My heart was racing and so was hers. I could tell as I held her small fragile body in my arms. She had started breathing rapidly as we rushed through the doors of ER. The triage nurse gave up trying to record her breaths as she wrote down, “Too numerous to count.”
She was hurried back immediately. Her temperature was increasing as time was ticking away. Upon examination, the swollen organs were easily found—her veins, not so much. After having to eventually hand over our seven-month-old baby to the medical team and leave the room with Dennis holding me up, dragging me away—they finally found a vein after seventeen tries. I counted each agonizing stick as the necessary torture went on.
Screams.
Thrashing.
Then lying limp.
I wanted to be there and be strong for her, but I didn’t want to watch. Her high-pitched cries of helplessness were too hard to bear. It was back and forth—me burying my head in my arm as I would hand her over to her daddy—then yelling, “No let me hold her!” Watching them poke her time and time again was ridiculous! The doctor finally told me to wait outside. I was too hysterical and they needed access right then. We were watching our child slowly deteriorate before our eyes and there was nothing we could do. The blood was clotting as fast as it was drawn. They had never seen this before?
We were frantically pacing the halls, her daddy and I—waiting for answers, anxious for results…
Chapter One
“Third Time’s a Charm?”
My doctor had already given us less than a 2% chance of conceiving again after having two healthy children and five miscarriages.
Needless to say, we were both surprised and shocked when we learned we were pregnant with Kristan, our soon to be third child. We did not realize at the time that percentages meant nothing. Only 0% and 100% matter and unless you are God, you cannot predict those odds.
The happiness of the positive pregnancy test was soon faded as I laid on the hospital bed while the ultrasound tech searched endlessly for a heartbeat. She walked away to get a “special” probe and said she would try again. I could see pity in her eyes and hear doubt in her voice as she tried to reassure me. I didn’t want to give up hope but I kept hearing the words of my doctor playing over in my mind—telling me that if we didn’t hear anything this time, we would schedule a surgery. That the pregnancy would be non-viable. Tears flowed down my face as I closed my eyes tightly and begged God.
The sound of the heartbeat we heard on the monitor left me breathless.
It truly felt miraculous.
The pregnancy from the start acted as though my body was trying to reject the baby growing inside my womb. The entire nine months was full of me trying not to lose her. From not being able to hear the heartbeat in early pregnancy and the doctor about to give up hope, to severe allergies, and being on bedrest three different times. Toxemia, along with a rough labor and delivery, made it a complicated pregnancy. The placenta was attached and had to be manually removed, leaving me almost needing a blood transfusion. It was extremely painful.
I thought I had felt pain.
I thought I had felt fear.
Nothing could have prepared me for what was about to come.
Seven months later...
We were escorted to a different area out of the rush of ER. We left our baby girl, Kristan, on the hospital bed with IVs and monitors all hooked up to her as we waited in one of those small stuffy rooms—the ones they put families in as they wait on results.
Everything but nothing was going through our minds as we knew the report was back. We tried to be strong for one another, then crumbled the next—two very frightened parents. I wanted someone to hurry and walk in and tell us something right away, but at the same time didn’t look forward to seeing the doctor or hearing what he had to say. Even though it had only been a couple minutes, I wanted to get back to my baby before she woke up and saw a stranger there with her. The nurse seemed capable, but it needed to be me there by her side. One of us would walk around the corner and peek in at her while we continued to wait.
As we sat there, I thought I had never prayed so hard in my life. Little did I know this was only the beginning.
“Didn’t look like leukemia but can not say for sure until further testing,” the ER doctor finally reported, after having received the preliminary results from the hematologist/oncologist. We were slightly relieved thinking at the time that cancer was the “worse case scenario.”
After going over all the paperwork, the three of us were taken upstairs to the hem/onc floor—the infectious side.
Did I know there was a whole floor for children with cancer? What kind of world was I living in to not know this, or did I just refuse to know it? It’s easier that way, right? To turn your head and not see? It made me feel guilty for being so consumed in what I thought was our “perfect little life” and not knowing. Let alone doing something to help.
Walked past a beautiful little girl who once had long blonde hair. I say once because they left the few strands that had not fallen out. The image of her holding her mother’s hand by the elevator is forever etched in my mind.
My heart was slowly sinking further down as my own fear tried to crush me. It was all in slow motion. My hope was dwindling. I was starting to feel the other parent’s pain. I knew they loved their child as we did ours.
We arrived at our room. So many in and out.
“Have you been to another country?”
“We will need to get a liver biopsy.”
“Cat scratch fever…”
Nurses hooking up IV medications. Infectious Disease asking more and more questions. Heard the word bone marrow aspirate.
It was total chaos. White coats coming in and doing assessments, nurses taking vitals—the smell of sanitizing wipes and alcohol filled the air.
And then we heard those words that sent us into an absolute panic and complete shock. “Her hemoglobin is 5.8. She has a low ANC, making her severely neutropenic…”
I felt claustrophobic as the intense feeling of dread tried to suffocate me.
“What? Wait! What is a normal hemoglobin and neutro-WHAT?”
Numbness was starting to set in—going through the motions…
Why is everyone suiting up in sterile garments and masks and why are we in isolation? I want my baby to be okay!
We don’t belong here.
She does not have cancer.
I had to focus. I had to give her complete history to the doctor, in hopes of finding out what was going on.
She needed me.
She needed for me to pull it together and not fall apart.
