A Rare Kind Of Day

February 28th is Rare Disease Day. This can be for any rare disease. We know Kwashi is rare. She is our special girl that we love with all our heart.

Hopefully, next month will bring us more answers as to what is going on with her little gi tract. We are confident in saying she does have a form of FPIES (Food Protein Induced Enterocolitis Syndrome). Her Feeding Team supports this, her allergist said she was on the "spectrum" and we have consulted a specialist in Columbus, Ohio who was certain she had a variant. Plus, the wonderful moms and dads in my FPIES support group have shared some much information. It has been eye opening.

I know everyone these days has a cause they want you to support. But today is for the "rare" ones. So wear some comfy jeans. You can still look professional. I know because her daddy came down sporting a pair. Guess he will be a bit more casual rounding today.

You Are A New Mom, It's All In Your Head: Part IV

We were in the hospital for 24 hours. NG was placed. Her doctor had no words of wisdom. He said he had never seen one like her. What is that suppose to mean? There wasn't a plan. Just to get her into feeding therapy that our insurance wouldn't pay for. The waiting list was long, maybe I could work on that. I knew the feeding tube wasn't the answer. 48 hours into using it. She started to vomit. She pulled it once. I placed it again. It was horrible. She fought me. I said enough was enough. If she didn't want to eat I wasn't going to force her. I couldn't hold her down and shove a tube down her. Over her 9 months of life I had to do it a few times. I wasn't willing to do it anymore.

Our baby was trying to tell us something. I tried a couple other foods after the NG. She would just vomit when I would place them in her mouth. Here we were with nothing to feed her, fighting to feed her bottles. On the weight roller coaster. NO MORE SOLIDS, that is what I decided. Gut rest until she was 12 months. Best decision ever. Although she fought us when it came to feeding her she was gaining again.

Around December I was encouraged to try solids again. We trailed beef and cherries with success. White potatoes were a fail. In that time we tried a medication to increase her appetite. It only made her throw tantrums and sleep all the time. It seemed to cause her pain.

At the end of December we held off for a few weeks until we saw the feeding therapy team. OH YES, finally feeding therapy. What a long day that was. They didn't tell us anything we didn't already know. They were in agreement that Kwash had some form of FPIES but now to get a doctor that understood. At therapy we decided to trail pears next.... And we all know how that went.

So that is our journey. We have no answers. We have no safe food. We wonder if her formula is all that safe. I sit here and struggle with all this. There was no fun birthday cake when she turned one. There is not sharing our food with her. There is just us saying NO or taking away foods she finds on the floor out of fear she will react.

You Are A New Mom, It's All In Your Head: Part III

By mid march I was defeated. I put the pump up and broke out the Neosure and rice cereal. Thickening my BM wasn't working.  I was getting less than an oz per pump. Her first bottle with rice she screamed and broke out in a rash. We had seen a speech therapist to evaluate Kwashi's swallow when she had her swallow test. They gave us a can for Thick'it. I gave that a try. She seemed to take to it better. In a few short days I had a screaming infant pooping 12x a day green/black, smelly, mucus poops. Being a new mom never around babies I didn't know any better. But she was eating and she ate for a solid week. As quickly as she started eating she stopped. Kwashi continued to decline.

April brought on dehydration and complete feeding refusal.We were advised to come into Children's Hospital for evaluation. Upon taking her in and the staff placing an NG tube we noticed how utterly uncomfortable she was while being tube fed. Her doctor told us she just had difficulty swallowing. So if she has trouble swallowing and we are now bypassing that function why is she so uncomfortable?  I questioned the staff about her poop, reflux, and her behavior for three days. Nothing was done test wise to figure out her issue. None of the doctors would look at her poop. Interesting enough the nurses, PT/OT, SLP, all said there is something very, very wrong.

We were d/c'ed home, with a NG, with no answers. My mom radar was going crazy. I called another children's hospital and got into a gi doctor that came highly recommended. Our insurance didn't pay for it, I didn't care. So I marched up to the new doctor's office he asked me if anyone had tested her stool. Looking dumfounded I said no. The test to 3 seconds. She tested positive for blood in the stool. He said she likely has MSPI but could not rule out a swallowing issue just yet. The dietician came in we started a plan. Alimentum powder mixed to 26cal/oz. We had the ng to work with. So we were hoping we could find her comfort more quickly. She briefly mentioned plan B and C if the alimentum didn't work. The dietician reassured me she has to rarely go to plan B and just about never went to plan C. 

