Extraordinary

I forget sometimes that this life is extraordinary. Being sick just becomes incorporated into your life. It is impossible to survive if you are upset about it every day. It just becomes part of your routine and you learn to live with it.

I had a new IV line placed last week. My port has been accessed continuously in the same spot for three years. My skin is indurated and paper thin over the access site. I accidentally tore the needle out last month and that further irritated the skin. Since I was likely weeks away from being able to literally see the port through the hole in my chest, we opted to place a temporary IV line for me to use so I could deaccess the port to heal the skin. They put in a midline last week and deaccessed my port.

I had a PICC line for a while before I had my port. The PA who placed it was pretty terrified of my mast cells. She had been warned by the infusion nurses at the hospital. The placement itself was uneventful but I will never forget having to reassure her. It was the first time I saw a provider scared of my disease. The following day, a home IV nurse came to change the dressing and check the site. She was also scared. She asked me to hold my epipens while she changed it in case of anaphylaxis. I reassured her, too.

While I am grateful to have IV access because it keeps me out of the hospital, I had forgotten what a royal pain the ass it is to have a line in your arm. The port is easier is so many ways. I can access it and deaccess it at will. I can change the dressing myself. I can get it wet. I don’t have to deal with my pump constantly squawking that the line is occluded because I bent my arm. Blood doesn’t back up in the port line. I don’t have to constantly lock the line with heparin. I forgot the way IV Benadryl burns when it’s pushed into a smaller blood vessel. The midline is temporary but obnoxious after years of having a port.

Having the midline has brought back a lot of memories for me from around the time I got the PICC placed. One of the strategies social workers recommend for adapting to a medical device or deformity or disease is to give it a name. I named my PICC because I had to convince myself that I could learn to live with it. I named my ostomy, too. I don’t bother naming things anymore. Because it has become routine.

Sunday night, I ended up in the ER after sudden onset severe GI pain. This pain is high in the tract and much more severe than what I have experienced before, both in intensity and in duration. I went to the hospital because the pain was so bad that I honestly thought I had ruptured something. It was the kind of pain that makes you think you are dying. I was literally screaming in pain.

I spent the next day in the hospital where my screaming pain was interrupted only by intense vomiting from the pain meds. We have no idea what is causing the pain. I am not convinced that it is mast cell related. I came home last night because the hospital couldn’t do anything for me that I couldn’t do at home. The nausea and pain were still there. So I left with no answers and a lot of pain.

One of my nurses yesterday was really horrified when I told him all the things I do on a daily basis to manage my disease. He in particular was horrified that I needed so much medication and was still left with debilitating symptoms. It is only in seeing this awe reflected in the eyes of people who see so much suffering that I remember how sick I am.

Today was the longest day of the year. In many pagan traditions, the summer solstice is the day when the land of the living and the land of the dead overlap. It is a day for seeing ghosts of those who have gone before us and specters of who we used to be. A day when the past whispers to you as you walk past.

I have spent all day reading through my journals from when I had my PICC line placed. I have thought about all the ways my life has changed. In many ways it has gotten better. But it definitely changed me. There is a before and after in my identity as a chronically ill person. That timeline splits along the line extending from that date.

What’s funny is that while so many things have gotten worse in that time, a lot of things have gotten better. I am much happier. I am much less scared. I am much more independent. I am much more in control of my disease and my life.

I no longer have to convince myself everyday that I can make it through the day with a central line that everyone can see. Because it is just part of my life and it’s no longer extraordinary.

Name your fear

Being chronically ill is an exercise in managing fear. There are so many of them and they all need attention in turn. You are afraid of the damage being done to your body. You are afraid of the damage being done to your mind. You are afraid of the damage being done to your relationships. And you are afraid of the damage being done to your life.

For me, none of these fears hold a candle to the one that looms largest in my mind: bloodstream infection.

