Diastolic Dysfunction – Yarn, Books & Roses

The Scleroderma Chronicles: This Drug isn’t Fairy Dust…

Oh, boy. I have been having adventures in my ongoing dice game with the Reaper. My (wonderful) pulmonologist started me on a high-risk drug towards the end of August to slow down the formation of scar tissue in my lungs due to my interstitial lung disease. Oh, boy. The side effects were not exactly great as I battled ongoing GI side effects and started to lose weight again.

My doctors get kind of worked up when I lose weight.

I know, like this is a bad thing? Here’s their thought process: there is a correlation between weight loss and poor prognosis, so my docs tend to focus on the one factor that I can control. Ugh. I sadly moved onto a diet of chia seed puddings, rice, and bananas while gulping down fiber tablets. After a week the tide seemed to turn, and I was eating more.

This is the new drug, just recently approved for the direct treatment of scleroderma-associated interstitial lung disease. Yay Ofev!

Ofev entered the scene just recently. I first heard about it in the early years of my scleroderma journey, and it was approved for use about three years ago. It took a few weeks for me to get enrolled to receive this drug and I need to go for mandatory blood testing each month before I can get my next month’s supply.

There is a significant risk of blood clots, and the pharmacist really stressed that I should watch for bruising.

A few days after my appetite returned, I woke up with a sore and swollen leg; I was also bleeding from both nostrils. When I looked at my leg there was a large bruise that grew to be about 6 inches across… not good, little BLZ, not good!! Yep. There was an immediate full stop on the drug for a week, and then I was started at a half dose a week later. A week after I had restarted Ofev my pulmonologist called to check up on me. No bleeding, but I was dealing with stomach discomfort.

He gave me the Fairy Dust talk. “There is no magic cure, and this drug is not fairy dust.” he said, “Drug companies can manipulate data to make drugs look better than they really are; I can’t stand for you to be miserable on this drug. There is a case to be made for focusing on quality of life as opposed to quantity of life.” (Well, that’s not discouraging at all, right?!) As he and I talked I remembered my conversation with the pharmacist; she had stressed how important it was to take the pills exactly 12 hours apart to maintain a steady blood level… I was taking the high dose only once a day at that point, so I was probably experiencing a spike every day in my bloodstream. I asked for a script for the lower dose and convinced him that I should try that for a month even though the total amount each day would be more. Over the last few weeks, I’ve had a little bleeding and a couple of little bruises, but nothing like I saw on the higher dose.

I moved to the lower dose twice a day three weeks ago. While that was going on my roses started blooming again.

A week ago, I went for more lung and heart testing. I bought green chili cheese fries and a chocolate shake on my way home because… my GI tract has decided that it loves me and I’m hungry again! Besides… green chile cheese fries!!! The results came in last Friday. My oxygen is better, and I can walk farther than a year ago! My pulmonologist doesn’t need to see me for 6 months. My heart testing and bloodwork was used by my cardiologist in a predictive model that returned a result of… low risk of pulmonary hypertension progression at this time. My heart failure numbers did double, but he isn’t all that concerned; he doesn’t need to see me for another 6 months.

I hung up the phone after the last call and thought to myself… maybe Ofev is Fairy Dust after all, because… I. Am. Better. This has been 2 months of NOT FUN, but the proof is in the pudding.

By the way, chia seed pudding, highly recommended by my pulmonologist, is just plain nasty! Chia seeds, without any doubt, are not fairy dust!!!

To be fair, it probably isn’t all Ofev; after all, it has been pointed out to me that it is not a magic cure. I started other drugs to control my pulmonary hypertension over the last year. I have made a lot of adjustments on my end to handle my lung disease. All of my down products are out of the house. I bought a new humidifier that can be easily cleaned each week. I put a high-grade filter into the furnace and bought an air purifier. I take all my drugs right on schedule and I do gulp down fiber-rich foods that are keeping any symptoms under control (except chia pudding… see above).

Okay, Reaper. You won a couple of the tosses, but this one is totally mine.

Pass the Fairy Dust and roll the dice.

This BLZ is ready to play!

