Pearl’s Story

 

A little over a year after my final hospitalization, baby Pearl was born on December 19^th 2013 as healthy and cute as can be. My health and pregnancy were closely monitored, but all went along nicely. I opted to hire a doula and went for a completely natural birth. Pearl was exclusively breastfed and I ate healthy foods, taking my own vitamins daily. I was going to give my all for her health. I had left my job as a banker and stayed home with my little bundle of joy. We were buds.

From the beginning, I noticed that she was a terrible sleeper and seemed to be writhing and grunting most of the night. It’s not uncommon for babies to do this so I started eliminating foods from my diet in case the proteins passed through my breast milk bothered her little tummy.

Desperate for sleep, I couldn’t wait for the day that she would start eating solids, in the hopes that she’d be sleeping through the night as happy as you please.

Little did I know, I wouldn’t be sleeping for a very long time.

The morning she turned 6 months old, I giddily marched to her high chair carrying a bowl of rice cereal. It immediately became apparent that she wasn’t interested in solids. At all. In fact, she never even put toys in her mouth like most babies. Nevertheless, I’d slip a bit of food in when she wasn’t looking and she’d spit it all out. Those nights, however, she’d be up crying more than usual and very gassy. Even though she didn’t swallow any food, enough must have stayed in her mouth to cause a tummy upset. Her general pediatrician wasn’t concerned as she was healthy and suggested holding off another few weeks and trying again. Of course, c-diff was always on my mind but was comforted by the fact that young babies simply don’t have the receptors to get a c-diff infection.  The next food trial was sweet potato and I watched in delight as she swallowed one bite. Visions of her eating handfuls of foods within the week danced in my head.

That night, I’ll never forget.  About 4 hours after she swallowed that one bite, she started stooling hourly.  I was starting to get nervous, but went on as usual and bathed her,  nursed her and put her to bed. Within an hour, a scream, unlike any I’ve heard from her, erupted from her crib. Her diaper was full of diarrhea and she was inconsolable. She’d nurse for a minute for comfort but stool the entire time, thrashing and in obvious pain. The frequency  increased to 25 diapers in one hour. I called the emergency number and they advised me to watch her very closely, if the diarrhea didn’t slow down I’d need to take her in but since she didn’t have a fever, it looked promising to quickly pass. I was about to take her to the hospital when she fell asleep in my arms and the stooling stopped. Pearl’s little body was exhausted and pale. I gently laid her in the crib and watched as she slept. Too afraid to leave.

Over the next three days the diarrhea stopped and she returned to normal.

Another month passed and I gave her a bite of oat cereal, and sadly, the whole process started over.

FPIES Diagnosis

Pearl was diagnosed with Food-Protein Induced Enterocolitis Syndrome (FPIES) when she was 8 months old by a specialist in Kansas City. The doctor had never seen a child with it, FPIES is very rare, but a colleague had.

FPIES is a non IgE mediated immune reaction affecting the GI tract. This is non-pathogenic; there are no tests for it, and the diagnosis is based on symptoms, but they are very obvious; the reactions are very startling and scary.

Children often fall into two categories: chronic reactors, and acute reactors. Chronic reactors have little or no ‘safe’ foods, generally having severe GI upset when in contact with certain or all food proteins. Acute reactors have more safe foods but have a more acute reaction, vomiting until they sometimes go into shock. Pearl was a chronic reactor.  Either way, children generally outgrow it. The doctor suggested I keep trying food but I was apprehensive. She was a good weight and nursing fine, and as long as she didn’t directly ingest food, she was very happy.  I felt like time was what we needed and thankfully our pediatrician agreed. I spent hours and hours reading medical journals or posting on the FPIES support group to solicit advice from the dear parents  living through this. It seemed to me that once a child reacted to a certain food, it would be lost as a safe food until outgrown, which could take years.  The most common first foods (rice, oats, sweet potato, etc) were also the foods with the highest number of reactions. New reactions rarely happened after age 1, so perhaps if I breastfed her long enough, she’d be less likely to react to new foods. So that’s what I decided to do.

It was a struggle supplying enough breast milk for a very active baby who was quickly becoming a toddler, but I’m so grateful I could. Since Pearl wouldn’t take a bottle, the feedings were all up to me.  I nursed her at least five times a night, every night.

Mealtimes became challenging. During the day, I’d eat lunch while she napped, and at dinner we’d sit her in the high chair, stacked with toys in hopes she didn’t notice her exclusion from food. I’ll never forget a certain play date we had. The other kids were joyfully snacking on apples, and I was eating as well. Pearl was playing busily at my feet when she suddenly noticed she was the only one not eating. She walked up to me and looked into my eyes and down at the apple I held in my hand, reaching for it. The sweetest, most innocent question spoke from her face. I could tell she was starting to feel confusion and it broke my heart.  I wept in the bathroom.

When Pearl was 14 months she successfully ate peaches. After a week, we gave her pumpkin. We were ecstatic! We very slowly introduced single-ingredient food – not even salt or flavoring could be added as that could cause a reaction. This graph helped me choose which foods to serve. I started with one tiny bite a day, increasing over several days. I always felt comfortable after day 5. Within a month, she had a nice variety.

I’ll never forget the day we finally had enough safe foods to eat a meal as a family . Roast beef, carrots and cottage cheese. Best meal of my entire life! We all giggled with every bite.

Pearl never had another reaction and by 18 months, she was weaned and eating on her own. We were overjoyed and grateful to have emerged from that stressful time.

