Bad paperwork

Tylyn decided to watch My Sister’s Keeper last night. She has already seen the movie and why she would want to see it again is beyond me. For those of you who haven’t see the movie, stop reading right now, for those of you who have, feel free to continue.

We were watching the end and Tylyn asked me why the daughter was consoling Camren Diaz. Her point was why shouldn’t be the other way around.

As I watched it for my second time, tears streaming down my face, I could not come up with an answer. It was a valid point but I could not break away from the mother’s pain of her sick child. To those of us who have children it is completely understandable, how hard it is to give up and let go, not in just times of death but in difficult times of sickness as well.

Always wise to his words Dan answered that the daughter got it, she knew she was going to die and had accepted it. It was the mother that was still in denial and fighting. His response made me cry even harder.

Earlier that night we had picked up Tylyn and gone for ice cream. Driving around we were talking about random things and the subject turned to her one day having children. I tentatively brought up a subject I wanted to discuss in Denver.

“Ty, I know you really want to have children someday, but what if you find out your child will have what you have? “

It isn’t a guarantee or even a fact. I just know her grandmother had GERDs and I have had problems with my throat and allergies that is worse than my mom and Tylyn is worse than me so……. Will her child be worse than her? A chilling question but a valid one I feel especially since many of the articles relay the information that GERDs is similar to EE and often is a misdiagnosis.

Silence came from the back of the car.

After a long pause she replied, “I’ll adopt. I don’t want anyone to have what I have.”

That is a very mature answer for 17 and humbling also. She obviously doesn’t know the costs of adoption or if there is even enough data to prove or disapprove whether this is hereditary although the link is strongly suggestive.

She does understand now that it’s not normal to be sick after eating meals and the average person doesn’t have pain constantly in their abdomen.

Today we started the Zyrtec. The Singulair trial yesterday was unsuccessful as she is itching profusely especially on her face and around her mouth. Because she is eating the same things as always I don’t think the increased allergies are either food or environmental as she was even experiencing it outside our home at lunch.

A quick internet search revealed the FDA updated safety labels on Singulair to include warnings about this suspicious behavior. Symptoms on the warning label include: aggressive behavior, hostility, hallucinations, night-terrors, tremors, irritability, anxiety, depression and even suicide.
Not something I want her on anyway it sounds.

Also today I discovered a letter from the insurance company requesting additional information about Tylyn from her GI & Allergist. I faxed over the requests and also sent them back to her PCP. In the letter both Dr. Fleisher and Dr. Furuta’s names are spelled wrong. Courtesy of the PCP to which the insurance company kindly requested the paperwork be corrected and resubmitted.

I’m hoping she is relaxing today, swimming in the pool, enjoying her night off from work. Perhaps I should join her and not think about allergies or anything else again until tomorrow.

It's all about attitude

I am exhausted. Burning the candle at both ends has worn me out. I am not sleeping well and I’m still trying to fit 36 hours in a single day.

Although my mood is not as good as Tylyn’s, I still, physically, fair better than her. I guess attitude is everything.

She called this morning to let me know last night was very rough. She could not fall asleep at all. We had purchased a second hand couch; something that was in great shape but still wasn’t an arm and a leg just in case the puppies and cat decide to destroy it.

She had wanted to sleep with the puppies and her room is hot. She tossed and turned for a while and at midnight she had to shower because she was so itchy. I’m disappointed my efforts of washing down everything including the walls and floor at 11:30pm didn't work out so well for her.

She woke this morning with no relief. The Singulair does not help at all. We had tried that because the allergist had given her some samples and we had forgotten to get Zyrtec. I will pick some up tonight on the way home for sure.

She is still feeling full after eating. Yesterday she complained of pains in the center of her chest near her sternum. Her direct quote was, “Like somebody is stepping on me. “

While her demeanor stays happy and an introduction to a coworker revealed she has great positive energy. My mood is not as chipper, I am annoyed when people ask if she is better. No, no change. No, they can’t change treatment. BTW did I mention THERE IS NO CURE?

Sigh.

I received her results from the Barium swallow study. Normal. Interestingly enough I spoke with a woman who has been tested for almost all the same things as Tylyn, many different doctor’s trips, similar complaints. It’s amazing how God works to bring people in your life.

Speaking of faith, mine is a bit faltered. I know this should be the time when I’m strong. This is the time that I sing all the praises to God and his glory. I’m finding that a bit difficult right now. Kind of like I am getting the short end of stick. It’s selfish I admit, because it’s actually Tylyn that is being robbed, not me.

Saturday on our way home from photographing a wedding Saturday, Dan and I were discussing Tylyn and her condition. Dan said that she would be soon in her twenties and needed to know how to take care of herself. I starting crying uncontrollably knowing that I wouldn’t be able to make everything all better for her forever.

