Upcoming Debulking Surgery with Dr. Spencer

"Sooo...this is the moment where I could look back on my life and say this is where it all went downhill, the great demise began..." I asked, my gaze meeting hers. "Yes." She said.

DEEP. FUCKING. BREATHE, ARI, DEEP. BREATHE.

"Okay, papers please."

"You are absolutely sure?

"Okay, i'm ready to sign."

I signed because despite all the complications she described, it was that or stay at the state I am at now that will only worsen and will lead to no mobility and being dependent on pain meds. So whatever happens from here, I am choosing my best shot at a FUNCTIONING life. For me that does not entail hopping from bed to couch and hallway to bed... that is no way to live for me. I am going to be an OCD specialist, and the best damn one in my field someday, just you wait and see ( : and if not, I died trying to obtain something I loved and believed in to the core. And, I may even get to look fabulous in a pair of heels (sp?) doing it ; ) I would be lying if I said I was not riddled with anxiety over the procedure, however.

Dr. Spencer will be doing a debulking surgery on my ankle and foot on January 26th.

Below are just a few pictures from my hospitalization late December that I promised to post a few posts back. I was hospitalized for Cellulitis in my buttock/thigh region. Sending you all so much love! - Arianna

Confessions

I was hospitalized this past week for a Cellulitis infection. Although, I do not feel as though I can call it that accurately without adding there was some controversy as to what it actually was from the Klippel expert himself, Dr. Fishman.

He will tell you he “simply doesn't know” in terms of whether or not it was a Klippel valve bleeding into another nor an infection. All I know is that I was on the phone with the VAC Clinic Friday with a dear VAC nurse I have known for quite some time now. She told me if it started to spread (the legion) or (got worse) than I could page the surgeon on call or come in. Unfortunately, things progressed in an unfavorable manner and the red, aching legions spread rampantly Friday night. I asked every person I met in the emergency room that night whether or not they were associated with Dr. Fishman and if they were, whether or not they deemed this an infection worthy of antibiotics. None of them hesitated in saying yes, we need you admitted and to treat this “infection.”

I escaped the hospital scene Christmas Eve and was elated to leave the downtrodden venue. There was one goodbye I did not say upon leaving though, it was too hard and I could not submit myself to it right then at an emotional level.

While there, I met a girl who was a Klippel Patient struggling for her life. We had contact prior, but this was our first time having physical contact. She still is inpatient, actually, and tells me she will never be released... One of my least favorite nurses I've ever had would not allow me to go visit my ailing friend a mere three floors beneath mine. Generally, I respect nurses and their word and would not forbid it. I explained to her the urgency of the situation and still, she resisted understanding my plight in a seemingly spiteful manner. She kept saying there was a person above her in the system she had to ask, as though she were referring to some vague deity... Nurses prior to her and nurses after her would instantaneously respond yes when I would ask to visit the ICU to see my sickly friend, but this one was different... Perhaps she lacked emotional decorum so vital to someone in her profession that I should feel bad for her... I hope to never have her again, she was uncouth in her actions and lacked sympathy.

I kept asking her, and finally she told me that the answer was no, I was to forget about it. I phoned my friend and spoke with her sister and told her what my nurse had said. Still, that would not be enough to contain me. Well, this nurse had 3 or 4 patients to deal with simultaneously and not enough time nor resources to keep me on lock-down. So, I snuck down to ICU to see my friend where I was greeted by my friend's ICU nurse that day. She was so kind that I had to disclose to her what me being there in that moment really entailed; lies, sneaking off my floor, and a seemingly spiteful nurse who would probably kill me if she found out where I was. 

I prepared to get a good verbal lashing in return, but the opposite happened, much to my surprise. “Thank you,” her nurse said. “Thank you for being here. She got so excited when she found out you were coming.” We shared a smile and she helped me conjecture a plan in case I were to be caught by my nurse; I could not believe the lengths she would go to in order to help me see my friend! Her heart was certainly in the right place, and she knew that treating my friend did not just need to happen in a medicinal manner... That's what separates a nurse from a good nurse, though, and I am proud that I did not let my vile nurse that day hinder my efforts. I never got caught, by the way. ( = Normally I would not brag about rule breaking but this is indeed an exception to the rule!

She slipped me into the plastic yellow protective gear one must wear while visiting a friend who is on precautions with outsiders. I was well-acquainted with this gear as usually I was the one on contact precautions. I gladly slipped into the yellow suit, mask, and gloves for a chance to see my friend yet again. She was unable to move, and her breathing was as unsteady as could be. In between breathes, she managed to speak her depression to me. She told me that “she was planning her own funeral” and burst into an explosion of tears. Where words failed, my hand didn't. I immediately reached out my hand for hers, and latched on tight. I don't know if there is much else in which I am willing to share of that moment...but damn, at that point you realize there is more at play here in life; there is more than that dress you want but can't afford, than the number of tiles on the ceiling, than how many pounds you lost in the weeks prior... I have had that realization prior, but sometimes the feeling wears off and one is reminded again...

Not many people will understand it, and seeing someone so close to their end demise (for me) is much different than coming back from those times where I almost died. Perhaps because I am at a more alert level...

