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AL AMYLOIDOSIS SUCKS

BACKGROUND:

For those who do not know, my husband Josh was diagnosed with AL Amyloidosis in April 2016. AL Amyloidosis is a rare blood disease where misfolding plasma cells in the bone marrow produce light chains in the blood called amyloid that attach to organs and cause tissue damage, (by no means an accurate description). There are less than 4,000 new diagnosed cases of Amyloidosis in the United States each year, which makes this an extremely rare disease. Amyloidosis is most closely related to Multiple Myeloma, which is a rare form of leukemia. As such, many treatments for the two diseases are the same. Most people diagnosed with Amyloidosis are in their 60s, but Josh unluckily discovered he had Amyloidosis at 37-years-old.

As a physician, Josh noticed swelling in his ankles tested himself to make sure he didn’t have a blood clot then tested his urine and blood and realized he had nephrotic syndrome. He went to a nephrologist who then ordered a kidney biopsy, which showed Amyloidosis. He then underwent a bone marrow biopsy, which also came back positive for Amyloidosis.

When Josh was diagnosed with Amyloidosis, I didn’t know what it was and immediately googled it. Don’t do this, a google search reports that patients diagnosed with Amyloidosis have 1-2 years to live. SHOCK! WHAT? HOW? WHY? I CAN’T LOSE JOSH! I cried for two weeks straight. I couldn’t wrap my head around losing Josh, I still can’t, and luckily two doctors assured me that I don’t have to. Two doctors assured me that Josh will live a long life with this disease. He will be around to see his children get married and there may be a cure in his lifetime.

“Why did young healthy Josh get Amyloidosis?” I asked Dr. Smith and myself a thousand times. “Is it just like randomly getting struck with cancer without a family history?”

Dr. Smith responded, “One out of three people will get cancer in their lifetime.”I don’t know the exact statistical chance to get Amyloidosis at 37, but pretty much Josh won the rare disease lottery. Although this is all bad news, the good news is with respect to this disease Josh also won the lottery, right now it is only affecting his kidneys (not the heart, liver, muscles, or nerves), which is amazing. He has little kidney damage and he is in amazing shape.

Due to his relative health, Josh opted for the most aggressive form of treatment to achieve long term remission and will be having a stem cell transplant. A stem cell transplant is a bone marrow transplant, except in his case they will be using his own stem cells. July 25th and July 26th doctors collected close to twenty million of Josh’s stem cells, which were then frozen at -130 celsius. He will be admitted to the hospital on July 30th and for two days will be blasted with high dose chemotherapy (melphalan) in order to wipe out his entire blood system. He will rest a day, and then be reinjected with his newly cleansed stem cells. The day of transplant is literally deemed his rebirthday. Every immunity he has acquired in the last 38 years will be completely wiped clean. He literally will be put on an infant baby’s vaccination schedule once his body begins to recover.

Josh will be in the hospital for close to three weeks, until he is deemed safe to reenter the world. He will be able to see me and adult friends and family, but the children are not allowed to visit him because of the risk of infection. He is heartbroken to be separated from the kids. He will be out of work for 2-3 months, which is sad because he just started a new job that he loves. We are focusing on the positive, looking at three months as a blip, like maternity leave, which women take all the time. (FYI, paid medical leave readers is not only for new mothers).

Originally, he was supposed to be admitted to the hospital on July 18th, but because of a lab mishap with Josh’s stem cells the transplant was delayed two weeks. We were upset about the delay and devastated because Josh was put through a brutal collection of stem cells twice, 40 million stem cells were taken within a two week period. The night of his second collection, I thought he might need a blood transfusion, he was sick, fatigued, cold (shut off the air conditioning), but a day of rest and he rebounded. Josh is a champ.

Due to the upsetting delay, we had more day dates than we have had since having children seven years ago. One of Josh’s best friends visited from Pittsburgh and spent the weekend with him. On Monday and Tuesday Josh gave 20 million stem cells, Wednesday we had a relaxing Boulder date and went shopping on Pearl Street, Thursday we played 18 holes of golf, Friday we went for a long hike near Chatauqua, and Saturday Josh checks in the hospital for his first dose of chemo therapy.

Friday night we had a Star Wars pajama party with the kids where they stayed up late, ate Chinese Food, S’mores, ice cream, candy, and watched movies. (Thank you, Sarah for the delicious ice cream). Josh’s last night of fun with the kiddos for a few weeks, he is the best dad.

