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.

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: Rare Disease Day, 2022

Well, this is a hard topic to write about. Quite frankly, I have been getting my butt kicked lately by my (wait for it) rare diseases. Still, I am trying to respond to the calls for publicity about rare diseases along with other members of the scleroderma and pulmonary hypertension communities.

You know, I feel like I should represent.

People with rare diseases are referred to as “zebras” in the medical community.

So, what’s a rare disease? A rare disease is classified as one that impacts a small percentage of the total population. Here in the United States that means fewer than 200,000 people diagnosed with the condition/disease. Perversely, there are a lot of people with rare diseases as there are almost 7,000 different rare diseases!  Some of these diseases are common enough that you may be familiar with them: albinism, achondroplasia (a type of dwarfism), and autoimmune hepatitis are examples. Others are very rare. Most are genetic in origin, and half of them impact children. More than 90% of rare conditions have no drug treatment.

The type of scleroderma that I have, limited systemic sclerosis, is considered rare as there are about 100,000 people in the US with this diagnosis. The latest diagnosis added to my medical history is of pulmonary arterial hypertension, another rare disease, and one that is a consequence of my scleroderma. Well, I am really rare now! I have struggled to explain my scleroderma to people when they ask; how can I explain in just a few sentences something that is just frankly causing horrific damage to my body and generating an ever-growing list of diagnosed conditions. Here’s my best answer at the moment:

Scleroderma is a chronic, progressive, uncurable, and often fatal autoimmune disease that causes scaring and damage to blood vessels, skin, internal organs, and muscles/joints. It is controlled and treated through the use of immunosuppressants and drugs that address symptoms. It is a life-altering diagnosis. It is my life.

So, I have blogged about Rare Disease Day several times in the past. Here’s what I wrote a couple of years ago, and what I wrote in 2018. In the past I have written about my symptoms and the struggle of living with a rare disease. It is pretty isolating. It is hard to get diagnosed and treated. I have also written about the difficulties to get funding for research for rare diseases and conditions, and the lack of treatments and cures because the patient population is so small.

This year I thought I would share some of the things that doctors have said to me since my scleroderma diagnosis. I’ve tried to organize these into chronological order to better reflect my journey.

  • My internist: It’s good to have a diagnosis, even if it is a shame.
  • My rheumatologist when I asked him what my life would be like in 5 years: Let me run some more tests, and then we can talk.
  • My ophthalmologist: Do you have a will?
  • The physician at the regional acute diagnostic center: This is a diagnosis like cancer. Of course, some cancers can be cured.
  • A physician speaker at a scleroderma support group presentation: this drug [the immunosuppressant that I take] can really give you a chance, as long as you don’t contract an infection.
  • My old rheumatologist: All you do is complain. Maybe I should order a sleep apnea test or prescribe antidepressants.
  • My internist, as I begged for an anti-inflammatory drug: I’m sorry. There isn’t anything that I can give you that won’t hurt your kidneys.
  • My dermatologist, as she prescribed an anti-inflammatory topical gel: This is unacceptable, and I am putting a stop to it now.
  • My new rheumatologist as she orders more testing on my painful joints: Why has no one followed up on this?
  • My rheumatologist two days later: You need to get a steroid injection in your hip joint as soon as possible.
  • The hip specialist: There is nothing more that I can do for you because your scleroderma is attacking all of your tendons and ligaments. You need a hip replacement, but it will fail.
  • The physician at urgent care: I don’t think they understood how complicated your medical status is when you were referred here. You need to be hospitalized because we can’t do the testing that you need here.
  • My pulmonologist as he walked me back to the waiting room: I really admire your attitude.
  • My cardiologist as I was being sedated for a right heart cath: Don’t worry. I’ll take good care of you.
  • My cardiologist as he started me on medication for pulmonary arterial hypertension: This is challenging, and we will need to be comfortable with “out of the box” thinking.
  • My rheumatologist last week, referring to herself, the cardiologist and the pulmonologist: We are your team!
  • My rheumatologist, also last week: We need to add a gastroenterologist to the team.