For any reading who question the validity of this story, I can easily tell you the patient's name and where to find her/her family online but I won't. The Doctor's at Children's would know that at the very least I am speaking of a real patient for I spoke/wrote to them about her while inpatient and they are the ones treating her. I was, however, met with blank stares and non-relies as they are under confidentiality when I tried to ask what more they could do for her. She is a very difficult case for them right now. And, of course, the nurses reading it would know what happened. I am not worried about the validity of my story, though, as much as I am for the patient's well-being. Notice how I emit details regarding people's appearance...for my goal is not to cast trouble or assign blame upon anyone. Her fate looks bleak and if there is a higher power, I call on him, her, it to heal her.

These are my confessions, for now. And I'm not sorry. I would do it all over again, for sometimes breaking the rules means being a better human-being and those dictating them simply cannot empathize with that.

I'm home, albeit in a ton of pain physically and emotionally. I feel so trapped, which is why I write. When I write, I have a chance to tell my truth, and perhaps it can help people to understand me better. I still believe that some of the best stories, though, are the ones untold...or the ones that are happening between the lines.

In case you have not yet come to figure this out, this blog is greatly uneven in content... I write very little about Klippel itself and more about the emotional journey it has led me on. KissesforKTS is more than just about garnering awareness of Klippel itself, but all of the components that come attached with it....

I will post some pictures of the hospitalization in my next post.

LOVE to you all,

Ari

She's Alright Now

I have a great deal in which I yearn to share, but am not quite sure where to begin. Therefore, I will just follow the waterfall of emotions in my mind and see where this particular post takes me...

It was very much so like any other day of surgery, with mom and dad and a bag of essentials in tow as I entered the hospital. For the second time since it had been instated at the opening of summer, I was placed into the IR waiting room prior to my procedure with my interventional radiologist. I was somewhat accustomed to these “peanut procedures” now with this particular doctor, and was greatly anticipating this one because of the bout of pain I had been experiencing the entirety of summer. October 1^st, in my mind, could not get here fast enough. Pain, as many of you know, is a vile force to be reckoned with, and I was tired of not being able to sit comfortably. The only comfortable position in which I could muster up on any given day was that of lying on my stomach. Not to mention, if I ever wanted to have a sex life of any sort, this surgery would be undoubtedly be instrumental in helping me to do so.

With that said, the morning of the surgery arrived and I kissed mom and dad goodbye with a mere peck on the cheek as the staff wheeled me into the operating room. I was not scared in the slightest, I had done this “process” (so to speak) too many times before. Not to mention, my trust was placed in the attending performing the operation. He had operated on me in much more dire circumstances prior for several years. For me, this surgery was almost symbolic of a new awakening; one in which I was addressing a problem that had been bothering me for quite some time now...not just on a physical level, however, but an emotional one, too.

I awoke in the ICU with my oxygen level in ruins; apparently, I had a horrific allergic reaction to morphine while under and some fancy word (relating to my breathing level) had dropped incredibly low. What was supposed to be perhaps a 1 night stay in the hospital turned into an 11 day one. I was dissatisfied, but I do not remember the vast majority of what I went through (thankfully). Nor do I blame any of the staff nor anesthesiologists, as their skilled handiwork is what got me through that operation.

I am still, however, having a hard time coming to grips with the fact that that operation could have been my last day on Earth due to the unfathomable allergic reaction I endured while under general anesthesia.

I will write more before long, however for now I am off to retire to bed. Since returning home in mid-October, I have been dealing with a great deal of other life matters (some pertaining to my Klippel, others not). This, I find, is a rather confusing time within my life but I am trying to embrace it with both arms (with the assistance of my family and friends).

I'm Back

Hey, everyone. I hope you are are genuinely well. I apologize for not updating for quite some time; I've been on a bit of a roller-coaster lately in terms of my mental and physical health. My last surgery did indeed take place, but went horribly awry in some aspects. It was nobodies fault by any means, but it turned into an 11 day inpatient stay at Boston children's hospital as opposed to that of of maybe day surgery or 1 or 2 days...

Writing is something I have been incredibly passionate about for several years now; the entirety of my life I have felt misjudged by the vast majority of my colleagues in a slew of ways. With writing, however I get to reflect and present myself in a way that I am too awkward to do so in a verbal manner...that, in itself, is incredibly soothing and cathartic to me. I also write about issues

I am exceedingly passionate about, like having Klippel-Trenaunay Syndrome and the ways in which it has impacted my life thus far. Mental health is another topic readers will see me discuss frequently; why does it take Robin Williams (May God rest his soul) killing himself for depression to make headlines? While many were aghast that such a loved man could not find strength to hold on, the truth is this disease impacts mere mortals everywhere. 

I look forward to sharing some of my experiences with you as my far as my Klippel, mental illness, experiences at CHB, and other personal matters are concerned. You will never see me holding a scalpel or be behind the laser of a machine that has helped save my life numerous times, but I hope to save lives in a different way. That is what envokes a burning flame within my soul. I have found my passion throughout the past several years, and despite my obstacles I hope to achieve them. 

In the meantime, though, I do believe in the kindness of strangers as the Klippel family has kept me afloat on many of my off days. They are no longer strangers, though, but treasured friends despite where they end up on this sometimes rather unpredictable, jagged journey.

