WARNING: Some of the images in this post show medical procedures and implanted devices. I have made the images smaller than in other posts so that you can scroll by quickly without seeing anything too graphic, please use discretion if you find images such as this disturbing.
You are not born with a fixed amount of resilience. Like a muscle, you can build it up, draw on it when you need it. “Sheryl Sandberg, IAMFearlesssoul.com
Well, according to the calendar, summer is soon coming to a close. Back into the autumn routine since Labour Day, it does seem that fall is already here, so time to post the last segment of my mini-series on “What I Did Last Summer” covering off some of the things that kept me busy over my blogging holiday. (In case you missed the first two, you can check out Chronic Illness & Post Traumatic Growth and 3 Good, Diverse Reads)
Apheresis: Therapeutic Plasma Exchange for Systemic Sclerosis
If you have been following the Sick With Optimism blog since the beginning, you will already know that I have a chronic illness caller Scleroderma, which may present in a number of ways, in my case the disease is diffuse (affecting multiple aspects of my body) and also referred to as Systemic Sclerosis. (If you are looking for more back ground on my experience with scleroderma, you could check out Sick With What?).
Back in the summer of 2017 my scleroderma renal crisis was escalating in spite of being caught relatively early and accessing the standard drug intervention. My nephrologist consulted with the hematologist our hospital’s apheresis clinic about the potential for putting me on plasma exchange treatments. Plasma exchange is used to treat a wide variety of diseases, including a number of autoimmune diseases where the treatment is favoured for removing antibodies from the blood.
Spreading Apheresis Awareness
As it turns out, this past Tuesday (September 17th) was designated Apheresis Awareness Day 2019 by the American Society for Apheresis (ASFA). Henceforth, Apheresis Day will be on the third Tuesday of September. The purpose, according to ASFA, “is to raise awareness of apheresis medicine and honor the many donors, patients, and apheresis practitioners that have dedicated their lives to saving others by using evidence based practice to advance apheresis medicine,” ( Aperersis Awareness Day, 2019, American Society for Apheresis website, http://www.apheresis.org).
How Does Plasma Exchange Remove Antibodies?
I am not a scientist or a doctor and a lot of this was quite mysterious to me for a long time! In simplest terms, the Apheresis machine is a centrifuge that spins blood drawn from your veins at such a rate that it separates into red cells (RBC), white cells, platelets and plasma. The machines can be used for a number of purposes from harvesting stem cells, to removing bad cholesterol, to helping organ transplant patients so that they will not reject their new organs. In my case, the machine is programmed to collect the plasma portion of my blood. The reason they do this for autoimmune diseases is that a good percentage of antibodies can be removed by extracting the plasma, where a significant volume of these proteins reside, and safely replacing it with albumin.
The Blood Viscosity Argument
In addition to the removal of antibodies or pathogens from the blood there is also an argument that plasma exchange improves the blood’s viscosity. According to an article on The Scleroderma Education Project’s website, patients with scleroderma/systemic sclerosis (SSc) tend to have thicker blood than healthy people, and hypothesizes that “Abnormally clumped red blood cells may be a significant component of the etiopathogenic processes in SSc, potentially contributing to the vascular damage” (Systemic Sclerosis – Is Blood Rheology The Key? 2018, The Scleroderma Education Project Ltd., http://www.sclerodermainfo.org). The article goes on to explain that plasma exchange improves blood viscosity, therefore, causing less damage to, and blockages of, microcapillaries, pointing out that ” In theory, any treatment that can reduce or eliminate RBC clumping should reduce or prevent the development of SSc-related symptoms.”
My History With Plasma Exchange “PLEX”
I have been on therapeutic plasma exchange (or “plex” or “TPE”) since August of 2017. At first I was admitted to hospital and had the treatments pretty much daily, tapering down to three times per week as an out-patient, then twice per week. December 2017 we tried to reduce to once per week and I ended up in the hospital as my heart began to fail. Spring of 2018 on a new cocktail of medications we tried tapering down to once a week again, and again, back in hospital, so since that time I’ve been pretty steadily going twice per week.
Ways to Access Your Blood for Plasma Exchange
Peripheral Venous Access
It seems that for most people, the blood is accessed from the vein in one arm and returned through a vein in another. Depending upon the patient, different types of needles or catheter’s are used. This is known as peripheral venous access and is how I started. In fact, when I was in the emergency ward prior to being admitted, a Registered Nurse from the Apheresis Clinic came to assess the viability of my veins before the hematologist approved me to try the treatment. If required, the nurses in my clinic can use a chair-side ultrasound machine to identify the best veins to use if they are not readily apparent.