I began, “At three months old, Kristan had gotten RSV that landed her in the hospital for a week. Once they released her, she had to return for repeat blood draws since all of her counts were low. Apparently the petechiae she had and the doctors feeling her tummy each time they came in must have meant something. We were told that viruses could hit the bone marrow and they expected it to rebound. It did.”
I was trying hard to concentrate and not leave anything out, while she was screaming inconsolably the entire time, making it a challenge.
I explained further, “With breastfeeding Kristan I learned she had extreme sensitivities and allergies to the foods that I eventually took out of my diet. She would have blood in her stool when I had any dairy or egg. We tried different formulas that did nothing but make her vomit. So I eventually took all dairy, egg, soy, beef, tomato, and citrus out of my diet—most allergens—to see if that would help. It would be better for a while and then she would go back to having diarrhea, twenty-five stools a day. Her bottom was staying broken down. We even ran allergy tests that came back inconclusive.”
Kristan, finally exhausted from all the constant screaming, laid sleeping in my arms. I kept watching her breathe while I continued giving, what I hoped to be, helpful information.
I mentioned, “At six months old she was sick again—this time CMV. Again petechiae covered her body, and the blood test results showed low counts. She had five days of fever. One more day and they were going to redraw labs. They were treating her like she had a virus.”
Kids get viruses, right?
Nothing too unusual.
So many things were running through my head as I was trying to help put the puzzle together and come up with something that could give them a clue as to what was slowly killing our child. Dennis was trying to help me remember dates—it was so overwhelming. We were beyond worried for our daughter as we both knew that this was life threatening.
I continued on through my tears, “I had kept telling my family that something was wrong. I didn’t like the constant diarrhea and this was addressed many times by our doctor. We sent off for cultures, checked the stool for blood, and was actually told to stop breastfeeding.”
I am glad I decided to continue since it was a great comfort for her, especially now.
I explained that our pediatrician, we had at the time, was in and out as she was in the process of moving. So we saw her along with other doctors and got different opinions. They were a great group and we appreciated all they did.
Infectious Disease kept asking numerous questions. We continued to go over her history—RSV, CMV, EBV?
I explained what led us up to this point.
Chapter Two
“The Call”
Seven days prior…
The last few days had been a total blur.
We put a call in after the first day of fever returning. Even though it had been a month since she had CMV—we were not liking it. Kristan was seen by her doctor and we were told once again that it was most likely just a simple illness, nothing more.
After day three of fever, they wanted to draw blood and check for EBV. The results were somewhat confusing since it is usually unreliable in young children. Day five of fever and I was begging them to see her again since Tylenol and Motrin were no longer cutting it—at all. Labs were repeated, for our peace of mind, and we were sent home to wait on results.
It was our son Logan’s 4th birthday. I was too nervous and worried to plan anything big, so we threw together something small at my mom and dad’s house. All while waiting on the phone to ring with the results of the blood test.
The anticipation of the call left me feeling physically ill.
Kristan was getting sicker and weaker before my eyes. All she wanted was for me to hold her and was looking more pale to me than usual. Earlier that day I was unsure as to whether or not to call. I felt like, Am I being extra paranoid? I knew they probably thought I was overreacting. They said she had a virus. Plus, they had just drawn blood two days earlier.
I didn’t care if I may have been that overprotective, nagging parent. I was making that phone call. She couldn’t make it. She was too young and I was her advocate.
When the phone finally rang, I took it in my parent’s room for privacy. I could feel my heart beating out of my chest as I answered it.
“All of her labs look a little screwy. We need to send you to St. Louis for further testing to see what’s going on. Do you want Cardinal Glennon or St. Louis Children’s?”
I had no idea. After a brief pause, I chose Children’s.
That one decision—the choice of which hospital—could have changed the course. Amazing how choices and decisions we make can take us down different roads and change the path of our destination.
I was to call and make an appointment for Monday—this was on a Thursday. I was told she should be okay until then, for it was not an emergency.
I went back into the living room where everyone was waiting on me, so that we could all sing “Happy Birthday” to Logan. Our future hero. Tears rolled down my face as I watched him blow out his candles before I told anyone the news.
The next day, Friday—very sick baby, could not hold her head up sitting in the highchair. I called Children’s and pleaded for them to see her sooner. They assured me that according to the looks of the labs, she should be fine waiting over the weekend and they would see her first thing Monday morning. They did explain that we could go through our local ER if we needed to have her seen before then.
I kept going over this in my mind—declining, sick baby. High fever. Pediatrician office is now closed. Labs had been drawn twice within days—last time being the day before. No swollen organs. She can wait until Monday. I kept asking myself over and over, Why does this seem like so much more?
Dennis and I both stayed in the living room that night, leaving a lamp on to keep a close eye on her—he in the recliner, me on the couch. I tried to get some sleep as I held her on my chest. Many prayers were being said. She woke in a frenzy, screaming in agitation. We were about to take her to the hospital when she finally calmed down. At wee hours of the morning, she could no longer physically lay on her belly. I was panic-stricken!
I woke Dennis, who had just barely fallen asleep, and we made a mad dash out the door. We dropped our two other children off at my parents' and I headed to the hospital. He had to work so I met my sister, Holly, at the Peds clinic—where doctors see patients on the weekend.
Kristan was burning up with fever and sweating at the same time. Something was obviously not right. It didn’t matter if it was a wasted trip—wished it was, but knew differently. They were going to see our baby. I insisted over the phone to the doctor that was on call.
