Cataclysm

I have had a central line for four and a half years.  We placed a PICC line in early 2014 to facilitate vascular access in an emergency, to administer rescue meds at home during an anaphylactic event, and to allow me to use IV fluids at home to stabilize my blood pressure.

Shortly after I had my line placed, I was hospitalized for a five day long episode of protracted anaphylaxis. Because I had pretty much already tried every oral medication that could help manage my symptoms, trying IV medications was the obvious next step. I went home from the hospital with a prescription for IV Benadryl.

Speed matters a lot in management of mast cell disease. There is a tiny window of time during which immediate medication can stave off continued reaction or anaphylaxis. I learned how to dilute and push IV Benadryl during mast cell crisis.

Using IV meds was really nerve wracking at first, much more than I expected it to be. I’m an infectious diseases microbiologist by training and used to develop diagnostics for blood stream infections. I don’t think I can overstate how scared I was of getting sepsis. Every time I touched my line to flush or give meds, my hands shook and my heart pounded. But this was tempered by an obvious benefit: use of IV Benadryl as soon as a reaction started often prevented the need for epinephrine.

My health fluctuated over time. Eventually, I started using IV Benadryl as a baseline medication to manage daily reactions. It allowed me to exercise. It allowed me to eat. It allowed me to travel. It allowed me to work. IV Benadryl restored a much improved quality of life. I have used it ever since.

IV Benadryl is an old medication. It is off patent, cheap to produce, and made by multiple manufacturers licensed in the US. Overwhelmingly, it is used to manage anaphylaxis. Regular use as maintenance for patients was unheard of before the mast cell community began using it to manage reactions. Home use of IV Benadryl both for maintenance and for rescue – that is, to manage regular daily symptoms as well as to be lifesaving in the event of anaphylaxis – has become more popular in the mast cell community in recent years. I think I’m probably partly to blame for this – I have openly talked about how much it has helped me. I probably know a hundred mast cell patients who use it regularly.

In mid September, I got a call from my IV pharmacy. They were calling to tell me that vials of IV Benadryl were unable and they were going to dispense IV Benadryl in prefilled syringes. Fine. I didn’t care what package it was in. But I knew multiple organizations made this medication and I found it strange that vials from all of them would be unavailable at the same time. It was odd. But it was also true. Every manufacturer was reporting that it would be several weeks to months before the vials would be again be available.

A week later, my IV pharmacy called to let me know that the prefilled syringes were no longer available either. I found out on a Friday afternoon that come Tuesday, I would be out of medication. No one could get it. All distributors were out. I called over fifty pharmacies in Massachusetts trying to find some. I talked to the FDA. I talked to the manufacturers. I talked to hospitals nearby.

Despite being labeled a “critical backorder”, this phenomenon was something else entirely. It wasn’t “I can’t get enough of it.” It was “There is no IV Benadryl available anywhere in the US.” EMS is out. Hospitals are out. And every pharmacy everywhere is out or dangerously close. With only a few days warning, mast cell patients found themselves unable to get medication that literally kept them alive.

It is not overstating things to say that IV Benadryl is necessary to sustain life in a number of mast cell patients. I am one of them. Many people need it to maintain an airway. Others, like me, need it to recover blood pressure during anaphylactic shock. A lot of patients were only able to live outside of a hospital safely because they had access to this medication. The danger posed to these people is enormous. This is especially true because in an emergency, you can call 911 and have them take you to the hospital but they don’t even have the medication to treat you. This means that unless patients already have some, in a crisis, it is impossible to get more from literally anywhere. If you need this medication to survive, you are in real peril.

Compounding the issue is that IV Benadryl is the only potent H1 antihistamine available in IV form in the US. There are no alternatives for IV H1 antihistamines. It is IV Benadryl or IV nothing. It is also not used much outside of the US so getting it from abroad isn’t really an option.

Mast cell patients have been encouraged to use epinephrine as early as possible without IV Benadryl to potentially stave off a reaction. So we can all just use epipens anytime we react badly, right? Just kidding. Epipens are also on critical backorder. Okay, let’s use IV fluids to recover blood pressure during anaphylaxis and severe allergic symptoms. Haha, no. IV fluids are also on backorder. One of the safe narcotics for mast cell disease is no longer available. Certain benzodiazepines are no longer available. And we have no idea whether or not these medications will be available in the future.

The stress of this situation is paramount. I have patients getting trached to allow them to be hooked up to a ventilator. I have patients who can’t stand up without reacting or passing out. I have patients who are using epi multiple times a week. And I have patients who are scared to use their epipens so they gamble that they can control their reactions with something else. The single greatest risk factor for fatal anaphylaxis is delay in the administration of epinephrine. “Saving” epipens is dangerous.

Those of us who have won some stability through this medication are terrified of going back. I am terrified of going back. It’s already starting. After several months of debilitating symptoms and repeat anaphylaxis, I have been preparing to return to work in November. I love my job. I love my coworkers. I love my company. I can’t safely return to work until I have a guaranteed supply of IV Benadryl. And as of now, that could take months.

I am angry over this situation in a way that borders on holy fury. Mast cell disease is hard on its best day. It doesn’t need to be made even harder. Total loss of access to rescue meds is life threatening for many of us. In a country known for its premier healthcare, essential medications are completely unavailable. Mast cell patients were not even notified of an impending backorder to allow us to identify a source and stockpile. We were ignored entirely. This is the end result.