VasCath Central Venous Line
When I had my acute health crisis in 2017 I was put on hemodialysis as my kidneys had just about quit from the scleroderma renal crisis. I was given a type of access line (may remind you of the more familiar a PICC line) they called a “VasCath” central line which could be used for both dialysis and plasma exchange. At first it was a welcome relief not to have to be ‘poked’ in each arm for every treatment. It seemed that as time went on, accessing those veins became more difficult due to wear and tear, compounded by the scleroderma that hardened the skin from the hands up past the elbows.
The VasCath is a catheter inserted in the collar-bone area (sub jugular) that extends into the heart. The access port of the catheter is outside of the skin and splits into two lines (see image below), one in and one out that hangs above your chest. There were a lot of advantages to having a line! On the plus side, access was much faster and less complicated with the VasCath as there was no requirement to locate the veins, no discomfort from needle insertions, and no risk that the vein accessed wouldn’t work as, unfortunately, is sometimes the case with peripheral venous access.
Some Potential “Cons”
Risk of Infection
On the down-side, because the VasCath protrudes outside the skin there is an elevated risk of infection. For this reason I kept a dressing on the area at all times which was changed and the area carefully scrubbed clean by a specially trained apheresis nurse on a weekly basis. I understand that some people are able to go without the dressing once the area is healed, but even though I had the catheter for 18 months there would still be episodes of minor bleeding indicating it was not fully healed or that I had somehow put a strain on the area.
The catheter and dressing had to be kept dry at all times. This made washing up a little more complicated and time consuming, particularly as my scleroderma had caused me to become much less flexible and weaker. I tried many different strategies which would be enough for a whole other post! Another obvious no-no was that I could not go swimming.
Blood Clots & Management
Another risk posed by this access line is blood clotting. After having the line for about four or five months an ultrasound revealed blood clots forming in my jugular above the line. The hypothesis is that the line slows the flow of blood which can lead to clots above the line. I also wonder if the theory that those with scleroderma have thicker blood would contribute to this risk factor? At any rate, I was very upset at first! I was immediately put on subcutaneous blood thinner injections, which I could administer myself at home for about a week and then began taking the blood thinner warfarin orally on a daily basis. The whole thing was a little scary and terribly upsetting. I got home from the hospital and cried, and pouted. It was then that I first tried out a strategy that seems to work for me to this day: I gave myself permission to be upset, feel sorry for myself, and grieve. However, I set myself a time limit! Starting the next day I would move forward and do everything I could to remain optimistic. This would become an important self-care strategy that I have come to depend upon.
Anyone on blood thinners must have their blood monitored regularly to ensure they remain within the desired ratios – this was easy for me as I have blood work drawn weekly at the apheresis clinic through the line so, once again, no needle required!
Dog’s Best Friend?
A final thought, while I have never heard of this as a risk, this ‘con’ is strictly anecdotal, based on observations I made while having the line – dogs seemed to become a lot more interested in me. One example, a friend’s small dog that had previously paid less attention to me suddenly wanted to be with me all of the time, right up on my lap! Another more frightening experience at a small party, a friend’s large hunting dog came right up to me in a group of people, first growling ominously and then barking viciously. My daughter was terrified, “Mom protect yourself!” she screamed as the dog’s master pulled him away. I have to wonder if the exterior portion of the line made the smell of blood more obvious (making me more of a target, like a weak or wounded member of a herd of prey)?
Other Venous Access Methods for Plex
There are other ways to access the veins for plasma exhange, I have seen temporary catheters in the neck, A-V fistulas, and vortex ports. An A-V fistula can also be used for hemodialysis (most people refer to this as “dialysis” but I will explain in a future post that there are two types of dialysis, hemo and peritoneal, the latter does not require vascular access). A fistula is created by connecting a vein to an artery, making point of access that is large and strong once the area heals.
What Does this Have to Do With What I Did Last Summer?
In an earlier post I discussed my experience of being turned down for a stem cell transplant (you can read about that in Hopelessness vs Disappointment). It was at that point that the penny dropped, I realized I would need to have plasma exchange for the long-term, perhaps the rest of my life. I no longer required the line for dialysis once I switched to peritoneal dialysis in March of 2018 so at this point the hematologist suggested we look at switching my access line to a vortex port, which would be entirely below the skin.
The primary advantage from a clinical perspective would be an up to 90% drop in the risk of infection versus the line. As I am on drugs to suppress my immune system, the benefit was even greater. In addition, I could shower normally and even swim if I so desired. Secretly, I also hoped that it would camouflage the scent that separated me from the herd in the minds of my canine friends!
I have to say I was very excited and began taking a lot of photos as I hoped to document my journey to the third form of access for plasma exchange right here on my blog! I have read in other blogs and articles about how not being able to bathe adequately causes a lot of emotional distress and I completely concur. It also has physical implications as well, which I won’t go in to at this point. Suffice to say, I was truly looking forward to a good, long, proper shower!
