But a quick "life" update: 2016 was the worst year of my life. My dad passed away on January 4, 2016 from esophageal cancer. It was without question the hardest thing I have ever had to go through (and grief never really goes away...so it is something I am still dealing with 2.5 years later). I miss him everyday and would give anything for more time with him.
I am also a Registered Nurse, however, I realized early in 2016 that working in a clinical capacity (at least for me) was not possible with EDS, POTS, and everything else. I still have hope that I will be able to use my degrees and license to work in the field someday but I have not been able to work, aside from some volunteer administrative nursing work, since graduating nursing school. The priority has been to get my health back on track, which is proving to be very difficult.
POTS and PORT-A-CATH
(Background)- I was diagnosed in 2014 (during nursing school) with Postural Orthostatic Tachycardia Syndrome (POTS). POTS is a form of autonomic dysfunction (Dysautonomia). The autonomic nervous system controls the functions of the body that you do not consciously think about, such as heart rate, blood pressure, digestion, pupil dilation, and temperature regulation. POTS is not uncommon amongst EDS patients, and I have had issues with my blood pressure and heart rate for a long time (dating back to high school). We had hoped that the decompression for Chiari in December of 2014 would eliminate (or decrease) my POTS symptoms but that did not happen.
As a result, it became clear that EDS is the underlying cause of my POTS. Because of faulty connective tissue, my blood vessels are too stretchy and weak. When I stand up blood pools in my lower extremities instead of working its way back up to the heart and brain. This leads to dizziness, lightheadedness, tachycardia and a host of other symptoms.
(The video below is a simplified explanation of POTS)
Aside from upright posture, one of my main triggers is heat and dehydration. My body does not regulate temperature correctly and therefore it does not effectively cool itself down when it overheats. These are all automatic processes in the body, things that I cannot control. For 2 years I tried a combination of different medications and lifestyle changes, but nothing seemed to control my symptoms and I struggled to get out of bed. As a result, I had a port-a-cath placed in August of 2016 and began regular (weekly) home IV infusions of normal saline and Lactated Ringer's. This has had the biggest positive impact on my overall health, however, it is not a cure and I still have episodes where I feel like I am going to faint. I also have overwhelming fatigue and exercise intolerance. I did not truly understand how someone with chronic fatigue could be so tired all the time, until I experienced it myself.
The port is also great for EDS because with surgeries, we always struggle to find a vein that can hold an IV throughout my hospitalization. Two weeks after I had the port placed I had my fourth open reconstructive shoulder surgery and the port was used as the primary access point for fluids and antibiotics. It was incredibly helpful. I have had my port for almost 2 years and while it should certainly be used as a last resort when all else has failed, it was one of the best decisions we made.
LEFT SHOULDER
The shoulder surgery I had in September of 2016 was a relatively new procedure. It was also the first time my surgeon had performed this particular surgery. It involved taking a cadaver graft and anchoring it into the joint to act as the labrum. It was then screwed to the outside of the humerus. A MRI revealed that my labrum (cartilage in the shoulder joint) was virtually nonexistent. I was experiencing painful dislocations everyday and as a result of the wear and tear, my labrum became extinct. The graft provides more cushion and stability within the joint, as well as acts like an internal sling. Following the placement of the graft, my surgeon also performed another capsular shift (wrapped the capsule around the joint).
I spent 8 weeks in an abduction sling (pillow sling). Typically patients are encouraged to start pendulums as early as 1 week post-op. I was told not to move my arm at all for 8 weeks. With EDS, we do not want to encourage ROM as that generally comes back without any issues. Overall, the recovery process was very slow and frustrating, but today, almost 2 years removed from the surgery, my left shoulder feels stable (it still slides posteriorly at times but I can live with that) and I have full ROM. Hopefully the stability remains for a long time!
RIGHT SHOULDER
The right shoulder was actually more "unstable" than the left prior to surgery in 2016, however, the dislocations were easy (see below for my explanation of dislocations). I was having a lot of rib subluxations with the left shoulder instability and that triggered a flare in POTS and neurological symptoms (another reminder that everything in the body is connected). Because this is a relatively new procedure for my surgeon, we had planned on waiting at least 6 months post-op to determine if we should perform the same procedure on the right shoulder. Unfortunately, before we were able to address the right shoulder, my right hip starting dislocating anteriorly and that became the priority. We will address the right shoulder eventually as it dislocates multiple times everyday and at this point does not sit properly in the socket.
LEFT AND RIGHT HIPS
Right
Both labrums are torn again (I have lost count at how many times I have torn them). In July of 2015 (after I finished my nursing school requirements) I had hip surgery to repair a labral tear in the right hip. In less than a year the labrum was torn again. However, my left shoulder was wreaking havoc so I just tried to manage my hip pain as best as possible...until everything changed!
