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Archive for the ‘Immune Deficiency’ Category

Brief update: Other than a brief 24-hour fever (up to 103.5) this past Thursday, I have been fever-free for nearly two full weeks. Further, there have been no fevers since stopping the antibiotics last night (Saturday)! If I can make it through the next 12 hours sans fever, I will be released Monday afternoon. Monday marks 30 days since I was admitted and brings my total time spent inpatient since mid-August up to 95 days. There have been no real strides made and the doctors have reiterated that I will be back. But we’re trying to make the most of the good days I have and want to celebrate them out of the hospital as much as possible.  Thank you for your continued support, prayers, and love.  

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Like most across this country, I spent my time this past long weekend focused on the multitude of blessings in my life. These marathon hospital stays are survived only through the kindness of the people in my life. It is difficult to know how to appropriately thank you all for what you have done for us. You have inspired and humbled me.

I am incredibly thankful that my body has protected my mind against the effects of the recent medical hell and prevented me from remembering a large portion of the worst. I am thankful that the parts that I remember are mostly filled with good and love and family and friends. And I am thankful that my family and friends fill me in on all the life that I miss while incoherent. Thank you to the family members who leave messages and send numerous texts of love. Just pure love. Thank you so much for all the cards we have received. Your words fill us with warmth and remind us how much we’re loved.

Thank you to all my younger friends who work endlessly on the artwork that adorns my hospital walls. Thank you for the e-mailed iPad drawings and the goodnight videos. Thank you for my school pictures and the drawings which constitute my wallpaper. I get questions and admiration all day. And each little glance from the corner of my eye brings a smile to my face.

Thank you to all our visitors. Despite the fact that I sleep through roughly 2/3 of all visits, you keep coming back. You bring your joy, your stories of the outside world, your smiles, and your laughter. All those good things that help to ensure that the medicine used inside the hospital will actually be effective. Thank you for playing the latest strategy games with us for hours on end. Until security reminds us that we are, in fact, in a hospital. Thank you for spending your lunch hour with me. For stopping in after your own appointments in the area. For bringing me fuzzy socks and solitaire games. Thank you for talking about the latest movies and the book you read last month. Thank you for reminding me that life goes on.

Thank you to the family and friends that help to remind us that time is still passing while we’re still impatiently inpatient. Seasons change and holidays still happen. But thank you for making sure we’re still a part of the passage of time. Thank you to my mom who helped us decorate pumpkins and my room for Halloween. Just days after I had pulled a Halloween stunt that nearly took my life. Thank you to my mito sisters for spreading out the Thanksgiving love and for being thankful with us for three consecutive days.

Thank you to the Bush/Dalton/Mahoney household (aka our “Massachusetts family”) for allowing me and Keith to stay for the four days between major hospitalizations. Thank you for sharing the giggles and smiles of my dear little munchkins. Thank you for the love and the tears. Thank you for being our family when our family is so far away. Thank you Sarah for braiding my hair while I was in the MICU and bringing some beauty to White 9 with your Physics equations all over the white boards. Thank you Liz for crossing multiple state lines to bring some holiday cheer and sending me balloons to keep me company when you are unable. Thank you Stef for holding my hand through it all – even while I hated you deeply as the 108.6 fever made me the most obstinate human being in existence.

We are so moved by the actions of our loved ones. But some of the most moving blessings have come from people I hardly know – or don’t even know at all. From people I have only met in passing. Or from people who know of me solely through common friends or family members.

Over the summer, Keith and I travelled 5 hours to upstate New York to attend the Mighty Matthew Benefit. Matthew and I gathered quite an audience of his school friends and we fielded questions about mitochondrial disease and life with our “tubies.” Matthew told one of these friends that I was very sick and in the hospital for a long time. When Matthew came to visit again, he brought with him a get well letter from his friend included with a school photo. It brings tears to my eyes to know that Matthew is so well connected with so many kind and genuine children in his youth. And to know that those 45 minutes resonated with this child and his life was changed by learning more about mitochondrial disease. Thank you to this young man for your kindness and concern. And a huge thank you to his parents for raising such a proper and considerate young gentleman. It’s so reassuring and touching to that see children like Nate are in this world, making it a better place.