When I started this post, it was my first time writing since my last one. I was not even quite sure I would be able to construct a simple sentence let alone write in paragraph form...here is to hoping this was somewhat edible in content. 

I greatly anticipate writing more incredibly soon,
Ari

Post-Op Day Two, Several Pictures Included

Yesterday, I had my miniscule procedure with Dr. Alomari from interventional radiology done at Boston Children's Hospital. The focal point of the procedure was the ankle and foot, both of which are still in a extraordinary magnitude of pain despite the medicine in which I am on (10 mg, Oxy). In sum, he injected three different areas with medicinal needles, including the ankle, foot, and thigh. Just prior to the procedure, I inquired to him about potentially working on the thigh area as well (it has been leaking a substantial amount of lymph fluid in recent times). So (despite his grueling schedule that day), he graciously agreed to inject that area as well (as opposed to c02 lasering it, which we have done on that particular area in the past).

Prior to the operation, I was required to have a series of pulmonary functioning tests done. The was my first time ever undergoing this specific series of tests; the reasoning behind the testing was due to one of the chemo medications in which Dr. Alomari was going to be injecting within me. Even though the amount in which he injected was incredibly bijou, the testing was still a hospital requirement as a mere matter of precaution. Below are three pictures mom took while I was undergoing the testing; I had no idea she was even taking them until she texted them to me later!

Pf Test

Me and Katelynn after surgery yesterday

Now, onto some of the more personal details regarding the day... I awoke feeling a great deal of anticipation, keen to have the procedure done with as I could hardly tolerate the pain any longer. The throbbing and burning sensation throughout my foot and ankle was momentous in nature, and I had no abatement from the lidoderm patches the night prior...much to my disappointment. Just before the procedure, I was greeted by one of my favorite hospital staffers to date, Jenn from hematology. I first met her several months back prior to starting the Sirolimus. She is so incredibly kind in nature, and such a truly thoughtful person. Even though I am no longer one of her patients, she still took time out of her busy day to come by and (literally) hold my before I was taken into the procedure room. Tomorrow, I will be meeting with her and Dr. Trenor at 9 a.m. To devise a plan about safely resuming the Sirolimus sometime in the near future (more about that in a future post). I am quite fond of Dr. Trenor as well, and feel incredibly lucky to have such a world renown hematologist as part of my staff. The way in which I see it (based upon several testimonials I have heard from chronic illness colleagues throughout the past several years), a Dr. who is good at his craft is a dime a dozen. However, to have a Dr. who is both empathetic and skilled in his/her specialty is, well, incredibly rare. I can truly say that all my Dr.'s ooze both empathy and knowledge, which is why I continue to trust them with my life and will always speak highly of them.

Me and Dr. A right after surgery

Dr. A and the amazing Cindy K. from interventional radiology. Love these two!

Picture of thigh area right after surgery!

Me in recovery room following surgery

Before I get too off topic, though, there are some shout-outs in which I would like to give! One is to Jose, the interventional imaging specialist who has worked on me within the past when I was vehemently ill. He so good-kindheartedly stopped by the waiting room just prior to my procedure to say hello, something in which he has done in the past as well. He is such an incredibly kind man; if you are reading this, Jose, thank you for the visit and all of the graciousness in which you have shown me in the past! Also, I would like to say hello to Pradeep, who is training under the skilled hands of Dr. Alomari. Everytime I see him he is so refined in nature and genuinely seems to care about his patients' well-being... I have no doubt that he will be a patient favorite one day and that he will continue to excel at his occupation as learns from Dr. Alomari. Also, a major thank you to Cindy for all in which she has done for me over these past several years. Whether or not she realizes it, she has truly been such a calming presence throughout all of my anxiety driven episodes and a pillar of strength. Aside from this, she is so enlightening as a nurse practitioner and overall person in general.

Upon awaking from surgery yesterday, I noticed that my post-operation nurse was actually a familiar face...it was Katelyn from 10 North West (the surgerical floor in which I am nearly always hospitalized on when inpatient). Turns out, she made the transition from floor nurse to post-op nurse a few months back. Although my memories from after the operation yesterday are rather hazy, I recall how lovely it was to have her as my caretaker again. She is so sweet! If you are reading this, Katelynn, thank you for all of your good care in the past, it was a pleasure to see you again yesterday (at least given the circumstances!). Also, a dear friend of my family (Jodie) and her sister-in-law surprised me with a visit just prior to my operation yesterday. Her son, Ben (a friend and fellow Klippel patient), just had a major operation done and is currently hospitalized across the street from Children's Hospital. Despite all of the post-op stress in which their family has surely been enduring, they so generously came to greet my mom and I. It is people as endearing as them who truly who inspire me to be a better person... they are always thinking of others despite their own plights. Please say some prayers for my friend Ben as he continues to recuperate from his operation and regain some of his strength back. Wishing you nothing but the best, Ben!

Adorable stuffed animal the beautiful Jodie and her lovely sister-in-law brought to me just prior to my procedure!

And, as always, thank you to my other friends and family for being there for me through thick and thin. As I have said in the past, having a chronic illness is truly a blessing in disguise... It has aided me in helping to realize who my true friends are throughout the years! It is easy to be there for people throughout the good, but to continuously be there for them throughout their trials and tribulations is another... well, that is what being a genuine friend is all about!

