Organization: Medical Supplies and Portfolio

Let's talk about organization. Organization is my thing. Not only am I prepared for nearly every situation but I am ridiculously organized (almost to a fault). I suppose it comes with being a perfectionist. Perhaps its my way of trying to regain some control in my life. Regardless, for me, my life runs more smoothly when everything is in order. In fact, a lot of people have told me they wish I could organize their life.

My closet it color coded, my medical supplies are organized by categories, my medical folder (accordion) is organized by specialty, and I keep all of the most important documents (copies at least) in a portfolio that I always have with me. Even my backpack is organized (see post on What's in my "Purse": Chronic Illness Edition). Basically, everything has a place. I may be the only one who understands my system, but seeing as I am responsible for my own health and treatments, that's okay!

I will break this post down into different sections and explain how I organize each area. Keep in mind that it is not possible to break down every little detail but I will do my best to show you my system. Maybe it will help some people understand how my brain works (because this is how I organize my life...not just medical stuff).

Medical Supplies
I have A LOT of medical supplies. This is largely due to the fact that I have a central line and there are a lot of supplies that are needed to not only care for a port-a-cath but for running infusions through my line. I have a system in place that accounts for daily and weekly needs, as well as a storage system for as needed and extra supplies. This also makes it really easy for me to pack for trips as everything is broken down into categories and I can go through my checklist and grab what I need from my different bins.

Extra Supplies and PRN (as needed) Supplies
My bins are organized by category. I have 3 main bins in my room.
1) Port and infusion supplies

Bin contains: extra access kit, swab caps, alcohol swab sticks and skin prep, chlorhexidine wipes, extra dressings, emesis bags, masks, sterile and non-sterile gloves, Purell, hand wipes, alcohol wipes, thermometer 

*Not pictured- bin of extra infusion bags and extra boxes of flushes (saline and heparin)

2) Tubing for infusions
Tubing takes up a lot of room as I have two different types-- dial-a-flow (gravity) tubing and pump tubing. I also change my tubing with each infusion so I go through at least 3 per week.

Dial-a-flow tubing and pump tubing with batteries (for the pump)

3) Allergy and asthma supplies

I have allergic asthma and do daily nebulizer treatments in the Spring and the Fall so I go through this bin quite often. This is also where I store any backup rescue medications (epi-pens, inhalers, nebulizer solutions, benadryl, etc.). If it has to do with breathing or allergies, it goes in this bin!

Bin contains: rescue medications (for nebulizer), tubing and masks (for nebulizer), extra spacers, extra rescue inhalers, extra nasal sprays and other allergy medications and creams, extra epi-pens

How I store my masks (there is another door as well not pictured)

Weekly Supplies
I do weekly infusions (3x/week) and I tend to run my fluids at night while I am in bed. As a result, I have a side table with drawers that has everything I would need to run a bag of fluids. I simply restock these drawers from my extra supplies when I am running low. The drawers typically hold at least 2 weeks worth of infusion supplies.

Tubing, alcohol wipes, flushes (heparin and saline), batteries, tubing caps

Bags of fluid (Lactated Ringer's and Normal Saline)

Daily Supplies
I have various treatments everyday. While I may not be infusing fluids all the time, as long as my port is accessed I have to flush the line daily. I also have daily medications, breathing treatments, and various PRN (as needed) medications that I may take. This is all organized on my bathroom counter.

Daily flushes (saline and heparin), alcohol wipes, swab caps, extra biopatches and injection caps, PRN (as needed) medications, bandaids, Purell, syringes

*On the main floor of our house I have pre-made access kits. I recycle the zip-lock bags my infusion company sends me with monthly deliveries to make these ready-made kits. Because we access my port every Monday (downstairs), it is easier to have them on the same level.

Access bags, extra supplies for the kits

Medical Folder/Accordion 
I have accumulated a lot of files over the years. I have visit summaries from appointments, insurance documents (bills, claims, etc.), discharge summaries from hospitalizations, operative reports, physical therapy summaries, and more imaging discs than I can count.

I have organized the accordion by medical specialty and topic. I have a separate binder of all of my x-rays, MRIs, and CT scans. I ALWAYS ask for copies when I have imaging done so that I don't have to track them down later if another specialist requests to see them.

Imaging binder

I also request operative notes/reports at my first post-op visit so that I always have them on hand as well. In the past, I have had issues with some hospitals and getting records when requested. I have found it less stressful to ask for the records during appointments as they can usually print them right then and there. It saves a lot of time spent on the phone trying to figure out why a specialist never received your records.

Two accordion folders: (left) insurance files; (right) specialists and other medical

Inside look

Medical Portfolio 
My medical portfolio is a collection of documents I have found important to have with me for new patient appointments with specialists.

What I include:

• Complete medical history (combination of everything else on this list)
• Diagnoses
• Surgeries
• Current medications and allergies 
• Anaphylaxis protocol
• Specialist contact information
• Port-a-cath placement radiology report and x-ray disc (many facilities have their own policy on using central lines and almost everyone requires proof that the line is in the correct place)- I update this once a year 
• Neurosurgery operative report 
• Genetics letter confirming diagnosis of EDS 
• Current diagnostic criteria for EDS
• Information sheets on Dysautonomia, POTS, EDS, and Chiari Malformation 
• Recent labs 
• Pad of paper for notes 

 

Information on POTS, Chiari Malformation

EDS diagnostic criteria, information on Dysautonomia

I also have business cards for my specialists so I can give it to other doctors who may want to contact someone else on my health team. I make sure to carry a pen with me as well (both in the binder and in my backpack).

I am constantly updating my portfolio. If I come across something that would be important to have with me, I make copies and add them as necessary.

I am in the process of coming up with a "Treatment Protocol" that my PCP and cardiologist will sign to make it easier for me to seek help in an acute situation. With my complicated history, many facilities simply do not know what to do with me. Having a treatment protocol signed by two members of my healthcare team should help provide some guidance to new providers should I need to seek emergency treatment or care.

When you are complicated, it pays to be organized! 

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Welcome Back

Wow! It has been a long time since my last post; more than 2 years. As one could imagine, a lot has happened and changed in my life. However, I have been thinking about coming back to this blog for awhile now and I think I just may give it another shot. Given the significant time lapse, I will update on my joints and POTS in this post, and provide an update for other systems in a separate post.

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):

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)

Bad shoulder dislocation and the first time I ever needed to go to the ER for a dislocation!

In my next post I will update on my new GI diagnosis and headaches/cervical instability.

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