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.

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.

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.

PICC Lines: Dressing change and blood draw

PICC Line Sterile Dressing Change Procedure:

1.       Place arm on drape provided in dressing change kit.
2.       Open StatLock and Biopatch packages into the dressing change kit box.
3.       With gloves on, remove the occlusive dressing by pulling up the edges and then pulling the dressing toward the midline.
4.       With gloves on, pull up edges of StatLock.  Unclip the locks on the StatLock.  With one hand, lift the PICC line up, while pulling the StatLock off the skin with the other hand.
5.       With gloves on, remove Biopatch from around the PICC line at the insertion point.
6.       With gloves on, use alcohol (if appropriate) to clean any remaining adhesive from the area.
7.       Put on sterile gloves provided with dressing change kit.
8.       Disinfect the area around the PICC line (either with chlorhexidine or betadine.)  Allow to dry.  Do not touch the area while drying.
9.       Connect wings of PICC line to StatLock and apply StatLock to the skin.  Apply Skinprep to the the skin prior to pressing the StatLock to the skin (if appropriate.)
10.   Apply Biopatch to the PICC line near the insertion site.
11.   Apply occlusive dressing.  (I use IV3000.)
12.   Use adhesive strips from the StatLock package to secure the PICC line where it exits from the occlusive dressing.
 
PICC Line Blood Draw Procedure:
1.       Flush line with 10ml sterile saline.
2.       With the same syringe still attached, gently draw back to pull 5ml of blood into the syringe.
3.       Disconnect syringe containing blood.
4.       Attach needleholder/Vacutainer.  DO NOT STERILIZE PICC LINE CLAVE IMMEDIATELY BEFORE THIS STEP.
5.       Insert appropriate tube into needle holder and collect appropriate amount of blood.
6.       Remove tube.
7.       Disconnect needleholder/Vacutainer.
8.       Flush line with 10ml sterile saline.
9.       Flush line with 10ml sterile saline again.
10.   Lock line with 5ml heparin.

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: Nomenclature, fluid infusions and IV Benadryl

My PICC line set up and what the various pieces do:

 
How to hook up a fluid infusion: 
 
How to dilute and administer IV Benadryl. 
 
 
IV Benadryl should always be diluted as it can be damaging to the veins and can cause spasms of the airway if pushed too quickly.  It should always be pushed slowly. I generally dilute 1ml (50mg) of Benadryl with 9ml of saline for a total volume of 10ml, which I then push over about 15 minutes.  The flush after the Benadryl should also be slow.  I push that over about 5ml. 
I made a video of me drawing blood for labs through the line and then deleted it by accident because I am a fool.  Bah!  So I’ll make another one next week, as I don’t have any spare tubes and Vacutainers.  I am putting together a video on dressing changes as well.
 

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.

The danger of travelling

My life has gotten to be a lot of work in the last couple of years.  My chronic tendency toward dehydration via third spacing means that I’m at high risk for repeat bowel obstructions.  I anaphylax a lot, sometimes without a trigger.  I have a colostomy.  I am pretty much guaranteed to have a variety of medical supplies, syringes, IV meds, epipens and alcohol swabs on me at all times.  I finally have a reason to have a huge purse. 