You can see how rocky the start was. There is a lesson here, I think. To be rare, to be a zebra in a medical community that is designed to identify the most likely cause of symptoms in a herd of horses, is hard. It is really challenging to secure the care that you need when, no matter how hard doctors try, you do not respond to the usual treatments, and you never fit the usual profile. It is easy to be seen as a problem. It is hard to keep insisting that there is something wrong when all the test results say you are okay.

Even when you are blue-lipped and panting it can be hard to convince doctors that there is a problem.

And yet, it is possible to get there. Over time, with great determination and persistence, I have A TEAM of doctors who view themselves as active collaborators in my care. They message each other to discuss test results and possible drug interactions, and they loop me into their discussions. It is only now, newly diagnosed with a terminal condition, that I feel confident and hopeful about my care.

Today I went in for a blood draw and a little jaunt through the local bookstore. The sun was shining, I bought a Starbucks coffee, and it was a good day.

****************************************

My scleroderma-related diagnoses:

  • GI tract: difficulty swallowing, hiatal hernia, GERD, gastroparesis, chronic gastritis.
  • Kidney: stage 3 chronic kidney disease.
  • Lungs: pulmonary arterial hypertension, interstitial lung disease, asthma, partial lung collapse.
  • Heart and circulatory system: grade 2 diastolic dysfunction (a type of heart failure), Raynaud’s phenomenon, telangiectasia.
  • Muscle/Skeletal: fibromyalgia and severe joint damage.

This is scleroderma. I’m a zebra, and these are my stripes.

Happy Rare Disease Day, everyone!

The colors associated with my diseases are teal (scleroderma), purple (Sjogren’s), and periwinkle (PAH). It sounds like the start of a great sweater, huh. 🙂

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.

Then the doctor came in!

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 a long time coming!!

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!

Hannah and the CoalBear: Mateo does Caturday.

Hi. I’m Mateo (AKA the CoalBear).

I’m almost 10 months old now. What do you think of my ruff?

The Mother of Cats isn’t feeling well today so I am hanging around with her on her bed. I’m helping her write on her computer right now! I’m such a good boy!

I’ve been bringing toys onto the bed so she can watch me play with them.
I chewed on the palm plant downstairs to keep up my energy.
I then helped the Mother of Cats with her knitting. I’m such a great helper!!
I groomed my sister so she would let me sleep on the cat tree with her.
I’m the best CoalBear ever!!

Happy Caturday everyone!!

May you all have an excellent day!

Notes from the Mother of Cats:

I’ve gone off my immunosuppressant drug so that I can get my Covid booster on Monday. Sigh. There was a big pressure change yesterday with a cold front that triggered some symptoms along with the predictable flare of my disease that has me back in bed for the weekend.

I’ve been in lockdown for two years and I desperately want the booster because my next round of medical appointments starts in another week. I also want to start knitting with my new-found groups, too. I want some of my life back!!

Please think of me and the other people in a situation like my own (high risk, immunosuppressed or immunocompromised, and struggling with chronic conditions that complicates their lives on the best of days) when you are out in public.

And wear your mask!

ps: I threw out the palm last night. It wasn’t a match for the CoalBear and I was afraid it would make him sick. Next up: a Boston fern.

Goodbye 2021: Year’s End

This is the last update for the year 2021. You know, 2020 was a pretty bad year for both me and the world, but 2021 just plain outdid itself. THIS HAS BEEN A HORRIBLE YEAR!!!! Seriously, I have been reflecting on all the horribleness of the year, and it is multilayered in the just plain awfulness of it. Here is some of the angst, anger, and sadness in a nutshell.

If you aren’t wearing a mask these days, shame on you!! I have been in lockdown for two stinking years (!) and I am over all your anti-public health nonsense.

