Progress notes on coming to terms with life and death

As my therapist says, “how interesting” to see my life from the top of the mountain, from a god’s eye perspective.

I have been climbing up this rocky mountain my entire life. It is strewn with boulders and obstacles that have required me to go over, around, or through in order to keep making headway. Every now and then, at the end of a switchback, I glimpse a peek of what might be on the other side. I have spotted trees, a river, the sea filled with seahorses, squid and many other beautiful and magnificent creatures, I have swum with whalesharks, and I have heard the songs of birds. But as I try to climb over to get to that place where life is wonderful, another obstacle jumps in front of me – but I keep striving. This month, I have finally gotten to a place where I can see where life is worth living. Where there is goodness and joy, friends and a loving husband.

On Friday, I actually said that I have discovered that I want to live, and that threw me into a maelstrom of conflicting emotions. I asked how can I resolve the differences in my life and my doctor told me I don’t have to resolve them. I just need to accept them. Life is complicated. My life has been especially complicated, but I have worked hard to learn to live it with joy.

I have talked of how other people with MSA go to special therapists to try to learn to walk better, or to balance better, or to breathe better. I have done it myself. No, I am not perfect, but I have learned to balance well enough (except for those days I am too dizzy) to walk without fear of falling – I have learned how to catch myself so I don’t fall. I am reprogramming my brain. My therapist doc says I have taken the act of walking out of the brain stem’s automatic pilot and run new neurons around the top of my headin my cerebral cortex so I put “walk” into manual drive where I consciously control it. I have to think about it, watch where I put my feet, be aware of obstacles on the ground in order to not have a misstep, but I concentrate and get it done.

My pulmonologist said once you get hyperventilation syndrome, you can’t get better – except he has heard that those who meditate a lot and/or do yoga, have been able to learn to control that autonomic system. I have done a lot with that. I can slow my breathing (granted, it takes a few minutes if I have had to really struggle physically doing something – but I can do it.)

I still have more growth to work on. The major area of growth to concentrate on now – that I am worthy of anyone’s friendship and respect. That I do not have to listen to old “tapes” from parents about not being good enough, or about being afraid I will be judged by others. That I am not guilty of anything. That seems like a lot (looking at how often I say that I am sorry), but look at how much I have already done. More than I ever believed possible back when I started therapy. My psychiatrist asked me how far I wanted to take my therapy, and I told him, “all the way. As long and as far as I can”. And, I am doing that. I am working. I tried to say how much my herapist has brought me along and he said, “no – you have worked for everything you have gained. He has only been there to guide you.” So, even though there is more to do, I am doing it. To really accomplish changing the life of someone who has climbed that mountain, it takes many years of hard work to accomplish true growth and “rewire” the mind. In intend to change my mind and choose LIFE. To live with joy. To review in my mind, daily, the joyous and loving experiences of my life.

Toward a better understanding of MSA

I have Multiple System Atrophy (MSA). This is a progressive, degenerative, and fatal disease of the brain. At this time it affects multiple areas of my brain including my left cerebellum, my midbrain, my left frontal lobe, and my left temporal lobe. The autonomic system that controls blood pressure, balance, and speech, if the most noticeable.

Some of my symptoms include:

• Balance problems: Falling frequently and unexpectedly
• Vertigo and/or dizziness when standing or walking
• Unable to walk or stand for more than a short period of time
• Hyperventilation during movement
• Muscle weakness
• Inability to look up or to the side when walking, resulting in bumping my head on anything that projects out just above my eyes
• Speech problems, stuttering, unable to speak the words I am thinking
• Hesitation when asked a question as I translate the words
• Choking and swallowing problems, especially when drinking liquids
• Taste and smell differences
• Inco-ordination – knock things over and drop things frequently, writing is difficult
• Muscle stiffness after sitting for more than a short time
• Breathing difficulties

What is the progression of MSA?

Disease progression in MSA is quicker than in Parkinsonism. Almost 80% of patients are disabled within 5 years of onset of the motor symptoms, and only 20% survive past 12 years. The mean survival is roughly 6 years. Rate of progression differs in every case and speed of decline may vary widely in individual patients.

Patients usually have autonomic nervous system dysfunction first. When MSA begins with non-autonomic features, imbalance is the most common feature. This difficulty in maintaining balance may be due to either cerebellar or Parkinsonian abnormalities. Some patients complain of stiffness, clumsiness, or a change in handwriting at the onset of MSA.

MSA progresses over the course of several years to cause more widespread and severe symptoms. Orthostatic hypotension can cause fainting and falls. Loss of coordination, slowed movements, and rigidity can interfere with activities of daily living.

Some patients with MSA have mild loss of cognitive abilities, with impairments in attention and speed of thinking.

Complications include:

Progressive loss of ability to walk or care for self

Difficulty performing daily activities

Injuries from falls/fainting

Side effects of medications

Orthostatic hypotension (a large drop in blood pressure upon standing) is common and may cause dizziness, dimming of vision, head or neck pain, yawning, temporary confusion, slurred speech, and if the hypotension is severe, the patient may "faint" upon arising from a recumbent position.

In spite of low blood pressure while standing, it is common for MSA patients to have high blood pressure when lying down. A fall in blood pressure following meals or in hot weather or following infection is quite common.

The concurrent involvement in MSA of multiple brain systems subserving movement, including the striatum, cerebellum, and cortex, leads to the movement disorder as often being the most profound disability. Hoarseness or even vocal paralysis is relatively common, as are sleep disturbances, including snoring and sleep apnea. The ability to swallow foods and liquids may be impaired.

How long can I expect to live?

The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops. Early death is likely. The mean survival is roughly 6 years. Most people who are diagnosed with Shy-Drager syndrome die within seven to 10 years after symptoms begin. It is rare for a patient to survive 10 years.

The autonomic abnormalities are seldom the direct cause of death. A significant number of patients develop laryngeal stridor and difficulty swallowing, which can lead to pneumonia.

Pneumonia is the most common cause of death, although irregularities in heartbeat or choking may be responsible for death in some patients. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest are common causes of death.

In addition, many patients with MSA experience periodic respiration and in some cases this may lead to a critical loss of respiratory drive, so called Ondine's curse.

Pulmonary hypertension may occur during apnea. The most common causes of death in patients with MSA are pulmonary embolus, apnea, and intercurrent infection.

Treatment

Currently there is no cure for MSA and no known means to slow progression of the disease. A number of drugs can be used to treat the various symptoms of MSA, although they become less effective as the disease progresses.

Learning I had this disorder

This blog will try to explain what it is like living with several severe disabilities for more than 30 years, and with two fatal ones - kidney disease from birth, and MSA for the past 9 years.

It has been a long road of doctors and tests stating 5 years ago as my physical condition deteriorated until I finally had to leave my post as Library Director and go on Social Security Disability. After all the doctors (20 in all) I finally found one who diagnosed me with MSA. What small letters for such a big disorder. To go from a successful Library Director to someone barely able to walk or talk. I took to the Internet and discovered the Shy-Drager Syndrome Yahoo Group where I have learned all I know about this, and other, Parkinsoniasm Plus disorders.