Despite her being dependent on me more now, in regards of making arrangements, calling doctors and interpreting results, I want her happy and healthy. I guess I should count myself blessed that I have a daughter who is alive and happy.

I just received a text from Tylyn, she rejoiced in a halfway normal bowel movement. I guess it’s celebrating the little things that get you through because if you focus on the negative, it will bring you down.

Another lesson taught from child to parent.  

Reality: The Non-Fiction Side of Eosinophilic Esophagitis

Today is Friday. Thank goodness my daily job as a Method Transfer Analyst at a pharmaceutical company is over for two days while I shoot a wedding and edit pictures.

As much as I love photography, I have pulled back a bit. I’ve cancelled all my promo work. I told Dan last night I couldn’t talk about shooting a wedding on November 5^th because it is two hours away and I won’t return home from Denver until the 3^rd. I’m not putting my life on hold because of Eosinophilic Esophagitis or Gastroenteritis. I’m reprioritizing.

Okay, whom am I kidding?

The fact is no different than when we had 9/11 or Hurricane Katrina- I am addicted in formation. My current read? Gastrointestinal Endoscopy Clinics of North America edited by Dr. Glenn Furuta, Tylyn’s soon to be doctor in Colorado. I even dreamed last night I was shooting a wedding and was describing to the guests about the disease and he was there listening. Dr. Furuta stood up and explained it had all been a misunderstanding and he would make Tylyn better. I woke up feeling sad that it wasn’t true. It was here. Forever.

I’ve been contemplating going back to school in the spring after my company closes. Something I had considered before for retraining, but set aside because my passion is definitely photography. I now think about the possibilities. The research I could do, the things I could discover, the help I could lend.

Then I realize I’m obsessing a bit like the man in Love and Other Drugs movie. Am I trying to make Tylyn better to make myself feel better? Do I want a simply uncomplicated life again? I don’t know the answers to these questions.

My sister, the counselor, says this is normal. Tylyn is supposed to be heading more towards independence. Instead I’m constantly researching the disease. I have to be the translator for the doctors to my daughter and my family. I have to be the educator for people who look at you and say, “What is it again? In English please. “

Take yesterday for instance Tylyn called after her barium swallow study. It went well except the doctor was concerned because her stomach was slow to empty during the study. Normal is five minutes to begin; hers did nothing for 13 minutes and then had five minutes of doing nothing.

My immediate question was, “So did it start emptying at 5 minutes? “

“No, it waited until 13 minutes.”

“And then it waited five minutes?”

“I don’t know, “ was Tylyn’s response.

How am I supposed to decipher what that means? How am I supposed to provide comfort? I have requested the 6^th of July off so I can go speak with the GI. Anything else gets muddled and is not relayed correctly.

In addition I have a file for Tylyn; test results, daily sheets and more. I didn’t think I was going have to be a mom, photographer, chemist and medical secretary. Add more to the plate.

On a lighter note the book is a great read. Sure it’s full of all kinds of medical terminology but I’m geeky and love that kind of thing. Some interesting data that can not be ignored is presented in the very first article.

‘Kelly and colleagues in 1995 investigated 75 pediatric patients who had longstanding reflux who were unresponsive to medical therapy. They found 23 patients had persistent esophageal eosinophiliac despite medical treatment of reflux. They hypothesized that there may be an allergic component to this entity and placed patients on an elemental diet for a period of 6 weeks. Of the 17 that began the trial, 12 completed the trial and 10 underwent repeat endoscopy. If the patients had improvement in their symptoms, a repeat endoscopy with biopsy then was performed following food challenges. On completion of the elemental diet, 80% patients became free of long term complaints and all others reported substantial improvement in their symptoms. The median time for improvement of symptoms was 3 weeks. Seventy percent of patients had asthma or eczema. On repeat endoscopy, 60% showed complete resolution of endoscopic findings. There was a significant reduction of esophageal eosinophiliac in all patients and complete resolution in 50%. The mean Eosinophils per HPF before and after therapy were 41 and 0.5 respectively. The investigators showed a decrease in basal zone hyperplasia and papillary height in biopsy specimens. During a controlled reintroduction of foods, symptoms were recreated in 9 of 10 patients a median of 1 hour after the reintroduction of the offending food. The most common agents were cow milk, soy protein, wheat, peanut and egg. With this evidence, Kelly and colleagues suggested an association between EE and an allergic predisposition. ‘[1]

I find this data a bit staggering and would like to read more about additional studies. The scientist in my head wants to read more about the data, the trends and patterns. I want more information about the antigens, the alleles and genetic markers. I guess that is for another day.

On lunch today I also read: 70% of children with Eosinophilic Esophagitis are males. Once again, lots of conflicting evidence to what is found on the internet. I guess I need to read more. Off I go.  

[1] Kelly K, Lazenby A, Rowe P, et a Eosinophilic esophagitis attributed to gastroesophageal reflux: improvement with amino-acid based formula. Gastroenterology 1995; 109 1503-1512.