Thigh area today, no flash

Thigh area today, flash

Love you all so much, and will update soon!

Lots of love,

Ari

Put On Your Combat Boots

While inpatient, I could almost never sleep at night despite the cocktail of drugs I was on (which often included rather hefty dosages of oxycodone and clonazepam). Feelings of loneliness, guilt, and shame were some of my closest companions, and they liked to keep me awake for considerable hours on end. Sometimes, (if I were feeling strong enough bodily at that point in my recovery), I would weasel out of my room in my wheelchair around the 4:30 am mark. Mom was always just mere feet away from me fast-asleep, and I'd manage to exit the room without waking her (as was my goal). I'd push myself to the elevators, and maneuver myself to the downstairs lobby. Along the way, I would run into various familiar staff members and we would exchange polite greetings. Often, they would look at me in disbelief and say something along the lines of, “you're still up?” Despite the emotional storm brewing within me, I would often just respond to their remarks with a coquettish laugh that made it seem as though I did not have a care in the world. I would then proceed to sit outside for about an hour on end watching the doctors and nurses make their way into the hospital through the ER doors for rounds and what not.

I would be lying if I told you I found the sight of them anything other than phenomenally inspirational... Often a time, I felt as though I was a useless, miniscule discoloration present on Earth, despite all of the love and affection that was perpetually shown to me by friends,family, and medical staff alike. Still, I could not shake that substantial feeling of fruitlessness; it seemed as though my existence was only cause for chaos... rather that be to my family, friends, or doctors. None of them had done anything to make me feel this way, quite to the contrary, actually. Yet, what purpose was I serving spending the majority of my life in a hospital bed trying to tame a chronic condition that seemingly had every intention to demolish me despite several methods of medical intervention?

However, seeing the various medical professionals make their way into their personal playing arena incited great hope within me; it made me believe that perhaps one day, I too could go to work on a timely schedule and help make a difference within somebody's life. I believe that on the vast majority of nights, this is what gave me the willingness to continue my fight to get better, despite feelings of impending doom and a body that constantly said otherwise.

These professionals were people who had worked long and hard to achieve their goals, and they were constantly willing to learn. Often a time, they contained the whole package; not only were they competent in terms of their specialties, but they were empathetic and seemed to have a thriving personal life outside of the hospital. They, indeed, were perhaps one of my strongest motivators to recovery; of course, like all human-beings, I could assume they too had been met with several challenges along the way to their successes. Yet, here they were, day after day, walking into work ready to perform their duties and learn.

- Arianna

I needed a reason to belief in a greater purpose for my own life, and they helped give me just that. I was cautious to have myself back upstairs by the usual time of surgical rounds, and by then my internal attitude would experience a rather large shift...I felt inspired to begin yet another day of combat.  

Dying on The Inside: Depression

“I mean it, I want to die because I cannot take this anymore,” I texted to my sister as tears paraded down my lethargic face. It was 6AM in the morning, and I was sitting in a dark, abandoned hallway adjacent to the 10 North West Unit at Children's Hospital Boston. A swarm of tears cascaded my tear ducts, and I could not get them to cease; if I'm being brutally honest, I may not have wanted them to, either. Sometimes, after crying for a long while, I actually find that I feel a little bit better. There is a scientific reason behind this that I learned a few years back: When upset, your body gathers a plethora of stress hormones that cause you to feel lousy inside. Upon crying, the stress hormones are hidden within the tears. Therefore, they are being released from the body. No such luck this time, though.

I had been crying for nearly two hours now in the same obscure, forsaken hallway. I had called my dad earlier, but he could only talk for a few minutes because he was stuck in a work meeting. I choked through my words as I spoke to him, they weren't so much words as they were syllables. He could barely understand what I was saying because I was so inconsolable that I could barely manage to pronounce a single word without sobs overshadowing my words. My mom, meanwhile, was dozed off in my patient room located on 10 South. I continued to text with my sister as she prepared for school that morning. I was, at this point, seemingly devoid of all hope. Sobbing in the empty hallway seemed to be the only secure place to cry, as I did not want anyone to overhear me and catch onto my despair. The last thing I desired were nurses or doctors all over me because of my elevated depression and anxiety. I didn't feel as if there was anything they could say or do to make me feel better while there. That, however, is the very essence of how depression works. It wants to keep you isolated, to make you feel as if there is no way out. That's not true, no matter how much it may seem like it at that point in time. In fact, in moments like these, it is most important to reach out to somebody you trust wholeheartedly. Whatever you do, do not sit alone with your depression. It is, indeed, okay to admit that you are not okay.

I felt so incredibly lost; as a young child, I never thought much of my live as an adult. Perhaps that is why I was so astonishingly happy at the time despite all the medical chaos I endured as a juvenile.. I lived in the moment, instead of dwelling in the past or being terror-stricken over what may happen in the future. Even recognizing this, I could not seem to change my ways nowadays. I was brimming with despair and self-loathing, and felt as though I were not worthy of good things coming my way. I hated myself. In fact, I still dislike myself a great portion of the time. I either feel ugly on the outside or on the inside, usually simultaneously. It's quite terrifying because when living a lifestyle akin to this one, it is important to be one of your own best friends. 

