What are the steps to a life well lived?

You have to figure out your goals. You have to figure out what bugs you and come up with a plan to fix it. I'm doing it. I'm motivated and confident.

I am determined to lose significant weight and strengthen my weakened muscles. Tonight I joined a weight loss challenge at a gym with personal trainers and a nutritionist.

I have had some obstacles to overcome. Most of my muscles are very weak due to the hypermobility. You may not know this, but when you body isn't holding itself together properly, you have difficulty activating and strengthening your muscles, because things aren't where they are supposed to be and working as intended. 

Exercise has to be very specific - directed at the muscle, and avoiding rotations or stretches.

I am very prone to injury and a slow healer (as my ankle sprain/ligament detachment can attest.. I'm still healing from my injuries last November).  I'm also prone to muscle failure (complete failure) and sudden onset of massive fatigue.  I have to be careful. Having a personal trainer is going to be key for me.

This gym will modify exercises to meet whatever your special requirements are. I need that really bad.

Another obstacle I have are my massive allergies and things that provoke the mast cell activation syndrome. I get anaphlaxis. I get massive facial, body, and limb swelling (angioedema) and migraines. I get stomach upset and .. the downstream effects of poor gastric motility.

They have a nutritionist that works with you to ensure that you are eating the right amount and not triggering allergies. I have over 200 allergies plus a bunch of non-allergy food that provokes mast cell activation attacks. I really need the help.

I'm very excited to begin this challenge and gain friends in the fight.

20 pounds in 6 weeks.  I'm going to do it!

Happy Husband, Happy Life

I am not particularly tidy. I'm hygenic. But I leave stuff piled everywhere until it bothers me, and then I have to go on a wild cleanup ride before I can concentrate on anything else. Being married means that my behavior also affects my partner. Although I try to rein in my untidiness, it's not a easy thing for me to and consequently, my success rate is highly variable.

When my hubby starts getting anxious about the house being untidy, I've asked him to let me know and I roll up my sleeves and get busy. If the kids are available, I get them involved too.  I don't resent this. I feel like I'm getting my due for being messy. That's fine. I'm completely ok with cleaning stuff up.  I don't want to be nagged, followed, given a hard time, or have to listen to a long lecture about it. My 50% is cleaning it up and his 50% is letting me know timely and disappearing until it's fixed.  It works pretty well.

Except..

I have so little endurance now. I have to pick and choose very carefully which targets I hit hard because I run out of gas really fast. What does that mean? My heartrate shoots up well over 100. (The highest so far was 165 or so, I think).  It gets hard to breathe. My muscles start to burn and hurt really bad (probably because they aren't getting adequate oxygenation and because my skeletal structure and musculature don't provide enough support for the activities I'm doing), and my muscles weaken, making it difficult to lift, fold, bend, carry, walk, and go up and down steps.  The more I do, the worse everything gets until I get to a point (which I am learning to avoid) where I sink to the ground, have great difficulty breathing, and can't move my legs or arms very well.  It feels kind of like if you took off your arm and replaced it with a big wood stick and then tried to move it around.  It can take several weeks for me to recover to my baseline.

I feel guilty when I can't clean as much as I want to. I feel guilty for my chore list getting smaller. I feel guilty for only being able to clean 2-3 times per week and having to rest so extensively in between every 15-30 minute cleaning spree. (Usually I have to rest for several hours between and limit those activities to a maximum of 3 per day, 2-3 times per week.

I want to do more.

I feel driven to do more.

I used to do so very much more.

It's hard to let go, accept, and be loving to myself when I come face to face with these limitations and how they affect not only me, but also my family.  I'm working on it. I am blessed to have an understanding, loving family who helps me without resentment.

Talking About Illness

When you have a vast array of symptoms, they don't come on overnight, typically. Usually they come on slowly and snowball into a huge mess.

It is very hard to deal with health issues over and over and repeatedly having to set new baselines at a lower level for functional life. It is draining. It's scary. It requires a lot of change in your life. Not only do you have limitations that take away things you used to enjoy (sometimes even things that felt like huge, exceptionally important parts of your life), you also have a wide range of advice from doctors. If you fail to comply, they will sometimes drop you as a patient and mark in your chart the dreaded "non-compliant" - which can make new doctors reluctant to take you on a patient. In that sense, your life isn't your own anymore. You now have to be obedient to another person, who may or may not fully understand the ramifications and difficulties of the changes they want you to make.

These things drag at your soul. Depression, anxiety and fear are very common. Not only are you dealing with the immediacy of the now but also looking at the future with trepidation. If the past informs the future, then you know you are in for a rough ride.

It is helpful to be able to talk about these things - to share things you learn, to grieve over what is lost, to get encouragement about facing the now and the future. But these topics are not on anyone's top 5 conversations to have. People want to talk about good times and fun and things that touch us in good ways. That is now how these particular conversations go.

