Our daughter was born six weeks premature and spent 12 days in the NCCU after birth. While this experience was difficult enough, it was nothing compared to the medical journey ahead of us. Clementine quickly caught up in weight and development and was a typical, happy baby. Since we, the parents, work full time, she started at a local daycare.
It was not surprising then when she started to catch the occasional cold or virus – except that when a bug came through a classroom, she would catch every single one. The coughs would go on for weeks. She caught hand, foot and mouth disease twice in three months. Her eardrums kept rupturing from simmering ear infections. And then at 13 months, we woke up one morning to her left cheek swollen to the size of a plum. We immediately took her to the pediatrician who diagnosed her with an inflamed salivary gland. Eleven days later it hadn’t improved, and a second pediatrician sent us to the ER for an ultrasound of the lump. It ended up being a large abscess and required surgical draining and several days in the children’s hospital. From then on, there wasn’t a month that didn’t include another virus, visits to the pediatrician, and more antibiotics. Laryngitis, roseola, bronchiolitis, severe mouth ulcers… Our poor baby’s happy spirit was crushed under the weight of constant illness and fevers and we were constantly rearranging our work schedules to manage backup childcare.
Time and time again I asked the pediatrician, “Is all of this normal? It seems like she is sick a lot. I know I’m a first time parent, but this doesn’t seem typical.” Time and time again, she reassured me this was normal for a daycare baby. My gut said that something else had to be going on. It didn’t add up. Her peers were not this sick. We were suffering. Our baby would lose weight from being unable to eat from the mouth sores, her lips chapped from drool she couldn’t bear to swallow. Her bottom would bleed from the horrendous diaper rashes caused by back to back oral antibiotics. At our lowest point, she couldn’t gather the energy to play – lying on the ground in a ball, simply repeating “no, no, no” – not even letting us touch her or hold her.
Finally the pediatrician called for more thorough bloodwork and a review of past bloodwork. I was on my way to do a radio interview when she called me with the results. She was vague but said we needed to make an immediate appointment with the pediatric hematology/oncology department. “Is it leukemia?” I asked her very directly. “We don’t know,” she replied. I made our first appointment from the recording studio lobby and tried to keep it together.
It was not leukemia. A bone marrow biopsy revealed that our daughter’s body makes neutrophils and then immediately kills them. Her absolute neutrophil counts ranged from 200-700 over the course of the previous months, but this had been attributed to the fact that she was always in the middle of an illness when tested. Our hematologist explained that her neutropenia was likely auto-immune in nature as all evidence shows that her white blood cells were normal at birth. At some point, probably around 9 months, her body turned on itself in response to a virus that looked very similar to neutrophils. While serious, they sometimes refer to her type of neutropenia as Benign Neutropenia of Childhood – hers being less benign than most. We cling to the hope that she will outgrow this auto-immune response and experience a remission – a hope most people with neutropenia do not have.
During a month of watch-and-wait to determine if our daughter’s diagnosis was severe enough to need Neupogen, a series of skin abscesses put us in the children’s hospital once more. She began taking Neupogen twice a week at 22 months and we have not had a major infection or illness since. And like most daycare kids, she only gets the occasional cold.
Today, Clementine gets one small injected dose (0.2 mL) of Neupogen per week. Daddy and Clementine have a ritual of picking out the colorful bandaid she will use and which YouTube video she gets to watch during the quick poke. Most nights she will protest a bit and squirm away, but there are not usually tears. We had really hoped to avoid injections, but her counts were simply too low. With the medicine, her counts are in the almost low normal range. At the very beginning she had weekly blood draws as the doctors determined her optimal dosage. Then, it was draws once a month. And then, draws every three months.
Basic sanitizing and hand washing is key. We have worked with her daycare to make sure that the other children are washing their hands too and not coming to school sick. We watch her wounds like a hawk for signs of infection or abscesses. Honestly, we are probably too lax now that she has been well for so many months. She’s a rough and tumble little kid, like most active toddlers. She has not needed any specialized care or accommodations at daycare or home.
With a colicky new baby in the house, our neutropenic toddler is the easy one! If she is experiencing pain or fatigue from the neutropenia, she is unable to articulate it. Since she is 2.5 years old, we try to keep her on a typical toddler routine with healthy food eaten by the whole family and a mandatory afternoon naptime (or play semi-quietly in your room time). Neupogen changed everything for our family. It’s incredible how much healthier she is compared to this time last year.
Last week I had to describe Clementine’s illness to a new Sunday school teacher. “Clementine has a very weak immune system. She has a rare blood disorder called neutropenia,” I usually begin. They look surprised since she is already bouncing around the room, going from toy to toy with enthusiasm. “Her body does not make enough white blood cells to fight infection. It will be important that everyone in the room washes their hands well.” We’ve never had anyone question the information or want to be anything but helpful. If anything, we have to assure others that it’s ok for Clementine to be around the kid with the runny nose or travel from home.
We know that our child’s health outcome could be worse. Many families in that same hematology/oncology wing of the children’s hospital have it so much worse than us. That being said, there is no way to prepare yourself for having a child with a chronic illness. How is my baby anything other than perfect? The deep grief we have felt at not being able to protect her from pain, and that it took so long (although really just months) to get her the right help, cannot be understated. We have suffered from this illness, but at least we now know what to call it. We have learned to recognize the many faces of this enemy and are so grateful to have a medicine with which to face it.
To the family or person with neutropenia, it is ok to grieve and to be angry. I am angry that I know where the best toys are in the children’s hospital. I am angry we had to cancel our annual Thanksgiving trip because we were too afraid to fly with our daughter on an airplane. I am angry my daughter looks at me with big blue eyes every Wednesday and Sunday and says, “Mama, I get my shot now. I get a boo boo (bandaid). I cry maybe?” But after you are done crying, you must look toward hope. One of the best things I did in the early days was reach out (through the Neutropenia Network) to a family in New York whose 18-year-old son has chronic severe neutropenia, diagnosed at age 2. Here was a family, many years into their neutropenia journey, providing evidence of a child who was thriving and happy.
Our daughter Clementine is smart, curious and confident. She reads dozens of books a day and loves to explore outside. She lives for special treats and surprises and loves her baby brother. We don’t know if she really will outgrow her neutropenia. I do know that with proper management and support, it will not define her future.