donate my DNA to the 23andMe Systemic Sclerosis Research Project. My DNA has safely arrived and is in the lab getting sequenced right now. I was motivated to contribute because Covid-19 is creating so many new patients with fibrosis that may benefit from this research in addition to people like myself with autoimmune disease or people with other fibrotic diseases.

Monday I had an urgent echocardiogram done and once again an eerie connection between my disease, systemic sclerosis, and Covid-19 appeared. My test was started a little late so I asked the technician if things were busy. He told me that they were very busy because there were so many Covid-19 long haulers who needed testing. After a while, thinking things over, I asked if these patients were getting heart damage. “Well, not their heart muscle, but they were developing pulmonary hypertension,” he said. Oh, oh. That is the very reason I was there getting an echocardiogram; as a systemic sclerosis patient I am high risk for pulmonary hypertension and pulmonary arterial hypertension, and I know that those are serious and life altering/ending conditions. After thinking a little longer I asked him how many Covid-19 long haulers were getting that diagnosis. “It’s in double digits,” he replied…

Double digits. At this one medical center in the heart of Denver. That means that there are potentially hundreds and hundreds of patients getting that diagnosis across my state.

I wished that I had thought to ask him how old those patients were…

The next day my rheumatologist called to let me know that I was being referred to cardiology as my echocardiogram results suggested pulmonary hypertension and that further testing was required. There is also an issue with fluid around my heart… It was what I expected, but not exactly what I was looking forward to. The only problem right now is getting me into cardiology, because, all of those Covid-19 long haulers…

It has been impressed on me that I need to double mask now each time I go out into public. I have a nice N95 level mask, but I’m also putting a medical grade mask on top of it.

Be careful, people!!

Stay safe and wear your masks!!

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: My Second Year Report

Well, here I am at the end of my second year since my diagnosis of systemic sclerosis, a life-threatening form of the autoimmune disease scleroderma. Last year I blogged about my illness: I had come through the worst of the grief and horror at the initial diagnosis, had made my way through some scary incidents that sent me flying across town to emergency centers, and was pretty upbeat about where I was in the progress of the disease. I was sick, but I hadn’t developed any of the most serious, life threatening complications. My heart and lungs were fine. I had just been started on some serious immunosuppressant drugs (the same ones that are given to kidney transplant patients), and while they are risky, I had been told that they could really make a difference in my 10 year survival rate.

Hey, you roll the dice and you take your chances. No sense worrying about the unknown future. I drugged up and slept like a baby at night.

Star Trek Meme — The Kobayashi Maru test, as all Star Trek buffs know, was a no-win scenario; it was meant as a test of character. Systemic sclerosis, an incurable, progressive, disabling and potentially fatal disease, can also be considered such a test.

Ready to hear about my second year? Let me give you a hint: buckle your seat belt, because we are in for a bumpy ride.

November-December: the drugs begin to kick in and as they beat my immune system into submission my skin starts to harden up. I hurt everywhere!! I can hardly bear to comb my hair. The place where my flu shot went in hurt for weeks afterwards.
January: I caught the flu. Talk about insult to injury! Here’s the short version: antibiotics, off the immunosuppressant drugs so my body’s immune system can fight back, and then trouble breathing, chest pain, heart palpitations, and a partridge in a pear tree. What a mess! The month passes in a blur.
February: I’m still pretty sick and struggling to breathe. I get bounced back and forth between doctors as the debate about the root cause of my symptoms rages. I begin to pressure my doctors for answers and there is much testing. Oops. All is not well with my lungs and my heart is accruing damage. I get sent to a pulmonologist and she give me an inhaler to help me breath. She also tells me I am in serious trouble and refers me to palliative care. The doctors increase my immunosuppressant drug dosage.
March: Why, hello, Sjogren’s Syndrome. We forgot all about you! In the concern about my systemic sclerosis, the bad boy of my autoimmune twosome, everyone forgot that I also have Sjogren’s Syndrome, another serious autoimmune disease that causes dry eyes and mouth. As it turns out, it can also cause small airway disease (think never-ending asthma attack) and it has pushed me into chronic respiratory failure. Hello oxygen machine. You are my new best friend.
My new best friend!
April: new lung scans are back, and while I am diagnosed with interstitial lung disease, it appears that it is only mild. Huge sigh of relief!! There is also consensus that my pulmonary hypertension has not advanced. Both of these diagnosis, while still early and mild, are very serious, and the decision is made for palliative care to continue to follow me. Bummer!
May-July: Sunshine! Heat! Burning muscles, aching joints, gastritis, dizzy, dizzy, dizzy, and I notice that my lips are blue. I’m on oxygen 24/7 by the end of July.
August: my internist changes my meds to bring my heart rate up, and suddenly I have enough oxygen. The heart palpitations stop and after more testing I come off the oxygen. The 6 month Sjogren’s-driven asthma attack is finally over.
Summer quilt and socks for my poor hurting feet.