Evelyn's body had a different take on things. Evey's NG stopped working. She ripped it out and I was done with putting it back in. She drank the new formula for a few days. Her stools improved but her mood did not. I was told to give it to her unthickend for two weeks. That it took time. So I stuck with it. But why did she seem to get worse? After day eight I said enough is enough. Let's go to plan B: Alimentum Ready To Feed, a corn free formula that some kids tolerate better. Well, not Evelyn. We finally went to plan C.... Elemental formula., 50 plus dollar a can formula!!! WTF??? She took to it ok. Her poos improved, her mood picked up, feeding her was only kind of a nightmare. She was gaining weight again. We were happy with the improvement and so was her GI doctor. 

Fast forward 3 weeks later and the feeding refusal started again. She started pooping more frequently, she started screaming more. I started crying more. The doctor told me "It takes time, sometimes 6 weeks." Ok, but why was she doing ok and now not? He also wanted me to start solids sooner rather than later. I didn't agree but there came a point of desperation in me... Maybe she would take better to solids. She was almost 6 months adjusted. What could it hurt?

Haha, I started giving her bananas. We had a ton in the house. She didn't take to them well. She tried a little here and there. Then one day ate a bunch. The following days brought on visible blood in the stool. Super cranky. No sleep. I consulted the doctor and we all agreed it was time for a scope...

We had the scope and it  was.......... NORMAL. I was told to keep trying Elecare. No kids reacts to it. And really start trying the solids. I didn't start the solids. We started thickening her feeds again because after the scope she flat out refused to eat. And oh she ate with the thickened feeds!!! But within a week EXPLOSIVE MUCUS filled poops started again. And we were now starting to slowly put together the puzzle pieces. Her stools tested positive for blood again. We switched to another thickener which causes constipation and more blood. So what was the common link between the two thickeners??? CORN! They were corn based. I asked the doctor about Kwashi possible reacting to the corn in Elecare. I was shot down. My husband and I found a new thicken called GelMix. It pretty much saved us from another NG tube. Kwashi started chowing down on thickened Elecare with GelMix. Her mood improved so much and the blood stopped.

Since we had a "happy" baby, happy for Kwash, I decided to go back to the solids. Next was apple, seemed to go well. Sweet potatoes seemed a win, squash OH YEAH. We seemed on a roll. Kwashi seemed to like the foods. We hit a little snag with carrots. She vomited a bunch on day three. I didn't push those any further. Then "The Great Mango Incidence" occurred. Mangos = Blood in the stool after one bite! Then Kwashi stopped eating her "safe" foods. Started gagging and vomiting when I tried to feed her. She didn't want that food in her belly. No way No how. So here I was with a 8 month old who refused to eat solids and started refusing her bottle AGAIN!!! He doctor just looked confused. I brought up FPIES and his Fellow said... She doesn't vomit... 

By September her bottle refusal drove me so mad I opted for the NG again... But at the same time we also changed her formula for a third time. The doctor wasn't too keen on it. I didn't care what he had to say anymore.

You Are A New Mom, It's All In Your Head: Part II

So we made it HOME. All the magical happiness of motherhood was going to start flying out my ass and sprinkle down on everyone. Haha... Right...

My husband and I live where family support was limited. We were alone with her, with no help, already very exhausted from the NICU stay. I was very, very alone in my own drugged out mind. No help from mom but that is a different story. My husband and I could do it! We lived through a long distance relationship, medical school, residency, pharmacy school, a house fire and an emergency c-section WE CAN DO ANYTHING!!!!

Things were exhausting as I expected.  Keeping a three hour around the clock feeding and pumping schedule was easier at home vs. the NICU. I didn't have to wake up from my 1.5 hours of sleep and walk a good half a mile to the NICU in the freezing cold while still recovering from the c-section. This was an improvement!

We went on like this for a few weeks. I tried to breastfeed but was obsessed with knowing how much she got. She would only feed on the breast of ~10 minutes. It was too exhausting for her. So I continued to not sleep for the sake of her getting that yummy BM. We were instructed for fortify her BM to 22 cal. But something wasn't sitting right with me. I noticed she became increasingly fussy, spitty, gassy, crabby on the fortified BM. So I cut it out and she continued to grow. No harm. A step towards a more natural approach of feeding.

 

Kwashi only did better for a short time. She eventually started to fuss again and take longer and longer to feed. I was so insane about feeding her and pumping. I cut out dairy and soy. My weight was drastically dropping and I was starting to lose my milk supply when she was two months old. Pumping for 10 plus hours a day and trying to feed her for the same amount of time left everyone with little to no sleep.