But…that was about to change.

Here We Go Again

It was a beautiful September and I was 6 months pregnant with my son. Fall was in the air and we were off to the zoo. By then Pearl was a happy and fun 21 month old. I noticed sweet little Pearl stooling more than usual and took note, understanding kids’ bowel movements are always changing.   It started with just a few extra poops a day but I became apprehensive when they slowly changed to include mucus, and the frequency grew to 8-10 a day. I brought her to the pediatrician who thought perhaps it was toddler’s diarrhea, and tests for parasites and the usual bacterium came back negative. Pearl had dropped to the 3rd percentile for height, but she was still healthy otherwise.

Before long, her diapers became full of completely undigested food, covered in mucus and with a terrible odor. Everyone kept telling me to relax, and I tried, but every time she disappeared, I found her crouched in the corner pooping again. 12 times. 14 times. I thought perhaps her FPIES was back and brought her to another specialist who examined her and found her healthy,  and re-ran the general stool test. I kept calling their office, worried out of my mind until they finally told me flatly to chill out. I began to wonder if I were crazy, that maybe I was being too uptight, though the nature of her stools were undeniable.

It was Halloween and we took Pearl, who looked a little pale and thin, out in her bunny costume. I noticed she appeared weak when walking but she was smiling and went to bed happy.

Pearl awoke in the middle of the night, screaming. Dane and I rushed into her room where we found her diaper completely soiled, full of stench, mucus and undigested food. I’ll never forget the look in her eyes. Her lips were completely white. Nobody was going to tell me again to relax and we rushed her to the children’s hospital.

The Dreaded Diagnosis

I’m not sure if you’re the praying type, but I am and so are our families. Pearl was in the ER, lethargic and white, but didn’t have a fever. Initial x-rays and tests showed nothing abnormal, and neither did initial fecal tests. The doctor and I were chatting, and I told him about my c-diff history and her FPIES. I told him I was absolutely not worried about c-diff as she had NEVER taken any antibiotics. He agreed that c-diff wasn’t likely and we were to bring her back to a specialist. She could go home.

My heart prayed as I desperately wanted an answer to solve this mystery. “Whatever you are, show yourself to me right now.”

The ER nurse was busy working on the discharge paperwork as Pearl was slowly eating a banana. She suddenly erupted in screams and stooled again. Blood. Pus. Mucus. Pearl had suddenly spiked a fever so the decision was made that she’d be admitted to the hospital.

Thank God.

Pearl was very, very sick. Her sweet little body had no energy and our hearts broke to see her endure the confusion and pain of blood draws, IVs, x-rays and strange faces.  Every time the door opened, Pearl cried in fear.

The GI doctor came to visit and I again told him our histories. He listened as I relayed to him that maybe her FPIES was returning. Gently, he told me that it was certainly not. A fever does not represent itself in FPIES. He had done his research and I was beginning to trust him.

A few hours later, the doctor returned with an answer to all of our questions: Little Pearl had a c-diff infection. I wanted to argue that this wasn’t possible – she’s never had antibiotics.  But my heart knew. I almost passed out and sat silently watching my daughter.

C-diff. My demon.

All the horrible memories of my struggle were now right before me as my innocent daughter, who I would die to protect, was given Flagyl, put  in isolation and also on complete gut rest (no food or drink).  All night and all day, vile green sickness poured out of her as it did me. I could hardly handle it. My body was so stressed, now 8 months pregnant, and I was having intermittent contractions. If Dane hadn’t been there with me, I wouldn’t have been able to cope. I hadn’t showered in days because I couldn’t leave her. When I finally did, I opened the door to our home and saw her little bunny costume where it had been. I’ve never cried so hard in my life.

Pearl was hospitalized for 5 days and we were finally sent home with Flagyl. Proactively, I had asked the GI about a recurrence and if he supported the fecal transplant. He said the recurrence was unlikely and he did not support the transplant.

Two days after finishing Flagyl, c-diff was back.

Pearl’s Fecal Transplant

My beloved mother, who silently works behind the scenes, found Dr. Bartlett from the Mayo Clinic who was one of the only doctors performing fecal transplants on children. They were so kind and understanding. I was able to fax all of her records and they set  up her appointment over the phone. I’ll never, ever forget their compassion and willingness to help us.

By now, December of 2015, the FDA had approved the fecal transplant for recurrent c-diff infections. Pearl’s local GI doctor still did not support that route, but her general pediatrician did.  Pearl was now on Vancomycin to keep the c-diff at bay until we arrived at the Mayo Clinic the following week for her fecal transplant. Though the Mayo clinic does provide donors, Dane volunteered to be Pearl’s and the screening was easily completed here.

I was 9 months pregnant and unable to travel. The stress on my body was starting to affect my pregnancy so the doctor told me to stay home. It was difficult to watch Dane and his mother load up our darling girl and head out, and I wondered if Pearl thought I’d abandoned her during this scary time. Meanwhile at home, I was standing on my head trying not to go into labor.

Pearl, being almost 2, had to do a colonoscopy prep which was difficult. After that little hiccup, the colonoscopy and procedure went perfectly well. Like my own story, she slowly improved. Every day was a tad bit better but the fatigue was very hard on her. Suddenly, her hair started to grow. She gained weight and shot up to the 26th percentile in height.

As soon as they returned, I immediately went into labor and Oren was born the next day. Surely the new baby would lack GI issues, right?

Click here to read Oren’s story