About ten years ago, a young girl named Brittany contacted me. She was a good friend of my very close cousin and had recently been diagnosed with chronic fatigue syndrome. As I was dealing with my own chronic medical issues, my cousin passed on my contact information, and Brittany and I helped each other through a particularly difficult time in both of our respective lives. It helped both of us to have another young girl dealing with health issues. Although we lost contact over the next few years, my cousin contacted me to let me know that Brittany had lost her life in a car accident. Her death radiated throughout her community. I am reminded yearly of the love she left in this world as my cousin’s family participates in an annual run in her honor. This year, I felt her love even closer as her uncle, Geb B., completed his first Iron Man competition with my name written next to Brittany’s on his sleeve. I feel so honored and loved to have a place next to Brittany’s. Thank you.

My mom has made the 3000-mile trek from her home in California to our Boston-based hospital twice since September. The second time she came she noticed that I bring my own pillowcases from home during each stay. A combination of allergies and homesickness inspired this tradition. In order to keep my sanity, I make sure that my pillowcases are the most vibrant and happy ones that I can find. Shortly after that visit, my mom put a call out to the long list of family and friends who have been following our story and this Thanksgiving, we celebrated with the blessings of nearly two dozen different families who sent the most vibrant and unique pillowcases that they could find. In addition, my aunt sent a beautiful ribbon quilt and a family friend crocheted a bright and happy blanket.

I just don’t know if I can say it enough. Thank you. Thank you to everyone. You are all so amazing. Thank you, thank you, thank you. To those who have called. To those who have visited. To those who have sent letters, e-mails, and care-packages. To those who have sent their prayers and positive thoughts. To those who have placed us on prayer lists. To those who have spread my story and the need for awareness about mitochondrial disease. To all those who have shown such compassion and care. Thank YOU.

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This is far overdue. I have done such a poor job updating here (or anywhere for that matter) in the last few months. So much has happened and so much has changed – and is changing, daily – that it’s difficult for me to make sense of it all. It’s difficult to identify the meaningful moments and not get lost in the drudge of daily life. I’ve sat down so many times in the last couple of months to write an update, but the words are difficult to write and the ideas are so difficult to grasp.

As many of you know – and probably an equal number do not – one of my college degrees is in mathematics. The last few months have been scary and painful and disheartening in so many ways – and yet uplifting, encouraging, and reassuring in many other ways – that one of the only ways to make sense of this time is through numbers. Numbers don’t lie or change. Numbers have no biases or inert fear. Numbers just are.

So I figured that if the best way I could make sense of this time was through numbers and that the easiest way to share was through these numbers. So without further ado, the last few months. In numbers:

  • Since August 13, 2011, I have spent a total of 65 days inpatient. That is over 3/4 of the last three months.
  • There have been a total of 5 admissions. The longest admission was 36 days.
  • I’ve spent time on 5 different floors. Six consecutive days were spent in the MICU (Medical Intensive Care Unit).
  • I have encountered sepsis caused by 8 different bacteria and yeasts. Three times I went into severe septic shock. Twice they thought I wouldn’t make it.
  • The highest fever I reached was 108.6 degrees. It resulted in a 6-minute seizure. Other than my typical brainwave slowing (evidence of my ongoing encephalopathy), I don’t have lasting brain damage other than mild episodes of confusion. I am very lucky.
  • During the last three months, I’ve had acute failure of three different organs: my liver, my pancreas, and my heart. The lasting damage, thus far, has been relatively mild.
  • I have been placed on 9 different antibiotics at a single time. They handed Keith a page with antibiotics listed and had him cross off all that I am either allergic to or with which I have adverse reactions. They placed me on all that remained.
  • I have only been off antibiotics for 25 hours during the last three months. I then spiked a fever of 104 and was immediately placed back on antibiotics.
  • The longest amount of time I’ve been home since mid-August is 13 days. The shortest amount of time is 4 days.
  • Today, I returned to MGH – just four days after I was last discharged on November 2nd. Keith had to call 911 as my fever spiked to 104 (from 101.8) in about 40 minutes. I became unresponsive while vomiting bile and tremoring intensely. My heart rate was 180 beats/minute during this time. This is 2-3 times what it should be. I was taken to our local hospital to be stabilized and then transferred to MGH 90 minutes later. I am now stable and my fever is under control, while being monitored closely. My pancreas and liver are showing signs of acute failure. All of my liver function tests are quite elevated and my pancreatic enzymes are over 20 times higher than the upper limit of normal.
  • The recurrent sepsis (bloodstream infection) is caused by two things: (1) an accumulation of “bad” bacteria in my colon which “translocates” (spreads from my colon to bloodstream); and, (2) a severe immune deficiency affecting my T-cells (white blood cells that recognize and remember antigens in the bloodstream).
  • During one of the admissions, I had surgery to removed my gallbladder, which was inflamed and causing pain. During the surgery, I also had a tube placed directly into my small intestine. I now have two tubes on my abdomen: a gastrostomy tube (in my stomach, used for venting) and a jejunostomy tube (a tube in my small intestine, used for minimal medications and “trophic” feeds of 5 ml/hr, 4-8 hrs/day, 3-4 days/week).
  • My “battle scar” from the surgery (an open cholecystectomy and j-tube placement) is 12 cm long and consists of 22 staples. It spans from my belly button to my lower rib cage.
  • Due to my poor peripheral access, need for intravenous nutrition and medications, and rate at which I need to infuse fluids and medications, I can’t be without a central line longer than 2-3 days.Since August, I have had 10 different central lines (semi-permanent IV lines): 1 port-a-cath, 3 IJ lines, and 6 PICC lines.  The longest amount of time I was able to keep one was 15 days.  The shortest amount of time was less than 2 hours.  Port-a-caths can be permanent, PICC lines can usually last 6-12 months, and IJ lines can typically remain for up to 2-4 weeks.
  • In the last three months, I have required 5 blood transfusions due to my extremely low blood counts.  Thank you, blood donors.
  • While I was inpatient, our hometown (North Chelmsford) received 6-8 inches of snow and 80-90% of the town lost power. Our apartment lost power for over 48 hours. Luckily, we were unaffected as we were still inpatient. When I was discharged and home for 4 days, we still had 1-2 inches of snow. It made me smile.
  • My mama traveled 3000 miles to come visit me while I was in the hospital. Twice. Definitely a “Best Mama Award”-winner.
  • Other than the nights that my mom stayed with me, Keith spent every night by my side. 58 nights.
  • The strength gained from the thoughts, prayers, and love sent and felt by our family, friends, family friends, acquaintances, and even complete strangers has been immeasurable. We remain strong because you fortify our strength. Thank you.

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In the depth of winter, I finally learned that within me there lay an invincible summer. ~Albert Camus

Sometimes those of us with chronic illness get so caught up in the can’ts. The shouldn’ts. The mustn’ts. I decided to focus this summer on the things I can do. From embracing the lives of loved ones lost to adventures in lands (not-so-)faraway. So we celebrated, joined, conquered, ventured, traveled, laughed, cried, and smiled. And we experienced life. It didn’t matter that I did most of it hooked up to at least 3-4 tubes at a time. It mattered that I did it.

Now that the summer comes to a close (thank you, labor day), I thought I’d go through the tradition of writing the back-to-school essay “What I did this summer.” Then I rethought. And decided I’d show you.

What I did this summer:

{And wished her Happy Birthday}

{At the 2011 UMDF Symposium in Chicago}

{With my Great Aunt Arden at the Sears Willis Tower}

{Lab safety is a must while tutoring}

{Sisters Day Out – Southwick Zoo}

{First time at Niagara Falls – Maid of the Mist}

{Seneca Falls, NY – With two of my most influential people – On the bridge from one of my favorite movies, It’s a Wonderful Life}

{For the first time since my license was revoked for seizure activity}

{TPN bag, enteral tubes, and all!}

{White Mountains in NH with my favorite in-laws}

{Haven’t attempted this since I was a gymnast – take that mito!}

{At the 2011 Mighty Matthew Benefit in Plattsburgh, NY}

{We love you, Princess Eithene!}

{So blessed to live in Boston, the Medical Mecca of the World!}

{With 4 adults, 2 kiddos, 2 dogs, and a partridge in a pear tree}

{Disappointing from the weather standpoint, but fun nonetheless}

{With a grand closing to a grand summer!}

And no one can say I didn’t do. Because I did. I really did.

