Submitted By: Natella Nickel
AMYLOIDOSIS: THE JOURNEY By: Natella Nickel. We go through many journeys in life. Some are happy, full of promise of a good life and future! Meeting someone special, getting married, the arrival of a new baby. Some journeys are sad and heartbreaking. When someone in the family gets ill or passes away or when a couple divorces. And then there are journeys that bring you to your knees.
That make you hit rock bottom and getting out of this pit can become a struggle of life and death. My name is Natella. I am 68 years of age, a florist, and have lived in Edmonton for the last 50 Years. I immigrated from Germany in 1959. Keeping fit and eating healthy has always been a top priority. My husband Rudy and I love hiking. From 2000 until 2007 we did extensive hiking in our Rocky Mountains, in Utah, Colorado and Arizona.
About 5 years ago I began to see the first signs and symptoms of a very rare illness called Amyloidosis. I would get the occasional black eye, bleeding around the eye, that would come and go. At first it was very infrequent and the doctor would tell me I had a weak blood vessel in the inside corner of my eye and it was nothing to worry about. But other changes began to happen, my tongue got larger, it started swelling under my chin and in the tonsil area, and it began to hurt.
My skin started changing. Hundreds of little red spots would appear all over my body. At first they were small, but then some of them would get larger and grow into huge red blotches. And still no doctors were alarmed in any way. Usually a blood test was taken and the result looked normal so I was O.K. I was still in good physical shape during 2005. As a Floral Design Instructor for Edmonton Public Schools I was working full time.
But gradually the symptoms got worse. The bleeding around my eyes was now constant, I began to experience severe pain in my finger- tips and the soles of my feet and I started feeling fatigued. It didn’t matter how much I rested, I was always exhausted. In December of 2007, I had to quit my job of almost 30 years.
It was a gut wrenching decision because I really loved my work. I was one of the lucky people that couldn’t wait to get to work in the morning. By now the symptoms had increased enormously. My tongue was now double in size and pushing up against my teeth leaving painful indentations.
Blisters appeared on the inside of my lips, cheeks and tongue. Eating became a real challenge. Gone are the days when my lunches where mainly raw fruit and veggies, now everything has to be soft, cooked and bland, with no spices. But even speaking became difficult. Because my tongue is so much larger it does not move as fast as it should when you’re eating or speaking. My speech became slurred, slow and laboured. I was accidentally biting my tongue on a daily basis. Doctors are now paying attention.
Between 2007 and 2008 a number of biopsies were done: skin from my forehead and shoulder, tummy fat, intestinal, bone marrow, my lip, my tongue. These are the most common areas where Amyloidisis is detected, but it can also show up in urine, blood and major organs. I was now seeing a clinical Oncologist at the Cross Cancer Institute in Edmonton. He was the first doctor who actually believed that my suspicions were right, that we were looking at Amyloidosis. The problem now was that we had to find amyloid deposits. All test results from all biopsies came back negative, nothing was found.
If there is no official diagnosis there is no medication and also no treatment. Back to square one; it was devastating. A lot of people would say to me: well, if every test came back negative then that is a good sign, then you don’t have this illness. For those of you who have gone through this ordeal you knew that something was terribly wrong.
The illness kept progressing and there was nothing I could do to stop it. I cried a lot and I prayed a lot. But I knew all about Amyloidisis because my mom passed away from this illness in March of 1999. I knew what was coming at me, I knew what lay ahead. I started to have severe pain in my stomach in the early part of 2008. I was diagnosed with acid reflux syndrome.
Stomach getting really bad, passing black stool. Admitted to Gray Nuns Hospital and two days later I was told by the Gastroenterologist that they found the Amyloidosis in the upper part of my stomach and in the duodenum, the first section of the small intestine. I came out of the Dr.’s office and got into my Van and sat there for a long time, I was stunned. It was raining outside. I could hear the rain pounding on the roof of the van and I just sat there.
The tears started flowing and I could not stop them. So many emotions bubbling to the surface. Anger and rage, devastation, fear. I guess a small part of me kept hoping, please Lord, let it not be this. And yet I had known for a long time what it was. We had the official diagnosis: Primary Systemic Amyloidosis. As I became calmer there was also a sense of relief, now the fight for my life could begin.
