Flare-ups, Emotions, Self-Care, & Alternative Medicine

I haven't posted in a very long time but it's time to get writing again! A lot has happened in the past year as far as my FAVA journey goes and I'm excited to start writing again. I've been in school and have had a very functional last 6 months as far as pain management goes. I've been mainly using my diet as my primary source of pain management which has been extremely effective except for the last few weeks. I'm learning that with high stress levels in school, I need to practice more self care and go back to some of my older pain relief strategies. The good news is that I have a few blog posts almost ready to go (including pain relief strategies) so those will be posted soon!

I'm currently experiencing a flare-up which has taken a toll on me physically and emotionally. It's been a while since I've struggled so much having this illness and I find it important to recognize that it's ok to not be okay sometimes. Having a chronic illness can be emotionally taxing on you and whoever is involved and it's okay to take a day off just to practice some self care however that is needed. For me that involved taking a few days off from school (very inconvenient since it is mid-semester and have lots of things to do) but it's proved beneficial so far at least.

Normally my flare-ups involve excessive swelling and some bruising/discoloring which inhibit my walking and include throbbing, pulsing pain and  some nerve pain. This recent flare up lasted for more than 2 weeks which was extremely abnormal so I spent the day at Boston Children's with two of my surgeons who have operated on me in the past to find out what was going on. We did an ultrasound that showed that my FAVA hasn't changed much for the past 8 months which was good news in a way but upsetting because we didn't know what caused the flare-up!  I had always had pain in two specific areas in my butt/thigh so had always imagined that those were the only two FAVA spots left since I had the gluteus maximus muscle removed from my amazing surgeon Dr. Spencer, however I never realized that the disease is more complicated than that. The ultrasound showed the FAVA was infiltrated throughout the majority of my tissues in that area and that those two specific masses were extremely inflamed to the point where it showed up like a black hole on the ultrasound. So even if we tried to resect those two masses which might not be worth it due to the recovery, scar tissue, and how difficult they were to find in the past, I'm still essentially full of FAVA in that area.

So here comes the plan. I'm scheduled to meet with Dr. Trenor at Boston Children's in a few weeks to discuss the possibility of starting sirolimus which is slightly terrifying yet I'm excited. My previous surgeons said that they could try to do the procedures again that didn't work for me but I'm not willing to go through that again. So sirolimus is essentially the only treatment left and wouldn't be started until February if I'm eligible. So until February, since conventional medicine hasn't been effective, I will be relying on alternative medicine to deal with the pain so I can be functional. Medical acupuncture, lots of supplements, essential oils, yoga, meditation, a dietitian, and possibly even aromatherapy is on the list so far! Also, stress management is of number one utmost importance so I will be taking incompletes in some of my courses to reduce my academic course load and focusing on my health right now to bring this flare-up down! Multiple studies prove that stress and pain are heavily connected and this is what we believe had caused the flare-up so I know I need to make a major lifestyle change. Talk about a lot of work!

Hopefully I'm eligible for the sirolimus because I can't express the need for more treatment options for this illness. I know I'm not the only one effected because I've met and talked to so many others who suffer but we need treatment options! So this holiday season, instead of buying pointless gifts for others, please consider purchasing something like a candle or T-shirt from the FAVA foundation where some proceeds go towards the foundation for providing help to those who suffer with FAVA. 

http://www.thefavafoundation.com/donate.html 

https://www.facebook.com/thefavafoundation/?fref=ts 

Some good news is that I'm fortunate enough to be doing my own independent research at the University of New Hampshire about the correlation between mindfulness techniques and chronic pain in young adults while co-facilitating a chronic illness support group on campus that I'm extremely passionate about. Also, my boyfriend and I are taking a vacation to Europe in December and January together while we have the chance before I (hopefully) start the sirolimus!

Felt like I needed to end on a hopeful note,

Best wishes #favahugs

Cryoablation (again), Steroid Injections & Links

Hi everyone! I haven't posted frequently because I was in school and was overwhelmed with classwork. I did well in college managing the pain levels this semester but in March my pain levels got worse. I'll cover my college accommodations and pain management techniques in another post. Early February I took a trip to Boston Children's yet again to talk to the Interventional Radiology team about more possible treatment options to help with the persistent stabbing, throbbing, and nerve pain that accompanies my FAVA in my upper thigh and buttock. After having my previous cryoablation done last September like I mentioned in my "New contrast shows more cryo" post, the ultrasound showed treated lesions next to untreated lesions. Therefore, it was uncertain whether or not my pain was coming from the treated lesions, untreated lesions, or both. In simple terms, we were trying to figure out whether or not the cryoablation was successful in eliminating my pain where my lesions were and labeling it a successful treatment option for me or not.