Depression is so purely manipulative; from living with it for several years and majoring in it at school, I knew that. Still, I could not seem to move past it. I was on antidepressants that helped a great deal with my OCD, but still left much to be desired in terms of my depression. When I was not in the hospital, I was meeting with my doctor and talk counselor at least once a week. I had just started seeing them over the summer season; what made them unique to other people I had seen was that they actually specialize in working with patients who have chronic illness. I would suggest that any and all patients dealing with this combative disease see a talk therapist at the very minimum. Fight for the help you need and deserve. Don't every allow anyone to make you feel inferior for seeking it, either.

Did you know that 1 in 6 adults receive some type of mental health care in any given year? There is no shame in doing so, and yet our society still stigmatizes people who suffer from mental disorders. Indeed, there is a high (or rather positive) correlation between those with chronic illnesses and mental illnesses like depression and anxiety. When you live your life in a constant state of pain, it is easy to fall into negative thinking patterns. Which is why, according to the Cognitive Model of Psychology, it is adamant to adjust internal processes.

Later on that night, I visited my old nurses on 10 North West. I had seen them earlier in the evening, but it was so busy that we barely got a chance to talk and catch up like we usually do. So, I went back to the main desk to see the nurses and desk staff. At this point, they feel like good friends as opposed to workers. They all hold a special place in my heart as they have seen me at my absolute worst and helped nurse me back to health. We've exchanged countless stories about our personal lives, and somewhere along the lines they became more than just staff, but friendly faces as well.

“You always have a smile on your face,” said one of the girls. “You have such a great attitude despite everything.” "Not always," I laughed softly as I stared down at my feet. I felt a smile widen across my face, but inside I knew it was a facade. All in which I was trying to do was keep it together, to not fall on my knees and start uncontrollably sobbing in front of the staff that was there. 

“But thank you,” I replied. “You guys are honestly the sweetest.” I meant what I said, but the smile on my face was in deep contrast to my dying insides.  

I think it's not so much about wanting to die, as much as it is just feeling as though you cannot handle the burden of the physical and emotional pain anymore. You feel as though you are drowning in tumultous waters, and answers are far-fetched, if they even exist at all. Still, you need to fight to stay afloat until that lifesaver reaches you. I promise in the end it will be worth it, although it may seem like all is lost in the moment. Just keep pushing through, and you will be alright. Just keep pushing.* - A

To Sit Comfortably, Once Again

Hey everyone! Since I last posted, I have been discharged from the hospital, come home, been re-admitted, operated on and discharged once again. And this is all within a week's time period!

It's true, the life of a KTS patient can be absolutely insane and anything but ordinary. But, as I continue to shovel my way through this difficult passage in life, I am coming to terms with the fact that almost everyone has been dealt an unlucky hand of cards in some way or other. This is just mine, and it is prepping me for the future in which I hope to help others and possibly inspire them. I guess it is not so much the problems in which we are handed, but the way in which we deal with them.

For now, I will give you guys a somewhat brief update on what has been happening with me medically.

I got discharged from the hospital this past Friday in a great deal of discomfort. The MRI showed some cyst areas that correlated with the places in which I was having pain. So, since every medication I was taking was done orally and my vitals were under control, the teams decided it was viable for me to go home for the weekend. Then, I would come back on Tuesday and have the areas operated on by my beloved Dr. Alomari. When I had the MRI and I was in a large amount of pain inpatient, he was out of town for a couple of days to attend a medical conference. I was freaking out, absolutely petrified that I was at a dead end and that if this issue couldn't be fixed , I may never again sit comfortably on my butt and/or thigh again. I already have a great deal of problems standing for more than a few minutes of time, and sitting was often the only relief I got from that pain. Now, it seemed possible that sitting may no longer be an option. Inside, I felt so incredibly lost and it seemed as though all my hope had vanished.

On Tuesday, prior to the operation, Dr. Alomari came into the room and we talked things over. Aside from the pain, I also had some deeply embedded vesicles that had been leaking lymph fluid and blood for several weeks. I had not mentioned them much before as I was just hoping they would merely close off and become a distant memory. Unfortunately, they persisted with great vengeance, constantly leaking through all of my pants throughout the day and evening with little to no breaks in between. So, at the last minute, we decided to do C02 laser to eradicate the stubborn vessicles which could act as a portal for infection.

I woke up from the operation in great shape - I was walking, talking, and even laughing! Of course, some of that powerful pain medication eventually wore off later that night and I felt some pain. Nothing too overwhelming, though. In fact, the only area that is sore is the thigh area in which I had the C02 laser done. The rest of the areas have improved dramatically! I am sitting solidly on both butt-cheeks while writing this, barely on any pain medication whatsoever. I have not been able to sit like this in months. I feel so blessed to have the amazingly talented Dr. Alomari and his fabulous staff (shout-out to Cindy, Dr. Alomari's "right hand" as I call her, for always having been there for me through all of my various moods) working on my behalf, as I have now regained some sense of normalcy!

I will leave you guys with some pictures below from the past few days. Love to you all and thanks so much for all the kind wishes that have been coming my way; I could not have asked for better friends.

Right before the surgery Tuesday morning. Inside, I was freaking out but I tried my hardest to maintain my composure.

