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

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

Appointment at Children's Hospital Boston with Quick Video

Here is a brief video I shot the other day while making my way up to the IR clinic at Children's the other day with Dr. Alomari. Being at the hospital (for the first time since my month long June admission) ignited a lot of vile emotions for me; despite knowing I was safe and my condition stable, I could not stop shaking throughout the several hours I was there. I will be updating more soon about the appointment and what is to come for me in the near future! Hope you all are well. - Arianna

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.  

Update on Mental Health Petition for BCH

Hi lovelies! I have so much in which I want to share with you guys... unfortunately I will not be able to do it within this one post. However, this post is certainly a start, anyway!

Perhaps you remember the petition I initiated a few months back, the one where I wrote about the need for the VAC Clinic at Children's Hospital Boston to have an informed psychologist as part of their staff. As a well seasoned doctor once told me, 

“what good is it to have a patient who is physically healthy if they are so emotionally wrecked inside that they are unable to get out of bed in the morning?” 

Chronic illness and depression practically go hand in hand, but with proper treatment from trained professionals (as research demonstrates) one can indeed live a life engulfed in prosperity and jubilation.

The reality is, though, that the mental health portion of this condition is largely ignored. In result, a great deal of patients struggle with hefty amounts of depression, anxiety, and other disorders that can be utterly crippling. No one should have to live life that way, yet so many people with Klippel do as they are unable to find the proper help in regards to dealing with this condition (and, unfortunately, there is not much available). That is why I asked Boston Children's Hospital to step in and, well, step up.

I sent my petition to Dr. Fishman recently and was so touched by how responsive he was in regards to this matter. He agreed with my reasoning wholeheartedly, and was willing to help me fight for this cause, as he too believes it in very deeply. For those of you who have not met Dr. Fishman, I will tell you this much: he is a phenomenal human-being. Aside from being incredibly skilled as his work, he is a fabulous humanitarian that genuinely cares about the needs of other people.

I am not quite sure he would want you to tell me this, but he can get pretty emotional when it comes to his patients. At the last meeting, he cried when he saw images and what not of how much Sirolimus has helped various patients of his; I know this to be true because he told me when I ran into him the other day.

Back to the petition, though. Dr. Fishman sent out an email to an array hospital leaders. He also had a 45 minute meeting with the Chief of Psychiatry, David Demaso. Dr. Demaso now wants to have a meeting with me in regards to this matter, and is very receptive in helping to find a tangible solution to this problem.

Had Dr. Fishman not been so active in spreading the word about my petition (along with being incredibly supportive and giving me the courage to believe in myself), I would not have this astounding opportunity to meet with such a high-ranking Dr. within the psych. Department. Dr. Fishman and Dr. Demaso are incredibly willing to implement the change in which is so desperately needed in order to give VAC patients the best all-around care possible.

So, basically, change is immenent people! ( : This is fantastic news for patients/family members of patients of Vascular Anomalies patients at Children's Hospital Boston. I will keep you guys updated as to how my meeting with the highly-esteemed Dr. Demaso goes! I do not yet have an appointment set up with him but plan to make one tomorrow.

To read my previous thoughts on this matter, please click here to read my Calling You Out BCH Post I did several months earlier
Also, click here to ready the petition I did

Below is a picture of Me and the lovely Dr. Fishman at the KT Conference approximately three years ago. Unfortunately my eyes are shut in it! : (

More soon, Arianna

Inpatient for Cellulitis

Hi everyone - unfortunately I am writing this post from my hospital bed on 10 NW at CHB as I was admitted for a cellulitis infection. Last night, I took a spontaneous nap. Upon awakening, I had this familiar, horrible pain radiating from deep in my left buttock. I knew this pain well, and therefore had mom take me to the emergency room immediately. I was still taking Keflex 1000 mg daily - but apparently that was not good enough for my body. This morning I had a 101 fever, chills. severe pain in both my left buttock and thigh (It spread down there throughout the course of the night), and redness. At present, I am on 4mg of Morphine every two hours which I have been told is a hefty dosage. Below is a picture I took earlier today of the area. Please pardon the fact that it's of my butt haha. From what I have been told, it has gotten redder since this picture had been taken, and of course I had to take it with my lousy blackberry camera... - A

Continued Antibiotics, etc.

Hey everyone! I know I have not written in a bit, much to my deepest regret. Access to a fully functioning computer still proves to be challenging at this point, but I will have a new one by Christmas!

I have been out of the hospital since the end of September now and it has been such a delightful treat. Just earlier today, I spoke with one of the VAC nurses Erin in regards to my antibiotic situation. Initially, upon coming home, I was taking 2000 mg of cephalexin daily (twice a day). We then lowered it to 1000 mg of cephalexin daily and I was due to finish it today. However, due to my unchallenged success on these oral dosages, my doctors have made the decision to keep me on it for another two months as a prophylactic. I was quite pleased with this decision as I have my sister's wedding towards the end of December. The medicine does make me feel slightly sick, but probiotics can help counter this.