Long before I learned about the intricacies of mast cell biology, I was an infectious diseases microbiologist. My first job out of grad school was developing rapid diagnostics for bloodstream infections. I spent thousands of hours studying pathogenic organisms like MRSA, VRE, E. coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, and Candida albicans and learning how to find them as fast as possible. I learned a lot about how often these infections occur and the sepsis they are and how fatal they are. (Pretty fatal, in case you’re wondering). The science around bloodstream infections feels very much like my old stomping ground if my old stomping ground was a burned out car in a Mad Max wasteland.

I have a central line permanently implanted in my chest for the purpose of giving myself IV medications and fluids daily. I had my port placed in September 2014. For several months before that, I had a PICC line in my arm to give IV access. Having a central line massively increases the risk of bloodstream infection. PICC lines are generally considered riskier than a port but ports are not a whole lot safer if it is accessed all the time like mine is. The likelihood of a line infection is not insignificant. It is on my mind every day.

In the 3 ½ years I have had a central line, I have never had an infection. There are basically two flavors of infections associated with lines: local site infections, where the place that the line crosses through your skin gets infected by organisms on the skin, and line infections, in which the inside of the IV line is contaminated. Both can be serious but site infections are less serious. In many instances, a site infection can be cured by pulling the line and putting in a new one somewhere else. Line infections are so dangerous because the bugs inside the line get pushed into your bloodstream and pass through your heart. Pulling the line often does nothing and most people end up in the ICU for several days or longer.

I am a maniac about my line. I exert a huge amount of effort to keep my site sterile and immaculate. I spend a lot of time sterilizing the end of the line before I inject any meds or hook up an infusion. I am extremely careful when I dilute medications to inject. I use a checklist when I access the line to avoid contaminating anything. If I think there is even a miniscule chance that I contaminated something, I throw it all out and start over again. But the most important way I protect against infections is by not letting anyone who isn’t me touch my line. My home care IV nurse is the only person aside from me that I trust to touch my line. I avoid going inpatient or to the ER at all costs because there are so many more people and the risk of contamination skyrockets.

Despite all of this, I work myself into a frenzy a few times a year in which I convince myself that I have a line or pocket infection. (A pocket infection is a kind of site infection you see with ports, which are implanted under the skin). Naturally, these frenzies occur when I am traveling because otherwise they would be no fun.

Deaccessing the port means taking out the needle so that I cannot inject medication into the port. The port is connected to my bloodstream. Without the needle, the port is pretty impervious to infection. Putting a needle back into the port is called accessing. It is a sterile procedure and involves sterilizing the skin and then putting a sterile dressing over the needle. Like many mast cell patients, I have very sensitive skin. I react to the betadine and alcohol I have to use to sterilize my skin. (I have tried other sterilization procedures, this one is the best for me because I react much more with others). I also react to the adhesive of the sterile dressing, although it’s much better than the alternatives. (I use IV3000 dressings and many mast cell patients have luck with them.)

I have to deaccess my port to go swimming. If I go swimming every day, this means that I have to take out the needle and pull of the dressing every day. When I need to use the line again later that day, I have to sterilize my skin and put on a new dressing. My skin reacts badly to doing this daily. I often get hives and it’s hard to clean off the adhesive residue left by the dressings without using a lot of alcohol, which I also react to.

When my skin reacts like this, it doesn’t look that different from a site infection. It is red and itchy. It sometimes hurts. I sometimes get hives. It can make it much harder to figure out what’s actually happening.

I’m in Mexico right now. There have been a lot of hiccups on this trip but it is insanely beautiful here. It is a special place. It is also incredibly hot here so I have been swimming a lot. I have been deaccessing for 4-6 hours at a time. Yesterday, as I will removing the needle so I could swim, a little bit of white fluid came out with a few drops of blood and the needle. It kind of looked like pus. I spent the next several minutes pushing on my port and trying to assess for signs of infection with thinly veiled panic.