If you are one of the doctors who told me that there wasn’t anything that you could do for me (and to not come back), or that I had sleep apnea, or that I needed to exercise more, or that my tests showed that I was fine and you would just continue to monitor my symptoms… shame on you!!! I finally received credible diagnoses this fall (from new doctors) that explained my symptoms. You know, the ones that you ignored or dismissed all those times I came to get help… It is good to get diagnosed, but in this case it is also a mixed blessing as my condition cannot be reversed and management is going to be difficult. Shame. On. You!!!

If you are one of the people who believe that the election in the US was stolen and that the answer is to impede the ability of American citizens to vote, or to create a provision that allows the legislatures of some states to just overturn the results of elections, or to resort to violence… shame on you!!!!

Did you notice that the number of exclamation marks kept growing? That’s how much shame is attached to those targets. There, I got that off my chest and let’s hope that things go a little better in the coming year.

True to its rotten black heart 2021 went out in absolutely dreadful and ironic fashion. First the dreadful.

We are in an extreme drought where I live and everything is brown. The grass, the fields, the bushes and even some of the evergreen trees are now brown.

Last Thursday, December 30th, we had a high wind event in my state of Colorado as a major weather front pushed its way towards us over the Rocky Mountains. In the area around Boulder, Colorado the gusts were extreme (one was clocked at 115 mph) and the sustained winds through the day were around 75 mph. Several fires started and swept through dried fields and brush towards housing developments and towns with horrifying speed. There was nothing that could be done as subdivisions, stores, and hospitals were evacuated; the fire crews set up base in the parking lot of a mall where they could best defend themselves while waiting for a break in the wind. No joy there throughout the afternoon and early evening. There were heartbreaking scenes of burning homes by others covered in Christmas lights. People parked along the major freeway hoping to see if their home was still standing. The historic downtown area of a town I have frequented in the past was lost.

At around 3pm I realized that the worst fire was near my son’s home in northern Westminster. As the evacuation zone continued to grow to within a mile of him we started to make plans to get him and his pets out safely. It was a nightmare as firetruck after firetruck rushed north towards the fire line in the night past his windows. By midnight the winds had died down, the fire stopped its spread to the south, and my son was safe. Daylight the next day showed that the damage was just horrific. Almost 1,000 homes have been lost, thousands are displaced, and the hunt for the missing is ongoing. There is information about all of this here.

Then the storm arrived on New Year’s Eve.

In a cruel twist of irony, after failing to deliver any real snow all fall and early winter, the winter storm arrived New Year’s Eve with snow, icy roads, and bitter cold. Like, we started the day at 3 degrees Fahrenheit yesterday. Serious, serious cold after weeks of warm sunny weather. All those poor people who escaped with only the clothes on their backs in the wind/fire event now have to deal with this. Bad 2021, bad!!

And that, thankfully, was the end of the year.

I also finished my Kevat sweater in the final days of the year.

It is wet and blocking in this photo, but not finished. See the loose ends of yarn?

I did finish the sweater with the ribbing and I-cord to make nice open and clean edges. I decided to block and try on the sweater again before knitting on a little lace edging onto the bottom; if the length is exactly right there won’t be any lace added. I did do a little math, however, and I do have exactly the right number of stitches to do the lace. It’s a sign, right?

Mateo: Happy New Year

Hannah and Mateo (AKA the Coalbear) and I all wish you all a Happy New Year. May things take a turn for the better with the coming weeks and months.

And 2022, you had better behave yourself or I’ll be sending you out on a walk of shame, too. I do have to warn you, however, that I am a little concerned with how you are starting out…

Because this year we are going to learn how to cope with a million new Covid cases a day in the US, and I am going to deal with this whole broken heart thing that I have going on, and we are going to f*cking save democracy. Shape up quick 2022, because this is the tough time, and you had better show some backbone, because I expect you to fight like you really mean it for the things that are really important.

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.

Hannah: I should have a crown!!