The Facility

It’s 8:00 am. While most high school kids are either working or sleeping this summer morning, Tylyn is in Rochester at Strong Memorial Hospital getting a Barium Swallow Study done. She hasd fasted since midnight and will have to drink the radioactive liquid and wait another 4-6 hours to eat. She napped on the way there and complained she can’t even sleep during the study. After it is over she will return home only to head off to work at a convenience store a mile from our house. She has worked there since August so the amount of intellectual requirement is little, it is the five hour shift that will bring her home tired at nine and send her directly to bed. Some how I feel that Tylyn’s aspirations of acquiring another job and working more is a moot point right now.

She jabbers of heading off to Darien Lake or swimming in the pool we just got. In addition her summer is already beginning to fill with AP summer assignments including seventy-five AP Calculus problems. She also will be studying for her retake of her SATs. Her first attempt landed her a 1470, a number she and I are both happy with. She is happy with it because it required little studying or SAT course prep and still scored better than her average classmate. I am happy due to it being such a tumultuous spring.

Her next try will be in November. I know this because she wanted to know when our visit to Colorado would be so she could reschedule.

Colorado. Four months and three days. To her it seems like she has time to study later, and for me it can not come soon enough.

When we visit we will actually be at two different hospitals.

The first is at  National Jewish Health and will be seen by Dr. David M Fleischer

Dr. Fleischer is an allergist that works in conjunction with the CH in Aurora Colorado. He has a great understanding of EE & EG and Tylyn will spend the first three days in Colorado in this facility to undergo allergy testing after her initial intake appointment with the allergist, teh GI doctor and a dietician if needed.

Afterwards we will head over to Aurora Colorado to The Children's Hospital In Colorado. There she will be seen by Dr. Glenn Furuta  in an outstanding program they have for patients suffering from Eosinophilic Diseases. For more information about the Gastrointestinal Eosinophil Disease Program click  Here. 

Dr. Furuta has an impressive resume including affiliations with the following professional membership and societies:

American Gastroenterological Association- Member
Chron's and Colitis Foundation of America - Member
International Eosinophil Society - Member
North American Society of Pediatric GI, Heptalogy and Nutrition - Research Committee, Editorial Board

Society of Pediatric Research - Member
The International Gastrointestinal Eosinophil Researchers  - Chairman

Other Helpful Links:

Cincinnati Childrens Hospital

More about Dr. Marc Rothenberg and the program at Cinninnati Children’s Hospital.   

Rare Diseases at National Institute of Health

Families Affected By Eosinophiliac Disorders (FABED)

 

The Testing Continues

April 2011

Tylyn’s abdominal sonogram revealed no anomalies. The HIDA scan also was negative for gallbladder problems, leaving us clueless and Tylyn still suffering. Mid April we moved forward with the Endoscopy and the GI doctor in Rochester was now convinced that Tylyn had something going on.

Her blood work showed Eosinophilia (an increased number of Eosinophils in her peripheral blood). In fact her counts went from 9% to 11% to 14% to 20%.

He wanted to biopsy her esophagus, stomach and upper small intestine. Dan had planned on going to California so I accompanied Tylyn to the doctor’s for her procedure. Despite everything she had gone through she was amazingly positive and of course I snapped a few pictures to document the experience, including a picture that was in her chart showing the furrows in her Esophagus. The GI doctor gave us a preliminary diagnosis if EE.

It would be a couple of weeks before received the diagnosis. Eosinophiliac Esophagitis. Despite being ready for this, it took me over. I had been combing the internet for the past week trying to find information and had come up short but everything that I had researched prior to this point now started to fall into place.

Tylyn would now need to have a Colonoscopy done to look at the lower part of her small intestine and colon. The GI doctor was hoping to rule out Chrons and Celiac disease as well.

May 2011

I took the call from the GI doctor while I was at work. He informed me even though Tylyn’s colonoscopy had been textbook; the biopsies reveal her small intestine was ‘infiltrated’ with Eosinophils. I would receive a paper later on confirming the second diagnosis- Eosinophiliac Gastroenteritis. This would be shortly before what I would later on discover to be Eosinophiliac Awareness week.

She would be started on a course of Steroids for the Eosinophiliac Gastenteritis. I was not pleased with the GI’s decision to place her on Entrocourt (Budesonide Oral). I reluctantly agreed after hearing that they were hoping the steroid would knock her Eosinophil levels back to normal providing relief. He expressed that many patients respond to this treatment and in ideal circumstances she wouldn’t have to remain on it long. In addition he reassured me this particular steroid had minimal side effects because it was metabolized quickly. I was partially relieved that the Eosinophiliac Gastroenteritis would be brought under control, helping alleviate the discomfort of her chronic diarrhea.

