John Ball- Amyloidosis Story

April 10, 2012

I was an otherwise healthy 64 year old Caucasian male. I have had no unusual medical issues and no family history of any. Recently I had noticed brief periods of breathlessness during my weekly exercise routines, but nothing that would have prompted me to tell my doctor. In mid-December, 2009 a routine annual physical evaluation reported elevated levels of protein in the urine. My primary care physician, Dr. Robert Brusky monitored my protein level and adjusted my hypertensive meds accordingly but, the protein levels remained above normal limits for several months. Dr. Brusky suggested that I seek a consult with a Nephrologist, so I self-referred (luckily I have a PPO insurance plan) to Dr. Hamid Sadeghi, M.D. of the California Kidney Medical Group, Simi Valley, Ca. on July 27, 2010. Dr. Sadeghi ordered blood tests and a kidney ultra sound, both of which returned negative results. After close monitoring, a kidney biopsy was ordered on September 14, 2010 and a diagnosis of AL Amyloidosis was rendered by the Cedars Sinai Lab where the samples were evaluated. I met with Dr. Sadeghi on September 23, 2010 to review the biopsy results and was told to seek an immediate referral to a hematologist/oncologist. When asked whom he might recommend, Dr. Sadeghi stated that my condition was so rare that he suggested that I see my GP for a referral.

My wife and I returned home and proceeded to search the internet for the Centers of Excellence in the country that treat Amyloidosis. We found a website called Amyloidosis that provided a wealth of information on the condition and where the leading treatment centers were in the United States. At that time, there were only three major treatment centers in the US listed for the treatment of amyloidosis; Sloan-Kettering, New York; Boston Medical Center, Boston, Mass; and Cedars Sinai Oschin Cancer Center, Los Angeles, Ca. Dr. Robert Vescio, M.D. and the Oschin Cancer center at Cedars were listed as the only specialist/facility treating Amyloidosis on the West coast. Since then a number of other treatment facilities and physicians have been added to the website. Since I live in the Los Angeles area, I called for an appointment. I met with Dr. Vescio on October 11, 2010 where he immediately began a diagnostic workup with a bone scan and bone marrow biopsy on October 18, 2010. Both confirmed the Amyloidosis diagnosis. For those afraid of the bone marrow biopsy, don't be; it was no big deal. Twenty seven infusion treatments were approved by Anthem Pre-treatment Review on October 19, 2010. I began a chemotherapy Infusion program of 26 treatments on October 20, 2010 and finished my last infusion treatment on February 11, 2011.

On February 11, 2011, I was referred to the Cedars BMT Coordination Team for a BMT medical work-up. Dr. Vescio was planning on having me start the stem cell mobilization process on or about March 3, 2011. During the benefit verification process with Anthem Blue Cross, I learned that, due to contractual and financial negotiations between Cedars and the health plan, Anthem withdrew their in-network certification status for the Cedars BMT Center; thus forcing all current BMT patients into a limbo status. I was ready to proceed with the BMT Medical Work-up, but could not do so until the financial and contractual dispute between Anthem and Cedars BMT Center was resolved. Not knowing when that might happen, Anthem gave me a list of facilities where they would cover my BMT, so I went to Dr. Vescio and asked for his advise and referral. He told me that given the options available, if it were one of his family members, he would send them to City of Hope for the SCT. He told me of a highly respected colleague in this field at the City of Hope named Amarita Krishnan, MD, who he consults with on Amyloidosis cases. He offered to contact her and facilitate my referral. Dr. Krishnan's office contacted me two days later and I was scheduled for an appointment with her at City of Hope on March 3, 2011 to continue my bone marrow transplant work up and procedure. Because so much time had lapsed between the work ups in October 2010 at Cedars and the insurance approval in March, 2011, I had to repeat all the work ups at City of Hope, which delayed the bone marrow transplant for another 2 months. Thank heavens my disease had a relatively slow progression.

Because a bone marrow transplant requires many blood tests, chemotherapy infusions, and bone marrow infusions, a vein access device (VAD) was surgically installed in an outpatient procedure. The VAD is a tube device that is surgically inserted directly into your neck artery then extends down into your heart ventricle. The tub exits your chest above the breast area and has a transfusion valve on the end which enables doctors and nurses to inject medicines and withdraw blood without having to insert a needle in your arm every time. Of all the procedures done to me, this created the most apprehension and in the end, it was no big deal. The VAD requires daily maintenance with self administered Heparin injections to prevent any internal blood clotting. Once a week the entrance site must be cleansed and resealed with a special antibiotic preparation and dressing. You can't get the VAD wet, so you have to cover it with plastic every time you bathe. I had the VAD for three months when it was removed the day of my discharge from the hospital in May, 2011. It was a pain in the ass, but no literal pain. It did, however, put a crimp in my scuba diving and swimming routines, but what are you going to do?

