Monday, April 2, 2012

Ella's Evans Syndrome Story

Contributed by Libby Gray

For the first two and a half years of her life, my daughter Ella Rain was healthy. She was my first child and watching her grow up has been an experience sad and thrilling and terrifying all at once.  For the first two years of her life she a healthy, happy, beautiful, outgoing little baby girl.

New Year’s of 2004, when Ella was 29 months old and I was pregnant with her little brother, we went to a friend’s house for dinner.  My toddler ate nearly as much as my friend—a large man in his late 20s! That intense hunger was probably the first symptom we noticed.

Over the next several days she began asking for cup after cup of water, and was going through more diapers than she had as a newborn.  I contributed it to a urinary infection, or a growth spurt, or her being jealous of me and the baby.  I took her to the doctor, who said she just had a yeast infection in her diaper area and gave me some steroid cream that did no good.  My little girl was becoming different…she was whiney, tired, clingy and thirsty, thirsty, thirsty.

On February 9th, 2004, Ella woke up vomiting in the middle of the night. She cried incessantly, begging for water, drifting in and out of sleep and complaining her eyes hurt, her legs hurt, her head hurt.  I tried to brush it off as just the flu, but my heart wouldn’t let me. I didn’t know what, but I knew it was something bigger. That afternoon, my husband got on the phone with the doctor who, in turn, told us to go straight to the hospital.

To shorten this long story, it was on that day that Ella was diagnosed with Type One Diabetes, an autoimmune disorder in which the immune system destroys the insulin-producing cells of the pancreas. Her blood sugar was nearly seven times the normal level, her blood chemistry was messed up, she had lost 8 pounds in six weeks, and was less than 12 hours away from a diabetic coma.  Suddenly we were thrust onto a steep learning curve of insulin injections, carbohydrate counting, and constant monitoring and vigor to balance a delicate hormone that could save a life or take it.

As time went on, diabetes became our “new normal”, and started to fade into the background of our everyday life. Ella was diagnosed with other autoimmune conditions over the next few years: Hashimoto’s hypothyroidism, severe allergies, and asthma. 

But our Evans story begins around December of 2008, when Ella was seven years old. At first I thought she might be getting sick, or in a growth spurt: She began experiencing relentless high blood sugars, having nosebleeds and being so weak and tired during the day she was taking napping more than her baby sister.  Next came the bruises that covered her body as if she’d been in a fist fight, the cold and ear infection that never quite went away, and the way I noticed her working just a little harder to take each breath.   

I was used to seeing a rainbow of colors from Ella: pink cheeks flushed from excitement and playing in the sun; ghost-like paleness from a low blood sugar; greens and browns of dirt and grass staining her knees from a hard day of play; marker, pencil, and paints of various shades covering her hands because her creative brain felt like making something.  But this was not a good rainbow to spot: she appeared deathly grey, with dark circles under her eyes; covered in purple bruises; and the whites around her hazel eyes just slightly yellowed.

Shortly after the New Year of 2009, Ella woke up with a 103F fever, blood in her urine and out her nose, and 450 blood sugar (her range is 80-150, and we rarely see numbers that high unless she’s very sick, we seriously miscounted carbs, or there is a problem with her insulin pump). She just looked like death warmed over.  Completely terrified, we decided not to waste time at the pediatrician’s office, and I took her straight to the ER.
They immediately started running blood work and found her hemoglobin and platelet counts to be severely low. She was admitted, given antibiotics and a blood transfusion, and the whirlwind of tests began to find out what was wrong.

I remember the first night: I sat by her bed and just sobbed. Why Ella? Why my family? She’s such a good girl and she’s already been through so much. Why can’t she just be left alone? The C-word was thrown around, but I refused to let it enter my mind. Lymphoma and leukemia—such big, horrifying diseases that just don’t happen to little girls who spend their time crafting, reading for pleasure, and doing cartwheels through the grass.
On January 10, 2009, after almost four days of testing on her urine, blood, and bone marrow, a direct Coombs test revealed the duel diagnosis of ITP and Autoimmune Hemolytic Anemia—Evans Syndrome.  I asked my husband, who was at home with the other two kids and the computer, to Google this bizarre disease.  He found nothing but horror stories and brief descriptions. I so wish we had found the Evans Syndrome Community Network (ESCN) at that time.

The pediatric hematologist explained the vicious attack her immune system was inflicting upon her body, and that the best way to stop it was a strong course of steroids to combat the attack. Steroids can make even a non-diabetic resistant to insulin, so her already-high blood sugars skyrocketed to five and six times the normal levels. I worried about the formation of dangerous ketones. Ketones are the by-product of high blood sugars as the body attempts to break down fat instead of glucose for energy.  She was already sick and developing trace ketones. I didn’t want to risk DKA (diabetic ketoacidosis:  a life-threatening level of ketones in which the blood is acidic and there is no way for the body to get energy from food.)