A picture of the C02 laser from a day ago. This is part of the area on my thigh that had the deep vesicles bleeding out. 

This is from yesterday, on my way home from the hospital, sitting comfortably in the car!

Finally, it seems as though I may be home to stay for a little bit (barring any complications). Here, I was snuggled up under a large blanket on my living-room couch. I fell asleep for a few hours right after I took this. Sleeping in the hospital is often hard for me to achieve, so I was utterly exhausted!

- A

Round and Around and Around We Go...

Seems these are rather tumultuous times for me and a multitude of my Klippel sweethearts. Many of them are inpatient at present; as of today, I am too. Once again, I am fatigued emotionally and physically. As for what I am being treated for, I cannot say with total certainty at this point in time. We have yet to rule out infection/deep clot/both. The good thing is I was able to catch it before I got systemic...which very well may be attributed to the 26 day intensive antibiotic regimen I have been on.

In other words, that may be masking things from getting out of control.

In my last post, “last evening”, I chronicled my initial flare-up that led to this hospitalization. While the overt systemic systems died down at rapid speed, I experienced yet another problem this morning. I awoke around 1 in the morning to a throbbing in my thigh that felt cellulitic (based on previous infections). Several hours later and the pain persisted. Between the butt and the thigh both being in abnormal pain, I felt it adamant to be seen and now I am here yet again. There is no striking redness, although there is a feint tint of some.

While I am not one to ever speak poorly of the beloved hospital that has saved my life dearly so many times, I must say I was not impressed with a couple of the vascular anomalies staff members today. I will not mention names, nor go into all of the specifics, but I was talked down to quite a bit (as was my mother) and even toyed with mentally to a degree. I am not referring to the specialists themselves by any means, but rather a couple of the people who work beneath them. Both who mistreated me (especially knowing the complexity of my situation these past 6 or 7 months) lacked empathy and compassion entirely. I just have to remember that I am indeed here for the specialists who are so expert at their crafts.

For now, pain medicine awaits along with bedtime. It was another 8 hour seemingly endless day in the ER prior to being placed into a room.

As always, love to you all - A

Hello Again, Hospital = (

Hey everyone! I am, as some of you may already be aware, back in the hospital yet again with another cellulitis infection. Here is how it all started: I awoke Thursday afternoon with an intense pain that spread from my back down the middle back of my KTS impacted leg. It felt as though someone were skinning that part of my body alive; it was as though someone was carving into me with ten knives throughout the area. I have had that feeling many a time before with cellulitis, but this time the pain level was so amplified that every passing moment felt like sheer torture.

My dad drove me into Boston immediately, where it was proven through my WBC that I was indeed infected. I already knew I was, but unfortunately in the emergency room you often get doctors who are unfamiliar with your situation. I, however, know the feeling of infection so well that I trust my intuition when things aren't right. Shortly after arriving to the hospital, I sparked a 39.3 temperature, and my heart rate was down into the 80's. The redness came with vengeance, albeit after hours of being in the emergency room. I was told the blood cultures were already growing back a bacteria; this was rather odd to hear as I have only grown something back twice in my life before. In fact, I think Dr. Fishman said I was the only one of his KTS patients who has grown back any specific kind of bacteria. In a way it's good, as it tells you exactly the bug that needs to be treated. However, it also proves the infection is in my bloodstream, which is rather frightening. I have had sepsis several times before, and of course it is incredibly trying... However, this infection is by far the most painful and stubborn one I have had thus far. By the way, I grew back Strep B Gram Positive in my culture.

There is so much more I want to tell you all about this infection, but due to how fatigued I currently am, I will continue this post either tomorrow or the next day, Wishing you all nothing but well! The only blessing about this hospitalization is my incredibly strong and brilliant KTS friend Rachel is here on the same floor as me and her lovely mom is staying with her. It is so nice to be able to get to see them while here, they truly are such brave, beautiful people. However, Rachel is going through a tough time herself so I would love it if you guys could open your heart to her and say some prayers.

My eyes are starting to shut by themselves, so that's my signal to go, for now at least. I look forward to writing more soon.

Until next time, - A xoxo

In A Moment's Notice (Part 1)

When I last wrote, things were seemingly getting better; albeit slowly. Still, everything was being managed just fine and the infection appeared to be responding well to intravenous treatment of antibiotic. Then, I woke up (while still inpatient) with the chills; it was previously planned I would go home that day... prior to the events that were unfolding at a moment's notice. Suddenly, without even the slightest of warning, I was wandering the vast mountains of Antarctica in nothing but a tank top and shorts. There were not enough blankets in the world to contain the numbing cold that occupied every crevice of my body. My temperature shot up to just under 104. It seemed the infection had progressed, and there was certainly no question now that it had entered my bloodstream. The usual people were involved in treating me, including Dr. Fishman, Dr. Alomari, infectious diseases and the allergists.

The next few days were utter hell as we fought to control the infection. I couldn't get out of bed to use the bathroom; when I stood up, it felt as though I was in a wind-tunnel. Standing would simply not suffice, for I could not maintain balance of any sort. Bed pans were a staple throughout this time period. Even getting my shivering body up on the bed pan was a challenge in itself, especially given where the infection was located (my back thigh). My energy was tossed completely, and lifting my head was something that seemed to be a tremendous feat.