In preparation for the actual bone marrow transplant, on March 28, 2011 I started the stem cell collection process. This is an out-patient procedure where they hook you up using your VAD to what reminded me of a heart-lung machine. It extracts blood from your heart, runs it through a filtering device that removes your healthy stem cells and then pumps the balance of the blood back into your body again. It's a totally painless procedure, but extremely boring as you must lie in a bed for up to 4 to 6 hours at each collection session. You usually need 4 to 5 sessions to collect the stem cells needed for a transplant. This is where my first emotional disappointment came. I was told that the stem cells they were removing were not adequate for them to do the transplant, and I would have to redo the process after two weeks of recuperating, and more self injections of Neupogen. I met with Dr. Krishnan on April 12, and she scheduled me to enter the hospital for an overnight procedure that would boost my stem cell counts to assure that my next collection effort would be successful. On April 15 (yes, tax day!), I had an IV of chemotherapy and was sent home the next day. I was ordered to resume my Neupogen injections and scheduled for another stem cell extraction series that began on April 25, 2011. I had the stem cell extractions daily, through the 27th when I was told that they had enough for a successful transplant.

On Friday May 6, 201, I was admitted to City of Hope for my Autologous Bone Marrow Transplant. That evening, I received high dose chemotherapy to destroy all the bone marrow and remaining stem cells in my body. On May, 9th, fondly referred to as day zero, my previously collected stem cells, after being frozen, stored, and then thawed in the sink outside my room (very high tech), were transplanted back into me through the VAD IV. I believe I received three bags that day. Interestingly enough, as I was going through the process, my wife noticed that the stem cells I received were from the first extraction. They never used the cells from the second extraction! Throughout the chemotherapy and stem cell transplant, I never had a really uncomfortable day nor did I lose my hair in clumps as I thought I would, although it did thin a little. At one point, I was concerned about not losing my hair as I was wondering if the chemotherapy was working! I was repeatedly told by my nurses not to worry as everyone responds differently to the treatment, although they had to admit that they had never seen anyone come through with a scalp so unscathed! I told the doctors before my treatment that I used to get motion sick as a kid, and I was very concerned I would not be able to handle the anticipated nausea. That was my worst fear! They gave me pills and injections when needed/requested and I never experienced any serious nausea to speak of throughout all of the treatment that I received.

Because my immunity had been destroyed along with my bone marrow, I was now susceptible to any germs and infections that I may encounter. The bone marrow transplant patient rooms were located on a dedicated wing of the hospital with restricted access and have a positive pressure air conditioning system and HEPA filters to keep the germs out. You cannot have visitors for the first week, other than your spouse. I was trapped in an 12 x 12 room for the first two weeks and could only access the outer hallway with my IV stand, urine bag, face mask, gown and slippers which was especially challenging for me since the hallway seemingly had beautiful nurses at every turn. On the upside, the food was amazing for the first week. I called the kitchen to compliment the chef. But as time grew on and stem cells slowly started to rebuild in my body, the food started tasting worse and worse. By the 25th of May, I told the doctor that I could not eat one more thing or I would puke. I could not handle the smell of hospital food served in those plastic covered containers. This is not to say the food was bad, but there was something about my treatment that made me gag at the smell every time they removed the serving dish cover and I inhaled the steam from the food. They started serving it to me in styrofoam, which helped for a while, but eventually, that too made me gag. It was something about those food serving dishes because when I was finally home, I did not have that problem with food at all. In my research on what to expect during this process, I read another man's journal who also complained of this malady. The treatment affects your smell and it is an unpleasant reaction. The last week of my confinement, each day, I begged the doctor to let me to go home. Finally, on the 27th, a team of doctors showed up to discuss it with me. I pleaded again with them. I promised to do anything they said and take all medicines and self injections needed. Reluctantly, they released me, and I went home to my wife's cooking and had no further problems. I continued my post SCT treatment at City of Hope as an outpatient until June 22nd, when my insurance company allowed me to return to the care of Dr. Vescio at Cedar- Sinai.

The recovery continues, though I am still very susceptible to germs, even now, almost a year later I can't be around kids with sniffles because I have the immunity of a newborn baby. The last cold virus I had in January and February went to my lungs and required heavy antibiotics to get rid of them It was hard this last year to turn family and friends away for fear of becoming ill. It was especially hard on the grandkids who didn't understand why they couldn't see grandpa or why they couldn't hug him or play "monster" with him on the back lawn! It's painful, but it's absolutely necessary to make rules about who can see you and when. I had to have some of my childhood vaccines redone earlier his year.