Labs were drawn immediately—the doctor could see urgency in my eyes and hear panic in my voice. He examined Kristan to find an enlarged liver and swollen spleen.
He sent us straight up to St. Louis Children’s Hospital. Had we known that we could have went on our own through their ER without being a patient—we would have taken her sooner.
We just didn’t know.
Doctors, residents, students, nurses—in and out of our room.
“Needs a blood transfusion stat!”
“Bone marrow aspirate in the morning.”
“Spinal tap is being ordered…”
They were not only feeling her tummy but measuring, marking, and recording.
With each new theory came more tests. It was really hard sending her to have the spinal tap as we waited outside the procedure room. She was so gravely ill—her pale skin, extended abdomen, and raging fevers. She would scream until she couldn’t anymore, then she would lay limp.
Her leaving my arms made me feel so helpless as I felt the realness haltingly set in. I found myself slowly falling to the floor as I slid down the wall holding me up. I lost it—right there in the middle of the hallway—frantically weeping, begging God to hear and answer our prayers. Asking people as they passed by to pray for our daughter. No one looked at us funny—they all knew. They had been there.
Tube down her throat to administer medications.
She continued to get even sicker. As her liver enzymes were skyrocketing, her eyes and skin became more and more yellow.
She was so swollen all over and in so much discomfort as each organ was being attacked. As awful as she felt, she continued to nurse. Not for the fact of being hungry but for comfort. It was hard to hold her without feeling like we were adding to her pain. That was all she wanted, to be in my arms. I could feel how high her temps were as she laid her precious hand on my chest, and her nursing was actually burning me.
Nearly six days… no answers, only more questions. Her ceaseless temperatures reached 106 as she was getting closer to liver failure. How much more could a helpless infant take?
Chapter Three
“Our Worst Nightmare”
On June 14th, 2002, our world stood still. It was a parent’s worst nightmare.
Two doctors walked into our room and sat down. They asked us to do the same. My mother was there—she had come up to be a support for us and witnessed our world as it came crumbling down.
The silence was broken when one of the doctors began, “We believe your daughter has a disease called Hemophagocytic lymphohistiocytosis…”
As they spoke a burning pain radiated down my entire body. My ears started to feel hot and my face flush. My heart fell to the floor. I could barely catch my breath. I felt as if the wind had been knocked out of me. I had to make myself breathe.
I was trembling from fear as I stumbled towards the hospital bed where I reached for my daughter. I could barely focus my eyes. The room seemed to grow darker all around me and all I could see was my baby. Everything else faded into the background. The doctors’ voices seemed to be a low distant mumble that I refused to hear. I picked Kristan up and held her in my arms and started talking to her as if the doctors were not even in the same room. I told her that I loved her so much and that she was going to be okay. She HAD to be okay! I held her close and kissed her delicate skin. I leaned across, laid my head down beside her on the bed, and cried what tears I had left.
Our child, our precious daughter…Why? How could this beast take over her body?
I knew that they would fill me in on everything that was talked about. I could not listen to it all at once. It was too much to take in. I was going into shock. It reminded me of when you learn a loved one had died. That is how I felt. Like I had been told that someone I loved had died. I was numb, paralyzed by what I was feeling.
Quickly I snapped back into existence when I heard them say, “She will eventually need a bone marrow transplant.”
And so our journey began.
We left the infectious disease side, after she received her first blood transfusion, and crossed over to the hem/onc side. As we walked down that long lonely hallway, turning our heads at each open door and glass window, we saw child after child hooked up to machines—tubes coming from their chest, no hair on their heads, and suitcases in their rooms showing they would be there a while. It really brought us even more into reality and out of what felt like was some kind of delusion, a terrible nightmare that we were wanting desperately to wake up from. Some of the precious children had faint smiles on their faces, others showing sadness.
Blood products were being brought in. IVs and medications were being given. Nurses were rushing into rooms and doctors were gathering at the front desk. The children in the hallway had on masks and were being pulled around in wagons. They were still hooked up to their poles—they must have needed that small escape.
I was so afraid as I had never witnessed such terror and sorrow all at one time. I saw a certain stare in the eyes of every parent that we passed. It was like they were reliving their lives of when they were the newly diagnosed. I saw sorry in their faces that we too had entered that floor.
We were about to become part of that family.
Each and everyday at some point I would leave the room. Sometimes it was when she was resting and I knew it was okay to leave. Other times it was when I needed to rest my mind and find some form of peace so that I could be strong for her. During this time I took my Rosary beads that my sister-in-law, Misti, had sent. They were special to me—the beautiful blue crystals with Lourdes water in the connecter. I held my beads and paced the halls back and forth. Walking with my head down—never looking up. I whispered the mesmerizing prayer aloud with my eyes barely opened enough to see the floor. It was a small freedom, the fifteen minutes I was gone, and brought me such comfort. The cards Misti and her family sent, assuring us that Masses were being said all over for Kristan, helped to give me some peace as well.
Fever, swollen spleen and enlarged liver, bi-cytopenia (low blood counts in at least two cell lineages), high triglycerides and/or low fibrinogen, hemophagocytosis, low/absent NK-cell-activity, high ferritin, and high-soluble interleukin-2-receptor levels—altogether, five out of these eight must be fulfilled. Unless family history or molecular diagnosis is consistent with HLH. These were all part of diagnosing the disease our daughter had.