I questioned reflux. We got the swallow test and an upper gi to see if she had any anatomical issue. All normal. The doctor wouldn't give me reflux meds based on the test. She encouraged me to stop pumping for my own mental health and relax. I begged to differ about the reflux since I watch Kwashi puke in her mouth and swallow it many times a day. Kwashi's refusal to eat became such an issue in the house my husband, who is in the medical proffession, consulted the only GI doctor at our children's hospital. The GI doc said she most likely had microaspriation that the test didn't pick up and we needed to thicken her feeds. He advised I stop breastfeeding and switch to formula to make thickening easier. Breastmilk breaks down thickeners. I was insane from little sleep. It all sounded like a wonderful plan. But we all know now what I wish I would have known then.

You Are A New Mom, It's All In Your Head: Part I

I know we don't have an official diagnosis but like I have said many times chronic reactors are tricky. You need to see a specialist that treats FPIES kids. Most of these knowledgeable doctors are in Philly or Boston. Ohio isn't the FPIES capital of the world. Although this is fact we are seeing our third GI doctor in April who treats FPIES kids in our area... Nervously awaiting that visit.

So how did it all start...? Do I really want to remember? There is a lot I have blocked out but if I think about it I can remember those first 10 nightmarish months.

Around the third trimester I was riding high. Passed my pharmacy boards. Excited to be pregnant during the holidays. Super excited to get to see my baby in February. Around my shower, just a week or so before Thanksgiving, this momma wasn't feeling so hot. My shoes wouldn't fit. I got tired super quick. I felt like a water balloon. I told myself this was normal. My doctors didn't seem concerned. I plugged along, volunteered at our local charitable pharmacy and ignored my body screaming at me to slow down. At work my legs would swell so much I would have to leave. But this is all normal?!? I gained 7# in the first 22 weeks or so of pregnancy. Then I started to gain weight crazy fast. I wasn't eating a lot but I was drinking a ton!!! And peeing very little although running to the bathroom every 10 minutes. Around Christmas I noticed my hands and face would go completely numb and on a lark I decided to take my blood pressure. Wouldn't you have guessed it the cuff read 200/100. AMAZING. I rapidly declined over the following two weeks. At Christmas I cried and begged to go home, as we were out of town. My pressures were staying up for longer periods of time and I knew something was very wrong when me pee started foaming on Christmas Eve.

After an admission, being sent home, then coming right back to the hospital the next day E.R.C was born at 33 weeks via c-sections because momma had got the pre-e. We were lucky to get it in time because HELLP syndrome was about to happen. And that my friend is a very, very bad thing. Lucky for me and Miss E we got the steroids in before the crash c-section. Baby come out breathing on her own. Momma was in the magnisum haze. All was ok?!?

I had a rough go in the hospital. Being drugged out of your mind with completely uncontrolled blood pressures while trying to pump for a premature infant was difficult.  But NOTHING was going to keep me from providing the natural goodness of breastmilk to my child. NOTHING. I got through it. My milk came in and she got all that hard work in her belly... through a feeding tube. OH SO NATURAL. The NICU staff still amped up the calories with fortifier. Which I didn't realize until two weeks into our stay. I wasn't happy with the fact she was receiving processed formula. But she was growing, yet still not wanting to breastfeed. Plus most of her diet was my BM.

Over the three week stay I became increasingly frustrated. I watched smaller babies breastfeed like champs and go home quickly. Here I was still struggling getting my little one to breast. Finally we decided to give her a bottle. It was "easier" She took to it well the first day. Then she stopped finishing the bottles. I was convinced it was the nursing staff. I was super mom! I could get her to eat! I wanted out!!! And so I did. I pumped, never slept and fed our LO at every feed. Every poky, labor intensive feed. I would stare at the clock because she had to do it within 20 minutes. Being constantly on edge about your child eating is a curse of the NICU.  They do it to everyone, even those who are feeding well.  All the nurses and doctors kept telling me "It will click. It always does. You will be amazed." But the day of D/C I knew it wasn't clicking and the crazy amount of work I put into feeding her I hid from the staff because I wanted to go home!!!

FPIES, What PIES?

Kwash has officially failed pears. Now we are on two week gut rest, again. We have had so many fails it is mind blowing. The Feeding Team wants her to be evaluated by yet another doctor. They feel she has FPIES... Come again? Food Protein Induced Enterocolitis Syndrome. Now there are two avenues when it comes to FPIES.

