The Sirolimus Diaries Post #2

Hi everyone! I apologize as it has been a bit over a week since I last updated this particular part of my blog. As I mentioned in my maiden Sirolimus Diaries post, I will be updating in regards to my experience with this medicine on a weekly basis. Taking a medicine of this nature is certainly new territory for me; as trite as this may sound, it is my only beacon of hope at this point in regards to my seemingly unappeasable Klippel-Trenaunay Syndrome. I have now been home from the hospital a little over 2 weeks, and that in itself is something to celebrate. Typically, I end up inpatient again right around the 2 week mark. Whether or not this can be attributed to the Sirolimus or is merely good fortunate is a distinction in which I am unable to make.

Although the official trial period of using sirolimus to treat complex vascular malformations has just ended about a month or so ago (it lasted 2 years in total, I was not a part of it), there is still much to learn about this particular drug and how it can potentially be of great use in cases that are somewhat treatment resistant, like mine. Again, hats off to Dr. Adams of Cincinnati for her amazing innovative thinking... if I did not have my competent team of Dr.'s who I trust and admire wholeheartedly at Children's Hospital Boston, she would undoubtedly be my next in line physician. I had the pleasure of meeting her at the biennuel Klippel Trenaunay Conference a couple of years back and it was a truly remarkable experience. Aside from excelling radiantly at her craft, she is implausibly kind and well-composed and just an all around lovely person.

Anyway, I will jump right into the update now!

Th Sirolimus has now had time to build up in my body to a therapeutic dosage. I got my blood levels checked approximately 6 days ago and was told my levels are indeed perfect. However, the past week has been somewhat of an anomaly for me, as I got sick in a way that I never had prior... My family always jokes with me and says that I never get “normal people sick.” Up until now, however, they have mostly been right. Over the years, I have endured the occasional cold and/or short term virus, but never have I gotten a bug that lasts for more than a day or so or one that wreaked utter havoc on my body (aside from all of my infections and other Klippel related illnesses, of course!).

A few days back, I started vomiting uncontrollably and had extremely sharp pains in my lower abdomen. I also experienced diarrhea in conjunction with a low grade fever, relentless headaches, and dizziness. For the three days that followed, these symptoms persisted and I even contemplated going to the emergency room. However, my symptoms eased up as of last night and for the first time in days I was able to hold down my medicines. So, I had three days where I was off the sirolimus and my other medicines (which made me incredibly nervous). Now that I am on the mend from whatever vile bug that was, I am able to resume the medicine as normal. I just spoke to one of the nurses, Jen, from the hematology department who works alongside Dr. Trenor. She is the person I speak to in regards to questions, concerns, or anything else to do in relation with the Sirolimus. She has been so incredibly helpful and kind to me throughout  arduous points in my life as of recent (most notably when I had the thrombophlebitis all throughout my upper leg) and I am so appreciative of her.

It is interesting to me that I contracted this bug right after the Sirolimus reached a therapeutic level in my body. While Sirolimus may be key in taming my Klippel, it does in fact hinder the immune system in other ways and makes one more susceptible to other illnesses. To help combat this, I am taking bacterum three times a week. It is vital all patients on sirolimus take this antibiotic while on the drug or else there is an elevated risk of contracting fungal pneumonia, among other things...

Other than the vomitting bug, I do not notice much difference in terms of my daily life thus far. I still have leaking of lymphatic fluid from an area on my Klippel, and rectal bleeding along with chronic pain. It will be interesting to see if the Sirolimus helps to correct these invasive issues over time. I should note that I am sleeping a great deal, however I cannot for sure say that is from the medicine itself. Chronic pain (which I, along with many other Klippel patients, have) tires one out greatly. Plus, I am sill recovering from my last infection and surgery that took place a couple of weeks back with Dr. Alomari. I also deal with a large amount of depression and anxiety, which can be mentally and physically exhausting.

So, for now, no bizarre side effects that I have noticed but I will keep you guys posted!

Hope everyone is doing well and getting to enjoy some (hopefully) nicer weather. I spent most of last summer in the hospital with a Strep B blood infection (among several cellulitis infections) so am hoping that I will get to enjoy the warmer weather as it approaches this year.

As usual, please feel free to comment on this post with any questions or comments you may have. Below I will leave you with a few pictures from the past week. Talk to you soon! - Arianna

I got my hair done professionally for the first time in a while; it felt good!

Sinead & I goofing around in one of the kid carts outside of Children's Hospital. I used to be wheeled around in these all of the time as an inpatient when I was younger!

Eating dinner with Sinead; one of my medical friends who was in town from Toronto, Canada for a few days!

Another picture of the two of us before we went out for dinner.

Reunited

It wasn't as surreal as you would imagine it to be – at least it didn't feel that way. It was almost as though we picked up where we had left off...we had first met in person in Minnesota a couple of years ago. Skype, Facebook, texts and phone calls were a constant between us ever since then, and perhaps that it why it was not quite so overwhelming to see her in person yet again. As the car pulled up, I saw Sinead and her mom waiting for me outside the Longwood Inn. Because I am on so many medicines for my KTS at present, I have been battling feelings of nausea, sweating, insomnia, etc. I had only slept about 3 hours the night prior and felt so incredibly fatigued, but I did not care. I did not care that my leg was aching beyond belief and that my butt still felt sore from the last infection and surgery. Nothing was going to get in the way of me seeing one of my best friends for the first time since the KT Conference a couple of years back.

It was so nice to see one of my dearest friends I had met from the online medical community. I wish I had felt better, but unfortunately I did not have a say in the matter and tried to ignore all of my yucky symptoms to the best of my ability. Having friends who understand your daily struggles has been so incredibly life-changing, and it makes me feel as though I am not so isolated. Below is a couple of pictures from Sinead's visit to Boston. - Arianna

Introducing “The Sirolimus Diaries”

Hi everyone!