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: The BLZ gets a CPET

The BLZ had some anxiety going into this test…

Last month I had a right heart catherization that showed an unexpected problem with my heart… there is a cardiac shunt that is allowing blood from the left side (you know, the one that is always colored red because that blood is rich with oxygen) to shoot over into the other side of the heart where it disrupts blood flow and adds pressure to the (blue) right side of the heart; that is a little tough on the right side because it isn’t built to handle the extra pressure. Houston, we have an explanation for all that blue panting that is going on.

Cool. Finally we were getting some answers and my doctors were taking my symptoms more seriously. My cardiologist ordered up some more tests to collect data and clarify the issue.

The test that I have been the most concerned about was the cardiopulmonary exercise test that was scheduled for last Monday. I hunted for some cool links to explain the test to use in this post and came up with a bunch of technical articles for physicians and specialists. Okay. Let’s not go there. I’ll try to explain what this test was all about. I was hooked up to a bunch of monitoring equipment that tracked my breathing and heart while I was riding a bike. As part of the test my lung function was tested, my heart monitored (like in an EKG), my blood pressure was tracked, my respiration rate, and the actual amount of oxygen I was using and the carbon dioxide that I was expelling for each mL of blood pumped. It was a lot!!

I was a little concerned because of my bad boy hip, but that wasn’t really a problem at all.

I shared my catnip with the Mother of Cats so that she wouldn’t have any trouble with the test.

The test went great for about 7 minutes: then there was an emergency stop. My blood pressure had suddenly zoomed up into the stratosphere at about 6 minutes into the test, and then 30 seconds later I began panting like crazy and the technician pulled the plug. Remember that cardiac shunt they found in the cath lab? When I exercise the direction of blood flow in that shunt reverses and blood from the right side is shooting into the left side where it interferes with the pumping of oxygenated blood to my body. No wonder I turn blue and pant.

The BLZ is both happy and sad at the same time.

The specialist who did my CPET was great at explaining the data to me; when he sent the report to my doctors he also included me in the email group. For the last week I have been reading the back and forth discussion by my doctors about the test and what the next steps for me will be. I am really pleased to be included in this process and feel that this option should be available to everyone; it is also kind of scary as I get a glimpse of what is coming down the road for me later in the testing department.

The gear and set-up for the CPET was pretty impressive. I joked with the specialist about it and he told me that there was an even more extreme version of testing that included a right heart catherization at the same time as the exercise test. Yep. You guessed it. That’s what my docs are considering to do next. It is clear that I have a cardiac shunt but they haven’t located it yet. The BLZ is pretty bummed. The word “profound” was used to describe my symptoms. There has also been some speculation about neuroendocrine tumors… The good news here is that no one is even considering sleep apnea or me needing antidepressants because, you know, I complain too much…

The BLZ is both happy and sad at the same time.

I keep my eye on the Mother of Cats while she reads her email.

So, it was a kind of tough week. I pulled myself together on Tuesday and went to a new yarn store for a little pick-me-up and was so distracted I missed my turn twice and had to detour through Starbucks before I actually pulled up in front of the store. I almost didn’t go in I was so worn out by then, but I did a little mental slapping to put myself into motion, pulled out my cane, and went in…

…to discover a woman dragging out three big bags of yarn that kind of screamed “community knitting” because they weren’t the yarn being sold in the store. “Hey! I want to community knit! Please, can I come play with you guys?” I immediately said.

That is how I ended up in a new local yarn store this morning knitting with the most wonderful group of ladies in the world. They are all current or former employees of the Kaiser medical system that I go to for my care, and they are producing hats for all of the Kaiser clinics that have infusion centers; I know those centers because they are the same ones that rheumatology patients go to. These ladies are all vaccinated and they all wore masks because they knew I was taking a risk to come. They had donuts! I have found a new knitting home when I needed it like no other. I actually cried a little with joy and relief as I drove away at the end of the meeting.

