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