There was not just one single test to prove positive or negative—it was more of ruling out other diseases and meeting five of the criteria. Ferritin being high seemed to be the contributing factor for her. The warning to go forward with treatment and after an MRI to check for CNS involvement, we were ready to start chemo.
Chemo, going into our 7-month-old infant.
She was just a baby. Soft skin and giggles! Just last week she was playing with her brother and sister on a blanket on the floor, the two showing her toys and making her laugh. Her daddy read her a book as she pointed to pictures and made babbling sounds. I was just getting the hang of taking three children under the age of five to the grocery store and loving the challenge of making it all work—the meals, the housework, the time for the kids and the spouse. Bath time was always fun, followed by a relaxing lavender lotion massage.
Now she was going to have to go into surgery to have a line inserted in her little chest, with tubes hanging out, so that chemo could be given. I saw no soaking nor splashing in a bath for her in any near future. I was nauseated at the thought of toxic drugs flowing into my baby girl’s precious body—the one I breastfed to try to give her what I thought was the best, keeping her out of the sun without sunscreen, and all the other precautions a parent takes to keep their child safe and protected from any danger.
The chemo would be given through an IV until she had her broviac put in.
My mother, Dennis, and I sat there waiting for them to bring the chemo into the room. As I watched them hang the drug that had a bright yellow sticker on it reading—CAUTION—I held our daughter tighter, and prayed that everything would be okay. The colorless drug slowly dripped, drop by drop, into the clear vial. It then ran down the tube, which entered the IV that was inserted into our little girl’s head. They could not find a usable vein anywhere else on her body, and had no other option—they had all collapsed.
Things seemed to be moving in slow motion as we stared in disbelief at what was happening. And then…
Suddenly, Kristan had a terrible reaction to the first dose. She went from burning-up-hot with fever to so cold that her temperature would not register. The nurses rushed warmed blankets in and we cuddled closely with our baby as the necessary infusion went on. Finally, we got a reading. The blankets alone were not enough. I held Kristan next to me, skin to skin, as the nurses wrapped us in the covers, like a tight cocoon. It was very frightening to feel your child have no body heat. I kept expecting to see a crash cart at any given moment.
We missed our other children desperately and needed to see them. Talking over the phone was not nearly enough. We wanted to hug and hold them, to make sure they were okay.
The day they came up to the hospital to see us was when I experienced our first miracle. We had not slept in days. It had been a week since we had been admitted. We had just learned our child had a rapidly fatal disease and could die. We thought it could not have gotten any worse.
And then it did.
We were missing our other two children and our old life. On top of all of this, more doctors had met with us earlier that day to explain that the disease we were dealing with… was genetic.
Nothing hurt more than learning that!
What that meant was our other precious children—Morgan, age six; Logan, age four—could also have it. This disease that kills and has no other options but a risky BMT.
The thought of knowing this—that at any given moment our other two children, whom seemed so healthy, could get the deadly disease that ravaged their sister—was too devastating to consider. It was bad enough to learn there was a possibility of them having it, but also there was no test at the time to know. We would have to wait and see if they ever got symptoms.
In the mean time, we would always have that worry in the back of our minds. We were not sure how we were going to be able to live like that—not knowing. Every waking moment the mere thought of it haunted us. All we could do was pray. Watch for symptoms, and pray.
Kristan’s genetic testing showed nothing but she had absent NK cells, which led them to believe it was most likely the familial form with a 25% chance of the others being affected.
Now I had to meet our children that Grandad, my father, was bringing up to the hospital. I had to try to explain to them that their sister was about to have surgery to insert a line near her heart that she would be given numerous doses of medications through. That we were going to have to take all kinds of precautions to help not bring any germs to her—to tell them how sick she was.
How do you explain to children so small that their world as they knew it was no longer going to exist for a while? That everything was about to change.
I was going to be looking at them through eyes that saw disease almost kill their sister within a few short days. From the time Kristan began her first fever, that is what the disease was doing. It was starting its attack at destroying her. She would have most likely died, had we waited another day. Every hour counted.
Now I was going to get to hold my healthy children and pray they would never see the beast HLH.
As I walked out on the bridge that overlooks the street below and is connected to the parking garage, I held my head down.
Walking.
Praying.
Talking to God.
My days all began with a Dear God… and my entire last several days were a non-stop prayer that ended each night with an Amen.
I was desperate.
I was at my lowest low, wondering how I could get enough courage and strength to do this. To even let our children see me like this? I could not pull it together long enough to let my face and eyes clear up for more than a few minutes without losing it again. I didn’t want them to see me so upset.
As I stood there and prayed, I stared out at the cars below driving by. I closed my eyes and begged God to please not only give me strength but to send me some kind of hope, some kind of sign to know everything was going to be alright.
I had never done that before—ask for a sign. It must have been out of desperation and sleep deprivation. I needed no sign to show me there was a God; I had complete faith in that. What I needed was something, anything, to let me know Kristan was going to be okay.
As I opened my eyes, that were blurred from the tears, I could see this brightly shining cross that hung on a rearview mirror of one of the passersby. The colors were brilliant as the sun shined down perfectly on the cross, making it look iridescent and glowing. It was beautiful. Truly magnificent.
It was as if the lulling glow erased the darkness of the near death hours that I had seen. It made my eyes more open. I could feel the heat from the sun’s rays, through the glass on my face, as my tears began to dry.
I—at that moment—felt for the first time since diagnosis…
HOPE.
I embraced our children, whom I missed so much, with my new found strength and determination. I let them know that their daddy could not wait to see them and that we were going to get through this as a family.