To this date, my tests have been normal, and although the disease is still barely seen in the molecular testing that's done, Dr. Vescio claims that I am currently in remission. I visit him every three months for medical updates and hope all continues in a positive direction. I've discussed the "What-ifs" with Dr. Vescio and he says that should the disease start to return, there are several treatments that have been very successful and new ones are coming on-line all the time. I continue to remain positive. My wife and I are planning a cruise to the South Pacific later this year or early next. As my strength continues to return, I'm getting back to being myself. As a side note, I was told by my doctors that part of my success was due, in part, to my overall good physical condition. For the past 12 years, my wife and I have done cardio and strength training at a local gym three times a week. I was also given exercises to do in the hospital while I recovered. It is imperative to do the exercises and not just lie around in bed. You have to keep moving.

As I write this it is April 10, 2012 and about a month away from my one year SCT anniversary. I can't tell you how much I appreciate what Dr. Vescio has done or me. He has an amazing way of communicating confidence and hope for a complete recovery. It also helps that he is one of the top oncologists in the world treating amyloidosis. Because of his positive outlook and friendly personality, I have never once doubted that I will have a positive outcome. Having said that, there is no question that this is a life altering disease. I look at life differently now. I don't assume there will always be a tomorrow. If I want something, I get it. I find myself enjoying people's company more, enjoying going out more, just enjoying life more and not taking things for granted. I also don't sweat the little things as I used to. I don't know how long this feeling will last, I hope forever, but right now, I feel a certain urgency to really live. Let's face it, at 65 how many good years does anyone have left? I want to enjoy every one of them for as long as I can.

UPDATE: December 23, 2012

I've been having quarterly check up visits with Dr. Vescio at Cedar since my SCT in May of 2011. The Lambda Light Chains started in the low teens, then jumped up to the low 20's (normal is under 24) and six months ago went up to 36, however the ratio remained normal. At my September, 2012 visit they actually held steady at 35, again, with a normal ratio so we all held our breath hoping that they might be leveling off and this could become my new "normal." I had my December quarterly lab tests last Tuesday and we just heard for my doctor that my Lambda Light Chain numbers are going back up to 52.8, still with a normal ratio. My wife and I are attending an AL symposium at Cedars on January 5th where the world's expert on Amyloidosis, Dr. Morie Gertz from Mayo/Rochester, will be a keynote speaker. Dr. Vescio and I are taking advantage of this serendipitous gathering to meet with him to get his perspective and see if I qualify for a clinical trial study on a new medication for controlling this disease. I am still feeling well and have no symptoms. There are still a lot of options open for me so I am feeling good about the future. I will keep this running narrative updated as conditions change and as time permits.

April 29, 2013 Update

My wife and I attended the AL symposium at Cedars on January 5th where Dr. Vescio and I met with Dr. Morie Gertz of the Mayo Clinic. We all agreed that I would be a good candidate for the upcoming phase III trial of MLN9708with Dex Vs. Physician's choice. I scheduled a visit with Dr. Vescio for February 29, 2013 to see if I qualified for the trial and to begin some testing and paperwork. While in the doctors' office, he reviewed the trial's clinical qualifications and said that, unfortunately, I did not qualify to participate at the present time. My Lambda Light Chain numbers where not high enough. I was at 53 and the study had a minimum of ~70 or there about. While I was there, we decided to do another blood test to see if by any chance my light chains had possibly gone up since my last test taken on December 18, 2012. The Nurse contacted me later that week with the test results and much to everyone's surprise, my Lambda Light Chains had gone down to 37.3! Well, that was good news but, I certainly would not qualify for the clinical trail now, so we are back in a waiting game to see what direction they are going before further treatment is initiated. I see Dr. Vescio again on April 30, 2013 for a follow up visit and another series of tests and hopefully, we will have a better idea what direction to take and the treatment options available for me.

May 14, 2013 Update

I met with Dr. Vescio on May 2, 2013 to review my latest lab test results taken on April 30th. My Lambda Light Chain numbers had gone back up to 53.6 but, my ratio was still normal. My total protein decreased from 3873 mg/24 Hr in December 2012 to their current level of 2205 mg/24Hr, a 43% reduction in total protein. This is very good news because the kidneys reflects the body's burden of amyloid deposits. When the protein goes down, this indicates that the kidneys are able to keep ahead of the amyloid deposits which is a good thing! I am not scheduled to see the doctor again until late July, and I will update this report at that time.