The Scleroderma Chronicles: Rolling Dice with the Reaper

I’ve been collecting new diagnoses over the last year like an out-of-control yarnaholic shopper at a fiber festival. Yeah, that bad. That’s how my yarn stash got so out of control… I mean, you never know when you will need that fabulous color in the future, it is one-of-a-kind, and it’s cashmere… Anyway, let’s get back to the topic at hand. The last year has been really eventful, and the new tests, diagnoses, and drug interventions keep rolling in. Here’s the chronicle of events:

An early summer echocardiogram last year showed that I had developed a type of heart failure called diastolic dysfunction. Scleroderma is causing scar tissue to form in the muscle of my heart.
On September 1st I had a right heart catherization that showed that I had a hole in my heart that was disrupting the flow of oxygenated blood to my body. Oh. That explains those blue lips I’d been sporting around town.

Follow-up testing showed that I had exercise-induced pulmonary arterial hypertension. I was started on drugs to treat the condition.
I went into a flare of my systemic sclerosis (scleroderma) symptoms because of the increased blood flow due to the drugs.
Testing in February to hunt for the cardiac shunt (hole in my heart) revealed that there was something going on with my lungs. A subsequent CT scan in March showed that I had developed autoimmune pneumonia in both lungs, a condition called interstitial lung disease. The cause of the ILD was confirmed by a lung biopsy in May. Yep. It is scleroderma related, but I was still forced to get rid of all my down products in the house. I miss my down comforter…
All summer long I have gone through a staggered course of drug interventions as my doctors worked to get my lung disease under control.

This sunflower is a pretty good metaphor for how I’m doing at the moment. I’ve taken a beating, but I’m still blooming, by golly!

So, I’ve been taking a lot of drugs, and all of them carry some side effects. I became dizzy from one, my blood sugar zoomed up, and my vision became foggy. Another is causing hot flashes. One of them causes muscle pain, and a couple of them are seriously interfering with my sleep. Two drugs cause edema, so I’m taking another drug to combat that. Two weeks ago, I started the last drug on the list, Ofev, which has a list of side effects that made me pause a few days before taking the first pill.

Nausea, diarrhea, vomiting… well, okay. That’s stuff that I deal with all the time because of scleroderma…

Liver damage, heart attack, stroke… say, what? Seriously?

I had to go through a 30-minute phone interview with a pharmacist before the first 30-day shipment of the drug could be sent to me. I will need to get blood work done every single month to check my kidneys and liver before I can receive the next 30-day shipment. I need to be vigilant about watching for bruising and the symptoms of blood clots, including heart attack and stroke symptoms. One of my sons is checking in on me daily.

Time for a baby bunny picture, don’t you think? One of the best parts of the summer. 🙂

So, here’s the deal. I’m in a lot of online support groups where people are afraid to take drugs to treat their systemic sclerosis (scleroderma) because of the side effects. We live in a world where people are afraid to get vaccinated because of possible side effects. We live in a world where people are resistant to wearing a mask because of… well, they will tell you. They have more reasons than I have time to list, quite frankly.

This is kind of nuts, in my opinion. Did you see that list of diagnosed conditions? Yikes. Not good, little BLZ, not good. I’m facing down some really serious stuff here, and I will take these drugs, get through the side effects, and fight this like the hail-battered and grasshopper-savaged sunflower that I am!

Two weeks ago, I gulped down the first Ofev capsule. All those nasty GI symptoms arrived and had their way with me for several days, and then I was through it. I’m now on the final doses of all my drugs, and the sleep disruption is improving, the dizziness is gone, and the muscle pain is receding. I’ve learned to never, ever drink elderberry juice, and that green chili actually improves GI inflammation. I can do this, yes, I can!!

My niece referred to my battles with my disease and the drugs that I take as “rolling dice with the reaper”. I guess that is a one way to look at it. The other way is… you have to play to win. I chose to take the drugs; I chose to play. Hand over those dice, reaper!!

Last week I went to get my first follow-up CT scan of my lungs. “You can take off your mask, if you’d like,” said the technician. Nope! No one with a list of diagnosed conditions like mine should take off their mask in a diagnostic facility attached to an urgent care center. I roll more dice than I should as it is! Grumpy, the technician did my CT scan while I kept my mask on.

The message from my pulmonologist came last night. My lungs show improvement, and there is no new fibrosis.

Take that, reaper! I win this roll!!