I went to the pharmacy later that day to pick up the script. Due to wide limits of income, Tylyn still receives Child Health Plus. Price with insurance for a 30 day supply (90 pills) $0. Price if she didn’t have insurance- $1140. When I relayed that information to my family when I returned home she sullenly looked up from the movie she was watching and stated that she might need to change her career choice to guarantee she would always have insurance. This is a depressing thought for anyone, let alone a seventeen year old choosing a profession. This was not a name brand drug, merely a generic.

Working first hand at a pharmaceutical company I know the millions of dollars that it costs to develop a drug, bring it through clinical trials and the market. I am familiar with the steps of drug development. IND (Investigative New Drug), Phase I, II, III & IV Testing and NDA (New Drug Application).

In addition I have worked with the top of the line instruments; HPLC’s (high performance liquid chromatography), GC (gas chromatography), analytical balances, MS (mass spectrophotometers), and FTIR (Fourier transform Infared Spectroscopy) to name a few. I know the costs of columns, reagents and standards. My experience encompasses all these areas but I am completely disgusted over the FDA and federal regulations regarding these patents and release of these products. I can say with certainty the analysts and research scientists are not over compensated and place most of the blame on the pharmaceutical companies who are netting millions of dollars on product.

Alas I have gone off on a tangent. Time to refocus.

She was on the Entrocourt for only a week when she saw a marked increase in her symptoms. Diarrhea and abdominal pain increased significantly along with a new side effect: headaches. The latter was confirmed with the pharmacist as the most common side effect. I made a call to the doctor’s office the morning of my Grandmother’s funeral. The GI doctor was sympathetic in our loss but accounted for Tylyn’s change in symptoms to the latest upset in our family. Annoyed I expressed that it was not related to the funeral at all and proceeded to hang up and tell Tylyn she needed to take it in order to feel better.

The following week we visited a new allergist that the GI doctor wanted us to change to. Off Dan and Tylyn went. The time spent there explained that the Entrocourt could increase the diarrhea and abdominal pain she was having and the dose she was on was a lot but not enough to make a difference. He proposed putting her on Prednisone. His theory was that she was trying to put out a fire with a squirt gun and not a fire extinguisher. She needed to get rid of it once and for all and finally feel better.

Tylyn liked him because he was cute, not enough to win me over at this point. When she texted me the information he had told her I called the doctor. He posed his argument and I rebuttaled with hard data about steroid use, particularly Prednisone. He explained his point once again and said he thought it was a good thing Tylyn would be feeling better in 24-48 hours after starting the Predisone. A small time, he noted after suffering for years and possibly having both the Eosinophiliac Esophagitis and Gastroenteritis since birth. I said I wouldn’t tolerate long term use of two steroids. He agreed and said the Prednisone was only a short term use, two weeks, with a high dose at the beginning for seven days and a gradual wean.

Against my better judgment I agreed, though not before informing him that if she did not have an improvement she would be going and seeing the clinic in Cincinnati or Denver. He downplayed my threat but reassured me he knew Dr. Furuta and this was the best care she could receive in this area and ‘this wasn’t his first rodeo’. I will leave out my own colorful words here. He also decreased the Entrocourt at this point to one a pill per day instead of three.

Day 1- No change. Diarrhea, headaches, nausea, stomach pains.

Day 2- No change. Diarrhea, headaches, nausea, stomach pains.

Day 3- No change. Diarrhea, headaches, nausea, stomach pains.

Day 4- No change. Diarrhea, headaches, nausea, stomach pains.

Day 5- No change. Diarrhea, headaches, nausea, stomach pains, joint pain.

Day 6- No change. Diarrhea, headaches, nausea, stomach pains, joint pain.

At this point Tylyn and I headed Florida. I had booked a trip for just her and I back in April to get away from doctors, tests and the drama that seemed to follow us from week to week. So much for 2011 being better. I was dealing with a girl who was upset she left her prescription anti-diarrhea medication at home even though it provided little relief.

Spending time with Tylyn in Florida made me realize how much Ty used the bathroom. We had flown in on Friday and visited Ft. Lauderdale, Miami, The Everglades, The Keyes and were going to leave on Monday, Memorial Day. Sunday night as we laid in separate beds getting ready to fall asleep I listened as she complained about stomach pains and how bad she felt. Exhausted she concluded it was because she was now weaning off the steroids and therefore body wasn’t receiving as much of a dose.

Tylyn fell asleep while I laid awake thinking of the 1000mg of Tylenol she had donned to get her daily headache to go away. She wasn’t happy and I couldn’t fix it. I felt like a failure as parent and as we flew home on Monday and Tylyn visited the restroom during our three hour flight I realized I thought once we had a diagnosis it would change everything; the way she felt, how she was treated for the Eosinophiliac Esophagitis and Gastroenteritis and hopefully a change in lifestyle for her.

I was wrong.