Seeing pus come out with the needle usually means a pocket infection, an infection under the skin around the port. But if you access a port while having a pocket infection, it can push some of the infection into the bloodstream. As I am heavily dependent upon using the port for IV meds and infusions daily, it’s not safe for me to not have IV access. After trying to collect myself, I called my IV nursing team at home. We talked through some scenarios and the likelihood of infection.

After some deliberation, I went to the doctor on staff here at the resort. I was very nervous that he would be unable to help or not want the liability. He ended up being fantastic. He ordered the high dose oral antibiotics my home team requested. He works at a local private hospital and was able to arrange someone to start an IV for me daily if the port did end up being unusable. Alternately, I could go to the private hospital daily and they would give me my fluid infusions and IV meds through the IV they placed.

After some more discussion, my home team felt it was okay to try and access the port that night if there were no more signs of infection (especially not getting any white fluid out when pushing on the port). If I accessed it, I could use it normally. If there were signs of infection, I would keep it deaccessed and stop using it until I got home. Then I would have an IV placed and we would discuss IV antibiotics at the hospital.

Last night, after several hours of deaccess and worry, I was able to reaccess my port. It is working fine and has had no other signs of infection. I’m still not sure what the liquid was and it’s possible it was the start of an infection. It is also possible that the white liquid was from a burst hive, or a precipitate formed by the betadine on my skin reacting with the water here, or some stray sunblock that hadn’t gotten cleaned off. Just something to keep things lively.

There are a lot of obstacles in the path of anyone who travels with major health issues. The fear of needing help and not having it readily available is the biggest one for me. Understanding all the ways something can go wrong is so often a hindrance. It is much harder for me to just take things at face value and not worry about it.

So I don’t really know what happened and I’m probably never going to. This morning, I was just grateful to wake up with a working port in a coping body in this beautiful, special place.

The absence

My port is currently deaccessed. It has been accessed in the same place, 24 hours a day, 7 days a week, for three years. Except when the needle is changed weekly, or it is briefly deaccessed for another reason, like to go swimming, it is accessed all the time. I feel much safer with it accessed as it gives me ready IV access in case of bad reactions or anaphylaxis. As much as I do not like needing a port, I am very happy to have it. It makes me feel much safer and more secure.

Last weekend, I accidentally tore the needle out of the port. I already have a permanent hole in my skin from being constantly accessed and it made the hole bigger. I was able to get it accessed again safely but something will have to be done about my access site. I may need to get a temporary line placed so that I can deaccess my port for a few weeks and give my skin a chance to heal.

For now, I’m deaccessing for three hours three nights a week and slathering my site with MML (magic masto lotion – recipe at the bottom) to soothe the skin and the site. Meanwhile, I just finished doing yoga on my living room floor, and am sitting here, thinking about my port, the absence of the needle, and the other absences my disease has rendered me.

 

I didn’t immediately realize what was happening when I started losing my hearing. I imagine it is that way for many people. It was springtime and I was training to once again walk 60 miles in 3 days for a breast cancer fundraising event. I walked a lot, 8-10 miles at a whack, people watching and getting lost in my head, music loud in my ears. When I noticed that the music wasn’t as loud in my left ear, I assumed my headphones were broken. I bought new ones and shortly realized that I had the same problem. Because I am not overly bright, I bought new headphones AGAIN, only to discover that I was being targeted by a complex conspiracy to deprive my left ear of sound.

It was still a few days before I realized that all of these headphones worked fine and that it was my left ear that wasn’t. I could hear in my left ear but it was dampened. I was also beginning to have balance issues. I will never forget the moment when I understood that I couldn’t hear well on my left side and that there was no obvious explanation for it. I somehow just knew that this would not be something that could be readily fixed. I felt this wave of panic, electric and silent, hidden under the beat of my quickening pulse. Always there, waiting without a sound.

I was pretty terrified while I was losing my hearing. Over several months, I lost all the hearing in my left ear and most of the hearing in my right. I listened to music compulsively, constantly. I noticed the blank moments where there used to be notes. I noticed the empty spaces in words. It grew. This hollowness grew and swallowed all these pieces of the world where there used to be sound.