It is hard to maintain friendships because of this. Not talking about something that is a huge part of your life is hard and, to me, feels fake. I can say, "I'm fine" when someone asks how I am. But, honestly, I feel like a liar and like I am unable to be my authentic self, and unable to just own up to that I feel like crap because it hurts to move, I can't breathe, and I itch all over. 

Another component of this is having a genetic disease. I have many genetic diseases. I want my extended family to know about them. I want them to know that these issues run in our family and what they look like so they can get prompt care if they recognize the symptoms. I don't want anyone else to have to wait 30 years for diagnosis and treatment. I don't want anyone else to have to put their financial health in jeopardy by going to hundreds of doctors visits - most of which are not helpful. I don't want anyone else to have doctors look at them in disdain and tell them that they have a psychosomatic illness. It's hard. Actually, it's more than hard - it's humiliating and crushing. It was very hard for me, personally, to keep pushing to find out what was wrong with me. I quit a hundred times. The light that kept me going was wanting to be the best person I could be for my immediate family. I couldn't know if I were doing that without knowing what was wrong with me -- whether it was curable or treatable. Waiting sometimes causes irreversible damage. I didn't want to be kicking myself later to find out that I had a treatable illness, but missed the window to get better because I stopped looking. 

But ... back to talking .. family members are just people. They don't like having the conversations either.  Social skills are not my strength. But I am pushing past my own discomfort to try to help others.

There are many aspects of living with chronic illness that suck.

Mast Cell Activation Syndrome - And How to Have a Really Really Flexible Life

I have Mast Cell Activation Syndrome (MCAS). This means that my mast cells, which are a function of the immune system, get way over excited and produce too much of the many types of hormones they can produce. Unlike allergy triggers which you can often identify through testing and the process of elimination, mast cells can flare at just about anything. Stress?  Check. Too little sleep? Check. Oh, liked apples yesterday but not today? Check.

When mast cells flare it triggers a cascade that looks just like an allergy reaction, which means it can look like a lot of different things. Anything an allergy can do, a mast cell reaction can do better and longer.

I had a big flare two days ago. Hives, welts, swelling, asthma, intense itching, headache.

Now, I know I have MCAS and I am under treatment. I take 5 medications every day to prevent allergies, hives, itching, tamp down those mast cells, and treat my asthma. That was not enough.

I ended up having a trip to the allergy/immunology specialist I see who is amazing, but who is 1.5-2 hours away. I had to drive myself, which I don't like to do because of my daytime sleepiness, but it was an emergency - the hives and asthma were getting worse despite all of the drugs in my arsenal, and no one else could take me. He ended up giving me a shot of benadryl, so hubby had to take off work and come and get me anyway.  (Then he went back with a friend to pick up my car today.) I also got steroids to take for 12 days, which the allergist warned me would probably not be enough. (My research showed that treatment resistant hives can last for several weeks.)  I got one of my medications switched out with another one because it's causing swelling (angioedema). But the new one - benadryl again, has to be compounded by a specialty pharmacy because I'm allergic to the dyes and preservatives in the over the counter benedryl. I also got another inhaler to add to my other two.

This is life with mast cell disease. My medications regularly change due to adverse reactions. I have several that are specially compounded for me with hyperallergenic powders and no dyes or preservatives. Even hypoallergenic products often cause adverse reactions. And I never know when I eat if my body is going to like what I put in it or not.

All of this makes my life really random. I never know how sleepy I will be, if I will have insomnia in addition to the normal daytime sleepiness, if I will have an adverse reaction, need to be near toilets or ice packs, or if I will be really out of breath, and I need to always have access to water to take medication. I take medication a minimum of 4 times a day.

I plan things, but I often require flexibility in making the plans happen. Dinner with Mom may be put off for a month. I may sleep through movies with the kids or be up all night and dead to the world all day. I really just don't know. I may plan to work on drawing and clear my schedule for several hours only to have blurry vision or tremors and have to go do something else instead.

This is life with mast cell disease.

Type B Discipline - Is Not Really Discipline

Since I first became ill all of those many years ago I have worked with a platoon of doctors. Each has their own recommendations about diet, exercise, medication, and lifestyle. Sometimes they agreed or disagreed or gave conflicting advice. As I became more ill and ended up with a small cohort of doctors, my list of medications and recommendations grew to gigantic proportions. I think it is underappreciated by doctors and by people generally how much change, adaptability, and sheer discipline is required when you are ill.

First, medication is required on a specific schedule. It must be taken a certain number of hours apart and sometimes with food or without food or in liquid. (This means that food and liquid need to be immediately available and the food and liquid have to be part of the carefully crafted diet.) Reduced calorie and special nutrition diets need careful counting and have to be consumed across hours in such a way that blood sugar doesn't surge or drop. You also can't just thoughtlessly grab whatever in the fridge or pantry looks good. It needs to be the food that best meets your caloric and nutrition requirements when taken in consideration of the dietary whole. This often requires being near a refrigerator and/or stove/microwave. Some diets do not lend themselves well to packaging or room temperatures.