September-October: why does it hurt to walk? What is up with my feet? And this whole barfing in the middle of the night is getting downright annoying… My internist tests me to see if I have an H. pylori infection.
November: Well, doesn’t this beat all. The H. pylori test came back negative and I am diagnosed with gastroparesis. The muscles of my stomach are too damaged by systemic sclerosis to work correctly; the damage is irreversible. I start eating a very limited diet of soft foods and dairy. Ironically, I can now eat jelly donuts, but not fresh baby carrots. I’m losing weight anyway.

Little Greenhouse — The gastroparesis diagnosis hit me hard even though I kind of knew it was coming. I stopped to get a little cheer-me-up on the way home. Check out the little greenhouse I put together for the kitchen window. As always, MacKenzie had to help out with the picture.

Flowers — Here’s the flowers. Aren’t these cute? They were sold at the local nursery to put into “Fairy Gardens”

and how could I resist adding the little cat and the mushroom? Those plants are miniature Kalanchoe that should eventually bloom again.

See, a bumpy ride that is still going, but a year that was also rich in gifts. Palliative care forced me to face the future with more courage and to make end-of-life decisions for my family and to start cleaning out my house of junk. I talked to family about my medical power of attorney. I enlisted one of my doctors to manage the medical team and I began to feel more in control of my basically out of control disease. I began to knit gifts for those I love with a purpose: everything now is a piece of me. In my mind the shawls that I am making for everyone I know are the “Good-bye Shawls”. I am on fire to make as many fingerless mitts for other scleroderma patients as I can. At the end of the day, this year was not one of struggle and heartbreak as I dealt with the endless march of a disease that has no pity or remorse. Rather, it was one of care, giving, creative fire, good friends, and the meditative peace of knitting.

Okay, I do get cranky at times, and there has been some crying. I get short with annoying salespeople because it is so hard for me to shop. I told my ex-husband I was tired of hearing about his “stupid-ass” motorcycle. I yelled at the cats. I hate when people say, “Well, you look great!” in a way that suggests that I’m not really all that sick at all. I wish that they were there to hold my hair when I hobble to the bathroom at 2am to throw up that nice meal that I hopefully ate but couldn’t digest. There. I got it off my chest, and I feel much better. Aren’t you relieved to hear that I can be petty and mean from time to time?

Tomorrow is Thanksgiving Day. You know, it is easy to focus on the day: travel, turkey, family and the descent into wild Christmas shopping. Sometimes we forget the history of this national holiday; thanks for a good harvest and the blessing of probable survival through the coming winter. It is also a time to reflect on the bounty of the last year and to be grateful for the gifts it brought.

In spite of all the bumps of the last year, I am grateful for all of the gifts I have received.

Happy Thanksgiving everyone!!

The View from Here: One Year of Scleroderma

It’s been a year since I was first diagnosed with scleroderma. Actually, it has been decided that I have the form of scleroderma called limited systemic sclerosis (the disease formerly known as CREST). Sounds kind of fraught, huh. That’s because it is; no matter what I think I know about my condition this week, by next week things will probably change. It has been quite a journey this year and now that all the dust has settled from the latest rounds of medical tests I wanted to share my thoughts.