The only time I would start to feel any relief was when the oxicodone, morphine,and moltrin were administered and started to kick in. Eventually, though, my fever would progress back to its just under 104 state, and the unrelenting chills would return along with the dizziness, nausea, intense headache, all over achi-ness, etc. Not to mention the thigh infection itself... My body wasn't done surprising me yet, however. I woke up the following day with two huge clots, one in each arm. They as well were infected. I wish I was joking. No doctor had ever seen anything like it before and are still having difficulty grasping the concept of how this may have occurred.

When people see my leg, they think it is just my leg that is impacted by this lovely condition we call Klippel-Trenaunay Syndrome. Fair enough, but for those of us who know better, we know that is far from the truth. We know it can get into our bloodstream and cause sepsis, travel to other realms of the body and wreak utter havoc. We know better, unfortunately.

I returned home from the hospital yesterday afternoon and will be writing a lot more about my time during my ten day stay. My KTS family as always pulled through for me and were the most incredible support group I could have asked for. Never could I have imagined people with such endearing hearts and souls being there for me day and night, that which I am incredibly grateful for. Think of this as somewhat of an introductory post.

My KT loves, whether you are a family member of a patient or a patient yourself, may you be at peace right now. If you are not, may your pain soon subside. May you know you are not alone and have a bundle of people in your corner at all times, no matter how isolated you may feel from the outside world at any given moment.

Love always,

Arianna

Why I Have the Best Interventional Radiologist EVER

Hello everybody! Earlier this week, I had an unplanned (unless you count the day before as having planned...) surgery in which Dr. Alomari once again performed his unrivaled magic. For those of you unfamiliar with his name, he is an extremely talented interventional radiologist at Children's Hospital, Boston. For the past couple of months or so, my Klippel-impacted foot had become almost impossible to walk on. I was on blood thinners for it -Lovenox- but to no avail as the extreme swelling and pain ultimately remained. Dr. Alomari had ultrasounded the area not too long ago, and determined that a slew of clots were stuck within a veiny region that spread throughout my foot. Sometimes, he said, these cause no problems for patients pain wise; when this is the case, operation is not usually necessary. However, in my case where they were causing me a momentous amount of pain, there was nothing left to do but operate. The alternative would be living in great discomfort for the next several months while the situation resolved itself – no thanks!

A couple of days prior to the surgery

So, Dr. Alomari performed the surgery flawlessly. Essentially, what he did was made a tiny incision in my foot (I didn't even need any stitches, that's how small it is) and squeezed as many clots as he could out from there. Look below to see what he was able to get out! Pretty incredible, right?

My foot has already returned near back to its normal size previous to all of this clotting hoopla that erupted several months ago. I could not be more pleased by the results and this is just yet another testament to how grateful I am to have Dr. Alomari as one of my doctors. He is, without a doubt, the best of the best. - A

P.S. I will update soon with a picture of how my foot looks now!

I'm Back

Since I last updated, I was admitted to the hospital again because I was having a hard time recovering from the last infection. It was not the infected area that was hesitant in recuperating, but rather my body as a whole. I felt so incredibly weak and nauseous among other things. I was severely dehydrated and not eating. However, I am doing much better now and I figured I would kick off this post with a new picture of how my C02 lasered area healed (as some of you may recall I had this work done by Dr. Alomari in June).

Below are before pictures (just a couple of days after surgery) and now. I'll update more soon.

- A

Physical Therapy

Hey everyone. So there has been no improvement in my foot yet; therefore, it has been determined the clyndamycin antibiotic is not working. In the meantime, I am being kept comfortable with a heavy dosage of pain medicine. Today, I started physical therapy. Even with the pain medicine in effect, I am having a hard time walking on my foot and therefore I need to be taught effective methods for maneuvering my way around until the foot starts to heal. The physical therapist came in and basically just taught me how to use the walker from my bed to the bathroom. It is extremely simple; just move the walker, take a step with your left foot, and then your right one follows. Below is a picture of me in progress with the physical therapist. - A

Picture Update: Current Cellulitis/Healing from Surgery

Hey guys, so just a quick post showing some pictures of my leg at present.

To begin with, here is the infected area. The redness is not very prominent; rather, it is more of a muted pink rather than a fiery red. However, the pain is so incredibly rampant and relentless. There is also a great deal of swelling.

As far as the healing from my last surgery in which I had co2 laser done along with schlerotherapy and some other injections, here are some pictures:

There is still a fair amount of bleeding associated with these. - A

Back in Hospital

Hey guys - I am back at Children's Hospital Boston due to another cellulitis infection. This one is primarily in my foot region. Pain wise, it has been one of the worst ones I've had in a while. As far as systemic symptoms are concerned, I have been experiencing nausea, tiredness, overall fatigue and low-grade temperatures but nothing striking. For me, each infection has something that stands out about it. For example, with some infections it is the incredibly fierce crayola redness that imparts upon the infected area. For others, it is how sick I got systemically. For this one, it is the relentless pain that is rampant throughout the foot and ankle area. I've been here a day now, and I still cannot bear any weight on it.  It is swollen and red, although the redness is not especially vibrant by any means.