That it was going to be okay.
Miraculously… I believed that wholeheartedly.
She was hurried back immediately. Her temperature was increasing as time was ticking away. Upon examination, the swollen organs were easily found—her veins, not so much. After having to eventually hand over our seven-month-old baby to the medical team and leave the room with Dennis holding me up, dragging me away—they finally found a vein after seventeen tries. I counted each agonizing stick as the necessary torture went on.
Screams.
Thrashing.
Then lying limp.
I wanted to be there and be strong for her, but I didn’t want to watch. Her high-pitched cries of helplessness were too hard to bear. It was back and forth—me burying my head in my arm as I would hand her over to her daddy—then yelling, “No let me hold her!” Watching them poke her time and time again was ridiculous! The doctor finally told me to wait outside. I was too hysterical and they needed access right then. We were watching our child slowly deteriorate before our eyes and there was nothing we could do. The blood was clotting as fast as it was drawn. They had never seen this before?
We were frantically pacing the halls, her daddy and I—waiting for answers, anxious for results…
Chapter One
“Third Time’s a Charm?”
My doctor had already given us less than a 2% chance of conceiving again after having two healthy children and five miscarriages.
Needless to say, we were both surprised and shocked when we learned we were pregnant with Kristan, our soon to be third child. We did not realize at the time that percentages meant nothing. Only 0% and 100% matter and unless you are God, you cannot predict those odds.
The happiness of the positive pregnancy test was soon faded as I laid on the hospital bed while the ultrasound tech searched endlessly for a heartbeat. She walked away to get a “special” probe and said she would try again. I could see pity in her eyes and hear doubt in her voice as she tried to reassure me. I didn’t want to give up hope but I kept hearing the words of my doctor playing over in my mind—telling me that if we didn’t hear anything this time, we would schedule a surgery. That the pregnancy would be non-viable. Tears flowed down my face as I closed my eyes tightly and begged God.
The sound of the heartbeat we heard on the monitor left me breathless.
It truly felt miraculous.
The pregnancy from the start acted as though my body was trying to reject the baby growing inside my womb. The entire nine months was full of me trying not to lose her. From not being able to hear the heartbeat in early pregnancy and the doctor about to give up hope, to severe allergies, and being on bedrest three different times. Toxemia, along with a rough labor and delivery, made it a complicated pregnancy. The placenta was attached and had to be manually removed, leaving me almost needing a blood transfusion. It was extremely painful.
I thought I had felt pain.
I thought I had felt fear.
Nothing could have prepared me for what was about to come.
Seven months later...
We were escorted to a different area out of the rush of ER. We left our baby girl, Kristan, on the hospital bed with IVs and monitors all hooked up to her as we waited in one of those small stuffy rooms—the ones they put families in as they wait on results.
Everything but nothing was going through our minds as we knew the report was back. We tried to be strong for one another, then crumbled the next—two very frightened parents. I wanted someone to hurry and walk in and tell us something right away, but at the same time didn’t look forward to seeing the doctor or hearing what he had to say. Even though it had only been a couple minutes, I wanted to get back to my baby before she woke up and saw a stranger there with her. The nurse seemed capable, but it needed to be me there by her side. One of us would walk around the corner and peek in at her while we continued to wait.
As we sat there, I thought I had never prayed so hard in my life. Little did I know this was only the beginning.
“Didn’t look like leukemia but can not say for sure until further testing,” the ER doctor finally reported, after having received the preliminary results from the hematologist/oncologist. We were slightly relieved thinking at the time that cancer was the “worse case scenario.”
After going over all the paperwork, the three of us were taken upstairs to the hem/onc floor—the infectious side.
Did I know there was a whole floor for children with cancer? What kind of world was I living in to not know this, or did I just refuse to know it? It’s easier that way, right? To turn your head and not see? It made me feel guilty for being so consumed in what I thought was our “perfect little life” and not knowing. Let alone doing something to help.
Walked past a beautiful little girl who once had long blonde hair. I say once because they left the few strands that had not fallen out. The image of her holding her mother’s hand by the elevator is forever etched in my mind.
My heart was slowly sinking further down as my own fear tried to crush me. It was all in slow motion. My hope was dwindling. I was starting to feel the other parent’s pain. I knew they loved their child as we did ours.
We arrived at our room. So many in and out.
“Have you been to another country?”
“We will need to get a liver biopsy.”
“Cat scratch fever…”
Nurses hooking up IV medications. Infectious Disease asking more and more questions. Heard the word bone marrow aspirate.
It was total chaos. White coats coming in and doing assessments, nurses taking vitals—the smell of sanitizing wipes and alcohol filled the air.
And then we heard those words that sent us into an absolute panic and complete shock. “Her hemoglobin is 5.8. She has a low ANC, making her severely neutropenic…”
I felt claustrophobic as the intense feeling of dread tried to suffocate me.
“What? Wait! What is a normal hemoglobin and neutro-WHAT?”
Numbness was starting to set in—going through the motions…
Why is everyone suiting up in sterile garments and masks and why are we in isolation? I want my baby to be okay!
We don’t belong here.
She does not have cancer.
I had to focus. I had to give her complete history to the doctor, in hopes of finding out what was going on.
She needed me.
She needed for me to pull it together and not fall apart.