There is no question that the first months after my diagnosis were filled with waves of horror and grief. At first I was just stunned to discover that I might lose the use of my hands. Then it dawned on me that my ability to live independently might become problematic; I needed to make plans to activate a support system for myself at some time in the future. Eventually I found out about the fatal complications and the high mortality rate. Well, shoot. During this time I became a driven knitter: endless pairs of fingerless mitts rolled off my needles.

CARD — My sister, who has a somewhat warped sense of humor, sent me this card about the time that I learned about my risk for pulmonary hypertension, an often fatal complication of limited systemic sclerosis.

Yet, it has been a year and I am still here. I sailed through the medical tests and none of the frightening medical complications have manifested themselves. My lungs and heart are fine (huge woohoo!!). My GI tract hasn’t gotten worse. My hands are very swollen and the skin is getting hard (one of the hallmarks of scleroderma is hard skin that forms due to lots of collagen deposition and scarring; my arms and legs are getting hard too), but they work just fine and are actually much better than they should be. My rheumatologist has advised me to knit as much as possible; what a hardship! Other symptoms have improved and I have transitioned onto a battery of drugs that have good track records for improving the quality of life and increasing survival rates in patients with my condition.

So, what have I learned this year? Here is my reflective collection of observations as I look back from the one-year viewpoint:

People are more important then things. Period.
..but they just don’t get chronic conditions and they tend to underestimate the seriousness of conditions that they can’t directly observe. In general people on crutches or using oxygen activate concern, but if you have flaming gastritis, the dizzy wobblies, and your muscles are on strike it’s easy for them to adopt a dismissive attitude. <sigh>
Medical personnel can be appallingly insensitive. Last week the technician doing my lung scan mentioned that I had a lot of doctors because my disease was really rare; it was important that the doctors get to learn as much from me as possible. Good lord, I wondered. Maybe she was raised by wolves…
As a corollary of the above observation, I have also discovered that I can’t assume that the medical personnel that I come in contact with actually know about my condition. One nurse thought that “systemic sclerosis” was “multiple sclerosis”. A doctor gave me a pep talk about how other conditions were worse because some of those patients actually can die from their disease. (“Did you want to hit him?” asked my rheumatologist…) One thing I can count on, however, is that they will put me into some scary machine to look for a possible blood clot. What is up with all the blood clot hunts?
I do have some wonderful doctors! Because of crazy interactions with random medical people I have decided that it is best to be proactive: last month I put this tag onto my keys and the little card in my wallet has the business cards of my internist and rheumatologist stapled to it.
There are few things as empowering as discovering that you won’t live forever. Why sweat the small stuff when you are facing down the long odds? My drugs have a small chance of a fatal brain infection? Whatever. Hand them over, Sparky, I am on board! I never worry about money (bad when I’m in a yarn store) or running out of gas, and I am taking more risks than I was comfortable with before. I mean, what is the worst thing that can happen? This week I figured out how to identify all of the electrical circuits of the house and rewired and installed a new doorbell. I didn’t worry (more than once) about getting knocked on my butt by an electrical mishap. Ha! In my younger days I hated to even change light bulbs…
Elizabeth Zimmerman was right. “Knit on with confidence and hope, through all crises.” is a motto that I can live with.
No, I won’t be skydiving! I’m not that risk-seeking. 🙂
It is so much easier to be happy than sad. Plant flowers. Read books. Knit like the wind! Chase bees. Go to lunch with friends (and get dessert!). Watch meteor showers and be sure to catch the Super Blood Moon. Talk to strangers in bookstores and coffee shops. Be kind whenever you can. Never miss an opportunity to take a picture of a great sunset (or a cat). It is really, really easy to be happy, even when you’re in a crazy machine that is looking for blood clots. 🙂

I finally went to my first scleroderma support group meeting a couple of months ago. I’d never met another person with scleroderma; it was a little extreme but good. Like me, they all suffer from cold hands. Unlike me, most of them also talked about the struggle to manage pain in their hands, and I could see that several of them had limited use of their hands because the skin was so tight. Remember all of those fingerless mitts I knitted during the sad times? I think that I have found a home for them.