If I hadn’t made all those bad turns and the Starbucks stop this wouldn’t have happened.

My yarn stash is full of yarn that wants to turn into hats. Hats with happy colors and a dash of cashmere. I am on fire with purpose to produce as many hats as I can for other people who are facing down serious medical situations. I want to make them arm warmers and fingerless mitts. I have tapped into the best, most perfect group to produce useful gifts for other people like myself just when I needed to be grounded, inspired, and calmed by the peace of knitting.

The BLZ is happy.

The Scleroderma Chronicles: A Trip to the Cath Lab

The BLZ has been waiting a long time for this…

Five years ago I came down with the flu and ended up in Urgent Care struggling to breathe. I scored some antibiotics, steroids, and cough medicine. To fight the virus I was told to go off my immunosuppressant drugs for a few weeks until I got better, stay in bed, and load up on chicken soup: it took a couple of months but eventually I got back on my meds. Except… things weren’t quite right. I panted for air every time I came up the stairs and I noticed that my lips were turning blue. My blood pressure was too low and I had to stop taking my hypertension medicine. I felt dizzy and light headed sometimes.

I was a newly diagnosed systemic sclerosis patient and my doctors began running tests to see if my disease was impacting my lungs. Nope. Not my lungs. Tests were run to see if scleroderma was attacking my heart. Nope: my heart seemed to be normal. There were some anomalies, but my doctors decided to just monitor me through routine testing and see if things changed down the road. My red blood cell counts were way too high, and I had nocturnal hypoxia, so I was started on overnight oxygen. I was tested for various conditions that could account for the weird test results, but I always had a normal result.

I struggled on, battling for more testing, as my doctors kept reassuring me that I was okay. Hey, I had a blue face and panted for air when I climbed stairs; vacuuming could put me on the floor. How could this be “normal”? Ugh. Welcome to scleroderma, I thought.

I began to think of myself as the Blue-Lipped Zebra (BLZ). If I didn’t have a rare disease confusing the issue I would be getting better health care, it seemed. If doctors didn’t tend to apply most-common-cause thinking to my condition they might get to the bottom of things faster. It was, in my mind, a huge complicated mess as my doctors applied best practice (and rigid) diagnostic parameters to my symptoms or zeroed in on specific complications of my scleroderma and ignored other possible (and to be fair, rare) causes for my symptoms. I worried that my doctors had just parked me in a holding pattern as I slowly got worse; it is hard to advocate for yourself when you are sick and dependent on your doctors for help, even if you think that they are dismissive and borderline disparaging.

I struggled on as my face became more blue, my red blood cell count higher, my panting for air more common, and the occasional near-fainting event left me collapsed on the floor. I began to ask for a right heart catherization procedure to directly measure the pressure in the right side of my heart. “Oh. You don’t want that,” I was told. “That is really invasive testing.” Umm… I think I do, I would reply. Nope. Nope, nope, nope!! “Not even on the table,” one pulmonologist said.

During lockdown last year I got much worse; ironically lockdown also gave me the opportunity to reboot, fire my old doctors and acquire new ones. My new team of doctors this spring ordered up testing that showed definite issues with my heart and lungs. I am now a heart failure patient (the wall of my left ventricle are too stiff and scarred to beat well) and there were concerning findings that suggested that I had developed pulmonary arterial hypertension (PAH): there are areas of cell death in my lungs and my pulmonary artery is too big. There is too much fluid around my heart, a suggestion of ongoing inflammation. I have a hole in my heart between the atriums (a cardiac shunt) that is impacting blood flow. I was gently prepared for the PAH diagnosis, assured that there were great drugs that could help me, and a right heart catherization was ordered by my new cardiologist.

Yay!! About time!!!

