Heart Failure with Preserved Ejection Fraction

The Scleroderma Chronicles: Carrying Light

You know, I think that synchronicity is a real thing; you just have to pay attention to what is going on around you. Sometimes, if you take notice, the world hands you just what you need at that moment.

Scleroderma has been kicking my butt lately. Having improved dramatically over the summer and sailing through my heart/lung testing last fall, my doctors were pretty upbeat when I reported worsening symptoms while visiting them in late winter. They ordered some testing, but they were also very reassuring.

A week ago I arrived at a Kaiser facility bright and early for a routine echocardiogram and 6-minute walk test. The echocardiogram did not go well (usually they don’t hurt, and what was up with having to pause so I could pant a little to catch my breath…) and I was in the red zone (the pulse oximeter starts glowing red if your oxygen drops below 90%) after a minute of walking. The test was halted after 3 minutes, and the concerned nurse walked me out to the elevator.

Ugh. Not good, little BLZ, not good. Refusing to overreact, I went to my favorite yarn store on my way home, bought some great yarn, and then hit Starbucks by my house. It was a bright, blue day and I headed out to the deck to knit.

I took this picture of Hannah. Look at that bright, blue sky!

Do you know the quote by Elizabeth Zimmerman that goes “Knit on, with confidence and hope, through all crises.”? Yep. That is a quote to live by! I cast on a new hexagon for my blanket and started knitting. I felt myself settle inside, my breathing steadied, I began to process what had happened, and my anxiety faded away. Scleroderma is a monster, and by the time I was casting off the hexagon, I was ready to once again face it down.

This post had appeared in my knitting group on Facebook from Michelle Obama a few days before:

Well, look at that: Michelle Obama is a knitter! Yay! It looks like she also knows about the Zen of knitting and the ability it has to bring calm and purpose to a simple activity while you reflect upon and process problems large and small. I knitted every night to finish my teaching days. I knit in hospitals. I knit in meetings. I knit just about everywhere I can, and I especially knit to deal with the rolling shitshow that is my chronic illness. I went and bought Michelle’s book.

Thursday morning the results from the echocardiogram were posted and a couple of hours later my cardiologist called me. I was knitting and ready for the call.

For the first time the words stage 3 heart failure were used in the discussion with my doctor. My pulmonary pressure is higher than ever before and there is more fluid around my heart. It is not clear if my symptoms are caused by worsening pulmonary hypertension or pericarditis, but the only way to sort this out is to go back into the cath lab and directly measure the pressures with a right heart catherization. It may be both. I will need a different treatment regimen. An emergency referral was put in and tomorrow I’m heading back to the hospital for the procedure. This is what happened the last time I did this.

My pulmonologist, who works closely with my cardiologist and rheumatologist, saw me on Friday for a lung function test and office visit. My lungs are hanging in there, but my ability to diffuse oxygen into my bloodstream has dropped significantly. I told him about the upcoming trip to the cath lab, and he started checking those test results. I’m not going to lie, it is a little alarming when your doctor says, “No, no, this is not good. I am not happy with this at all.” More testing has been ordered. He emailed the other doctors on the team to start the discussion about what changes should be made with my meds.

I took this picture of my new Goldwing sweater in his office the day that I met him. If you are going to scary appointments, armor yourself in your favorite knits!

This weekend I started reading The Light We Carry and was amazed that it starts with… knitting. Serendipity strikes!! Michelle Obama began knitting at the start of the pandemic as she struggled with the lockdown: grief, isolation, loss, and everything else that happened in that time. It became an important vehicle for processing, recovery, and perspective for her. The daughter of a father with MS, she is very aware of disability and how it absolutely impacts how someone like me can view myself and the rest of the world. She talked about using tools such as the cane that her father needed to empower ourselves to deal with what comes our way. Her book appears to be a toolbox of different strategies to cope with the challenges in life.

For the first time since all this started happening last week, I cried. This book is absolutely, positively, what I needed to read right now as I pack my bag for the hospital and prepare for what is coming my way in the upcoming days and weeks. It’s like someone could see right into my heart and lit a light for me. I will carry that light along with its warmth and glow tomorrow as I join my doctor and the pit crew in the cath lab. Whatever happens, I am positive, I will glow, and my light will shine.

So, Michelle, whatever can I show off as a favorite knit? Every single item that I cast off my needles has left me with a sense of purpose, accomplishment, and fed my creative needs. Knitting helps me cope with adversity, plan my day, and work through problems. Knitting delivers calm in a time of crisis. Knitting allows me to deal with an unpredictable autoimmune disease that delivers an uncertain future. Knitting connects me to all the knitters in the past and provides gifts for others as I pay forward. It is essential for my being and a vehicle to connect with others.

Here are some things I’m really pleased with: Goldenfern, a knitted copy of a beloved (and lost) cat, baby booties gifted to a neighbor knitted from a pattern handed down through 4 generations of my family, the hats and PICC line covers that are donated to Kaiser infusion centers in my area, and Mando (and Grogu) mitts for a knitworthy niece.

Tomorrow I’m wearing arm warmers and knitted socks into the cath lab. Take that, scleroderma.

Behold, I carry my (knitted) light with me!

Did you wonder what a BLZ is? That’s me, the Blue-Lipped Zebra!

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: 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.