Swapping the Line for a Port
I was scheduled to have my old line and catheter removed and at the same time the new vortex port installed. This is a fairly straightforward day procedure which is guided by ultrasound. It was decided while I was on the table to leave the old catheter line in and just exchange the head of the VasCath with the vortex port. This was done under local anesthetic and some sedatives to keep me calm. I was awake and frankly quite happy throughout, the nurse commented after that I smiled the whole time!
Now, the new port could not be accessed for a couple of weeks until it healed, therefore, I was back to peripheral venous access. I was pleased that the nurses had no trouble accessing my veins. However, there was some concern about how the area was healing. We elected to keep it covered a little longer than usual. When I returned for plasma exchange about 10 days post surgery the steri-strips holding the wound together came off and it was apparent the nurse was not pleased by how it looked. She casually mentioned she would advise the doctor to have a look. The doctor examined it closesly and asked to send me back to Interventional Radiology where the procedure was performed to have a look-see.
After plex, I headed down to IR, a little annoyed because it had been a long day and my sister who had been kind enough to drive me was now having to wait even longer, the prospect of driving north out of Toronto at rush hour was my primary concern at that moment! The nurse that ushered me into the exam room advised me that they were wrapping-up for the day and there would only just be time to assess the site for me. I nodded and smiled, not really all that worried anyway and glad it was going to be quick.
When the surgeon came in and removed the dressing he immediately said that the area was compromised and the port would need to be removed. I was upset and argued I needed the port! The surgeon and nurse were both exceedingly compassionate. He took a picture of the area and enlarged it so that I could see for myself that not only was the skin not healing up but I could actually see the white plastic line of the catheter. The doctor told me that they would need to consider the area infected and could not risk leaving the device in any longer. He also assured me that when it healed they could try again, advising it might be a good idea to put on a little extra weight before that time.
Goodbye Vortex Port…
There were no day-procedure OR beds available, so a nurse agreed to stay late and a team hastily set up a mini surgery around me. The doctor assured me that this was a sterile room, as I had guessed (it was quite large with the exam table right in the middle and freezing cold). While the gowned team set things up my head was spinning! I was sad, disappointed, and frustrated that I would need to go through the procedure and recovery again (it had been somewhat more painful than I expected). I employed the strategy (mentioned above) which I had been using the past year and in that moment gave myself permission to cry, and wallow in a little self-pity.
To my surprise, I think approximately one tear escaped my eye. I realized that this, in the grand scheme of things, was not such a big deal. Peripheral access was working fine for plex for now and we could just try again in a few months. Yes, I was still disappointed, but I didn’t have any tears for something as surmountable as this! (I could save my tears for the baby diaper commercials on TV!) I was optimistic which in turn made me feel somehow empowered. Compared to the upset I had felt a year before when diagnosed with blood clots my reaction was much calmer and far less emotional, as though I had grown through all of this.
I didn’t even change into a hospital gown, they gave me a local and mild sedative and pulled the entire catheter right out. The doctor allowed me to see the device once it was removed, I even held it in my hand. It had an interesting weight to it, I turned it over in my fingers and admired the technology and compact design; “We will meet again,” I thought to myself…
I was so grateful I had my sister there to drive me home that day.
That Took Me Into August…
The first procedure was a week into July, by the time I left for my vacation the second week of August I was already healed enough to shower and swim, though some of the steri-strips were still hanging on. I have to tell you I relished that first long, hot shower! I joked with my doctor – when he asked about my first shower – that my neighbours couldn’t flush their toilets or water their gardens that day!
I am also pleased report that dogs are once again treating me with their usual indifference!
Chronic Illness, Resilience & Perspective
It may seem like a bit of a summer misadventure, but things have healed up just fine and heading back this week to try again for the vortex port insertion. I realize through this chronic illness journey that I am becoming more resilient to these disappointments. To truly grow, I have to learn to empathize with (and try not to judge) others for being upset or disappointed about what seem to be minor issues in their lives. I do recognize they have a totally different perspective; I remind myself that everything is relative, and in fact my disappointments are small compared to what many others have to deal with on a daily basis. I know I’ve said it before, but I try to be grateful for what is possible and good in my life which assists in gaining a better perspective and seems to improve my resiliency.
Things I am Grateful For…
- The Amazing Apheresis Team at My Clinic
- The Caring Team in Day Surgery
- My Sister
- 2nd Chances
- My Terrific, Extra-Warm Summer
- Ambivalent Canines
Hope you all had a great summer! Planning to publish an update this fall on how the vortex port insertion goes and my experiences receiving plasma exchange with the new device (better late than never!) – stay tuned! In the mean time, I would be very interested to hear from anyone who has the vortex port what they think of it; from folks who also receive therapeutic plasma exchange (for scleroderma or other disease); how chronic illness has affected your perspective and resiliency; or any other comments or questions! Thanks for reading!