I am good at advocating for myself and getting my needs met, in all venues of my life.  I knew that travelling would exercise those skills, but I honestly didn’t worry about it very much.  I am very, very familiar with FAA and TSA regulations and how they apply to someone travelling with a chronic illness/ medical device/ lots of medication.  The bottom line is that they are required to accommodate you provided you have the appropriate documentation.  Which I have.  In spades.
I called my airline last month and explained that I had mast cell disease and required several accommodations.  These accommodations included a wheelchair from ticketing to the gate, gate checking my larger suitcase (which also contained medical supplies), boarding early so that I could administer IV meds and set up my infusion, and that I needed to infuse for the duration of the flight.  The person I spoke with was extremely helpful.  She asked some questions about my infusion pump and the volumes and types of medications I would be bringing.  She asked that I bring a letter from my treating physician stating that these medications needed to be with me while travelling and that I needed to infuse for the duration of the flight.  No problem.
I arrived at Logan Airport and had a very painless experience.  They opened up all my bags and swabbed my hands and my meds for explosives. They patted me down.  They were very courteous and helpful.  When it came time to board, I was the first one on the plane and was happily infusing fluids when we took off.  It was really easy.
Then I arrived a week later for my return flight and it was a mess.   It made me really mad, especially in light of my previous positive experience.
The first problem was with gate checking my bag.  I explained to the agent that it had medical supplies and she tagged it appropriately and said it was all set.  Then another agent came up to me and told me that I couldn’t gate check my bag because it was too big.  I told her it contained medical supplies.  She argued with me.  After about two minutes, I told her again that it contained medical supplies.  “Oh, well you didn’t tell me that!” she exclaimed, rolling her eyes.  Whatever, lady.  Gate check my bag.
I got on the plane and pulled out my stuff to give IV meds.  A flight attendant came over and told me to put my backpack containing my infusion pump and meds under the seat.  I told her that I couldn’t because it was going to be attached to my body and I didn’t want there to be tension on the IV line.  I told her that I had spoken with the airline and that the infusion pump/meds are not considered luggage and can be with the passenger in their seat.  “The FAA isn’t going to let you do that,” she said.  I explained that I had infused on the flight from Boston to Seattle.  I offered her documentation and she talked over me.  “Well, you can let me know when you’re done and we’ll take off then,” she told me.  “It’s an eight hour infusion,” I told her.  She shrugged and walked away. 
A few minutes later she came back and told me that “the FAA sees things like this in black and white, and [I] can’t expect them to see things in grey because of people like [me.]”  I didn’t reply and she said, “I’m trying to be sensitive here.”  I do not think that word means what she thinks it means.
So I didn’t start infusing until I was flying.  This is important to the story.  When I started infusing, I noticed there were a lot of bubbles in the PICC line extension.  I realized that the extension was cracked and air was getting into the line.  This is very bad.  I stopped the infusion and had to trouble shoot the situation at 30,000 feet where I couldn’t just call my IV service and ask.  The fact that I couldn’t start infusing while on the ground turned a manageable situation into something very scary. 
While I was trying to identify the best course of action, the lady came over and asked if I felt okay.  I told her that I feel like I always feel.  “Do you need help?” she asked pointedly.  I just looked away.  Her behavior was making me mad, which was causing me to react while I was managing this situation.  I was really not amused.
I ended up disconnecting my extension and connecting the infusion directly to the PICC line without a clave to keep the end of my PICC line sterile.  This required some changing of hardware in the obviously less than sterile environment of an airplane.  I kept the clamps on the line and did not infuse fluids, which guarantees me a bowel obstruction in the next day or two, which guarantees me anaphylaxis.  I bought Wifi and messaged all of my PICC knowledgable friends because I didn’t know what else to do.
I’m now going to have to have a conversation with my doctor about this in the morning and the line will almost certainly be pulled and a new line put in because of infection concerns.  After I had to have IV meds administered to me by my friend (since I can’t access my PICC line without an extension), I put the cap directly on the line.  It is unusable until I get someone to come out to my apartment and fix it.  Thanks, airline lady!  I was so hoping to have more surgery this summer.  I also really enjoy having a line for emergency meds that I can’t use. 

The really unfortunate part of travelling while you’re sick is that it doesn’t matter if you know your rights if everyone else doesn’t know them too.  This woman’s behavior put me in danger.  It remains to be seen if I get a central line infection, but at the very least, she made it a serious possibility.  I am going to get a bowel obstruction because of this situation, which is massively painful and causes anaphylaxis.  This is unacceptable.  I don’t want this experience for anyone else. 