You learn to live around absence. It is an instinct to adapt to your environment. And even though my environment didn’t change, it changed for me. It was both terrifying and fascinating. The way I interacted with the world was fundamentally changed. I was present in a world full of absence.

As strange as it sounds, when I lost my hearing, I wasn’t terribly “sick”. Like I lost my hearing but my overall, day to day functionality was still very good. It was over the next couple of years that things took a serious turn. And you get sick, like properly sick, you lost things. It’s not always a lot at once but it is steady and unending. You lost friends. Opportunities. Money. Jobs. Dignity. A million little things and a lot of big ones.

One of the big losses for many of us with mast cell disease is food. I didn’t start really losing foods in a significant way until late 2013/early 2014. Things picked up speed and soon I was down to very few foods that I could keep down that wouldn’t trigger mast cell reactions or anaphylaxis. I have regained and lost a lot of foods in the last few years. This past winter was the worst patch in a while. I was mostly limited to liquids, and very few liquids, at that. I was still eating plain potato chips because without any other solids, I would wake up because of the hunger pains. I lost a ton of weight, a lot of muscle, and a whole lot of hope.

I started Xolair injections in late February. My expectations were pretty tempered but it was worth a shot. Within a week, I could keep down some solids. My stomach has become a lot of smaller and I still can’t eat a lot of food at once but I can eat again. I can eat things I haven’t eaten safely in years. I still react to certain foods but I don’t seem to react anymore to the process of eating.

I am very aware that I could lose solids again at any time. For now, I’m just trying to be present.
*Magic masto lotion (MML): a cream applied to the skin for hives, itchiness, eczema, really any type of mast cell skin irritation. It is made by mixing liquid cromolyn with whatever your safe cream or lotion is. Some of us have prescription liquid cromolyn ampules while other people making it using over the counter Nasal Crom. I put about 3 ml of cromolyn liquid in a plastic bag, squirt some cream in there, close the bag, mix it up, and then slather it on wherever I want. Always speak with your health provider before adjusting your treatment plan.

Do all mast cell patients need central lines? No. But some do.

A newer patient asked a couple of days if everyone with mast cell disease needs a PICC line, Broviac/Hickman or port for IV access. The answer is no, but I think we should talk about this a bit.

Central lines are usually given for people who need chemo or long term IV treatmet. These lines are not really designed to be left in your body forever, even ports. They generally are pulled once treatment is done, although ports can be left in for years as long as they are flushed monthly.

In my experience, mast cell patients get central lines for a few reasons:

  1. They have very poor IV access, so poor that it could delay treatment in an emergency (anaphylaxis).
  2. They get regular IV medications (this is not very common, although it’s hard to tell in this group).
  3. They regularly take IV medication that can damage veins if given frequently in peripheral veins (like Benadryl).
  4. They get them for IV hydration (it is not recommended to get a central line just for IV hydration, however some people do get them).

In the groups, it seems like there are so many patients who have these lines. Please keep in mind that those with more disabling disease are the most likely to be present in those forums. This group often also has other diagnoses for which central lines may be beneficial. On the other hand, the other group that is quite visible is the rookies. So the new patients see this very severe face on a disease which is quite manageable for many. You are seeing a subset of the population. Central lines in the mast cell community are not as common as it seems.

Regarding IV hydration, there are a few reasons why people receive this. Some of us vomit frequently and so fluids are difficult to get into us orally. Some of us have POTS or dysautonomia and have low blood volume, so the IV hydration stabilizes our blood pressure and heart rate. Some of us third space badly, and oral fluids end up in the wrong place.

This patient asked if they could just drink fluids. The answer is absolutely yes. If you can keep oral fluids down and are functioning, then I would do that. Receiving regular IV fluids can help with some symptoms, but there is no reason they need to be delivered through a central line. I used to get IV fluids at the infusion center with a new IV everytime. It is a pain but it’s not awful.