****

Today we checked into the hospital. It was surreal, driving to the hospital on a sunny day, young families shopping at the farmer’s market and riding their bikes on the bike path while  Josh will be stuck inside the hospital for one month. We checked in thinking we could get a cart and together go back to the car to get his things. Nope, once Josh entered his room, he couldn’t leave the hallway. I took the cart back to the car, called my twin, and cried. I can’t believe Josh will be in this small room for almost a month. I can’t believe he has to fight this shitty disease. I can’t believe small tragedies go on around us every day as the world keeps circling and the sun keeps shining.

Yesterday, I was brainstorming what to write as an update. We hiked miles with our dog, our shoulders burned by the sun, our lungs expanding to get us up the steep hills we have climbed so many times. I felt blessed. We are blessed in love, family, and friendships.

Today as we left our home, my five-year-old sobbing and her two-year-old brother imitating her screams. I felt heartbroken. This is officially the hardest day of my life thus far. Healthy Josh driving our car to the hospital to be blasted with toxic chemo and taken to the brink of death to be brought back to life with his own stem cells. It feels torturous. It feels unfair.

I wish, pray, hope for remission. I’m wishing that Josh can come home soon. I hope he will breeze through chemo and this seemingly endless stay in an artificially lit sterile room. I pray he stays sane. I pray my children feel loved and secure. I pray for a cure. It seems desperate and pitiful, but I pray and hope that everyone that has ever known or cared about me, Josh, his family, or my children, send us prayers. I want to believe in the power of prayer, healing thoughts, and love. Josh is private, but I want to rally all the love I can muster and direct it at him. Family, friends, world, we  need some magic sent in our direction.

The first round of chemo starts at 4pm, until then we will be playing Yahtzee, reading, and walking the halls, imagining ourselves in a claustrophobic train car heading through Europe to eat pasta and drink wine in colorful Positano, Italy.

****

For the next few months, I will keep family and friends updated on the blog. We appreciate all the friends and family who have rallied around us during this tough time – meals, ice cream, wine, texts, phone calls, emails, cards, have all been greatly appreciated. It is always nice to know people are thinking of us, so never second guess texting, calling, or emailing. We love it. Thank you, thank you, thank you…

 

 

 

15 thoughts on “AL AMYLOIDOSIS SUCKS

  1. We are thinking of you guys and wish you and the family the very best. Josh has been on of my best friends for a very long time. The kind of friend that I may not see for years but as soon as we get together it is like we never skipped a beat.
    Hang in there Josh. I love you and hope the next month flies by for you.
    Dave

  2. Oh, Justine, I had no idea. I will definitely be sending you all as much magic as I can muster- full of the breezes through the flowers, in the meadows above treeline. Big big hug to you.

  3. Beautifully written. Your words are clear. Sending positive thoughts to all of you. I’m caddy Harper’s sister who is a friend of Bonnie. We just found a fun computer game that we play on the iPad. Requires no energy to speak of and brings out the competitive spirit in all. I’ll have to ask my daughter the name but you could goggle internet game that Ellen Degeneres peddles. 2 can play. Xoxmaggie

  4. Sweet Justine, You are the most amazing woman. I am so glad that my nephew, Josh, has you to fill his life with love and care. The children you two have created are magical and beautiful. Please know that I will help in any way possible. My prayers are sent on waves of love.

  5. Dave and I are thinking about you all so much. And praying! Josh will get through this – heart warrior that he is!! Thanks for writing a blog to keep us filled in. We love you both and all! Love, Nan

  6. Yes! we are all at your back sending MUCH love and support. You all are in the middle of a huge journey and you all WILL get through it with flying colors…especially Josh! Thinking of you and sending prayers and love and magic !!! Love Abby

  7. Hi Justine,
    I know we have never met but have heard great things about you and your children. I am David Hinish’s little sister, Lauren. I loved getting to know Josh as a kid and looked forward to seeing him through the years. He was always so fun to be around.
    You all have to be on such an emotional roller coaster. Keeping your eyes to the future with so much to get through first. We are all looking forward to the good news coming and hope that you all get through this, knowing you have so many people pulling for you. Even those you have never met:)
    I look forward to meeting you and your beautiful children when Josh is healthy again.
    Take care and thanks for keeping us updated!
    Lauren Hinish

  8. I am sending you & Josh many positive thoughts and love every day
    Thank you Justine for keeping everyone up to date on Josh. Much love😘
    Laurie

  9. Oh my goodness Justine, I’ve been thinking about you since I first heard from you about what’s been going on. What an ordeal that will (please God) have a happy ending. I know you never ever imagined using this space here for updates like this. However, I’m glad you have the format here to communicate as people near and far will absolutely want to hear what is going on and how they can help in any way. You are all in my thoughts and prayers.

  10. Thank you for writing and sharing! I know it will help others too. Love and prayers to you. I am thinking about you guys and sending them all the time!!!!

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