This is a dark time for our community. It is a time for fear and anxiety. It is a time of uncertainty. It won’t last forever. But that is cold comfort when you are terrified.

It is also a time to stand up and fight. It is a time to be visible. It is a time to be loud. It is a time to shame these organizations for letting this happen to us. Because if I have to suffer, I will damn well make them watch.

I know a lot of us are struggling. Be careful with yourself. Don’t take risks. Wait it out.

Take care of yourselves. Remember: it won’t always be like this. You don’t have to beat it. You just have to outlast it.

The use of intravenous fluids for management of mast cell disease

I get frequent consult requests from patients specifically around the use of IV fluids to treat mast cell disease. I am often asked to provide references for papers that show its use and benefit. I am not able to provide any such references because there are none. There has been no organized study for the use of intravenous fluids to manage symptoms from mast cell disease.

Despite this fact, use of intravenous fluids in mast cell disease is increasing in popularity, largely because it works, and word of effective treatments travels fast in a rare disease community. While there is no firm answer for why it helps, there is a reasonable explanation: it treats both deconditioning and POTS and many mast cell patients have one or both.  I wrote a seven part series on why exactly intravenous fluids help in these situations. I have also written in great detail about the way that mast cell disease and POTS interact.

A paper published in early 2017 reestablished the finding that use of intravenous fluids helps POTS. Treatment lengths and infusion volumes varied from person to person. Despite these variations, use of IV fluids decreased symptoms and improved quality of life for POTS patients. The link to the abstract is here.

Many mast cell mediators are vasoactive, affecting the permeability of blood vessels. This means that mast cell activation causes third spacing, the loss of fluid from the bloodstream to the tissues, where the body cannot use it. This functional dehydration can cause a lot of symptoms, not the least of which is exhaustion and difficulty standing or exercising. For obvious reasons, this will be further exacerbated in a patient that is deconditioning or who has also has POTS.

Orthostatic symptoms can be very activating to patients and managing them effectively can help significantly. I have seen IV fluids work where more traditional methods like drinking lots of fluids and consuming lots of salt, or medications like fludrocortisone have not helped. Additionally, the first line tools for managing POTS, beta blockers, are contraindicated in patients at increased risk for anaphylaxis and therefore in people with mast cell disease.

I am a fervent supporter of IV fluids (also called volume loading) in the context of mast cell disease. I have seen it stabilize patients and reduce the frequency of anaphylaxis and severe symptoms, especially orthostatic symptoms and GI symptoms.

I personally use IV fluids. If I don’t receive IV fluids at least three times a week, my orthostatic symptoms become so severe that it is difficult to stand or even move. This in turn triggers mast cell reactions. The benefits of IV fluids to my personal health are significant. Many patients report the same.

While I support the use of IV fluids in the context of mast cell disease, patients should be aware that there are infection risks associated with repeated IV access or placement of a central line. The risks are much lower for repeated IV access as central lines have a host of other risks, including blood clots, and infections have the potential to be much more serious. However, IV access can be difficult for mast cell patients. The treatment value of IV fluids should be weighed on a case by case basis and IV access on a case by case basis.

For additional reading, please visit the following posts:

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 1

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 2

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 3

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 4

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 5

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 6

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 7

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 12

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 31

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 32

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.

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.
 
 
 

PICC Lines

I talk about PICC lines, why you get them and what the risks are.  Tune back in later in the week when I show you how to access it safely, change the dressing and draw blood for labs.

In this clip, I say that PICC lines are “inserted in central veins.”  This should be “peripheral veins.”  Sorry for the flub.

 

This is what being sick looks like


I don’t like when people tell me to be positive.  I am quite positive, generally.  But pretending that being sick is this perpetual ethereal learning experience that imbues me with this magical understanding of life is not realistic.  I am allowed to be upset.  Most days being sick doesn’t bother me.  Sometimes it makes me sad.  Today is one of those days.
I am aware that I often don’t look sick.  But this is what being sick looks like for me.

 
 
It looks like an entire cabinet full of oral meds, IV meds, ostomy supplies, PICC line supplies and miscellaneous medical stuff.
 

 

It looks like meds and epi at the bedside, always.

 
It looks like blown veins from poor IV access.

 

It looks like low blood pressure and tachycardia.

 
 

It looks like a medical alert bracelet.

 
It looks like a colostomy bag and swollen, hard, scarred abdomen during a bowel obstruction.

 

It looks like industrial strength equipment to take a bath.

 

It looks like pitting edema.  (This picture was taken five minutes after I rolled up the sleeve over my PICC line; the impression stayed for hours.)

 

It looks like flushing even after 120mg IV solu-medrol, 100mg IV benadryl, 40mg IV pepcid and one dose of epi.  (On top of daily meds.)

 
It looks like getting oxygen during anaphylaxis.

 

It looks like feeling like you’re winning when you get to infuse at home with your new PICC line.

 
It looks like needing IV benadryl in the middle of the night.

 

It looks like being grateful for a central line.

 

It looks like slow pushing IV meds.

 

It looks like weird rashes all over my body.

It looks like sharps containers full of reminders.
 
This is what being sick looks like.