I am incredibly excited to be starting this new journal series of my blog entitled “The Sirolimus Diaries.” Anyone who knows me well knows that I have spent the entirety of my life battling Klippel Trenaunay's relentless symptoms, but that this past year has undoubtedly been a game changer in terms of severity. When I first heard that a chemo drug was being used on Klippel patients in Cincinatti, I was flabbergasted. I had never imagined that such a treatment option would ever be viable for Klippel patients, but indeed it has proven to be. The lovely Dr. Adams (who Dr. Fishman openly refers to as his “sister”) spearheaded the initial treatment on a particularly treatment resistant vascular anomaly patient of hers, and this patient experienced fantastic results. A two year trial was then set up for other patients, and many also saw life changing results as well. At first, I was rather hesitant to begin taking this drug that could (or could not) possibly help my Klippel-Trenaunay Syndrome. A chemo drug?!? Never in my wildest dreams did I imagine I would be taking this for my Klippel! It seemed too tremendous a thought for me to comprehend all of a mere six months ago, but I now feel much differently. Below, I will explain why me and my doctors now feel this drug is a good option for my particular case.

My cellulitis infections progress much faster (which I did not even think was remotely possible) than they used to. Now, when sick, it feels as though there are literally steak knives going through my buttock and thigh area, and the pain is intolerable. I used to think the pain from these severe infections was already at an all time high, but I had no idea they could actually feel much worse. Every moment before I arrive to the ER (where I am continuously bombarded with an abundance of various pain meds before the pain starts to dwindle even little bit of a noticeable amount) feels excruciating and I literally get systemically sicker by the minute.

Perhaps what is even worse, however, is that these infections quite often happen while I am already on antibiotics to treat the last severe cellulitis infection (usually a high dosage of an oral antibiotic that was preceded by weeks of multiple intravenous antibiotics). Of course, this could be because my body has obtained a different bug in which the particular oral antibiotic does not cover. However, with Klippel, it is indeed very rare to grow back any kind of specific bacteria and therefore doctors are often not sure what bug they are even chasing (which, ultimately, leads them to putting me on broad spectrum antibiotics). This past summer, however, I did something incredibly rare for a Klippel patient; I grew back Strep B in my blood. I had a serious blood infection, and it refused to respond to antibiotic treatment until Dr. Alomari was able to perform a couple of dramatically helpful (and ultimately lifesaving) surgeries in which he drained cyst like areas in my left buttock, thigh, and leg. Much to my disdain, these cyst like areas keep coming back and Dr. Alomari has been forced to repeat this procedure several times.

I have continuous leaking of lymph fluid and blood from several areas despite surgical intervention. I have dealt with rectal bleeding for a multitude of years now, and it has always been rather treatable by Dr. Fishman performing a rather simplistic procedure. After he would perform said procedure, I would have stability (as far as the rectal bleeding was concerned) for about a couple of years time. These days, however, my rectum apparently has a mind of its own (I apologize for I just realized how incredibly gross that sounds) and bleeds out despite surgical intervention. This is rather dangerous as I am already incredibly infection prone, and this serves as a pathway for bacterium to make its way into my buttock. Also, it is rather uncomfortable and sometimes even painful. Lastly, but certainly not least, it has caused me to be an anemic (which I have been in the past due to different Klippel issues that were resolved several years ago) and in result zaps away a great deal of my energy. The rectal bleeding is just the tip of the iceburg, though, as I experience leakage of lymph fluid and blood from a bevy of other areas as well that have not responded to c02 laser and schlerotherapy.

Blood clots have also become an increasing problem for me throughout recent years. Most recently, I struggled with thrombophlebitis throughout the entirety of my upper left leg. While most people get it in a vein or two, I had it in a considerably widespread area that led to a month of me not walking (prior to surgerical intervention by Dr. Alomari). As one nurse said, “most people get thrombophlebitis in a vein or two, and you got it all over.” About a week ago was the surgery by the incredibly talented Dr. Alomari and I am still in a great deal of pain from it. Perhaps the worst, however, was the pain I was in before the surgery; this pain was so deep that I literally could not stand on the leg whatsoever. Ironically, the clotted veins were located in superficial areas and not in the deep venous system. However, superficial clots are extremely painful because they are so incredibly close to the nerve endings.

Throughout these past 9 years or so, my chronic pain has become much more drastic and invasive than ever before. Pain medication does not even do much justice anymore, and I find it hard to function on a daily basis like a “normal” 22 year-old-college student would. Because I am in the hospital more than I am out, I have had to take three medical leaves from college within the past three years.

My Klippel circumstances have indeed pushed me into a corner, and as one doctor put it I simply don't have stability anymore. Because of this, we started the drug about a week and a half ago despite months of me mulling over the idea. At first I was hesitant, but I now see it as my only hope in tangibly achieving some sense of normalcy again in the near future. My Klippel has indeed taken on a life of its own, and continues to put me in life-threatening and painfully excruciating situations despite treatment. Below, I will sum up the main reasons in which I have turned to Sirolimus as a feasible option for helping to treat my Klippel despite my initial hesitance.

1. The frequent occurrence of severe cellulitis infections despite treatment
2. Continuous leakage of lymph fluid and blood despite surgical intervention
3. Intrusive blood clots
4. Intense chronic pain

I will be updating this particular part of my blog weekly, and look forward to sharing my experiences while on it with you. Please do not hesitate to ask questions in the comments section below, as I will do my best to answer each and every single one to the best of my knowledge. - Arianna