I began, “At three months old, Kristan had gotten RSV that landed her in the hospital for a week. Once they released her, she had to return for repeat blood draws since all of her counts were low. Apparently the petechiae she had and the doctors feeling her tummy each time they came in must have meant something. We were told that viruses could hit the bone marrow and they expected it to rebound. It did.”
I was trying hard to concentrate and not leave anything out, while she was screaming inconsolably the entire time, making it a challenge.
I explained further, “With breastfeeding Kristan I learned she had extreme sensitivities and allergies to the foods that I eventually took out of my diet. She would have blood in her stool when I had any dairy or egg. We tried different formulas that did nothing but make her vomit. So I eventually took all dairy, egg, soy, beef, tomato, and citrus out of my diet—most allergens—to see if that would help. It would be better for a while and then she would go back to having diarrhea, twenty-five stools a day. Her bottom was staying broken down. We even ran allergy tests that came back inconclusive.”
Kristan, finally exhausted from all the constant screaming, laid sleeping in my arms. I kept watching her breathe while I continued giving, what I hoped to be, helpful information.
I mentioned, “At six months old she was sick again—this time CMV. Again petechiae covered her body, and the blood test results showed low counts. She had five days of fever. One more day and they were going to redraw labs. They were treating her like she had a virus.”
Kids get viruses, right?
Nothing too unusual.
So many things were running through my head as I was trying to help put the puzzle together and come up with something that could give them a clue as to what was slowly killing our child. Dennis was trying to help me remember dates—it was so overwhelming. We were beyond worried for our daughter as we both knew that this was life threatening.
I continued on through my tears, “I had kept telling my family that something was wrong. I didn’t like the constant diarrhea and this was addressed many times by our doctor. We sent off for cultures, checked the stool for blood, and was actually told to stop breastfeeding.”
I am glad I decided to continue since it was a great comfort for her, especially now.
I explained that our pediatrician, we had at the time, was in and out as she was in the process of moving. So we saw her along with other doctors and got different opinions. They were a great group and we appreciated all they did.
Infectious Disease kept asking numerous questions. We continued to go over her history—RSV, CMV, EBV?
I explained what led us up to this point.
Chapter Two
“The Call”
Seven days prior…
The last few days had been a total blur.
We put a call in after the first day of fever returning. Even though it had been a month since she had CMV—we were not liking it. Kristan was seen by her doctor and we were told once again that it was most likely just a simple illness, nothing more.
After day three of fever, they wanted to draw blood and check for EBV. The results were somewhat confusing since it is usually unreliable in young children. Day five of fever and I was begging them to see her again since Tylenol and Motrin were no longer cutting it—at all. Labs were repeated, for our peace of mind, and we were sent home to wait on results.
It was our son Logan’s 4th birthday. I was too nervous and worried to plan anything big, so we threw together something small at my mom and dad’s house. All while waiting on the phone to ring with the results of the blood test.
The anticipation of the call left me feeling physically ill.
Kristan was getting sicker and weaker before my eyes. All she wanted was for me to hold her and was looking more pale to me than usual. Earlier that day I was unsure as to whether or not to call. I felt like, Am I being extra paranoid? I knew they probably thought I was overreacting. They said she had a virus. Plus, they had just drawn blood two days earlier.
I didn’t care if I may have been that overprotective, nagging parent. I was making that phone call. She couldn’t make it. She was too young and I was her advocate.
When the phone finally rang, I took it in my parent’s room for privacy. I could feel my heart beating out of my chest as I answered it.
“All of her labs look a little screwy. We need to send you to St. Louis for further testing to see what’s going on. Do you want Cardinal Glennon or St. Louis Children’s?”
I had no idea. After a brief pause, I chose Children’s.
That one decision—the choice of which hospital—could have changed the course. Amazing how choices and decisions we make can take us down different roads and change the path of our destination.
I was to call and make an appointment for Monday—this was on a Thursday. I was told she should be okay until then, for it was not an emergency.
I went back into the living room where everyone was waiting on me, so that we could all sing “Happy Birthday” to Logan. Our future hero. Tears rolled down my face as I watched him blow out his candles before I told anyone the news.
The next day, Friday—very sick baby, could not hold her head up sitting in the highchair. I called Children’s and pleaded for them to see her sooner. They assured me that according to the looks of the labs, she should be fine waiting over the weekend and they would see her first thing Monday morning. They did explain that we could go through our local ER if we needed to have her seen before then.
I kept going over this in my mind—declining, sick baby. High fever. Pediatrician office is now closed. Labs had been drawn twice within days—last time being the day before. No swollen organs. She can wait until Monday. I kept asking myself over and over, Why does this seem like so much more?
Dennis and I both stayed in the living room that night, leaving a lamp on to keep a close eye on her—he in the recliner, me on the couch. I tried to get some sleep as I held her on my chest. Many prayers were being said. She woke in a frenzy, screaming in agitation. We were about to take her to the hospital when she finally calmed down. At wee hours of the morning, she could no longer physically lay on her belly. I was panic-stricken!
I woke Dennis, who had just barely fallen asleep, and we made a mad dash out the door. We dropped our two other children off at my parents' and I headed to the hospital. He had to work so I met my sister, Holly, at the Peds clinic—where doctors see patients on the weekend.
Kristan was burning up with fever and sweating at the same time. Something was obviously not right. It didn’t matter if it was a wasted trip—wished it was, but knew differently. They were going to see our baby. I insisted over the phone to the doctor that was on call.
Labs were drawn immediately—the doctor could see urgency in my eyes and hear panic in my voice. He examined Kristan to find an enlarged liver and swollen spleen.