June 2011

When we returned home I called the allergist and relayed that Tylyn was not feeling better but now was having a lot of joint pain. The message was relayed to the doctor who relayed back. ‘Why is she still on the prednisone?’ With irritation I replied, “Because she is only at day 10 and she is weaning off until day 14?” The nurse revealed that the doctor was booked for two weeks. If Tylyn was having problems, they suggested she could go to her PCP.

Infuriated I called the GI doctor. He patiently told me his thoughts of the course of action I should take. Wean her of the Prednisone and the Entrocourt as there was no change. I mentioned Denver and Dr. Furuta again. I also threatened to seek out a second opinion with Dr. Scagnelli that was still practicing. In fact I had already booked an appointment with him.

I was tired, exhausted and drained at being my daughter’s supporter, advocate and seemingly the only person who could look at the data objectively.

The GI surprised me and supported my decision to seek another opinion. He told me that because Eosinophiliac Esophagitis and Gastroenteritis was so rare, if this was his child he would take them to the Eosinophiliac Clinic in Cincinnati or Denver. He did not discourage me from seeing Dr. Scagnelli who had also treated this disease, he just suggested going to the best place due to its rarity. He also empathized with my frustration of empty promises by the allergist with no resolution.

I called Dan and discussed. After agreement I called both facilities. Cinncinati’s recording stated they worked on a volunteer basis and would return our call when they could. To date I have not heard from them, it has been three weeks.

Children’s Hospital (CH) in Denver called back that day. I talked in length to Kim, someone from the program. She was patient and understanding. She offered to pass my information onto the program coordinator, Joann.

Needless to say they have been true to their words. Kept deadlines, offer information and discuss their program. It took less than one week for Tylyn to be accepted, which included having all her records transferred from the five different doctors that had treated her over a three month period. 

Prior to her visit yesterday with the allergist yesterday she had been doing better since off the steroids. No joint pains, headaches are gone, weight gain subsided. The only problem abdominal pain is continues,as the diarrhea and fatigue.

But we made it miraculously through prom despite a rocky day prior. Good thing the boys baseball team won sectionals and they moved it. :) Now if we can only stay stable until our visit in Denver on October 27^th.

Above: Tylyn at Prom

Up Next: About the facility

Things Get Ugly

I had hit the snooze button this morning and had just cuddled back against my boyfriend to steal a few extra moments of sleep before heading to work when I realized yesterday I should have requested a requisition (req) for a Chemistry panel.

I had asked for a CBC slip from her PCP to check her Eosinophil levels because she has been so sick. She is off the steroids for a little over a week now and I wanted to know if they had climbed back up. I was instantly awake as I had made a mistake of not requesting a Chemistry panel as well. I wanted this mostly because of my interest in her potassium (k+) levels.

To say the least Tylyn’s conditions have made me a bit paranoid.

Symptoms of low potassium are:

Weakness or Muscle Cramping

Tiredness

Tingling or Numbness

Abdominal Cramping

Constipation

Palpitations

Passing large amounts of urine or feeling thirsty all the time

Fainting due to low blood pressure.

Delirium, confusion or hallucinations

Granted Tylyn doesn’t exhibit any of the symptoms other than the Abdominal Cramping & Tiredness but then again they were the only symptoms she exhibited the night she was in the ER. But I guess I’m getting ahead of myself now, perhaps I should start where our nightmare REALLY began.

March 2011

I love March. It’s the end of winter and of course Tylyn’s birthday. I was really looking forward to her birthday even though last year was the iconic sweet sixteen. My ex husband and I had separated on that day, so it wasn’t held to the highest esteem within our family. In addition she was almost recovered from the bad sprain during basketball season that landed her in the ER.

Above: Tylyn in the ER at MMH after spraining her ankle at a BB game

This year however we had managed to wade through several bouts with the flu and purchased her prom dress. My family came up two day’s prior to her birthday on Saturday, March 5^th. As we ate, laughed and played a fun board game, attention shifted to Tylyn’s prom gown. Due to my family being out of town we all piled in my parents van and went to the shop so she could showcase her dress.

After a brief stop there we hit a couple of stores and Tylyn expressed not feeling well. She stated she had to use the restroom with her usual post eating ritual. When my mother delayed being in a store Tylyn vomited in the car. We returned home, her going upstairs and leaving me to clean up the vehicle with my dad before my family having to return home in it. 

She was sick the rest of the night. Pale and violent intervals of vomiting and diarrhea. Sunday she was still ill, no appetite but the sickness had subsided. Diarrhea was unchanged. I restocked our supply of Gatorade which was normally reserved for her sporting events.

-Monday she tried to eat something but again became violently ill. She stuck with Gatorade.

-Tuesday Tylyn watched TV and ate nothing, only trying to keep Gatorade down.

-Wednesday she felt great, stayed home and ate little, keeping goldfish and Gatorade down.

-Thursday she went to school and ate a little, feeling the best she had in days.We went out for dinner that night and she picked at a  couple of fries with gravy, some cottage cheese and sherbet. She was happy, REAL food.