I am being treated on IV clyndamycin and am being given morphine for pain. One thing in which I have learned, however, is that IV pain medications do not last as long as the oral ones (or so I have been told). Therefore, given the intensity of the pain in which I am experiencing, they may switch me over to oral morphine.

Above is a picture from me in the ER last night. As usual, all of the nurses have been so incredibly friendly and helpful. One of my nurses, Christina from 10 NW, is so sweet and friendly to talk to! I had her last during my last hospital trip as well. A good nurse can undoubtedly make a huge impact - so never forget to smile and say thank you! : ) - A

MORE FREEDOM, PLEASE!

When you are in the hospital so much, it can seem like a fight to hold onto your own identity. Every hour of every day there is someone telling you what to do – an army of people dictating your every move, from what you can eat to how much you are able to bend your arm (if you are lucky enough to have an IV directly where it bends, that is!). I found that as I have aged, I have become more internally annoyed with complying to all of these things whenever I am hospital bound. It seems that just when you are about to fall asleep, finally (after hours of mainly strangers poking and prodding you), a nursing assistant comes in for vitals. That nursing assistant then wants to know how much you have gone to the bathroom, if you can sit up so they can listen to your lungs, what you are doing with your life outside of the hospital, and so forth.

Forgive me if I sound incredibly arrogant and unappreciative in the above paragraph as it is not my intention in the slightest. Those staff workers are the ones playing a vital role in chronicling my health's progress, and are merely doing their job. For all I know they too were unable to sleep prior to going to work and are just doing their best to make it through their work shift. I always try to be mindful of that with every hospital staffer I meet! Also, I never take my personal frustrations out on any nurse, doctor, etc. Inside I may be really annoyed at the simplest of things, but I do my best to keep it together. Ultimately, they are all there to help and assist, and for that I am grateful.

Sometimes, though, I just want to tug off my allergy bracelet and rip out my IV and go running outside! I want to be like a normal kid in their twenties, and not be bound by all of the rules in which I have to so meticulously follow while in house. Yet, the rules are that way for a reason, and each little rule adds up to a bigger picture. Therefore, I know it is vital to comply. Still, coming home is like a breathe of fresh air! Although there are still rules to comply with, I can stay up until 2 in the morning without someone documenting it in the computer or checking in with me every half hour.

Do any of you ever feel the same? - A  

Update, @ Children's

Hey everyone! I apologize as I have not posted in a bit. Since I last posted, I have been hospitalized at Children's Hospital for another cellulitis infection. I also had surgery yesterday with the incredibly talented Dr. Alomari. I have wanted to post while here, but was too fatigued to do so. Currently, I am writing this from my hospital bed at Children's. Because of yesterday's surgery, I am on pain medication which is making it feel as though the room is swaying back and forth. I promise to write more within the next couple of days. Below is a picture from when my sister visited me in the hospital the other night. I always miss her so badly whenever I am hospitalized. - A

Hospitalization due to Cellulitis

Hey guys! So basically I am back in the hospital with a cellulitis infection but I have already been here two days. Prior to now, I have been too fatigued to update. The good news is that I think I am going home tomorrow! I will update more later this week. - A

UPDATE: Just a couple of random pictures from this hospital stay.

Well, technically, this was the night before! But it just goes to show how quickly an infection can come on.

My sheets after they placed yet another new IV because they all kept  blowing!

Skyping with my sister. That cheered me up so much!  I had no idea this as being taken!

Let's talk about how much I utterly adore Dr. Fishman from Children's Hospital Boston. He just left my room a few minutes ago and I cannot help but feel incredibly beholden by him. He knows his material, and his display of confidence but lack of cockiness is quite appealing when it comes to discussing elaborate medical procedures. As the team of doctor's were leaving my room, I was sure to thank all of them profusely. Then, however, came my farewell to Dr. Fishman. "Dr. Fishman," I said, "never a pleasure." We all laughed. He knows I am absolutely kidding, and that I am very grateful for all of the work and care in which he has provided for me throughout the years. I love that I can banter back and forth with each and everyone of my doctors! When you have known them for so long, they become friendly familiar faces in a strange way, Ultimately, though, he knows I am grateful for his expertise and the time and work he has dedicated to me as a patient.
 By the way, in case you were wondering, he does not have a house in the Hamptons to kick back at during the summer season; I would know because I already asked to swim in the pool. ; ) Overstepping boundaries much? Yeah, well, I'm notorious for that. Not that a man of his magnitude would even have the time for that. During my last hospital stay, he showed me the quantity of emails he had received via phone since the ten minutes he had been in my room. There had to have been at least 40, no lie. I don't know how he does it, but he does it and he does it well. Not only does he manage work, but he has a wife and two children.

I should get to go home tomorrow. This does not surprise me in the least bit as this infection was quite tame from the start. Systemic symptoms were minimal, as was the pain. The cardinal symptom was indeed the redness in the lower left limb. However, I came to the emergency room shortly after noticing the redness so there was little time for the infection to progress into a more developed stage. With potential infections, you NEVER want to wait around. Supposedly, as recited at a presentation at the conference in Mayo Clinic this past summer, somebody died because he waited too long to get help for the infection.

I'll write more later because I am so fatigued right now as I got no sleep last night. -A

Room view from the kitchen