He sent us straight up to St. Louis Children’s Hospital. Had we known that we could have went on our own through their ER without being a patient—we would have taken her sooner.
We just didn’t know.
Doctors, residents, students, nurses—in and out of our room.
“Needs a blood transfusion stat!”
“Bone marrow aspirate in the morning.”
“Spinal tap is being ordered…”
They were not only feeling her tummy but measuring, marking, and recording.
With each new theory came more tests. It was really hard sending her to have the spinal tap as we waited outside the procedure room. She was so gravely ill—her pale skin, extended abdomen, and raging fevers. She would scream until she couldn’t anymore, then she would lay limp.
Her leaving my arms made me feel so helpless as I felt the realness haltingly set in. I found myself slowly falling to the floor as I slid down the wall holding me up. I lost it—right there in the middle of the hallway—frantically weeping, begging God to hear and answer our prayers. Asking people as they passed by to pray for our daughter. No one looked at us funny—they all knew. They had been there.
Tube down her throat to administer medications.
She continued to get even sicker. As her liver enzymes were skyrocketing, her eyes and skin became more and more yellow.
She was so swollen all over and in so much discomfort as each organ was being attacked. As awful as she felt, she continued to nurse. Not for the fact of being hungry but for comfort. It was hard to hold her without feeling like we were adding to her pain. That was all she wanted, to be in my arms. I could feel how high her temps were as she laid her precious hand on my chest, and her nursing was actually burning me.
Nearly six days… no answers, only more questions. Her ceaseless temperatures reached 106 as she was getting closer to liver failure. How much more could a helpless infant take?
Chapter Three
“Our Worst Nightmare”
On June 14th, 2002, our world stood still. It was a parent’s worst nightmare.
Two doctors walked into our room and sat down. They asked us to do the same. My mother was there—she had come up to be a support for us and witnessed our world as it came crumbling down.
The silence was broken when one of the doctors began, “We believe your daughter has a disease called Hemophagocytic lymphohistiocytosis…”
As they spoke a burning pain radiated down my entire body. My ears started to feel hot and my face flush. My heart fell to the floor. I could barely catch my breath. I felt as if the wind had been knocked out of me. I had to make myself breathe.
I was trembling from fear as I stumbled towards the hospital bed where I reached for my daughter. I could barely focus my eyes. The room seemed to grow darker all around me and all I could see was my baby. Everything else faded into the background. The doctors’ voices seemed to be a low distant mumble that I refused to hear. I picked Kristan up and held her in my arms and started talking to her as if the doctors were not even in the same room. I told her that I loved her so much and that she was going to be okay. She HAD to be okay! I held her close and kissed her delicate skin. I leaned across, laid my head down beside her on the bed, and cried what tears I had left.
Our child, our precious daughter…Why? How could this beast take over her body?
I knew that they would fill me in on everything that was talked about. I could not listen to it all at once. It was too much to take in. I was going into shock. It reminded me of when you learn a loved one had died. That is how I felt. Like I had been told that someone I loved had died. I was numb, paralyzed by what I was feeling.
Quickly I snapped back into existence when I heard them say, “She will eventually need a bone marrow transplant.”
And so our journey began.
We left the infectious disease side, after she received her first blood transfusion, and crossed over to the hem/onc side. As we walked down that long lonely hallway, turning our heads at each open door and glass window, we saw child after child hooked up to machines—tubes coming from their chest, no hair on their heads, and suitcases in their rooms showing they would be there a while. It really brought us even more into reality and out of what felt like was some kind of delusion, a terrible nightmare that we were wanting desperately to wake up from. Some of the precious children had faint smiles on their faces, others showing sadness.
Blood products were being brought in. IVs and medications were being given. Nurses were rushing into rooms and doctors were gathering at the front desk. The children in the hallway had on masks and were being pulled around in wagons. They were still hooked up to their poles—they must have needed that small escape.
I was so afraid as I had never witnessed such terror and sorrow all at one time. I saw a certain stare in the eyes of every parent that we passed. It was like they were reliving their lives of when they were the newly diagnosed. I saw sorry in their faces that we too had entered that floor.
We were about to become part of that family.
Each and everyday at some point I would leave the room. Sometimes it was when she was resting and I knew it was okay to leave. Other times it was when I needed to rest my mind and find some form of peace so that I could be strong for her. During this time I took my Rosary beads that my sister-in-law, Misti, had sent. They were special to me—the beautiful blue crystals with Lourdes water in the connecter. I held my beads and paced the halls back and forth. Walking with my head down—never looking up. I whispered the mesmerizing prayer aloud with my eyes barely opened enough to see the floor. It was a small freedom, the fifteen minutes I was gone, and brought me such comfort. The cards Misti and her family sent, assuring us that Masses were being said all over for Kristan, helped to give me some peace as well.
Fever, swollen spleen and enlarged liver, bi-cytopenia (low blood counts in at least two cell lineages), high triglycerides and/or low fibrinogen, hemophagocytosis, low/absent NK-cell-activity, high ferritin, and high-soluble interleukin-2-receptor levels—altogether, five out of these eight must be fulfilled. Unless family history or molecular diagnosis is consistent with HLH. These were all part of diagnosing the disease our daughter had.
There was not just one single test to prove positive or negative—it was more of ruling out other diseases and meeting five of the criteria. Ferritin being high seemed to be the contributing factor for her. The warning to go forward with treatment and after an MRI to check for CNS involvement, we were ready to start chemo.