Above: The day she ended up in the ER

After we came home I was raking the front yard and she came out on the porch telling me she had thrown up her dinner. She proceeded to tell me she was going to bed. It was 6pm. Not uncommon for the last few days.

Some time later I was in my office and I received a call from her. She complained of stomach pains and needed me to come upstairs. A quick assessment of her made my heart go in my throat. I went down and let Dan know that I needed to take her to the ER. I was thoroughly convinced she was having a gall bladder attack. In her words she was having stabbing pain under her rib in her upper right quadrant. Off to the hospital we went with her fading in and out of consciousness. I took her to our rural hospital due to the severity of her sickness as opposed to taking her to Strong or Children’s in either Rochester or Buffalo.

At MMH the night quickly became agitated as I tried to sign her in and Tylyn proceeded to throw up in the garbage can. It was flu season and evidently I was an annoying over paranoid mother. The nurse became more agitated when Tylyn wouldn’t respond to her questions, her eyes rolling back in her head. As a photographer and someone that had worked in the medical field, I snapped some pictures just in case she took a turn for the worse and nobody did anything. Later that night I told the nurse her fingers were blue and as they walked away they said over their shoulder “Perhaps she’s cold.” 

Above: Ty sick in the ER

Above: Tylyn's Blue thumb and nail beds

 It was three hours before we were seen. Five before we had blood results. Her potassium had bottomed out. They needed to give her IV fluids of 30MEq. Dan stayed with her while I ran home to get some sleep sometime after 1pm. I had to work the next day and was quite irritated when I found out they released her without her follow up labs.

After some hassle on Friday I managed to get my hands on her lab work. Everything was all over the place. She had bilirubin in her urine (not acceptable in any quantity) and had some immature WBC’s. They were only slightly higher but was a bit alarming as there were promyleocytes, metamyleocytes and myleocytes coming right out the bone marrow.

I know that sounds like a foreign language to most people but luckily for me my degree was in Medical Laboratory Technology. The three types of myleocytes in her blood are normally found in the bone marrow and mature there prior to being released in the blood. An increased count warrants a retest or at least a notation.

Results in hand I confronted her PCP who brushed me off. He said the more you had the better. I looked at him incredulously wondering if I should have continued my education to even begin to receive adequate treatment for myself or my family. He said that this is the first time he had heard of Tylyn’s diarrhea problem even though it was her regular doctor’s office.

The next morning the PCP’s office called and said the doctor had reviewed Ty’s charts and we needed to come in that morning, free of charge of course.

That visit revealed that he wanted to test Tylyn for Chrons and gallbladder disease. I had a bit of satisfaction at the look of concern on the doctor’s face when Tylyn revealed she hadn’t had a normal stool in over a year. It seemed someone finally took our “complaining” serious. We rejoiced even though he suggested seeing the GI whom we had already set an appointment with on Tuesday. The PCP said if the GI didn’t order the Barium enema, Sonogram or HIDA he would. Relief flooded us. An inch of progress and surely the knowledgeable GI doctor out of a prominent Gastro group in Rochester would agree that this was gallbladder issues. She had all the symptoms of GB disease and only one of Chrons which would be diarrhea.

Tuesday finally arrived, only to be a disappointment. The doctor ignored the lab work I brought with me, discounting any data that it presented. He interviewed Tylyn about sexual or mental abuse, suggested a sonogram and an endoscopy but refused to rule out Chrons or do a HIDA.

What was his focus in our 15 minute consultation? Whether Tylyn was emotionally distraught over a recent break up with a boyfriend or did she know what irritable bowel syndrome (IBS) was? He handed us information about the south beach diet and suggested an allergist. Through gritted teeth I informed him that she already had an appointment on Friday for an allergist and that she couldn’t have IBS if she didn’t eat anything. She had lost 13 pounds in 10 days at this point. Prescription anti-diarrhea medication did not even touch her condition.

He had nothing to say to her response that she had 4-5 BM on a good day, 15-20 on a bad day.Nor did he comment about if it was IBS or Chrons she should receive relief when she wasn't eating.

We left the doctor’s office a bit stressed and went to get something to eat. Ty ate off the south beach diet menu. We headed back home and Ty vomited up her entire breakfast. Like a trooper she wanted to get her blood work done that was being repeated to check her K+ levels. I scheduled the Barium enema for the next day due to her being mostly cleaned out from the constant diarrhea.

Wednesday the 16^th she underwent that procedure and missed another day of school. The results would later reveal no Chrons.

She attended school on that Thursday, the first day in week. Barely enough to keep up with two college level classes and three AP classes. Her course load was heavy- PreCalc, Spanish IV, AP English, AP Bio & AP US History. Her grades would suffer more before this was all over.