You have your "Classic" acute symptoms:
    "The classic pattern of an FPIES reaction is when a healthy infant or child develops symptoms    shortly after eating a food. There is a characteristic delay of 2-3 hours before onset of severe and repetitive vomiting and eventually diarrhea. The child may appear very ill and sleepy (lethargic), and may become pale or blue. When evaluated by a doctor, he/she may be found to have low blood pressure, seem dehydrated, and have blood tests that mimic infection (sepsis); which in some cases can lead to sepsis-like shock. Many infants who are eventually diagnosed with FPIES are initially suspected to have a severe infection or sepsis." (FPIES Foundation)
 
Then there is the ever tricky "Chronic" reactors: 
   "The second common pattern of FPIES reaction symptoms occurs when infants who are ingesting a problem food (usually milk or soy-based formula or proteins in breast milk) as a consistent part of their diet might experience increasingly severe vomiting, diarrhea, and poor growth, possibly progressing to an illness mimicking a severe total-body infection. Please note that each child is unique and your child may experience their own range and intensity of these symptoms." (FPIES Foundation)

    The range of symptoms are as follows: sleep disturbance, sensory issues, blood/mucus stool, acidic stools, congestion, limiting food intake, fussy/irritable. Fun right? 

None of it is fun. Doctors are unaware of the acute reactions let alone the chronic, which has even less medical date to support a diagnosis. 
 
Fortunately (or unfortunately) Kwashi has never had an acute reaction. But her chronic issues have left her hospitalized twice for dehydration due to feeding refusal. The first time she was barely three months old. She was drinking almost nothing for a week. She had chronic green, smelly, mucus filled, diarrhea at least 6x a day. She slept poorly. Upon taking her in and the staff placing an NG tube we noticed how utterly uncomfortable she was while being tube fed. Her doctor told us she just had difficulty swallowing and that many preemies in the NICU did. I questioned the staff about her poop, reflux, and her behavior for three days. Nothing was done test wise to figure out her issue. None of the doctors would look at her poop. Interestingly enough the nurses, PT/OT, SLP, all said there is something very, very wrong. I got the heck out of the hospital for a second opinion which unfortunately has sent us down another dead end path. 

Pears are a Bear

We started feeding therapy two weeks ago. We go once a week. Have a meal and talk about what to try next food wise. Last week we decided Pears. Kwash has been doing well with smooth purees, although still limiting herself to about 2.5oz. So I went to Whole Foods got some organic pears, cooked them up and prepared them to a smooth like texture.

I was nervous about the texture, the rate of FPIES reactions, and just well... feeding her. At therapy she did great. No gagging, no resistance, just happy eating an oz of pears. That gave me a ton of confidence. Until 5 hours later.

Kwash wasn't into eating her last bottle. After we battled through it she vomited all over me. Not just a little, a lot. And the thing is she doesn't vomit. She vomited with carrots, cow's milk, and rice. So these episodes have been few and far between. Since she slept ok I decided to chug along.

The next day she flat our fought me about eating the pears. She only ate 0.5oz.  My feelings of defeat returned.

Today she ate 3 bites which  caused her to vomit. I cried for 3 hours.

Feeding team wants to put the pears up for a while. Bring them to the next appointment to address if it is a texture or avoidance issue.  I say it's a fail. But who am I?

A Day In The Life

About a year ago my darling little baby was growing in the nicu. Although feeding was going slow, she was thriving on my breast milk I was so diligently pumping. Let's fast forward to today, as I sit in my child's vomit and cry. I often wonder where did it all go wrong?

I must say Kwashi (as we like to call her) is a tricky one. People don't look at her and think oh there is something, not right. She is a tiny 17 pound one year old that's a decent height and head that is just right. She crawls, started walking, has several words, and commands. She 'seems' happy when new people are around. So what is the problem? Why do I seem like I psychopath that can't leave the house, can't mess with her scheduled, that can't seem to take a shower most days or move on with my life? For any mother that has a special needs child you understand but for us many people don't. You can't see Kwashi's issues. They linger deep inside where no doctor or mother could ever see what is going on.

Kids with GI issues and intestinal allergies are as difficult to deal with as any special needs child. I spend most my day syringe feeding a child formula that she generally refuses. Most solids she gags on, vomits up, or if she will eat it days later she cries to be held all night because well..... She is in pain. And no one can tell me why this is happening. And no one will give her a diagnosis. She doesn't fit FPIES (Food Protein Induced Enterocolitis). She isn't just MSPI (Milk Soy Protein Intolerance). Maybe it's MFPI (Multiple Food Protein Intolerance)? Maybe I am a crazy mother and it is all in my head. Maybe I need to push solids more. Maybe I treat her with kid gloves. Maybe I should try this and that. Or maybe I should just stand on your head and say boo!

Whatever the case maybe I am exhausted. I need an outlet and this will be it. So if you stumble across this journey or if you are a friend of mine checking in. This is the good, the bad, and the ugly.