As far as debulking my ankle is concerned, I am planning to move forward with this procedure. However, nothing will be finalized until I meet with Dr. Spencer, Dr. Alomari, and Dr. Fishman on January 3^rd. 

I am still dealing with a great deal of anxiety and depression, however am working very hard to make it through each day and to be at least somewhat productive. The hardest part of being out of the hospital is readjusting to at home life while trying to maintain my health and reestablish normal daily habits.

Hope to update soon!

- Arianna

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

Home

Hey everyone! I am, at last, home! I have been for a couple of days now, and in spite of the constant pain stemming from the surgeries in which I had done, am enjoying being back in a familial atmosphere. If you keep up with this blog, then you are aware of the c02 laser Dr. Alomari did on my leg back in June. Those legions have scarred over and looks utterly fantastic now, (pics are on here in one of my more recent posts) but the pain in which I endured in order to recover was momentous! Dr. Fishman performed a very similar surgery, albeit in a more taboo area; we'll just say the thigh and regions of the buttock. He also did some schlerotherapy to help stop some rectal bleeding I have been experiencing for several years now. This is not the first surgery I have had done in relation to rectal bleeding; a lot of KTS patients, so I've noticed, are more hesitant to talk about this facet of this condition. I completely understand, as I was too for the vast majority of my teen-aged years. However, what many do not realize is that rectal bleeding is anything but ucommon in KTS patients. Some I have talked to have been rather surprised/delighted to find out that they are not the only ones who have been impacted by KTS in this way!

I am currently in so much pain from the laser in which Dr. Fishman did on my thigh and buttocks. The areas are still healing (they were done just over a week ago), but I know ultimately the surgery needed to be done. Lasering these areas was not done in an effort for the areas to look better cosmetically, but rather as a way of preventing infection. Some of these areas ooze out continuously (blood, lymph. Fluid) and allow bacteria to sneak in. Since cellulitis infections have been a tremendous burden on me throughout my lifetime, it is essential to have surgeries of these kind done every now and then to lower my risk factor.

It is currently not even 7am as I write this but since I could not sleep due to pain, I wanted to update you all on the surgery I had done with Dr. Steve Fishman. As many of you may be aware, Dr. Fishman and Dr. Alomari are both members of the vascular anomalies team at Children's Hospital Boston. I trust both of them immensely and am grateful for the incredible work in which they have both done on me, improving my quality of life drastically over the years. - A

Below is a picture of me and my sister right before I was discharged!

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!

Catching Up

So much to catch you guys up on! I have been inpatient since Tuesday, and since then have had two surgeries as well as a cellulitis flare-up occur within that time period. At the moment I am well, just in a great deal of pain due to my surgeries despite all of the pain medications I am on.

I don't want to write everything in one long post, so I will be spreading out the next few posts summarizing about all that has gone on throughout my time here the past several days. At this point in time, the surgical resident is saying I should be able to leave Monday – at the earliest, anyways. So naturally, I am going to try my hardest for Monday. I come from a close-knit family and not being able to go home with them at the end of the day is really tough on my heart. One of the hardest emotions to deal with while I'm here is that of homesickness/loneliness. 

Anyways, before I get too carried away on this post alone, I will leave you guys with a picture of me and my sister from when she visited the other night. I miss her a great deal and she always helps to cheer me up whenever I am in here. The picture below was taken in the recovery room just after my first operation.

Stay tuned, lots more to come! ; )

XOXO - A

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

15 days inpatient

I am currently still inpatient at Children's Hospital Boston because of the ferocity of the cellulitis infection I endured several days ago. I believe today marks my fifteenth day here thus far - just from this one admission. Basically, I am dealing with a massive clot in my foot and a widespread cellulitis infection in my left buttock. Never before has an infection caused me this much pain for this amount of time. The longevity of this infection is what makes it unique from all the others ones I have had thus far. Dr. Fishman, who I saw today, said this is the worst infection I've had yet and it will take a long time to return to baseline.

However, I have made heaps of progress and should be going home any day now (with picc line in tow, of course). My blood pressure is stable as is my temperature, and we have gotten the pain under control. I am even starting to walk with ease all by myself, something in which I was not sure I'd be able to do for a long time again!

With that said, I still feel crummy overall; fatigue and pain are rampant throughout my body. I must say though, I a very proud of myself for coming out victorious despite the events that have occurred in the past couple of weeks. I know I will continue to progress and I look forward to catching a glimpse of summer once I am released from here.

Hope you all are well - XOXO
-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