Finally, after 5 years of struggle, I was yesterday wheeled into a procedure room to a waiting team of specialists who hooked me up to equipment and took me though testing to get a better look at my heart: I learned in recovery that this team calls themselves “the pit crew”, and that is exactly what it was like. Within 5 minutes I had completed a breathing test, was on oxygen, wired up to a heart monitor, hooked up to an IV, medicated, prepared with surgical drapes, and swathed in warm blankets with a heater by my feet. There was music playing and the crew was cracking jokes as they darted in and out from the table getting me ready. I was knocked out for the echocardiogram imaging of the back of my heart that was done using a probe in my esophagus, but they woke me up for the main event: the right heart catherization. My cardiologist inserted a probe into the carotid vein in my neck and threaded it into my heart by way of the superior vena cava (blue side of the heart diagram above). People, this was the most amazing experience ever! There was a huge screen showing the progress of the probe and I could watch and ask questions as the line snaked through my heart; there was absolutely no pain. “Well, this is interesting,” said my cardiologist at one point, and there were more measurements happening and a flurry of new activity from the team. The BLZ felt a surge of elation: they had found something, and it was NOT what they expected.

Back in recovery my cardiologist caught up with me again. I had done great, he said, and he just beamed as he told me that I absolutely did not have PAH. This is great news, he assured me, great news!! There is another circulation problem in the lower part of my heart, between the ventricles. There is blood coming in from the left side of my heart and mixing with the blood on the right, disrupting the flow through the heart and robbing me of oxygen to my body. I have a second, more serious, cardiac shunt, and now it is a question of locating that pesky little guy and doing something to fix the problem.

It has been FIVE YEARS, people. If I hadn’t had my trip into the cath lab yesterday my doctors would still be nagging me to get more exercise (the BLZ just barks in laughter), offering me antidepressants, or insisting that I must have sleep apnea. I feel so validated!

My cardiologist is now going over my previous imaging to find the hole now that he knows what to look for. I was told that I may need to go through more testing to definitively characterize the opening, but this is huge forward progress. I suspect that I am facing open heart surgery down the road, but I am elated that the progressive and eventually fatal diagnosis of PAH is now off the table. Things are looking up since my heart failure will now be much easier to treat.

I am reminded of Elizabeth Zimmerman’s admonishment: “Knit on with confidence and hope, through all crisis.” Also, when the going gets tough, get a kitten!

Today I am waiting to hear back from my cardiologist who is going to email me with follow up instructions after he has finished going through the data and past test results. I’m on oxygen, knitting, and feeling pretty calm about all the new developments.

Almost exactly 7 years ago (August 28th was the anniversary day) I was diagnosed with Limited Systemic Sclerosis and Sjogren’s Disease. I have learned a lot along the way, but the best, most important lessons have been about self-advocacy, facing down the worst case scenarios, communicating with your doctors, and maintaining a good attitude.

Yesterday this all paid off for me big time.

Note: The fabulous BLZ graphic was made for me by my exceptionally knitworthy niece Melissa and her beautiful and talented daughter Eleanor.

The Scleroderma Chronicles: Matters of the Heart

The Blue-Lipped Zebra (BLZ for short) has been busy the last three months (once she was fully vaccinated for Covid-19) and lots of testing and doctor appointments have happened. Lots of diagnostic hypothesis have been pursued and tested; the BLZ has received several emails from doctors that let her know about good news: you don’t have pneumonia!! your heart looks good!! your kidneys are maintaining!!

All is good. Go visit your baby bunny and don’t worry about it…

Hello. BLUE-LIPPED Zebra. Chest hurts. Zebra is dizzy. Zebra pants as soon as she moves around. Zebra is absolutely sure that everything is not fine at all!

Did I mention that the BLZ got fully vaccinated? Ever since that happened (and the BLZ got a steroid injection for her ill-behaved hip) her fatigue and brain fog have receded into the background. Quite frankly, the BLZ is feeling pretty frisky and clear-headed these day between bouts of dizziness and panting episodes. She has decided that enough is enough and she is on the move to get to the bottom of what is going on!

