Lust for Life
A few year back when I was going through a particularly tough period in my life, my wife Kathy wrote me this poem:
Lust for Life
I want breadth and fullness,
Juices spouting sprays like rain.
Giant moons glowing, sagging the sky.
Preposterous flowers with aching stems.
Trees whipped by shrieking winds.
Waves swirling and reaching.
No hollowness, no withered core,
Or shrill and desiccated soul.
Shatter the brokenness and find life again.
Rage with anger.
Burn with love.
Let life consume.
That is Kathy through and through. Her passion for life during these 39 years has always been so evident and so beautiful. We thought we had faced our sole life medical struggle with years of undiagnosed infertility. But we were wrong. We were so wrong. Kathy was diagnoses with primary AL amyloidosis, localized in her throat, in June of 2000. Although it was a year we both want to forget, our struggle has only begun. Up until 2000, Kathy had been the perfect picture of a healthy woman. She had never stepped foot in a hospital as a patient other than one emergency room visit years ago.
In March of 2000, Kathy noticed she was becoming increasingly winded after her three-times-a-week walks with her friend. This came with a corresponding hoarseness in her throat that sounded like laryngitis. At that time, Kathy’s primary care doctor treated Kathy for what she thought was allergies. In May, as these symptoms were getting progressively worse, Kathy was referred to an Ear Nose and Throat (ENT) specialist here in Charlotte. He did a laryngoscope on Kathy and noted she had significant swelling in her throat, probably caused by acid reflux. He prescribed steroids and prilosec. The two week follow-up exam revealed a slightly worsening situation, which was confirmed with a CT scan the next day, June 1, 2000. In concern for Kathy’s airway, the physician suggested that she be given a tracheostomy (breathing tube inserted through her throat into her airway) and that the growth be biopsied. On June 5, 2000, Kathy was admitted to Carolinas Medical Center and these procedures were done. I remember the ENT surgeon saying that he had not ever seen a growth like that. It did not have the definition of a cancer tumor. He quietly mentioned that he would also test it for a rare disease called Amyloidosis, “just to be sure”. We are so lucky he had the foresight to do that! Kathy spent five days in the hospital while her trach site healed and we learned how to care for the trach. We went home without any final diagnosis – that would come the following week.
We met with the surgeon that Tuesday, June 13, 2000. “You have a systemic blood disorder called Amyloidosis. It is very serious and has a wide range of potential implications. It can be fatal.” He referred us back to her primary care doctor for a systemic work up. I remember those first sleepless nights with this fresh news in my mind. I remember planning Kathy’s eulogy all night long – I couldn’t get it out of my mind.
Between these physician visits, we jumped all over the internet trying to find out what this was all about. I know our friends did too, because they were all so quite as they learned about this tragic disease. I quickly learned of the great reputation and work of Boston University and sent Dr. Skinner an e-mail. She quickly responded saying it sounded like Kathy’s case was localized and that research is showing that localized cases rarely spread systemically.
Kathy’s primary care physician spent the weekend prior to our visit in the library researching this disease – she would never see a case like this again! After a tearful meeting with the primary care physician, we proceeded with a battery of tests that our fellow amyloid patients are all too familiar with: bronchoscopy, bone marrow biopsy, fat pad biopsy, 24 hour urines, liver function tests, echocardiogram, and other blood work. Everything came back negative for amyloid protein! We were elated. Kathy’s amyloid is truly localized in her throat. Now what do we do? Let’s cut it out, remove the trach, restore Kathy’s voice and get on with our lives. Wrong.
In August, we went for a work up at Boston University and a consultation with a Boston ENT at Boston Medical Center who specializes in amyloid treatment. By then Kathy had lost most of her functioning voice and continued to struggle physically and emotionally with the tracheostomy. A scope at that time revealed the growth closing off about 70% of her airway at and just below the vocal cords. We were obviously motivated to remove the trach, but not at the expense of the safety of Kathy’s airway. The Boston physicians recommended these priorities: (1) preserve the airway and reduce the growth in a series of conservative laser surgeries; (2) remove the trach if possible; (3) restore Kathy’s voice if possible. Doing nothing would mean eventual closing of the airway and 100% reliance on the trach to breathe. The biggest risk was scarring which may cause more problems than the amyloid itself. Our Charlotte ENT suggested doing nothing at this time because of these risks. After two months of praying and discussing, we decided to proceed with the surgery.
On January 8, 2001 we again traveled to Boston for our first of three planned surgeries. A scope the day before surgery showed significant progression of the disease compared to the scope done in August. During surgery the next day it was also discovered that the disease involved more of Kathy’s throat, extending further down to the trach site. Laser surgery was not going to remedy this problem without first slowing the amyloid growth rate. The surgery was discontinued at that point. In a follow-up meeting we discussed with the Boston ENT and a physician with the Boston Amyloid Clinic a recommendation to use low-dose radiation treatment on the growth to hopefully slow its rate of growth. They advised against chemotherapy and stem cell rescue due to the localized nature of Kathy’s case. We are currently waiting for a formal proposal from Boston University regarding low dose radiation.
In the meantime, Kathy traveled to Sarasota Florida in February 2001 for a consultation with a Chinese Medicine physician. He has prescribed several different therapies (supplements, teas, and tonics) to be taken to “bring her body back in balance”. We have forwarded this all to the Boston physicians, having promised them that we would do so. We are also waiting for their response on this.
In early March 2001, we visited Dr. Solomon at University of Tennessee. He has developed an antibody that dissolves amyloid deposits in mice. At this point, it has been sent to Europe to be modified for human trials. He expects to receive the new batch in May. Then, he will have to conduct more lab tests with mice to make sure it is still effective in its modified form. After that, he mentioned an August deadline for making application to the National Cancer Institute to begin the sometimes political road toward human trials -- which still may take some time. Although the antibody is on the fast track, the problem may still be in getting drug companies interested in manufacturing it because the disease is so rare. Although this is a long term option, it gives us great hope to know this work is out there and that we may be able to help our fellow patients.
Kathy and I both know that our case is unique. Some would call it one of the hopeful amyloidosis cases. There are days we feel very optimistic and days when we feel great futility and despair. The experts agree that amyloidosis itself won’t likely kill Kathy. Kathy’s issue is the safety of her airway and the potential for surgical scarring and infection there as the amyloid continues to progress. The amyloid grows in Kathy at a rate that is exponentially higher than what the experts have seen in most other localized cases. We will do everything we can to keep her airway safe. The desire to live without a trach, to sing in church, to read to her kids, to safely take a shower and to dive into the ocean again must take an emotional back seat for Kathy.
Our early life medical challenge of infertility resulted in our adoption of two beautiful children, Meg (now 7) and Will (now 2 ½). Maybe, our new and more life threatening challenge will bring something to our lives or to the lives of future amyloid patients that are unknowingly waiting in the wings. Early diagnosis & awareness and support for curative research are now our calling.
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