This is part of a series where Heather shares her story of being a mother of two daughters with chronic illness. Read part one and part two (Jewel's Story) to learn more of her story. Part one of Lily's story is here and part two is here.
In the first two years after her diagnosis Lily failed every treatment.
First the holistic approach failed. After that NSAIDs alone failed. To NSAIDs the rheumatologist added weekly methotrexate injections. Methotrexate is a common chemotherapy medication. People often say “At least its not cancer”. But kids with arthritis often go on Methotrexate for years at a time. So yes. At least its not cancer, but here's a cancer medication.
The methotrexate made Lily's hair fall out. Most childrens don’t experience that at the low dose she was taking, but Lily did. The methotrexate also did very little to alleviate her symptoms. The doctor increased the dosage until she reached the top dose. While she was at that dose she developed a bleeding ulcer, which is common from the combination of NSAIDs and methotrexate. She woke up one morning and threw up. She had become so use to being nauseated and throwing up that she didn't even wake me up. The second time she threw up I heard her and went into the bathroom to check on her. I flipped on the light and saw bright red blood in the toilet where she had vomited. The intense fear I felt when I saw the blood was overwhelming. My husband was at work at the time. I woke up my sleeping Jewel, who was 7 at the time, and the three of us drove to the hospital. What I found out was--and write this down--throwing up blood is not an emergency. They made sure she was stable and said as long as she did not continue to throw up blood she could wait to see the gastroenterologist. It was a bit shocking. It took many months for her stomach to heal. Even after it healed it’s never been the same. Methotrexate. The gift that keeps on giving.
Even though vomiting blood isn’t an emergency, it does qualify a person to “fail” methotrexate. Which means no more methotrexate.
Failing methotrexate is a big deal because after methotrexate you move into a different class of medications called biologics. I don’t actually know why they’re called that. I do know that that cost a LOT of money and insurance companies don’t like to pay for them. Getting insurance approval is like completing a labyrinth with lots of dead ends and tricks inside. The first biologic medication Lily took cost about $1200 per injection. After insurance our copay was $150 per injection. The pharmaceutical company then had a co-pay assistance card that brought our copay down to $10 per injection. The medication came in the mail. The box would come and I would look at it, knowing it cost more than we made in a month. One time I spilled one of the viles. Imagine looking at $1200 worth of medication on the floor. I called the pharmacy and they replaced it. They said “we know it happens”. Lily was six in 2015 when she started her first biologic medication. After starting the biologic she started coloring. I almost cried the first time I caught her coloring for fun. I took her picture. That same summer she was 6 she jumped for the first time. She was 6 years old and I’d never seen her jump. Her elbow also released.
For the first time she could bend her arm to touch her shoulder. Imagine spending nearly 2 years unable to touch your shoulder. Now imagine not complaining about it.
Lily hates for people to know she is in pain. For the first five years of her illness she denied all pain. Bright red swollen motionless joints? She said they didn't hurt. Can’t get out of bed? She would tell me she wasn’t hurting. Unable to hold a pencil? Not because it hurt. She said she didn’t want to hold the pencil. My preschool Lily was as stubborn as they come. While I was worried about teaching her to live with chronic pain, she just lived with it. People who have kids who are sick say the kids are extra tough. They are. Watching Lily manage her pain, manage her treatments, manage her fatigue is nothing short of a miracle.
As strong as Lily is, chronic pain crushes neurotransmitters in our brains. Chronic pain uses up all of the serotonin in our brains and when Lily was 7 she experienced her first episode of clinical depression. The neuropsychologist told me if we could get her out of pain he believed her depression would go away. I burst into tears when he said that. I’d spend almost half of her life and failing to get her out of pain.
Lily started crying at night. Every night. She would go to bed and I would hear her crying in her bed. I would go to her, but she was inconsolable. She didn’t know why she was sad. Her arthritis was better than it had ever been. But she was sad. She stopped eating. Nothing I offered her made her want to eat. She would push her food around on her plate. I would sit with her and encourage every bite. I would eat with her. I would try and distract her while she had her food. Only a few bites would go down. I took her to the pediatrician and she started her on prozac. Lily started counseling once a week and she had a full evaluation by the neuropsychiatrist. The conclusion was that my 7 year old with arthritis had clinical depression caused by her chronic pain. It made me angry. Very angry. Arthritis had taken so much from Lily already but now it was going to give her something: the gift of clinical depression. Just like I didn’t know three year olds could get arthritis, I didn’t know 7 year olds could get clinical depression. I learn something new every day. Most days, its something I’d rather not know. Lily responded well and quickly to treatment of her depression. Within three weeks she stopped crying at night and she started eating again. She started joking again shortly after that. Lily is such a silly funny kid, but during this first episode of depression her teasing giggle went away. The sparkle left her eyes. Watching her return from this depression was a relief. Watching her learn how to manage her depression with the help of her therapist has been incredible. Lily is such a strong amazing kid. Her therapist gave her tools for her tool box and Lily uses them each day to manage her depression and anger. I’m not sure I could be any more proud of Lily if she was a neuropsychologist herself.