I am reaching out to the airline and hope they will do something about this.  I will keep you all posted.

Ask me anything (kind of)

Someone asked me recently if I would do an Ask Me Anything, Reddit-style.  The answer is no, because there are things I don’t want to put out on the internet at large.  I am a pretty open person, but there are things about my health and life that I don’t feel comfortable sharing. 

I do recognize that a lot of people who read my blog/see my posts on Facebook probably don’t know very much about me as a person, so I figured I’d do a post about me.  I’ll answer some questions I’ve gotten at the bottom.
I was born and raised in Boston.  For college and grad school, I went to UMass Lowell, where I got a really excellent biology education.  I strongly urge anyone looking for a serious science school with affordable tuition to look at UMass Lowell.  My undergraduate degree is in Biological Sciences, my graduate degree is in Biology (concentration in Microbiology.)  I planned to go to med school, and took my MCAT, but was too sick and too unstable.  It is still my dream to go, but I realize that’s unrealistic. 
My parents are both from Cambridge.  My mother, Gail, works as in Operations for a non-profit.  Her background is in childcare, and we had a daycare in our house when I was growing up.  My father, Mike, worked in the automotive industry until he had a heart attack in 2008.  He is now disabled and does not work.  My sister, Kristin, is five years younger than me and has degrees in Mathematics and Economics from Boston University.  She works for a large financial corporation in Boston. 
I am very close to my parents and sister.  I live three houses down from them and spend a lot of time at their house.   I have a large extended family including several nieces who always cheer me up.
I have a dog named Harry who is eight years old.  He is extremely handsome but not very bright.  He enjoys walks and cookies.
I also have a rabbit named Sadie B, who is going to be nine in the fall.  She has a terrible attitude and is currently chewing a hole in my wall as I type this.  We are not friends right now.  She likes lettuce and electrical wires.
I put myself through college by working in a pharmacy.  At the time I worked there, having a federal license enabled technicians to compound, so I did a lot of that.  I worked in pharmacy for almost ten years.  It was often frustrating, but I learned a lot about health care, insurance and medication in that job. 
After grad school, I worked in Research and Development for a small biotech company that developed diagnostics for blood stream infections.  I helped to develop tests for bloodstream and urine infections that are now FDA validated or EU CE marked. 
I now work in the biomedical research division of Novartis, a very large pharmaceutical company.  Novartis makes some drugs that are used to treat mast cell disease, as well as a drug in clinical trials for mast cell disease.  I do a lot of operations work now due to my often needing to work from home.  I learned how to do some coding last year and have done a lot of that.  I like coding and it’s easy to do from home, so it’s a good fit.
How long have you had mast cell disease?
I was diagnosed with mast cell disease in 2012.  I had been actively seeking diagnosis since 2008, and had been sick for a few years before that.  It has gotten progressively worse and more disabling for me.  I am fortunate that some of the top mast cell doctors practice in Boston so I am able to get excellent care.
How are you able to work if you are so sick?
I live alone and still work full time, which a lot of people ask about.  I have a lot of help.  I can still drive, but only if I don’t take pain medication, so I generally prefer to have someone else drive me.  My father usually drives me to work, which is about twenty minutes away.  I either take the train home, someone gives me a ride, or I take a taxi, depending on how I feel. 
How are you able to live alone if you are so sick?
My friends and family help out with household chores, like food shopping and cleaning, as I often don’t have the energy to do these things.  I am also able to give myself IV meds/epi at home without going to the hospital if I have anaphylaxis.  My doctors are comfortable with this because of my lab background, which means I am trained in keeping things sterile while accessing my PICC line.  Mostly I am able to live alone because if I call someone and need help right away, they are going to come.  If I can’t get in touch with whoever I called, I’m likely to get in touch with someone who will help. 
Why do you have a PICC line?
My IV access is terrible.  Over the years, frequent blood draws and IVs have caused hardening of a lot of my veins.  There has also been “mast cell deposition” at the site of access, which feels like sand when you rub your finger over it.  This means that it is easy to get a needle into my veins, but you often can’t get blood return or fluid in.  The PICC also enables me to give myself IV therapies at home. 
Was it hard to learn to access your PICC line?
No.  I watched once and did it.  I have many years of lab training to back me up, so I am very comfortable with sterile technique and syringes.  I also have a good understanding of the medications and the procedure and how to troubleshoot it if necessary.  I realize it is not always so easy for everyone.
Can you do a video about your PICC line?  My daughter is getting one.
Sure. 