Today was April the 7, 2008. It was my Mom’s birthday. The Cross Cancer Institute called – I should come in and see the Oncologist a.s.a.p. Treatment options where discussed – there where only three. 1: To do nothing and let the illness take its course. 2: Cancer drugs and steroid treatment drawn out over years, if you last that long, with horrible side effects. 3: Autologous Peripheral Stem Cell Transplant.
We chose the stem cell transplant with full support from my family and Dr. Belch. The frequent bleeding in my stomach was of great concern, and I was admitted to the Cross on May 21. 2008 May 22 insertion of the C.V.C. (central venous catheter), under local freezing was very painful but was O.K. The C.V.C. provides access to your bloodstream, it is used for medication, Chemo, blood transfusions and other liquids. This has to heal for a few days. Please, let there be no bleeding. I am praying.
Day pass for May 24 & 25 – healing well. Great to be home. Back to the Cross May 26, chemo is at 2pm. As I was watching this clear and clean looking chemical cocktail dripping into my body, all kinds of thoughts went through my mind. What is in this stuff? Can it kill me, is it even safe for people? And promptly at 5:30 I had a nasty reaction.
My tongue started swelling so rapidly I barely got a chance to call for help, it was so huge I couldn’t close my mouth let alone talk. Everybody started running – doctors, nurses, but nobody really knew what to do. Finally, somebody said something about steroids. It took till midnight until my tongue was small enough so I could close my mouth, but speaking, drinking and eating remained difficult. Vomiting started at 2:am, I started to shake, felt hot, then cold – I have never felt so totally unwell in my entire life.
With the chemo, Melphalan, you are also hooked up to all kinds of other meds: Filgrastim, to help your bone marrow produce more stem cells, Mesna, to protect your bladder, Dexamethasone, pre chemo for nausea, Ondensatron, also for nausea. And morphine. May 28, 2008 Still vomiting, blood in urine, but I was told that was not unusual. May 30, 2008 Full blown bladder infection, up to the bathroom every 10 minutes, I am exhausted.
Moved into isolation this afternoon and now I am on heavy duty antibiotics, vomiting has finally stopped. May 31, 2008 Slept much better, bladder slowly recovering. Waiting for blood count to go up so that the stem cell collection can proceed. June 4, 2008 Hurray! Today is collection day, the numbers had jumped to an incredible 2.1 from yesterday’s 0.6. At 11:00 am, they took me down to the collection unit. Hook up time took 30 minutes and by 11:30 collection had begun. 5 hours of collection, 30 minutes for flushing and disconnect.
I got back to room by 6:00 pm, still vibrating from the apharisis machine and feeling exhausted. I was grateful that my daughter was with me. June 5, 2008. More good news, they collected enough stem cells yesterday and I don’t have to go for a second time. I was told I could take the day off. Sadly, Rudy’s Mom was admitted to the Gray Nuns Hospital this after- noon.
Things are not looking very good. She is 97 years old and her body is shutting down. June 7, 2008, Blood count too low, 3 bags of blood (transfusion), 2 Friday night and 1 Saturday afternoon. Lots of bone pain in the large bones, legs, back. Feeling very tired and weak. My body moves slow and stiff, like I have no control. I move like a robot. June 8, 2008 E.C.G. is O.K., blood count is O.K. and after 19 days I can go home for 2 weeks and rest. Get ready for round two. June 13.2008
Oma Nickel, Rudy’s mom, passed away early this morning at the Gray Nuns. Today is also Christopher’s, our grandson, birthday, he is 12 years old. June16, 2008 All my big muscles are cramping badly. I have trouble walking, feel stiff and it is very painful. My body is moving in slow motion, like a rusty machine.
My hair fell out in clumps so Rudy shaved it off for me. June 18, 2008 Oma Nickel’s funeral was today. We will miss her immensely , but for her it was a blessing, her body was just too worn out and she was ready to go. I will rest now until next week, I am exhausted. June 24, 2008 Back in the Cross, settling in for the big chemo, isolation unit 35. I am not as calm as I was the first time around.
There are too many memory flashes and I am scared. My personal directive and wishes for my funeral were all done before my treatment started. Just in case something went wrong. And so the fight for my life began. June 26, 2008 ‘Big’ , or ‘high dose chemo’, at 10:30 am. My tongue and throat started swelling again but they got on top of that right away.