With this question at play the course of action was to inject steroid injections into the locations of the lesions (combining the locations where I had the most pain with the ultrasound imaging with contrast) in order to provide me with temporary pain relief. The theory behind the steroid injections was if they injected the untreated lesions and I had pain relief, then they would know that the cryo had worked in relieving my pain and the chances of another cryo procedure being successful in the future would be significant enough to continue with that treatment option.

March 16th I had the steroid injections done at Boston Children's with high hopes of it either masking my pain or making no difference. I was a fairly easy day procedure that I did over spring break where I was sedated instead of having general anesthesia. The steroid agent used was 60 mg of Kenalog and 13 mL of Marcaine. To my surprise I woke up in a massive amount of pain and stayed until my pain was managed. It was expected for me to have immediate relief within a couple days however I feel like I took a major step backwards. My pain levels along with the frequency of the pain unfortunately has increased drastically since before the procedure and my walking was impacted greatly. I had to use my cane to walk for a few weeks. With time, the inflammation and bruising went down. It took about 2 months and I'm slowly increasing the amount I walk using my fitbit (that has been a lifesaver may I add).

So, now what? Since the steroid injections, interventional radiology has declared the previous cryoablation unsuccessful and we have canceled the plans for the future cryo that I had planned for the summer. They referred me back to my orthopedic surgeon, Dr. Spencer, who I admire and trust greatly. She is willing to do another resection and cut out the lesions since that has been the only treatment option that has been the most successful. I also contacted my plastic surgeon and spoke about the advantages to add more tissue/aloederm over the sciatic nerve for added protection as well.

FUTURE LINKS AND TREATMENT!

It was also brought to my attention in March that the team is thinking about doing a research study on possibly using Sirolimus to treat FAVA patients in the future. A recent article on the genetic mutations of FAVA was recently published claiming that Cloves syndrome, Klippel Trenaunay Syndrome, lymphatic malformations, and FAVA all come from the same genetic mutation called PIK3CA. They have been using Sirolimus for CLOVES, KTS, and lymphatic malformations which leads them to believe that it might be successful for FAVA patients. It's great to hear that they might have more treatment options for FAVA patients who don't respond well to cryoablation, sclerotherapy or who are unable to get resections. It's been interesting to see how many FAVA patients have had amputations as well due to the pain FAVA has brought into their lives. Although that is not an option for me due to the location of my FAVA, it is so encouraging to hear the positive results of being pain free from having the limb amputated.

Here is the PIK3CA mutation article

However, it is important to remember that EVERY case of FAVA is different and treatment results may differ from case to case. Unfortunately, resection is the only beneficial option for me at the moment but I've heard of patients responding well to cryoablation. Here is a study about cryoablation by my surgeon Dr. Shaikh who is so kind and who I respect and admire his dedication to helping FAVA patients with whatever he can do to help. There are other links to this study if you google it but this one was the easiest for me to link here. 

Also, FAVA is now on Wikipedia! So a simple google search will finally give a brief overlook of the illness! Yay! Take a look!

And yes, I will be posting more often,
Becca #favahugs

Returning To School

Last year I had two surgeries including a debulking and a cryoablation which made me take a full year off of school at UNH and although I'm still in a decent amount of pain, I am back to school for the spring semester before my next cryoablation in the summer. I mentioned in a previous post titled "Compromising Physical Health for Emotional Health & Cryo Update" how I was worried that trying to go back to school would compromise my physical health. I'm taking the risk to continue working on my degree and moving towards my goals. School, especially college, takes a lot of energy and brings upon a lot of stress. I'd be lying if I said I wasn't worried about what will happen this semester.

Fall semester 2013 I found out that the FAVA grew back and I was in incredible amounts of pain. I was taking four classes totaling 16 credits and ended up dropping a class. I became instantly discouraged when my body stopped letting me get out of bed in the morning. I would lay there in pain for hours and not be able to move my legs. It was hard for everyone in my dorm to understand what I was going through but I physically couldn't go to class anymore. I made plans to have my debulking surgery during winter break and I only ended up finishing one class that semester. The other two classes I took "incompletes" which meant that they would extend my deadlines until I felt like I could finish them. Thankfully my dean worked with me to get all the resources I needed. Physically, mentally, and emotionally I slowly began to fall apart. Hopelessness ran through my veins and I had an incredibly hard time staying social and hopeful. The pain was unbearable and my anxiety and stress increased drastically.