In late-January 2017, as I was getting out of bed, my right leg completely gave out on me and I had an excruciating pain in the front of my hip with a pop and feeling of immense pressure. I felt like someone had just pulled my hip off my body. As I sat on the ground trying to understand what had just happened, I realized that my hip had subluxated (partial dislocation) anteriorly (front of hip). It was the first time I had ever experienced a hip subluxation (despite having hip dysplasia) and I knew this was a problem. (REWIND: In 2010 and 2011 I had left and right PAOs to provide more anterior coverage of the femoral head--essentially to create deeper sockets and correct hip dysplasia--this should have provided more stability). I called and left a message for my surgeon in Boston, who wasted no time getting back to me. We talked about what I felt and he agreed that it sounded like my hip subluxated. He advised me to wear my hip brace until I could get back to Boston to be evaluated. In March of 2017, with a dynamic ultrasound (created at Boston Children's to evaluate hip instability objectively), it was confirmed that my hip was severely unstable. In neutral position, my hip was sitting out of the socket by 5.8mm (it should sit at 0) and that just increased as we moved through different degrees of extension (your hip should move no more than 1-2mm total--my position in neutral surpassed that). The severity of the instability meant that I would need an open reconstruction with an allograft to essentially recreate a capsule. I had that surgery in December 2017.
I spent 4 days in the hospital. Complicating recovery was the fact that they discovered DURING surgery that my rectus femoris (the largest quad muscle) had somehow detached itself sometime before surgery (we suspect when my hip first came out in early 2017). This explained why I could not activate my quad for the 11 months prior to surgery and also why the recovery was so painfully slow and frustrating. It took my quad 4.5 months to fire (I could not even advance my leg effectively with crutches), PT was excruciatingly painful, and because my left kneecap was dislocating too (I will get to that disaster later), I spent the first 5 months in a wheelchair.
Unfortunately, despite finally getting my quad to fire again, we learned in June 2018 that my graft had torn (likely in January/February) and did not scar back down. As a result, the surgery failed and another ultrasound confirmed that my hip was unstable again. There are likely multiple factors contributing to my right hip instability so simply replacing the torn graft probably would not work. My surgeon is discussing my case with several specialists in order to determine the best course of action. However, it will not be a simple solution.
Left
The labrum in my left hip has been torn for almost 3 years. It is painful. It pops and catches almost everyday. However, we really cannot go back in to fix it as the reality is it would likely cause more problems. My hip surgeon has a lot of experience with EDS and is a specialist in arthroscopic hip surgery. Because of my right hip situation (the capsule becoming virtually useless and my hip subluxating) and the fact that most of his EDS patients re-tear their labrum within a year of the surgery, he no longer wants to do hip surgery involving the capsule on EDS patients. Every time you open the capsule there is a greater chance that it will not heal properly and this probability is increased when the patient has an underlying connective tissue disorder. So although labral tears are painful, the consequences of surgery on an EDS hip are far worse. I personally would not recommend having arthroscopic hip surgery if you have EDS (particularly if you have a severe case), even if you have an experienced surgeon (which mine is). So...my labrum will remain torn. We have no options for my left hip at this time.
LEFT KNEECAP
Where do I even begin with my left kneecap? Back in 2010 I had an MPFL reconstruction with a cadaver graft to stabilize the kneecap from lateral dislocations. At the time I was experiencing 20+ kneecap dislocations a day. We elected to hold off on a Tibial Tubercle Transfer Osteotomy, believing that "less is more." After all, the MPFL reconstruction is not a minor procedure and my Q-angle measurement was borderline. We have learned A LOT about EDS and measurements since 2010 and in hindsight (which is always 20-20) we probably should have also done a TTT osteotomy.
I managed to go 6 years without left kneecap dislocations, which we considered successful. However, in late 2016, my left kneecap started subluxating laterally (to the outside) again. In early 2017 I was experiencing complete dislocations, which then progressed to lateral AND medial dislocations.
When someone dislocates their kneecap it almost always goes laterally (to the outside of your knee). Based on our anatomy, a kneecap should NOT dislocate medially. This is incredibly uncommon. In fact, there are only a handful of cases discussed in literature, most of which came following a lateral release procedure.
Both of my surgeons were skeptical at first when I was describing medial dislocations. However, when they were able to evaluate my kneecap in late 2017 and again in early 2018, it became clear that my kneecap was dislocating in both directions. Unfortunately, there is no surgical procedure to date that addresses medial kneecap instability. And as is the case with most of my problems, there are a number of factors contributing to the situation. I see my surgeon again this week to further discuss surgical options for stabilizing the left kneecap, but based on our last discussion, it will be a very invasive and complicated procedure.
Currently my left kneecap is the priority. I cannot walk on it without my kneecap moving. In fact, at rest the kneecap does not even sit in place. It is incredibly painful. For me to make progress with mobility, and to be able to address other problem areas, the kneecap needs to be stabilized. No procedure will be perfect. But at this point, something has to be better than this (see below):
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| Medialization of the patella (non-weight bearing) |
LEFT ANKLE
About 2 and 3 months ago I rolled my ankle. This prompted me to go back into a walking boot (though because of my knee I am not really weight-bearing anyway). I found out 3 weeks ago that I actually broke my foot again when I rolled my ankle. I have another avulsion fracture of the 5th metatarsal. My left ankle instability is severe. I have no lateral ligament support anymore. However, given the severity of some of my other joints, I am perfectly content wearing a walking boot and/or brace (when my foot is not broken) for now.
How I classify/describe my dislocations:
Easy- easy to reduce (relocate), not very painful, no additional immobilization or support needed
Bad- painful, difficult to reduce, usually requires immobilization, can take weeks to calm down, often leads to numbness and tingling for days, may also impact surrounding tissue negatively (Ex: ribs and scapula tend to shift with bad shoulder dislocations which leads to painful muscle spasms and tension that we work through in physical therapy)
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| Bad shoulder dislocation and the first time I ever needed to go to the ER for a dislocation! |
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