Friday revealed a slight skin allergy positive result to Milk. The allergist ordered an IgE serum test for milk and Anti- Islet Antibody test along with another antibody blood test. We planned on doing these on Saturday but it was moved to Monday after the PCP called us to tell us Tylyn had abnormal blood results. They needed to repeat testing. Her Eosinophils were 9% with the immature WBC’s still present.

Monday we had all her blood work done. The results were accidentally sent to the allergist which in turn delayed the PCP to revealing them to us. When they finally did we were referred to an Hematologist/Oncologist. The PCP who dismissed my concerns the first day after her being in the ER had no problem telling me over the phone they wanted to rule out Leukemia. I did not tell Tylyn but rather coordinated questions through my sister who works as a Counselor in an end of life clinic for cancer patients.

A little relief came from my sister’s boss and coworkers; doctors in the profession who felt that it wasn’t leukemia but rather a secondary reaction to something else that was going on in her body.

The Hematologist/Oncologist ordered repeat testing along with a RA Factor (for rheumatoid arthritis), ANA (anti nuclear antibody for Lupus and other autoimmune diseases) and another Celiac test.

Blood work was finally normal other than the ANA coming back slightly positive. To date no one has paid attention to this result.

Also tested this month:

Stool Samples- for Ovum & Parasites, C-Diff and Campylbacter

Multiple Blood Tests

Up Next: The Testing Continues

The First Appearance of Symptoms

As I write this my mind is not fully on my words. It’s about the pending insurance request for Tylyn to be seen by an out of state doctor. I had to call the PCP about this request this morning. It has been stalled for a week because someone let it slip through the cracks. “Bullshit” was the exact words that the nursing staff used when they discovered it, incomplete and unsubmitted.

Time is the essence now; a request could take days, weeks or months. We are in limbo to buy tickets for our upcoming trip to Denver in October. Waiting to see the staff at Children Hospital and National Jewish for Tylyn’s conditions is requiring more patience than I can muster today. My Monday was spent connecting with folks at both sites to coordinate our visit, along with the financial advisers who firmly let me know. “Her insurance is not covered.” Yes I knew there was a referral needed. Yes I had requested it from my PCP. Yes I realized I would be responsible for full payment. A big sigh and a heap of more things to my plate, hence the follow up with the agitated nurse this morning.  

Yesterday was also filled with additional calls to Ty’s local doctors: the GI and the allergist. An early morning appointment with the Nurse practitioner at the allergist revealed a prescription for a sinus infection, another one for nasal spray, a change in course from Allegra™ to Zyrtec™ and some lifestyle changes, like no nightly snuggles with the puppies. I’m anticipating some additional directions in the future. We will also be starting a daily log of symptoms and overall feelings. The GI will see us the 6^th of July to the discuss results of the test that have been ordered by CH in Denver prior to her visit.

But I have digressed; this is only our current circumstances. In the beginning it wasn’t always like this. In fact mostly doctors ignored any concerns that I had regarding how Tylyn felt.

Sometime in 2005 Tylyn started reacting oddly to food. I say that with 100% certainty because when a child eats something and immediately has violent diarrhea I don't sincerely think that it could be related to anything else other than the food, especially when that particular food is avoided symptoms disappear.

It all started with Burger King™. We would meet Tylyn’s father half way between Binghamton and Rochester for Ty to visit for a few days, week or a summer block. Usually this entailed catching a quick bite either with us or her father’s family. At first I associated it with how quickly she ate. She could make swift work of her food before I had even seasoned mine. Even though that habit was unchanged from when she was smaller I scolded her to slow down.

Soon it became clear Burger King™ was a culprit no matter how slowly she ate. Soon to follow was Pizza Hut™. The latter devastated her because she LOVED pizza. Pepto Bismol™ began to make a more frequent appearance in our household, appearing on the counter, in the fridge and an occasional road trip. We avoided certain fast food joints and stumbled through these uncomfortable embarrassments of stops only minutes after just getting back on the highway.

In 2006, Tylyn had the pleasure of joining my parents, my sister and her children for a road trip to Florida. There was a phone call to me later on why I hadn’t warned them of the Burger King™ syndrome. Tylyn had just about cleared out the car. I realized that Tylyn was too embarrassed to tell anyone that eating food made her sick. Even as adults we hate discussing any uncomfortable facts about the posterior let alone being a preteen girl.

So we muddled through the next couple of years. Tylyn’s only steady boyfriend was the pink Pepto™ bottle that accompanied her to amusement parks, sleepovers and smuggled into her school locker.  She would occasionally get sick at friends houses after eating so she made an effort to get through everyday things we take for granted. Regular visits to the doctor would downplay any of our concerns.

In 2009 I started a new job and made friends with a woman named Sandy. As we complained about our jobs and discussed relationships, work and children, Sandy empathized with my frustration with Tylyn being sick. She shared that her son had also had many of the same symptoms as Ty; abdominal pain, nausea and diarrhea. Sandy recounted the ordeal she underwent getting her son diagnosed with gall bladder problems. Many tests, doctor’s visits and ultimately two surgeries later to remove his appendix and gall bladder at 16 reiterated that Tylyn’s symptoms were not normal and I shouldn’t let anyone discount them.

