Chris' Journey to Health - Amyloidosis Blog: 2008-09-28

Monday, September 29, 2008

Day 100 - Thanks to all of you

Just a quick update (I will write more in the next day or two), but I wanted to say THANK YOU to all of you who took the time to send something to Chris. It was absolutely amazing to see the outpouring of love, the incredible comments of support, the variety of people, and the resulting stunned reaction from Chris. She was so pleased to read each and every card and realize that so many people were rooting for her. From high school friends, colleagues at work, neighbors, friends of parents and siblings, friends of our daughters away at college, the cards were fantastic.
Chris also received cards from people she did not know, some of whom have been following her journey on this blog, some of whom are going through the same journey themselves. Of course, the cookies, the cakes, the balloons, and the anonymous flowers (beautiful) also were well received and greatly appreciated by Chris. She honestly was shocked at how much people cared and are praying for her recovery.

Chris had a great day. To see the progress from as little as a month ago, would surprise many people. Just 3 months ago, Chris would eat a half slice of toast and a small glass of OJ and 30 minutes later she would lose it. She could not walk 40 feet without having to stop and take a rest, and she was almost in a deep sleep while listening and watching things going on around here. I was honestly scared that we would never get to see again the incredibly strong and fun-loving Chris that we knew just a year ago. Yet, she is really coming back strong in the last few weeks.

So today, we start a new chapter in this journey. The focus now is to:
-get strong and conditioned and feeling physically able to do almost anything
-get back to a normal routine and lifestyle
-get mentally prepared for December when we go back to Boston Medical and come to the next crossroad on this path.

As I have told her many times, her new full time job is to focus 100% on herself.

I cannot thank you all enough. You have been amazing and you have made a huge difference. Trust me. THANK YOU!!!!

What is Primary Amyloidosis?

