Even when I was the sickest, I was trying to think of others and it helped. —Linda Danielson
LOGAN — It was two days before her first and most critical chemotherapy treatment and Linda Danielson was visiting other patients at the hospital, handing out small gifts she had gathered prior to being admitted.
The tokens of kindness served as a diversion to a recent diagnosis of a rare and potentially deadly disease. Danielson, a retired nurse, was trying to deal with what was to come.
"Even when I was the sickest, I was trying to think of others and it helped," she said. "Little tiny things like that, when you go into a very scary situation, it can help you instead of just laying there and thinking about how sick you are and wondering if you are going to die."
At that point more than a year ago, Danielson didn't realize how important her positive mindset would be in getting her through what ended up being months in the hospital. Helping others has now given her life new meaning.
In the past two years, 59-year-old Danielson had gone through myriad medical tests. The elusive and mysterious sickness had attacked her heart, liver, kidneys, nerves, gastrointestinal tract and thyroid. Weight was "literally falling off me," she said, recounting a sudden 35-pound drop.
"I felt like my world was upside-down," Danielson said. "As sick as I was, I really wondered if I was going to die."
Doctors couldn't pinpoint a cause, and her "list of 18, odd things that had changed in the last two years," including constipation, anemia, unusual fatigue, nausea and severe back pain, wasn't helping to draw any conclusions for a variety of specialists who had become involved in her treatment.
"They didn't know what they were looking for so they didn't see it, but it was there clear back then," she said. "I was dying in front of my doctors' eyes and they couldn't find out what was going on."
Ultimately, a precautionary kidney biopsy revealed primary systemic amyloidosis, a rare disease that results from problems with plasma cells cloning themselves and leaving protein deposits within vital organs, said Dr. Finn B. Petersen, a hematologist and oncologist at LDS Hospital.
The head of the hospital's bone marrow transplant program said amyloidosis impacts only a few thousand people across the country each year, with most victims of the disease in their 40s or 50s. Amyloidosis is in the same family as multiple myeloma, but does not grow or expand like the cancer does in the bone marrow.
Instead, amyloidosis travels throughout the body, rendering various organs dysfunctional.
"It can totally impact the quality of life," Petersen said.
He said it is difficult to diagnose and to care for amyloidosis patients because of the involvement of multiple organs and the vital roles each plays in survival. Early diagnosis, Petersen said, is key to increased survival rates.
With treatment — a potentially life-changing but strenuous stem cell transplant — Danielson was given about two years to live. Last week, she happily celebrated her first year.
"How we look at anything in life, the choices we make, how we are going to feel and how we are going to act every single day can make a difference on how our life turns out," she said, adding that with the support of others, she worked through various stages of grief upon diagnosis.
"I chose to be as positive as I could be."
With some direction from doctors and hospital staff, Danielson sought out others who had survived the fateful illness, and landed herself in a seat at an amyloidosis support group that meets four times a year in Denver.
"What a difference it made for me and my family to know that there was somebody out there — they had speed bumps, they had a few problems, but they're alive and they're functioning and they're doing well," she said.
Approximately 2,500 individuals are diagnosed each year in the United States with one of five types of amyloidosis. With such distinction, it falls under the National Organization of Rare Diseases and actions are being taken in Washington to get grants to assist in research and increased awareness efforts for medical schools and practicing physicians.
The condition is likely so rare because it is not often considered in complicated patient circumstances, according to the organization. It is often just missed.
An online support group, www.amyloidosisonline.com, encourages early diagnosis, which leads to decreased morbidity rates, as the best response from treatment comes in healthier individuals who are not yet broken down by the disease.
"I'm alive," Danielson said. "I have amyloidosis for my entire life, it can awaken at any point and I'm never going to be free from it, but I am responding to treatment."
Treatment options are similar to those used in treating multiple myeloma and involve paralyzing plasma cells to stop production of the protein deposits and then reintroducing healthy cells to encourage bone marrow production after chemotherapy kills it all.
All of it is done under close supervision of physicians and at least a few weeks in the hospital, Petersen said. He said without the stem cell transplant, patients can expect to live a little more than a year, but with it, and no major organ damage from the disease, "the average life expectancy exceeds eight to 10 years."
LDS Hospital treats an average of six or seven amyloidosis patients a year, both from within the state and outside of Utah. The prognosis is different for everyone, but Petersen said the earlier treatment can get started, the better.
"You have to be in tune with your body," he said. "If something is wrong, have it checked out and the likelihood is that it won't be amyloidosis, but it is always best to alert a medical professional when something is awry."
Safety in numbers
In the course of her ongoing treatment, which includes rigorous blood tests every three months and an echocardiogram each year for the rest of her life, Danielson has learned a lot about the disease and is looking to teach others, and provide a similar support system that she believes saved her life.
She's been talking to survivors in India and the Netherlands, but would love more than anything to have a local support group, with friends to band together.
Denver has a survivor community large enough to host quarterly support meetings, which offer the latest research efforts, question-and-answer opportunities with physicians and caregiver support. But Danielson doesn't want to have to travel so far to find others with the disease and she believes there are people in the state who could benefit from such a friendship.
"Getting the word out publicly not only alerts family members of this disease, but it also increases awareness so that it isn't missed," she said.
Danielson is prepared to sell T-shirts, bracelets and pins to help raise funds for a local amyloidosis support group, one that could provide help to others battling the disease in Utah.
"I hope they get what I got, which is hope," she said. "So they can say, 'Hey, I can live, I don't have a death sentence, and I can contribute by encouraging people that I am still a functioning person and I can still learn and stay on top of the disease so it doesn't get the best of me.'"
A community of patients, she said, would give each of them another reason to go on.
"I'm going to be the one who's going to be around for a while," Danielson said. "I'm here because I'm supposed to be. I had several brushes with death and if I was supposed to be dead, I'd have been dead. But I'm not, so I'm sure I have something to accomplish still."
Danielson has four adult children who don't have to worry about the disease in themselves, as her type isn't hereditary, but likely brought on by a combination of environmental or viral/infectious exposure. But they now continue to benefit from her good deeds, something she learned the importance of during chemotherapy.
"It'll be interesting over time, to see if they ever figure out what causes this," she said, adding that she'll probably not live long enough to witness the discovery of a cure.
Amyloidosis symptoms (although they mimic those of other diseases): shortness of breath, fatigue, edema, enlarged tongue, bruising, tingling or numbness in the extremities, sudden weight loss, elevated cholesterol, weakness