Roller coaster

I got my first central line in February 2014. At that point I was at the hospital two or three times a week. Being able to manage my needs at home as much as possible was a welcome relief and so I welcomed the line.

I had a PICC line placed in my left arm about three inches above the elbow. The poor PA who placed it was terrified. She called me the day before to go over the procedure in pinched, staccato notes. She casually mentioned that she was considering doing the placement in the ER since they would have a crash cart nearby. The infusion nurses had regaled her with stories of my reactions and anaphylaxis history. I laughed and then felt bad about it. “Whatever you need to feel comfortable is fine,” I told her. It is the first time I remember a provider being scared of my disease.

The second time I remember a provider being scared of my disease was the following day when a home infusion nurse came to change my dressing. She was much more frightened than the PA had been. She made me hold my epipen while she did the dressing change. “You’re doing fine,” I reassured her, hoping that was true. Comforting trained professionals while they treat me is a special sort of pain, like pulling out a thorn. It shouldn’t have happened in the first place and half the pain is disbelief and feeling ridiculous.

That PICC saw a lot of action. But before I was a mast cell patient, I was an infectious diseases microbiologist, and I wanted that PICC line out. PICC lines are basically a straight shot to infections. I managed to keep my line sterile and my site immaculate and lobbied hard for a port to be placed. In September 2014, the PICC was removed from my arm and a port was placed in my chest.

I wanted a port for a few reasons, but the big reason was because I could manage it completely independently. I expend a lot of energy trying to stay infection free and a big part of that is staying out of the hospital. The PICC line allowed me to give myself meds and fluids but I needed someone else to change the dressing and it was harder to troubleshoot outside of a hospital. With a port, I could access and deaccess the line, change the dressing, and give meds on my own. That meant I only needed to have supplies and myself, and I could bring those just about anywhere. I could also shower more easily, take a real bath, and go swimming with the port. It was an all around win.

In the three years and three weeks that I had it, that port lived quite a life. I took it to my parents’ house and on long walks with my dogs. I took it to work on countless days. I took it to the beach and water parks. I took it to my pool on hot summer evenings. I took it to the hospital for appointments and surgeries and procedures. I took it to my niece’s First Communion. I took it wedding dress shopping with my sister. I took it on many long weekends in New Hampshire. I took it apple picking and trick or treating. I took it Christmas shopping. I took it to my girls’ weekend in Maine. I took it to the Garden of the Gods in Colorado Springs. I took it to California. I took it to Disney World. I took it all over Florida. I took it to the blue waters of Tulum and underground rivers and the Mayan Ruins at Coba. I took it on the Star Ferry and on long walks around Hong Kong. I took it to the Forbidden City and the Great Wall of China.

It wasn’t completely without issues. I had some trouble with my port and a line infection abroad is on my top ten list of things I never want to experience.  But the horrors my mind produced in response to my fear never materialized. I am fortunate to be able to say that my panic attacks have been the scariest things I have experienced with a central line.

My port was my security blanket. It still is. I could travel to all these places because I wasn’t terrified of anaphylaxis. I still took extreme measures to prevent anaphylaxis but I wasn’t paralyzed in fear by the thought of traveling. I had the meds and IV access and could keep myself stable. In an emergency, I could get myself to a decent hospital, and from there, I could get home.

I started having trouble with my port in May. My port had a small reservoir and was difficult to access unless you always put the needle through my skin in the same spot. After years of having a needle continuously penetrating the skin in the exact same spot, scar tissue formed around this spot and the site became indurated. Eventually, the skin at that site became ulcerated and millimeters thin. It was almost to the point that you could see the port through the hole. I developed a literal hole in my chest over my port.

I had a huge amount of anxiety over it. My port allowed me to control so many things about my life and I was really scared about losing that control. I spent a few months trying to get the hole to heal. It did not heal. It ulcerated and got larger.

I had the port replaced at the end of September. They were able to put the new port in the same pocket as the old one with the new line ending in the same blood vessel. They did a fantastic job on the ulcer repair and removed some additional dead tissue. All in all, it went as well possible.

It is staggering to think about how much has changed between the placement of my first central line and my most recent one. It never occurred to me that I would need a permanent central line. I figured I would have it to for a few months and I would get better and it would be removed. It obviously didn’t play out like that.

There have been some very low points. I decompensated a lot. I ended up needing more GI surgery. I transitioned to doing IV meds daily. I needed continuous IV fluids for a while. I’ve had a bunch of procedures, scopes, etc. I lost the ability to eat all solids. I needed several months of medical leave from work.

I’ve had plenty of high points, too. And in the past year, I’ve had a lot of them. Most things considered, I have steadily improved since the spring of this year. I am back to work. I am actively working on MastAttack again. I have been able to adjust my meds a lot as some symptoms have improved. I can eat again, and not just eat, but eat real food. It still takes a lot of work to keep this body functioning but it’s not as much of a constant struggle.

I no longer feel like I’m constantly flirting with anaphylaxis. I haven’t used an epipen in several months. It’s a reality for me that I will never be free of the risk of anaphylaxis. I still keep epipens out in every space in my home. I still carry around four epipens and IV rescue meds at all times. But I’m not waiting for it anymore. I’m not always afraid. That in itself has been unbelievably liberating.