In October of each year Lily often experiences a more severe flare of her arthritis and becomes unable to bear weight on her legs. She uses crutches, or a wheelchair or her electric handicapped scooter. So although medications have kept her arthritis from causing joint damage, they seem to only work for about a year each. Lily started her second biologic medication in 2016 when she was 8. The new medication cost $2500 per injection. Insurance again covered all but $150 of each injection and the co-pay assistance brought our cost down to $5 per injection. When the box of medication arrived it the mail it contained $10,000 worth of medications. Eventually Lily’s dose increased and she was getting two shots a week, which meant in a month she received $40,000 worth of medication. In a month. Lots of people make $40,000 a year and I would receive something worth that in the mail each month. It was a sickening feeling. Not only was the medication very costly, it was an extremely painful injection. I am told that grown men refuse to take the medication because of how bad it hurts. Not only does the injection hurt, but it continues to burn for 10 minutes or longer after its injected. By this time in her treatment, Lily would lay on the couch and allow me to do her shot. She never tried to wiggle away or fight me on it. She did however begin to cry and beg me not to give her the shot. While she was crying and begging I would give her the shot and she would scream while it went into her body. It’s a rule at our house that if something (a medical procedure) hurts, a person can yell or cry, but they have to remain still. She endured like a champ. As her mom I had to shut off part of my brain and my heart to endure with her. I had to ignore her cries and focus on the long term goal of preserving her joints and the short term goal of giving her the best quality life I could. Within a year and a half Lily hit the maximum dosage, again without experiencing remission of her arthritis.
In October of 2018 her rheumatologist decided it was time to move to the next level of medications, infusions. Before she could start the infusions we once again had to go through the preapproval process. If insurance don’t want to pay for a biological medication that is between $1200 and $2500 per injection, they for sure do not want to pay for an infusion that is between $5000 and $10,000 per infusion. So once again the doctors, nurses and mother (that me) worked on the preapprovals. Hours and hours of phone calls later Lily had her first infusion in November of 2018.
All of our anxiety was high that first infusion. There was a chance of a severe allergic reaction. There was also a chance that within the same week she would be feeling better. The arthritis in her knee had been active for nearly a year without quieting down. She’d developed bony overgrowth on that knee, in her elbow and in one of her fingers. She was back to being in pain all of the time. When she started the infusions she’d been on steroids for over a month which was longer than she’d even been on them before. Because of the high doses of steroids the nurses had extreme difficulty starting her IV and it took four tries. As a mom and an advocate I usually only let nurses try twice to start an IV. So allowing them to continue to poke her while silent tears rolled down her face was nearly unbearable. The infusion went extremely well. She didn’t have a reaction, she didn't get sick from it. She did get very tired and the tiredness lasted through the next day. Lily had three infusions in November, one in December and one in January. Although the medication is making her more comfortable, she still is not in remission. The second week of January I received a bill in the mail for $30,000 from the children's hospital. Looking at it I realized the insurance company had declined payment for the infusions which held a price tag of $5,800 each. I had a pre approval letter so I couldn’t imagine why they refused payment. I spent hours and hours on the phone to find out that the insurance simply was trying to find a way to not pay. It seems impossible to me, but insurance often gives people a run around because they don’t want to pay. I found out that the pre approval I received approved the administration of the medication, not the medication. There was no way for me or the hospital to know that unless we called the insurance company and ask to speak to a pre approval expert and they read the pre approval. While I made phone calls I spoke to the rheumatology nurse--who is a super hero among nurses-- and she said she had in her hand two pre approvals, one for the administration of the medication and one for the medication. She also told me that the insurance had indeed paid for one of the infusions. So even though they were telling me they wouldn’t cover the cost, they already had. It seemed again that they were simply giving me the run around because they didn't want to pay.
This road with Lily has been so much longer than I anticipated. Nearly seven years ago it began with what I thought was an ingrown toe nail, followed by the diagnosis of JIA with hopes that remission was just six months around the corner. Six years of failed treatments have left all of us changed. We’re less hopeful, more cynical, very tired, kinder, and more compassionate.
I have also learned to enjoy the good days when they come and to know that the bad days will pass. Its hard to look at the bad days and find good things about them, but I’m learning to do that. The days when the girls are too sick or unable to manage I’m learning to savor my moments with them. Some parents do not get those moments. The long appointments, car rides, days on the couch are opportunities for time with my girls. On the worst days we cry together, but we are together.
I don‘t know what the future holds and it seems scary, but I know we will face it together and I anticipate we will continue to find our strength in one another.
Written by Heather Fenner