I know that I still have untreated FAVA lesions in my thigh and I'm aware that I'm taking a huge risk (pain-wise) to continue to take classes before another procedure. Fortunately, I'm not putting myself at a health risk waiting. My degree is so incredibly important to me and I'm NOT willing to give up my dreams, hobbies, or talents in lieu of this illness. I understand that sometimes I'll have to set boundaries or limitations on things in order to physically succeed but my will-power to mentally succeed is too high to not try. I'm honestly petrified that my body will fail on me and not let me walk or sit through class this semester like it did the last time I was here at school. However this time I'm starting out with a different outlook and I'm not going to try to be superwoman and do things I know my body can't handle.  I'm constantly learning about what I can and can not handle mentally and physically and am learning that it is a long process and not just something you can learn quickly. I believe that there is strength, courage and bravery behind everyone with a chronic illness and you need to discover it in order to grow.

Henry David Thoreau stated  "As a single footstep will not make a path on the earth, so a single thought will not make a pathway in the mind. To make a deep physical path, we walk again and again. To make a deep mental path, we must think over and over the kind of thoughts we wish to dominate our lives." With a deep physical path along with a deep mental path of pushing through the struggles that I will continue to face with FAVA, I believe I can make my way through school and any other obstacle. But I know this mindset has to be used over and over again in order for me to grow.

 Strength. Courage. Bravery. Determination. Perseverance. I am a warrior and so are you. 

New contrast shows more Cryo

I had my imaging appointment at Boston Children's Hospital this past week and it was a much larger appointment than I had anticipated. It was the first time I went into an appointment by myself and took the trip into Boston via train and subway. It felt more real when I went in myself and hit me a little harder knowing that this was truly a chronic illness. Most of the time the people I'm with encourage me that maybe this will be the last time but this time I didn't feel that way. I spent two full hours with my surgeon throughout the imaging and discussing the next steps and actually felt like I got to know the team a little better. After all, it wasn't going to be the last time I saw them and I felt like I should  know them  better and they should know me better. It didn't feel like they were putting on a "professional show" for me; they were truly being themselves and made me feel more at ease with laughter and medical jokes.

When I got into the interventional radiology department, I was given an IV for the new contrast they were going to use with the ultrasound. Despite how friendly the nurse was who gave me the IV, she missed several times and I nearly had a panic attack in the bed because although I've had many IV's I am still not good with needles. They were concerned about me having an allergic reaction to the contrast because I have so many food allergies and the contrast apparently had something to do with eggs. They also haven't used it on many vascular anomaly patients yet, if any so this procedure was extremely new. They watched my oxygen levels, heart rate, and blood pressure and monitored me closely throughout the entire imaging. I got very nervous because if I was to have an allergic reaction, I was going to be admitted overnight and I was at the hospital by myself.

With all the monitors attached to me it felt as if I was inpatient. They gave me a marker to draw circles around the areas that hurt the most although it seems like when the FAVA hurts, all the tissue around it hurts so it's hard to pin point an exact location. In the room there were two surgeons, Dr. Shaikh and Dr. Palotier, along with two nurses and many med students. They were reviewing my old ultrasounds and MRIs and comparing them to the live regular ultrasound before they did the contrast. What was odd about the contrast is that the full effect only lasted about 10-20 seconds before it started to fade and they could only give me a certain amount every 10 minutes.  Therefore, they had to work quickly when the contrast was given. We did ultrasound imaging for over an hour and I had three syringes of contrast. The only side effect I had was dizziness, feeling lightheaded and a bit foggy but the feeling went away within about 20 minutes. It was very overwhelming hearing them talk in all the medical terms trying to figure out where the FAVA was and where it wasn’t but I loved hearing them discuss it in front of me. It wasn't as clear cut as we had hoped and there was a lot of confusion at first.

To my surprise we found the areas that were previously cryoablated and it showed in the ultrasound that it had worked and killed the cells! It appeared almost as a black hole where the cells were no longer there. To me it was pretty cool and reassuring that the previous procedure wasn't useless. However my pain is still there in the same locations like I posted about previously and we found multiple untreated masses close to the area that was previously treated. Thanks to the contrast, we found that both masses around the locations where I had previously had cryoablated were just missed from the previous cryoablation and they are superficial and will be treated next summer after my college semester is over. They did find another small mass that was deep and connected to my sciatic nerve and close to my hamstring which sounded worrisome to me but they reassured me that they aren't worried about it doing any damage because it was so small. However, it is untreatable because  the risk of paralyzation is too great to risk. Therefore, it may cause me some pain but as long as it doesn't do any damage I'll be content. It was scary but now I'm content and am trying to manage the pain before the next cryoablation in the summer.