The Scleroderma Chronicles: World Scleroderma Day, 2022

Wow, it is that day of the year again. The Niagara Falls will turn teal, Scleroderma organizations around the world are sending out messages and videos, and patients with scleroderma like me are wondering how best to showcase our conditions in a meaningful way. Here in the US the theme is Know Scleroderma. In Australia it is Shine Like a Sunflower.

The whole idea is to educate the public about this rare disease that pretty much flies under the radar to help secure support for patients, funding for research, and awareness of treatment options. Patients are encouraged to tell their stories and to do what they can to expand scleroderma awareness in the public eye.

Well, shoot. I do that all the time! I wrote about World Scleroderma Day last year and I kind of like what I wrote. I talked about what was going on in my illness and the progress that I was making in getting diagnosed and treated for the significant organ damage that was underway in my lungs and heart. I also mentioned the similarities between Covid-19 and systemic sclerosis (the type of scleroderma that I have), and the fact that people like me are still dealing with lockdown. You can read that post here.

So, what has changed in the last year and why am I typing away on my computer once again about World Scleroderma Day? Well… awareness and support are the messages that I’ve been urged to put out, but I’ve been reflecting on what I’ve learned this year and how it might be useful to others. This was a huge year for me… I was diagnosed with pulmonary arterial hypertension, a complication of systemic sclerosis that is developed by about 15% of patients, and I was also diagnosed with interstitial lung disease, another complication of systemic sclerosis that is also developed by about 15% of patients. These serious complications develop so routinely around the 15% mark that there is now a rule of thumb about it in treating patients with systemic sclerosis. There are other conditions that fall into the 15% rule, and I have two more of them: Sjogren’s Disease and diastolic dysfunction.

MacKenzie and I from a posting a few years ago.

So, I am getting a lot of experience in dealing with being sick in a way that is not visible to the public and is not the first thing considered when you head into a doctor’s office seeking help for debilitating symptoms that have no obvious cause. I have been successful this year in becoming an active participant in my own health care and I love my team! Here are my lessons learned:

Physicians tend to diagnose with the most common condition that matches your symptoms. You know, if you are struggling with fatigue, it must be depression or sleep apnea…
They also tell you to stay off the internet.
That works great up to a point. Get onto the internet!!!! Look up the symptoms and treatment options for the condition/illness that your physician is talking about. Do they really fit? Go ahead with the testing that your doctor orders but continue to educate yourself. Get the full text of any testing reports (those are your tests on your body, so do insist… nicely…) Look up crazy words that you don’t understand.
I should include here…DON’T PANIC… about any crazy-ass, scary condition that you run into on the internet that you think you might have. I mean, what could happen? You already are sick, and you won’t get magically worse overnight once you get a name for it. You might, however, get some really helpful treatment that could turn things around for you. That is, if you have that crazy-ass, scary condition that you really, probably, don’t have. DON’T PANIC!!
What if you get test results that say “you are fine” and your doctor pretty much is ready to stop there? Um… go right back to specific test results and symptoms to reset the conversation. You know, “my face is still blue, and the latest CT scan showed that I was losing tissue in my lungs. What other testing can we do to figure out what is going on?” is exactly where you should redirect the conversation.
Make a list of your symptoms and track them in a journal or on a calendar. Document stuff and then contact your health care provider (email works great!) with your concerns and the symptoms that you are noticing. Specific data helps a lot, and the written record makes you more credible and your health provider more accountable.
Do not let a medical health professional dismiss or disparage you. Kick them to the curb and get another. On the other hand, don’t go doctor shopping to get the diagnosis that you want; that is not productive for you or anyone else involved in your daily struggles.
Ask your physicians to communicate with each other and make sure that they include your primary care physician in any messaging.
Remember to be kind to others: your doctors, the nurses, your family, your friends, and yourself.
DON’T PANIC!!! at any time. Remember, feeling powerless and not knowing what is happening is stressful. Learn everything that you can, do everything that you can, and then sleep well at night. Hugs to anyone who finds this meaningful.

There. Those are the best, most excellent lessons that I learned this year. Today I am out of the serious flare of the winter and feeling pretty darn great. I am on steroids, and my immunosuppressant drug’s dose has been doubled; I feel more like myself than I have in a couple of years. I headed out on errands this afternoon, bought a Starbucks, and signed up for a Tour de Fleece team at my local yarn shop. I bought some new lavender plants that I am going to put into the ground this evening and I am cleaning up the spinning wheel to see if I can get some paco-vicuna spun next month before my steroids get stopped.