Chemo, going into our 7-month-old infant.
She was just a baby. Soft skin and giggles! Just last week she was playing with her brother and sister on a blanket on the floor, the two showing her toys and making her laugh. Her daddy read her a book as she pointed to pictures and made babbling sounds. I was just getting the hang of taking three children under the age of five to the grocery store and loving the challenge of making it all work—the meals, the housework, the time for the kids and the spouse. Bath time was always fun, followed by a relaxing lavender lotion massage.
Now she was going to have to go into surgery to have a line inserted in her little chest, with tubes hanging out, so that chemo could be given. I saw no soaking nor splashing in a bath for her in any near future. I was nauseated at the thought of toxic drugs flowing into my baby girl’s precious body—the one I breastfed to try to give her what I thought was the best, keeping her out of the sun without sunscreen, and all the other precautions a parent takes to keep their child safe and protected from any danger.
The chemo would be given through an IV until she had her broviac put in.
My mother, Dennis, and I sat there waiting for them to bring the chemo into the room. As I watched them hang the drug that had a bright yellow sticker on it reading—CAUTION—I held our daughter tighter, and prayed that everything would be okay. The colorless drug slowly dripped, drop by drop, into the clear vial. It then ran down the tube, which entered the IV that was inserted into our little girl’s head. They could not find a usable vein anywhere else on her body, and had no other option—they had all collapsed.
Things seemed to be moving in slow motion as we stared in disbelief at what was happening. And then…
Suddenly, Kristan had a terrible reaction to the first dose. She went from burning-up-hot with fever to so cold that her temperature would not register. The nurses rushed warmed blankets in and we cuddled closely with our baby as the necessary infusion went on. Finally, we got a reading. The blankets alone were not enough. I held Kristan next to me, skin to skin, as the nurses wrapped us in the covers, like a tight cocoon. It was very frightening to feel your child have no body heat. I kept expecting to see a crash cart at any given moment.
We missed our other children desperately and needed to see them. Talking over the phone was not nearly enough. We wanted to hug and hold them, to make sure they were okay.
The day they came up to the hospital to see us was when I experienced our first miracle. We had not slept in days. It had been a week since we had been admitted. We had just learned our child had a rapidly fatal disease and could die. We thought it could not have gotten any worse.
And then it did.
We were missing our other two children and our old life. On top of all of this, more doctors had met with us earlier that day to explain that the disease we were dealing with… was genetic.
Nothing hurt more than learning that!
What that meant was our other precious children—Morgan, age six; Logan, age four—could also have it. This disease that kills and has no other options but a risky BMT.
The thought of knowing this—that at any given moment our other two children, whom seemed so healthy, could get the deadly disease that ravaged their sister—was too devastating to consider. It was bad enough to learn there was a possibility of them having it, but also there was no test at the time to know. We would have to wait and see if they ever got symptoms.
In the mean time, we would always have that worry in the back of our minds. We were not sure how we were going to be able to live like that—not knowing. Every waking moment the mere thought of it haunted us. All we could do was pray. Watch for symptoms, and pray.
Kristan’s genetic testing showed nothing but she had absent NK cells, which led them to believe it was most likely the familial form with a 25% chance of the others being affected.
Now I had to meet our children that Grandad, my father, was bringing up to the hospital. I had to try to explain to them that their sister was about to have surgery to insert a line near her heart that she would be given numerous doses of medications through. That we were going to have to take all kinds of precautions to help not bring any germs to her—to tell them how sick she was.
How do you explain to children so small that their world as they knew it was no longer going to exist for a while? That everything was about to change.
I was going to be looking at them through eyes that saw disease almost kill their sister within a few short days. From the time Kristan began her first fever, that is what the disease was doing. It was starting its attack at destroying her. She would have most likely died, had we waited another day. Every hour counted.
Now I was going to get to hold my healthy children and pray they would never see the beast HLH.
As I walked out on the bridge that overlooks the street below and is connected to the parking garage, I held my head down.
Walking.
Praying.
Talking to God.
My days all began with a Dear God… and my entire last several days were a non-stop prayer that ended each night with an Amen.
I was desperate.
I was at my lowest low, wondering how I could get enough courage and strength to do this. To even let our children see me like this? I could not pull it together long enough to let my face and eyes clear up for more than a few minutes without losing it again. I didn’t want them to see me so upset.
As I stood there and prayed, I stared out at the cars below driving by. I closed my eyes and begged God to please not only give me strength but to send me some kind of hope, some kind of sign to know everything was going to be alright.
I had never done that before—ask for a sign. It must have been out of desperation and sleep deprivation. I needed no sign to show me there was a God; I had complete faith in that. What I needed was something, anything, to let me know Kristan was going to be okay.
As I opened my eyes, that were blurred from the tears, I could see this brightly shining cross that hung on a rearview mirror of one of the passersby. The colors were brilliant as the sun shined down perfectly on the cross, making it look iridescent and glowing. It was beautiful. Truly magnificent.
It was as if the lulling glow erased the darkness of the near death hours that I had seen. It made my eyes more open. I could feel the heat from the sun’s rays, through the glass on my face, as my tears began to dry.
I—at that moment—felt for the first time since diagnosis…
HOPE.
I embraced our children, whom I missed so much, with my new found strength and determination. I let them know that their daddy could not wait to see them and that we were going to get through this as a family.
That it was going to be okay.
Miraculously… I believed that wholeheartedly.