The nausea is something else, it comes in waves and it is spinning the room around. The vomiting is non-stop, convulsive and agonizing. With nothing in your stomach the acid comes up and burns your throat and your mouth and still you’re heaving. My face is black from the bleeding around my eyes and from the constant convulsions there is bleeding around my mouth as well.
My lips have huge blisters and are black and blue. I can not describe the pain, even the morphine did not take the pain away. Please Lord, walk with me and hold my hand. June 27, 2008 Bad night, not much sleep. Stem cell return was at 10:30 am.
This is day 0. Four small bags are partly thawed and infused through your central line, it took all but fifteen minutes and felt really cold in my chest. The preservative that is used for freezing your stem cells has a very strong odour. It is in your room, on your clothes and on you. It makes you gag. More nausea, vomiting, fever and chills. Meds are given for these side effects as well. At any given time there are about 6-10 bags of medication hanging from your I.V., including morphine.
It takes 7-15 days for the stem cells to re-grow (engraftment). During this time your bone marrow is suppressed and there are no longer white blood cells to fight infection. Visitor restrictions are in effect: people with colds, flu, etc are not allowed to come and visit, no fresh flowers or fruit. By now I was too ill to keep a daily journal, I could not even sit up in bed without assistance.
My daughter Gaby and my husband Rudy had arranged an angel watch around me, where 15 people, family and friends, would take turns staying with me at all times even at night. I felt deeply humbled by so much love and kindness. I called them my angels.
They came with hugs and smiles, taking care of me for 48 days. To this day I feel humbled, grateful and so blessed, I was never alone and it Gave me comfort and strength and I knew the Lord was walking with me. My brother, Ernst, and sister-in-law, Ingrid, came from Germany for 4 weeks to help our family and to be with me. I can not find words adequate enough to describe and express how grateful I was.
Every day I got worse. I got a fever of 42.9 and they just couldn’t bring that down. I gained over 20 pounds in fluids and developed bacterial pneumonia and breathing became a real struggle. These 10 days were a big blur, but I remember lying there, struggling so hard and saying, please God, just let it be done. But a small voice kept repeating one word: fight, fight, fight! And so I kept fighting.
They took the central line out of my chest because bacteria had grown in there. By now I am on 4 different antibiotics, but no improvement. There was one thing I had a very hard time with – and that was guilt. Even before the treatment began, and long after it was finished, I felt guilty because I had put such a huge burden on my family. I could see the worry in their eyes and the frustration because they couldn’t help me. It made me really sad. Usually I was the caregiver.
All my life I looked after everybody, I was the one who made things better, and now I am flat on my back unable to turn or sit up in my bed without help. I remember feeling very hot and very swollen. The fluid was everywhere – my legs, arms, midsection and my face. I looked like a black and blue beaten up pumpkin.
Oxygen helped a little with the breathing problem but I could not eat. Slowly the antibiotics began to work. They also did a procedure called Thoracentesis where they siphon the fluids out of the areas where it is most highly concentrated. They did that two days in a row and took out about two litres of fluid in total. After that breathing became a little easier.
I was sitting up in bed now and was also able to get out of bed with assistance. It is very hard work to learn how to walk again when you’re so weak and it will take a long time to rebuild your body and muscle tone. I had lost 24 pounds. Slowly, every day got better. I was determined to beat this, I will get strong again. Blood count too low, 6 bags of blood and platelets are given and it did improve my overall condition. August 11, 2008 Finally, today I can go home.
After 48 days of lying flat on my back and without my buddy, the Intravenous, it felt rather strange. Still very weak and shaky, I had developed an allergic reaction to some of the anti-nausea medication, I am so looking forward to going home. A heartfelt thank you to the doctors and nurses at the Cross Cancer Institute, for there professional and loving care I received.
I will be forever grateful for all the love and kindness that was given so freely and generously by family and friends, but most of all I am grateful to be here. Every day I can sit outside for a short while, I can enjoy the flowers and the garden, just feeling the breeze on my skin and listening to the sounds of the wind and the birds gives me such joy. I am grateful for that.
Thank you Lord for walking with me. In writing about and sharing my journey with Amyloidosis, I hope I can help anyone who is struggling with this illness. If it is only one person who I can reach or help, that would be great. I will be updating the journey periodically. Love Natella