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There is nothing like looking, if you want to find something.
The Hobbit, JRR Tolkien

I had to look back at my last post (which was just six short days ago) because so much has transpired over such a short time. Although there were some frightening periods, we have much to be grateful for. The most important is your support. It means so much to me to read notes of love and encouragement. I could not make it through those difficult times without it. Thank you, thank you, thank you!

I’ll try to go through looking and the finding of the last six days as methodically as possible, but there’s much I don’t remember, so I apologize for the holes.

Just minutes after I posted on the latest saga with the positive news that my fever broke, I spiked another fever at 102.3. Despite 2 different antibiotics. Despite round-the-clock Tylenol. Although the fever was miserable, it seemed the treatments finally took hold on Tuesday morning and I went fever-free for just over 24 hours prior to discharge. During that time, we saw the Infectious Disease (ID) doctor who told us the bacteria grew out as MSSA, or Methicillin-Sensitive Staphylococcus Aureus, which is far more favorable than the nasty MRSA, aka Methicillin-Resistant Staphylococcus Aureus. We thought we were in the clear. Although he clearly still wanted my port removed, I was able to doe-eye the hospitalist into allowing us to try to save it so that I could swim and enjoy the last three weeks of summer. We got our discharge papers on Wednesday afternoon and my home health nurse met us at home for a three-and-a-half-hour review.

(more…)

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I’ve been home for nearly two weeks now. Recovery has been slow and has sapped me of most of my excess energy so many apologies. Because there’s so much to cover – and I’m having difficulties organizing my thoughts – we’re going to go about this bullet-style.

  • I’m home. I was discharged with a four-week course of IV Vancomycin (a very powerful antibiotic) at a very high dose. Because we never got those sensitivities back (i.e. what bug we’re fighting and what antibiotic is best to fight it) and my reaction to the infection scared them (though I assure you, it did not scare nor surprise me or Keith), the doctors are playing it safe. I guess that’s what they do best. I was also on Cipro (another antibiotic), but I have finished that course.

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Considering I am currently back at MGH for a central line infection (which is being treated as sepsis due to my plummeting blood pressures and skyrocketing temperatures and heart rate; this could, however, be due to my really screwy autonomic nervous system), I thought I’d address the issues we’re currently encountering with my current former central line (a PICC in my left arm) and the debate over what kind of line is most appropriate for the long term use we’re envisioning.

How did we get here from there?
My GI tract (from stomach to colon) is essentially paralyzed. After numerous tests and a 25-day hospital stay when it officially shut down, a GJ-tube was placed. Five days later, I was readmitted partially because the GJ-tube was not providing enough of the necessary nutrients. The solution was to place a central line (a peripherally inserted central catheter or “PICC”) so that I could receive calories and vital nutrients intravenously. At this point, the PICC and TPN were planned to be short-term aids until we could build up the feeds to my jejunum (the middle part of my small intestine). A couple months later, my GI doctor said it looks like TPN is the most viable long-term option for me.

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When it rains, it pours.

It often seems like all the bad stuff gets clumped together in one big barrage of awfulness. This hospital stay is surely no exception.

First, we’ve dealt with a few episodes of dystonic storms. These are often triggered for me by exhaustion, physical pain, and exertion. When these three things are combined, the storms are worse. Dystonia is a movement disorder that causes involuntary movement and posturing of different muscle groups. I’ve suffered from dystonia in my left side for about 9 years. Recently, the storms have caused me to have bilateral leg involvement. It’s painful and the treatment often given cannot be used on me due to the underlying mitochondrial disorder. In short, it sucks.

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