If your family is Irish, why is your last name Klimas? 

This is my grandmother’s married name from a previous relationship, which became my father’s last name, and so on down the line.  The name Klimas is Lithuanian.  We are not Lithuanian.
Do you get Novartis drugs for free?
I have very good insurance and yes, I get Novartis drugs for free.  Yes, I realize how lucky this makes me as a mast cell patient. 

Does anyone else in your family have mast cell disease? 

My sister has had anaphylactic reactions to specific medications.  She has been tested for mast cell disease and is negative.  She does not have ongoing mast cell type symptoms.  My father has a hematologic disorder and autoimmune disease, but has been negative for mast cell testing, including bone marrow biopsy. 

Do you do research on mast cell disease?
No, but I know people who do, and they are often able to answer questions I can’t find the answer to. 

Are you Christian?  Do you mind that I pray for you? 

No, and no.
Has any of your research been published?
Yes.
You talk like a teacher, were you one?
Yes.  I taught microbiology at UMass Lowell while in grad school.  (This is how I paid for it.)  I also taught ASL classes out of my home for several years.  I like to teach.  Explaining things helps me understand them better. 
What do you miss the most from your life before getting sick?
I miss not having to plan every aspect of my day in advance.  It’s exhausting.  Also being able to try new foods without risking anaphylaxis.
What food do you miss the most?
Seafood.  I was actually saying yesterday that I’m going to pick a day this summer, premed the day before, give myself some IV Benadryl and have some.  I will probably need epi, which I have accepted.  I grew up eating seafood all the time and I miss it so much.
Do you speak Spanish?
Yes.  I have been speaking Spanish for almost twenty years, mostly with native speakers.  I generally speak with a Puerto Rican accent and slang, but am familiar with other dialects.  Castillian Spanish is the hardest for me.  Last time I took a proficiency test was in college and I was rated “near-native fluency.”  In college, I took literature, history and politics classes taught in Spanish.  When I lost my hearing, it became harder for me to understand spoken Spanish.  It has improved, but it’s still not always easy for me.  I read books in Spanish a lot.
Are you married?
No, and I’m not in a relationship either.  I also have no children.
What’s the best advice you’ve ever gotten?
I think this was masto related, but the best life advice I’ve ever gotten is to not do your favorite hobby as your job. 
I love languages and have been taking classes in various languages for pretty much my entire life.  I was originally a Modern Language major in college and switched to Biological Sciences after getting this advice.  I took three years of Italian and three years of German in college.  I took French in high school, four years of Russian over the course of my life, three years of ASL as a kid, a semester of Mandarin, a semester of Ancient Greek.  I spent a few years learning Arabic with the help of a native speaker.  I’m taking Hindi now.  My Irish is quite terrible, but I can exchange pleasantries.  Same for Danish.
What is your biggest dream?
To travel around the world.
Are you a microbiologist or molecular biologist?
Both.  There’s a lot of interplay between the two.  The diagnostics I helped develop were molecular tests, but my training is in microbiology.   
What is your favorite movie? 
Nightmare Before Christmas.  The Crow.  I have a lot. 
What is your favorite TV show? 
I love a lot of TV shows.  All time favorite?  Probably Battlestar Galactica. 
What are your hobbies?
I like playing games (card games, etc.)  I read a lot.  I also watch a lot of tv and Netflix now.  I enjoy hiking and rock climbing, but can’t do them anymore.  I walk a lot.  And I write.  Obviously.
If you could change one thing about your life, what would you change?
I don’t really know how to answer this.  If I could regulate my sleep, that would be great. 
Can you exercise?
This year has been kind of a disaster health wise, making exercise really hard.  Yes, I can, but it’s very difficult for me.  I walk a lot, at least 2-3 miles a day.  I am starting to do yoga again after a long hiatus for various reasons.