When you have bad reactions to food or medication, your body may need a time out to get back to normal. The inflammation needs time to go down. You may need to do an elimination diet with food and/or medication to try to determine which is the trigger. (Sometimes the trigger can't be identified.) Then you end up off of your medication/dietary schedule and need to go back on again. You may also have a specific sleep schedule to try to adhere to. If so, this may also come with advice about how much time before bed devices and lights need to be turned off and have medication requirements tied to hour of sleep.

All of this requires planning. It requires careful execution. It requires staying tuned in to how much medication is left in a bottle and ensure that new meds are ordered in a timely fashion. It may require filling out medication holders. It is not easy. It seems like it should be easy. Really, it is a lot of steps that need to happen whether you feel like it or not. It requires discipline. If you are a Type B person, forcing yourself to be disciplined can be extraordinarily hard. You may feel like you are having to be someone else in order to make the magic happen. It can be an uphill battle. It is for me. I find I have to take breaks from discipline so it doesn't break me. I can do one thing repeatedly without problem. Two is harder. Ten feels impossible. But I try. Every day is a new day to try and get it right.

Who Am I? Am I Still Me?

Chronic illness has changed my appearance a lot. I went from being an average sized girl with cute features and active lifestyle to an overweight, lumpy, swelling person with very limited activity. It is increasingly painful for me to walk, even with crutches, two doors down from my house. The longer I try to walk, the more pain and dysfunction in my legs and pelvis I experience. I have difficulty bending down, crouching, and of course, getting back up again. It's not just a matter of obesity. I strongly suspect I have lipedema. The tissue in my legs is very painful. It doesn't have to be touched to hurt. My legs, hips & butt are lumpy and swell regularly. When I was young I was determined that I would be the kind of Mom that sat on the floor and played with my kids. I would be fit. I would be active. I never even dreamed that something like chronic illness could take all of that away. I went in 2 years from being cute and having people look twice at me to having a swollen, doughy face and doughy body. But it's not just my appearance that was stripped away. I went from being very smart to being very average. I have difficulty with my memory and processing skills. But I can adapt. And really, if I want to look back on my life without regret (my goal), then I MUST adapt. I need to find a way to be at peace with that stranger in the mirror. I need to dig deep and find within the things that make me unique. Things other than being smart. Things other than being creative. Things that may be less tangible. Because this is life. And in life there is no giving up. Time keeps pushing us forward. We keep moving or we get rolled over.

Life, She is a Changing ... and I am Changing with Her

The last few years have brought tremendous changes in my life. I went from travelling every week for work to finding my dream job locally. I was well respected in my field and loved my work. My family is wonderful and likewise was doing very well. I found a very comfortable niche. In the midst all of this life goodness, I continued to get sicker. I went from playing pick-up soccer to roller skating to walking to doing not much at all because my legs began to fail me. I have been gaining weight despite dieting, experiencing a lot of pain, migraines, breathing problems, so many things. Getting assistance from doctors was difficult for a long time. When you are overweight, that is all they see. As my body continued to spiral downwards, finding compassionate doctors who were willing to look beyond the obvious and really dig in with me was very difficult and there were a lot of humiliations along the way. Finally, when my thyroid died in 2015 I was able to get doctors to take me seriously. It was the beginning of putting together a crackerjack team of doctors who finally figured out what was wrong with me. For those of you who do not know me, I have been experiencing increasing debilitation across most of my body's systems for almost 30 years. It wasn't until I found a good immunologist, neurologist, and family doctor that I was finally diagnosed with the conditions that have plagued me for years. I have over 12 conditions diagnosed so far. 5 or maybe 6 of them are rare conditions. It is completely overwhelming. Despite being overwhelmed with my diagnoses and the things I need to do in order to feel better (and the reality that I can only feel so much better and it will likely continue to get worse), I try to stay focused on life. I have a great family, many things to do, etc. But, I have to admit, it has been difficult in the face of increasing illness. This year I finally became sick enough that I could no longer work. It was very demoralizing to reach that conclusion and to face the scary future. Being disabled means different things to different people. For me, it has stripped away all of the things I used to self-identify myself - being super smart, cute, active, successful. I have had to dig deep to find what still makes me unique and different and special and focus on bringing those things into the light. Without that, I would probably have succumbed to despair. I have many ideas of things I would like to do - from advocating for animals to writing books, but I am currently grounded by fatigue. I hope to one day be able to fly up high with my ideas and put them into action. In the meantime, I am learning about my conditions, advocating for myself, and trying to develop the discipline to take care of all of my conditions properly. I hate discipline. I hate even the idea of discipline. But discipline is necessary. It is a careful balancing act. I am hoping to blog more. I would like to improve awareness of my rare conditions. I would like to connect with others who have similar stories. I would like to change the conditions that make diagnosis of rare illnesses so difficult. Early intervention is critical. Waiting 30 years is too long. I hope you all are doing well in your own lives, and if you are not - I hope that you make great strides this year. All of our challenges are different. Namaste.