In an acute situation, IV fluids can be very helpful to mast cell patients. Long term, you need to be monitored properly as it can affect your electrolytes and for some this may raise kidney concerns. I would not get IV fluids based simply upon “feeling dehydrated”. If you “feel dehydrated” and also your blood pressure is wacky and you can’t keep down oral fluids, I think that then regular IV hydration might be useful.

I know it is frustrating to feel that you are not doing as well as you used to, but if you have mast cell disease, it is very possible you never will again, even with IV fluids. I am sorry, but that is the reality. You need to adapt to the level of ability you can manage currently.  Get some stability and things will improve.

If you and your doctor feel that IV hydration is appropriate, I would try it outpatient for a few weeks. If they then feel you need to do it at home, placing a PICC line is a good place to start. If you have a problem with the PICC line, it can be pulled without much trouble. The other lines are implanted and require surgery to remove them. The risk of bloodstream infections from central lines is real and these are very serious situations with long term effects.

Mast cell patients also run the risk of reacting to the materials used to make the line. They can also react to the maintenance of the line, such as flushing, use of heparin and alcohol swabs. This is a real problem for some people. So any time you can avoid an indwelling line long term, that is the better option.

Lastly, central lines require maintenance so you need to be sure that if your doctor wants to order one, they will also order the solutions and nursing care needed to keep you safe.

Becoming reality

I scheduled my colostomy surgery about six weeks before I had it.  That afforded me a comfortable window of time to overthink it and work myself up.  I am a logical person.  I am a scientist.  I understand the risks and rewards of procedures and meds and so on.  But I am also human.  While I knew it was the right decision, when I was alone, I often thought about all the ways it could go wrong.
One of my friends asked me if I was ready for surgery a couple of weeks before I went in for it.  “I wish I could just do it right now,” I answered.  “Once it’s my reality, it’ll be fine.  This thinking about it all the time is exhausting.”  I think that sums it up well.  I just need these things to happen because once they become my reality, I just deal with it and move on.
Part of why mast cell disease is scary is because so many things can go wrong.  That doesn’t mean they ever will, but even if you feel confident you can manage your symptoms, you can’t help but think about all the horrors lurking in the dark places of the world.  But it’s not productive or comfortable to live your days living afraid of all the terrors that might befall you.  When living with mast cell disease is your reality, you just do what you have to do to get through your day.  It’s okay to worry as long as it doesn’t keep you from living.
I got a port placed today.  I have known this was coming for some time and I know plenty of people who have them.  It was not something I was logically worried about. 
But last night, the mental gymnastics started and suddenly I was worried about IV contrast accidentally being used and my friends and family reading my journals after I died from the reaction.  There was no reason to think this would happen.  It was pure ridiculousness.  But that doesn’t mean it’s not scary. 
I told a friend about it.  “Oh, I thought I was the only one who did that!” she said.  No, you’re not.  We all do it, whether or not we admit it. 
This morning I arrived at my hospital at 6:30am to have my port placed.  I met with the PA doing the procedure at 7.  He had read my entire history (“Which is really long and interesting,” he noted) and did some research on masto.  He went through the entire procedure, what materials would be used, what meds would be pushed, and made necessary changes.  (No Tegaderm, no chlorhexidine, absolutely not under any circumstances IV contrast.)  He asked what I wanted for premeds and ordered them for one hour before the procedure exactly as I requested, right down to the diluted Benadryl pushed over 10 minutes followed by a slow flush.  The nurses and technologist were excellent and the procedure went very well.  I am very sore and tired, but I have an accessed port and no PICC line and no reaction to speak off.  That’s what I call success.
Whenever my body changes in a noticeable way, I show it to my animals and let them investigate it.  Tonight I sat on the floor and Story came over and sniffed at my port and mouthed at it a little.  “It’s okay,” I told her.  “My body used to look different but now it looks like this and it’s okay.”
And you know what?  It really is. This reality is not so bad at all.