I have some really serious conditions that carry a significant risk of a poor outcome. And yet, I feel a little like an imposter as I laugh and interact with other people that I encounter. The man at the drive-through window at Starbucks traded cat photos with me. The lady at the yarn store and I laughed and talked about spinning wheel misbehaviors; are the wheels worse if you name them? Behind the mask, I am still me, the old me; I may have scleroderma, but it doesn’t have me. I am kind of the poster child for what an invisible illness looks like, and that’s why there is this campaign today to “Know Scleroderma.”

Those serious conditions that I mentioned… they are complications of scleroderma, but they happen for other reasons, too. Some are rare, but some are not. Knowing about scleroderma can help with research efforts into these other conditions (sadly, some are now more common because of Covid long haulers), and perhaps the lessons I have learned will help others in their efforts to secure empowerment and medical treatment.

This is World Scleroderma Day.

Hannah and the CoalBear: Caturday, 6/25/22

Hi. CoalBear here.

I’ve been entertaining the Mother of Cats all day because, frankly, she is in a slump.

Well, now that I think of it, she should be in a slump. The only day this week she was frisky was Tuesday and look at what she did to me!!

People, this was the most horrible thing that has ever happened to me. I got stuffed into the carrying crate and before I knew it, I was in the car. We ended up in a strange building with other people and DOGS and I kept crying, and the Mother of Cats just ignored me and took me to a little room where I had to COME OUT OF THE CRATE!!!!! I got weighed, physically cathandled by a strange lady, and then there were the SHOTS that I absolutely did not deserve because I am the best boy ever. You want to hear the worst thing about all of this…. Hannah exercised her right to refuse and didn’t have to go the vet. I think that she was laughing when we got back home again.

Okay, back to the week. The Mother of Cats is taking lots and lots of new pills now and parts of her don’t feel good because of the side effects (let me tell you about the side effects of those SHOTS!) so I am doing my best to be cute.

She finished her little quilt and hung it up on the wall behind her knitting chair.

The Mother of Cats used clear plastic push pins to secure the tops and corners of the quilt. I have been pulling the pins out of the bottom of the quilt and taking them upstairs to play with. I left both of them on the bathroom floor where she would be sure to find them this morning so she can put them back into the quilt again. Wasn’t that good of me?

I’ve also been trying to help her with her knitting, but she seems to be in a slump. She isn’t knitting much, and the stuff she has going is… boring.

Do you see how boring this knitting is? The pink blob is a sweater… maybe. The blue is going to be a pair of socks someday, and that pastel smish of funny colors will someday be a hat. Where are the cat toys? Hello? How can I be cute and entertaining if I don’t have all the toys in the world…

Although, I think that I am pretty darn cute with the toys I have now!!!

Well, I guess that is all for now. It is almost time for the baby bunny to come out in the back yard. Show time!

Mateo the CoalBear

Notes from the Mother of Cats:

I did get Hannah into the crate at least three times, but she was too strong for me and managed to push her way back out before I could get it zipped closed. She has another appointment next month and I’ll try some new strategies.
I’m in the middle of a big drug push to get my lung disease under control; I am breathing much, much better and I have more energy by far than I did a couple of months ago. The downside is… side effects. My doctors are running frequent blood tests to monitor my progress, adding more drugs to control symptoms that are concerning, and so far, things are going well, but I am pretty much homebound with pain, blurred vision, and dizziness. Two more weeks of the steroid push to go and then I start to get tapered off. Yay! I’m dealing with a lot of tendon pain as my immunosuppressant dose is doubled, but that has happened before and I’m hopeful that there is an end in sight.
What type of side effects, you ask? I gained 12 pounds in two days and my blood sugar soared into the high 100s. Opps.
I am getting out into the garden a little and there is a new rose bush waiting for me to plant it. Someday soon, little guy.

The pictures above are my morning pills, the braces that I now am back in because of tendon pain, and my new, beautiful rose. The name of the rose is… Easy Does It!

Just the rose that I needed for the week.

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…

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: 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.

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!

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.

Happy Caturday everyone!!

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?

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: 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?!!!

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!!