Amyloidosis
What is amyloidosis?Amyloidosis is a disease in which an abnormal protein (amyloid) builds up in organs and tissues, impairing their function. Amyloid is a protein polysaccharide substance with starch-like characteristics.
What are the forms of amyloidosis?There are different forms of amyloidosis, distinguished by the type of protein fibrils (small fibers) that are deposited. The forms of amyloidosis include:
Primary amyloidosis -- occurs independently of other disease (no specific cause or disease is related to the abnormal protein deposits). Primary amyloidosis typically affects the skin, tongue, thyroid gland, intestines, liver, kidneys, spleen, lung and heart.
What causes amyloidosis?The cause of amyloidosis is unknown. Amyloidosis is thought to result from many factors.
Is amyloidosis a form of cancer?No. Amyloidosis is not a form of cancer, although it may occur in some people who have multiple myeloma.*
Multiple myeloma is part of a spectrum of diseases called plasma cell dyscrasia. Plasma cells are responsible for forming antibodies against bacteria and foreign proteins. For reasons that are unclear, these cells lose their ability to respond to controlling signals from immune cells. Plasma cells then divide and form abnormal proteins that damage the bone, bone marrow or other organs of the body.
What are the signs and symptoms of amyloidosis?
Weakness or fatigue
Weight loss
Heart damage (congestive heart failure)- Shortness of breath- Swelling of the feet and legs (edema)- Chest pain- Irregular heart rhythm- Lightheadedness (due to blood pressure lowering during sudden position changes) in the arms, feet or legs
Kidney damage (renal failure)- Swelling of the feet and legs (edema)- Shortness of breath- Decreased energy level- Anemia (low hemoglobin level)
Skin changes (such as a non-itchy rash, unusual sores or bumps, or thickening of the skin), especially on or near the eyelids, ears and body folds
Abnormal nail changes
Unusual hair loss
Unusual or easy bruising
Abnormal sensations or numbness in the feet, arms or legs
Swelling of the lymph glands
Enlarged tongue (uncommon)
Symptoms related to damage of the autonomic nervous system (autonomic neuropathy) including bloating or diarrhea -- these symptoms are especially present in the neuropathic hereditary amyloidosis (or "familial" amyloidosis)
Bone damage and bone fractures (if large amyloid deposits are present)
Changes in vision (caused by amyloid deposits behind the lens of the eye) may occur in familial amyloidosis
Sudden drop in blood pressure following changes in posture
Who is affected by amyloidosis?Amyloidosis is a very rare disease. About two-thirds of patients with amyloidosis have secondary amyloidosis; less than 5% have primary, familial or senile amyloidosis. There are about 2,000 cases diagnosed in the US yearly. In patients with primary amyloidosis, 95% are over the age of 40 and 66% are men.
How is amyloidosis diagnosed?The diagnosis of amyloidosis is often delayed either because the cause is unclear (as in primary amyloidosis) or the signs and symptoms of the underlying chronic disease mask the signs of amyloid deposits (as in secondary amyloidosis).
Upon physical examination, amyloidosis should be suspected in the presence of any combination of the signs and symptoms listed to the left or when there is:
Slow onset of unexplained protein in the urine
Unexplained kidney failure
Enlarged liver or spleen
Malabsorption of food
Nerve damage
Unexplained congestive heart failure
What tests are performed to confirm the amyloidosis diagnosis?
Blood testsThe results of blood tests can also indicate amyloidosis.
A high platelet count occurs in 5 to 10% of people with amyloidosis and may be a clue to the diagnosis.
Levels of serum alkaline phosphatase (an enzyme in the bone and the liver) may increase and occurs in about 25% of the patients with amyloidosis.
A low hemoglobin level (anemia) is not a prominent feature of amyloidosis but may occur due to kidney dysfunction, multiple myeloma or stomach bleeding. Low blood protein levels could occur because of a loss of protein through the urine and could result in increased cholesterol and triglyceride levels.
ElectrocardiogramAn electrocardiogram (EKG) is a test that detects and records the flow of electrical impulses throughout the heart. When an EKG is performed on a person with amyloidosis, low voltage and false signs of heart attack may appear.
BiopsyA biopsy, which can detect the presence of amyloids in tissues or organs, is necessary to confirm the diagnosis. The most common biopsy sites include abdominal fat, rectum, gums and bone marrow. Biopsies of the kidneys, nerves, heart or liver are sometimes performed to confirm the diagnosis.
How is amyloidosis treated?There is no known cure for amyloidosis, and no treatment has been found effective in reversing the deposits of amyloids. However, there are treatments that can slow the progression of amyloidosis and reduce its harmful effects on certain organs.
Because each type of amyloidosis is different, the treatment will be tailored to each individual. The treatment will depend on several factors, including the organs or tissues that are affected; the severity of symptoms; and the person's age, overall health and medical history.
In secondary amyloidosis, treatment is aimed at relieving the underlying disease. People with renal amyloidosis may be candidates for kidney dialysis or transplantation. Localized amyloid tumors can be surgically removed, generally without recurrence.
Treatment with chemotherapy that is known to be effective against multiple myeloma and other related diseases has been attempted. More recently, therapy with high dose steroids and alpha-interferons appear to be showing promising results and is currently under investigation. Treatment with cochicine (a drug used to treat some forms of arthritis and cancer) has shown to slow the progression of amyloidosis.
Preliminary research shows that high dose chemotherapy followed by bone marrow transplantation may be an effective treatment. However, further investigation is needed.
What is the outlook?Amyloidosis is chronic and usually progresses slowly over a number of years. The severity of the disease depends on which organs are affected by the amyloid deposits. Amyloidosis can be potentially life-threatening when the kidneys and heart are affected.
Early diagnosis is the key to managing the disease before it becomes fatal. The outlook for people with primary amyloidosis has improved due to earlier diagnosis, improved supportive care, and advances in medical management of the disease.

Boston Medical Amyloid Center

Chris is undergoing treatment at the Boston Medical Amyloid Treatment and Research Center. This is one of two centers in the country that have dedicated Amyloid programs ( the other being the May0 Clinic in Rochester, MN). The link to the program overview and a great deal of information about the people, treatments, and research are available at this site: http://www.bu.edu/amyloid/index.shtml

There are many sites on the web that describe the disease, the treatments, the research, etc. With great urgency, we did extensive research on Amyloid programs and are convinced that this program is the best in the world. It is amazing that it is just 20 miles from our home in Holliston.

Chris' Journey to Health - Amyloidosis Blog

Monday, September 29, 2008

Day 100 - Thanks to all of you

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Chris' Journey to Health - Amyloidosis Blog

Monday, September 29, 2008

Day 100 - Thanks to all of you

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Stephen's Amyloidosis Reseach 1500 Mile Bike Ride

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What is Primary Amyloidosis?

Boston Medical Amyloid Center

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