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.