I am working on decreasing my IV meds and IV fluids very, very slowly. My long term goal is to get to a place where I do IV fluids overnight three nights a week and only access as needed for IV rescue meds or procedures. It will likely take years to get to this point, even if I continue to improve. It has been hard because these things have kept me safe for a long time. I think I will never be free of them entirely and that’s okay.

But my relationship with my port is changing. I am very slowly trying to transition to a person who uses my line but who is not completely dependent upon it for safely. Part of this is training my body. Part of this is training my mind to stop catastrophizing and to let go of my anxiety. It’s not obvious yet which part which be the hardest.

I get Xolair every four weeks at my immunologist’s office at the hospital. Last week, his nurse and I were chatting about my recent port placement and decreasing my IV meds. I shared that the idea of not having a continuously accessed port and therefore ready IV access was scary. “Imagine that,” she said as she gave me my injections. “Imagine just accessing a few nights a week. Imagine if you could have it removed!”

I can’t though. I can’t imagine it. I think I will always have a port and will always need some IV support. But the idea that I could only need it to be accessed for three nights a week is mind blowing. A year ago I was passing out if I stood up, my stomach was newly paralyzed, and I couldn’t eat at all. Now I’m back to work full time, working on the courses for MastAttack U, planning international travel for next year and eating chicken pot pies for dinner. Bananas.

As I get more comfortable with this process, the fear is still fresh, but there is now an edge of exhilaration. Like an amazing ride from a high height. A long drop with a safe landing.

A roller coaster.

Hole

Ports were developed expressly for the administration of chemotherapy to cancer patients. They addressed a couple of specific issues in cancer care.

Chemo can be very damaging to blood vessels. Many drugs are caustic and can cause sclerosis of the blood vessels and damage to soft tissues around the vessels. Port lines terminate in very large blood vessels close to the heart so the risk of damage was much lower.

Chemo patients are also at a significantly increased risk for infection. Ports are implanted between the skin and the muscle so they are entirely within the borders of the body. In order to use the port, you push a very large needle through the skin and into the port. This is called accessing the port. When you are done using the port, the needle is removed and the port no longer has contact with the outside environment. They were designed to be accessed occasionally for treatment and to be deaccessed as soon as that treatment was done.

Like so many other things in medicine, ports were not designed with mast cell patients in mind. I am part of a cohort of patients that currently have ports for chronic, continuous use. My port is accessed all the time. There is a needle going through my skin and into my port all the time. The needle is changed once a week and I take the needle out for a few hours sometimes if I am going swimming or taking a bath. Otherwise, the needle is never out.

My port is a bit unusual in that it is very difficult to get the needle into the port without going straight in through one spot. Most patients who have accessed ports move the needle to a different spot when they change it weekly. This allows the skin to heal a bit between accesses. This has never worked for me. For this reason, over the last three years, I have developed a literal hole in my chest over my port where the needle is placed.

Last spring, I accidentally tore the needle out of the port. It made a hole that was already pretty big much bigger. I started deaccessing for a few hours a couple of times a week to try and help the skin to heal. I slathered cromolyn cream all over it and hoped for the best.

In June, I had a temporary IV line called a midline placed in my arm so that I could stop using the port for a few weeks and give my skin a rest. Unfortunately, that didn’t go well. Aside from all the pain in the ass things about having a line in your arm (which I had forgotten), using IV Benadryl through the short line caused phlebitis, a form of vasculitis that affected the vein where the line terminated. I was diluting it to a ridiculous extent (1:50) and it was STILL causing really severe pain both while I was pushing it and for hours afterward. We pulled the midline after a week.

The result of always accessing in the same spot is not just a hole but a hole surrounded by thick scar tissue. My nurse was able to get it accessed at different angles by pushing through the scar tissue but it was shockingly painful. The port was getting inflamed and it was getting to a point where the inflammation looked just like a pocket infection. It wasn’t infected but the fact that I could no longer tell the difference was alarming.

The pain surprised me. I can tolerate a lot of pain. This is unbelievable. The hole is now large enough that my body recognizes it as a wound. I have been really tired and achy lately and I think it’s because my body is expending energy trying to close this hole.

I did not expect the emotional effects of this situation. Memories from 2014 keep cropping up. My life is unrecognizable compared to the life I had when it was placed. I knew it was necessary but I was really nervous. I didn’t like having a PICC line but I was finally used to and troubleshoot it without panicking. I was worried that the port would get infected or I wouldn’t be able to access it myself. It was the coda to a year of abysmal health.

It’s funny to reminisce about how anxious I was about getting it placed because my port is my lifeline now. It gives me confidence. It makes me feel secure. I have travelled to the other side of the world with this port. It has allowed me to work full time. It has saved me countless ambulance rides and admissions. It is part of me now, a part that has given me back a lot of my life. A part I don’t want to use.

Because we are concerned that the hole makes me much more susceptible to infection, we decided to just cut our losses and pull the port and place a new one on the other side of my chest. I will be getting this done in the next few weeks. I think things will stabilize without the inflammation.

For me, Septembers will always be tied to pumpkin flavors, the smell of falling leaves, harvest moons, and ports.

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.