She contacted her doctors and health providers and had them forward her the entire text of her test results. She read these results carefully and then spent some time consulting with Dr. Google to figure out what some of these words meant. The BLZ is so grateful for that biology degree and years of related job experiences.

The BLZ has limited systemic sclerosis. What she learned was…

  • A general rule of thumb, the 15% Rule, can be used to describe the number of systemic sclerosis patients with serious complications associated with their illness. For example, 15% of patients will have Sjogren’s Disease, or digital ulcers, or lung disease, or maybe pulmonary arterial hypertension. These complications are sometimes rare in the general public, but for systemic sclerosis patients they can be common.
  • A large European study found that the majority of systemic sclerosis related deaths were from heart complications (26%) or were pulmonary arterial hypertension (26%) related.
Time to stop and smell the roses. This is a little disappointing… my doctors have been reassuring me that all is fine because they are focused on lung disease. There are a lot of bread crumbs in the test results that suggest heart problems.

Then then BLZ made an appointment with her internist (the primary care physician) to go over the test results with her and to help her prep for her cardiologist appointment next week. Don’t you think that was smart?!!!

Mateo: Very smart!!

Here’s the summary of my appointment with my wonderful internist. My face was blue and I struggled with dizziness in her office: she entered a new diagnosis into my chart that says I’m cyanotic and told me to press the cardiologist for a prescription for day time oxygen so I can carry portable oxygen with me. (“Now we’re talking!!!” barked the BLZ.) She read the test results for my CT lung scan and echocardiogram and agreed with my understanding of what the test results were saying. She told me what tests to ask for from the cardiologist at my appointment. Here’s the summary:

  • I have physical findings in my lungs that consistent with pulmonary arterial hypertension. The summary results of that test say “mild to moderate” and even say that the loss of lung tissue and an enlarged pulmonary artery are due to PAH. Huh. Look at that. (“I’m just shocked, shocked!” snarks the BLZ).
  • The tissue of the heart (the muscle) is scarred and too stiff to beat well. This condition is called diastolic dysfunction and is a type of heart failure. The echocardiogram states that my diastolic dysfunction is Grade II, which is moderate. Scleroderma is attacking my heart; 15% of systemic sclerosis patients have diastolic disfunction. While there are lots of reasons why people develop diastolic dysfunction, for me the picture is different as it is a common complication of my systemic sclerosis and not a result of say… uncontrolled high blood pressure.
  • The estimated pulmonary pressure from the echocardiogram is difficult to measure in my case (Dr. Google had to teach me about incomplete TR jet and other obscure heart-related terms) and is most likely being undermeasured. The number now is the upper limit of normal; twice in the past it couldn’t even be estimated.
  • I have a newly developed hole in my heart called a cardiac shunt.
  • My heart is broken damaged by scleroderma.

So what should the BLZ do about all of this? The internist and the BLZ hatched a plan in which she should insist request direct measurement of the pressure in the right side of her heart (right heart catherization) and another echocardiogram that looks at that cardiac shunt while she is exercising standing up. Like, maybe the BLZ needs to be climbing stairs… The BLZ is just thrilled… Also, the BLZ wants day time oxygen-to-go. Yes, please. Right now, please.

Also, the BLZ is considering taking someone with her to the appointment and will have the cardiologist send the internist his notes following the appointment.

Also, when life get tough, get a kitten!! BLZs love kittens!

So, this is an adventure in progress, but I do have some gems to share with others struggling with their own medical misadventures. Get your own copies of your test results and physician notes after appointments. Google like crazy to learn what the obscure medical terms mean. Educate yourself about your illness/condition. Stay off social media as you do this and read journal articles from legitimate sources like the Rheumatic Disease journals and articles posted by the NIH. Ask another knowledgeable person to review your test results to help clarify/validate your thinking. If my journey here can serve as a roadmap for even one other person battling their way to a diagnosis, then this post was a success.

And remember to be brave.

It is good to have a diagnosis, even if it is a shame.

Zebras are brave!!