She was released from the hospital four days later, and for the next several weeks we walked a dangerous tightrope. The steroids stabilized her hemoglobin and platelets to around 10 and 30,000 respectively, but they sent her blood sugar soaring. I doubled, tripled, quadrupled her insulin dosages around the clock, but days were rare we saw a number under 200. In addition to the immediate risk of DKA, the paralyzing fear of future heart, eye, kidney, and limb damage from relentless highs kept me wide awake at night. As soon as we began tapering her off the steroids, her blood sugar would come down, but so would her hemoglobin and platelets. She ended up back in the hospital three times in two months from low platelets, low hemoglobin, or scarily high blood sugars with ketones.

By March, we’d all grown frustrated beyond belief with the battle between diabetes and steroids and I longed to see my daughter well.  It’d now been four months since she felt like a normal child—she was always tired, nauseous, thirsty, and dizzy from the high blood sugars or the Evans.  We both cried a lot in that time. Our whole family was missing the spunky, creative, energetic little girl we’d known just a few months prior. I begged our hematologist for another option. He suggested IVIG.

In April, we began the monthly infusions. After some trial and error with working out the horrendous headaches she would get from them, she started to respond very well. Her platelets went up to 60,000; 80,000; even 110,000 and her hemoglobin climbed, too.  While she didn’t particularly enjoy sitting for 5 hours in a doctor’s office every three weeks, we were all thrilled with the progress she was making. With the exception of our treatment days every few weeks, life was getting back to normal. Ella was getting back to normal.

On June 15th, exactly one week before her eighth birthday, we began seeing dramatic drops in Ella’s white blood cell counts. We knew it increased her risk of infection, so we tried our best to keep her isolated…but in a houseful of small, social, germy children that was nearly impossible.  So began an uphill battle with chronic sinus and ear infections that would take two and sometimes three rounds of antibiotics to clear up before they would just return again. The infections also would drop her platelets and hemoglobin, trigger her asthma, cause roller coaster blood sugars, and make her just feel miserable. She went back on short courses of steroids, during which we struggled to control her blood sugar even more.  I tried every method I knew to protect and clean her sinuses and ears to prevent these infections: Neti pot washes, saline spray, fresh juice, homeopathic ear drops, garlic oil.  It helped some, but her weak immune system just couldn’t fight off these infections.

At the end of the summer, a nurse at our endocrinologist’s office recommended papaya leaf extract for low platelets, saying she knew a young leukemia patient who had great results with it for his platelets. A big fan of homeopathy, I ordered a bottle and gave it a shot. I gave her a large spoonful each morning  on an empty stomach, and her platelets shot up just as well as any IVIG treatment. We used it for about four or five months and her platelets haven’t been below 80,000 since then, even after stopping the extract.

While the papaya leaf extract stabilized her platelets, Ella still struggled with repeated infections, low WBCs and recurrent anemia. Her numbers would go up for about two weeks, then crash steadily in the remaining week or two before her next treatment. In October 2009, she was hospitalized for a week and a half for bronchitis, complicated by the Evans Syndrome, diabetes, and asthma. What a nightmare! In the prior months, we’d also had several overnight trips and 24 hour stays in the hospital to correct high or low blood sugars, ketones, and dehydration from stomach viruses and other infections.

We continued with steroid bursts, papaya leaf extract, IVIG, and round after round of antibiotics for the next year. She had a period of about two months in early 2010 when her numbers were very stable and treatment was working well; then her counts started to fall again.

In May of 2010, shortly before she turned 9, Ella had sinus surgery and ear tubes placed in an attempt to relieve the chronic infections. It didn’t get rid of them completely, but greatly cut down on the number of infections she got, and improved her quality of life.  The IVIG kept her stable for another month, so much so that her hematologist wanted to begin lowering her dose. That didn’t work well at all. Her counts fell drastically, her symptoms returned, and it took another round of steroids (and super high blood sugars) to pull them to safe levels.

After a failed, vomit-filled trial of Cellcept in August and September, Ella began the drug Rituxan in October 2010.

(Now might be the time to mention that in the midst of this chaos, we had a baby girl in August, and began fostering a four-year-old boy the week before Ella’s first Rituxan treatment. Instead of dragging all these kids with me to the hospital every two weeks, my husband graciously took Ella to all of her twice monthly infusions.)

Rituxan was a wonder drug for Ella. That, in addition to all our other measures, stabilized her counts drastically in a very short amount of time. For the first time in almost two years, she could play like a real little girl again—she could run outside with her siblings and not get tired. She could turn cartwheels and not be covered in bruises and blood. She wasn’t so sick anymore. She was Ella at last.

She took her last IVIG on December 10th, 2010, and her last Rituxan shortly after the New Year.

On February 9th, 2011—ironically the 7th anniversary of her type-one diagnosis—Ella was officially declared in remission and has remained there for just over a year. 

I thought having a toddler with diabetes and a new baby was the hardest thing I ever went through. Little did I know I would deal with diabetes, Evans Syndrome, and five young children (now six—we adopted again in March 2012). 

Ella Rain is a trooper through all of it and the strongest person I know.  On our journey with Evans, she cried, she got angry, and she got scared. But through all of it she found a way to be her quirky, outgoing, positive self, even when dealing with the worst of it all. She’s been recently diagnosed with Celiac Disease (January 2012) and is adjusting to a gluten-free life with grace. 

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

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