Have you gained a lot of weight from your medication?
Yes, in particular from long term high dose steroids.  I weighed 145 lbs when I started and now weigh 170 lbs after weaning down to 5mg prednisone.  It always takes me years to get back to my normal weight and I really hate steroids.  None of my other meds have caused weight gain.
I heard that long term high dose steroids are bad for you, and that doctors who prescribe them don’t know what they’re doing.  Why were you on them?
This is an example of things I’d rather not project onto the internet, but I get asked this really frequently so I’ll give you a pseudoanswer.  Long term high dose steroids are only worthwhile if the benefits outweigh the risk.  I was sick enough that my doctors and I felt it was worth it.  I do not regret doing it, even though weaning has been a nightmare, because I think I would have spent a lot of those months in the hospital otherwise.  Mast cell disease is not the only chronic disease I have, and the steroids were necessary to control all of the things happening in my body.
I feel bad asking you questions all the time, do you mind when people ask you questions?
Not even a little.
What things really bother you?
Rudeness and unfairness.  I am a very fair person, and it bothers me a lot when I give people the benefit of the doubt and they treat me poorly in return. 
It seems like you still have a lot of friends, how did you manage this?
My friends are amazing people.  I have lost a lot of friends, including some close ones, but I am lucky to still have a lot of very close friends.  A lot of them have been on this journey with me since the beginning. 
Is your colostomy permanent?
Yes.  I decided I did not want to try to reverse it and risk needing another surgery to reverse the reversal.  It improves my life.
Are colostomies common with mast cell disease?
I wouldn’t say they’re common, but I’m certainly not the only person I know who has mast cell disease and an ostomy.  Everyone’s disease process is different, so the need is very individual. 
Was it hard to adjust to having a colostomy?
Not really, for me.  I was so relieved to be able to go to the bathroom that it made the transition easier.  It depends I think on whether or not you feel it improves your life.  Sometimes before I shower, I catch myself in the mirror and see my stoma and it makes me feel kind of weird.  I am also not easily embarrassed and never have been, so the idea that my colostomy will fart in a meeting is not disturbing to me.  It’s just funny.  And yes, it has happened.
How do you know so much about mast cell disease?
A lot of this is having connections to researchers and also having really good journal access.  When I got sick, I spent months learning this stuff.  I took immunology in grad school and always pretty current with recent happenings in that field.  I literally read about mast cell disease every single day.  So I guess I know this stuff by brute force.
You seem very happy, do you take antidepressants?
I take doxepin for its antihistamine properties, but it is an antidepressant, so yes.   I have taken Effexor in the past, but not for years.  I get upset and mad and sad about life.  I just don’t do it all the time.  Having appropriate dosing of mast cell meds helps a lot.  I don’t really have an explanation for how I can be happy other than I like my life and think I’m lucky to have it. 
Can you travel?
We’re about to find out.  After a two year moratorium on air travel, I am flying to Seattle next month with my best friend to visit one of our dear friends.  To achieve this, I have to ship twenty pounds of medical supplies ahead of me.  Literally.  I also would not be able to do this if I did not have IV meds and IV access. 
What’s one piece of advice you would give to someone with mast cell disease?
Don’t worry about it.  It’s going to be fine, and if it’s not, worrying about it’s not going to change it.  I know this is easier said than done, and I certainly worry sometimes, too.  But I honestly don’t most of the time, and maybe that’s why I’m happy.
 
Have more questions?  Ask them in the comments.