Back to the doctor we went. She had blood work done and they ruled out Celiac’s. All other blood work was normal so it was not pursued no matter how persistent I was. So we stocked up on Pepto™, avoided certain locations to eat and concentrated on sports and other things.

In 2010 Tylyn was very ill in school and wasn’t allowed to play a basketball game. She was very angry that the principal would not let her play even though the coach and athletic director wanted her to. The school’s reasoning? She had diarrhea. No amount of explaining to them would budge the decision despite my relaying that Tylyn frequently had diarrhea.

Later that night I came down with the flu and chalked it up to she really had been sick and I was being a stubborn parent and should have listened. If I only knew then what I know now.

Our highlight of her sophomore year was being inducted to Honor Society.

Above: Tylyn and I the night of her Honor Society Induction Spring of 2010

Up next: Things get ugly.

Years 2-10 The quieter ones

Once a child gets past the terrible two’s most parents breathe a sigh of relief. Children are sleeping through the night and they have given up on the “NO!” word that is a response to even the slightest parental request. In addition they have begun to find themselves and have created countless of imaginary scenarios everything from friend’s to the boogeyman under the bed.

After being weaned off the bottle at 20 months, Tylyn said goodbye to her formula at around two years. Dr. Scagnelli was pleased that the times in between visits became longer as ear infections faded and she gradually began her solid diet. There still was the occasional doctor’s visit that required extra testing.

The first one was the blood work, followed by a bone scan. Tylyn wasn’t growing very fast. They did a sonogram of her ovaries to see if she was either under or over developed. The bone scan revealed her body was growing just as it wanted to. Other than these occasional visits she progressed wonderfully. She was happy and adjusted despite her father and me separating. She was a little small still, giving way to my new nickname for her “Tiny Tylyn”.

In 1997 I relocated to Rochester. During the transition Tylyn lived with her dad. On one particular visit in the spring of 1998 Ty had wanted to show me how “big girl” she was by washing her own hair. She did a great job, completing the getting ready by herself and combing her long blonde hair.

Determined to continue with her usual independent way and assist with making pancakes, she dragged over a stool to the stove. While flipping a pancake I put my hand on her shoulder, causing her to cry out. An examination reveal bubbled and beet read skin along her neck, hairline and shoulder I had touched.  I trip to the doctor confirmed what it appeared to be- a chemical burn. The culprit? Flex shampoo that she had used that morning. That was the beginning of many years of non perfumed or beauty products most of us take for granted.

In July Tylyn came to live with me permanently again.  That year she suffered from the occasional ear ache and had a nasty bout with strep throat. They were unsure if the strep had turned to scarlet fever because it seemed that she always at some point had a rash of some kind on her body. By this date Tylyn had seen four different pediatricians.

That summer she was still wearing 2T’s. As most parents know a child should be in XT’s for their age, X being 2-5. She should have been wearing 4T’s but alas they were too large. In the beginning of September of that year Tylyn woke up crying complaining that her legs were extremely painful. No amount of consultation could relieve her of the discomfort. It continued for a week. That month I kept checkmarks of her growth progress on the inside wall of her closet as I always did. By October my little girl had grown three inches and had gone through three shoes sizes.

The next few years were uneventful with only an occasional visit. She remained small for her age but not too bad. She had a love for seafood, milk, popcorn but disliked chocolate. Tylyn and I traveled to Maine and a few other places. She became independent and a self sufficient child. She visited me at my new pharmaceutical position and took up an interest in chemistry. She excelled in school and it seemed to be we were out of the clear.

I now know it was the lull before the storm.

Above: Tylyn at my take your daughter to work day at a chemistry lab that was my employer in 2000. 

Up Next: The first appearance of symptoms.



 

The first 2yrs- What it meant then and what it means now.

Tylyn was born March 7^th, 1994 after a pretty uneventful pregnancy and delivery other than a scare when they tried to induce me two days prior.  I had a reaction the medication they had used which has since been pulled from the market. She arrived at 7:45am with strawberry blonde hair. Tylyn was red, wrinkly and weighed 6lbs and 14oz even though she was two weeks late.

Now I’m not going to ramble about the euphoric feelings I had when I saw my child. As a new mother I was pretty clueless about responsibility and too naïve to be scared about what it really meant to be a parent. I was 20 years old and pretty ignorant.

Luckily for me Tylyn, or as I affectionately call her-Ty, was a great baby. She ate like a horse, gaining two pounds in the first two weeks of her life. At five weeks she was sleeping from ten until five without waking and rarely cried or fussed,  a dream baby for any parent.

As she grew older, she always seemed hungry. At two months she only weighed nine pounds. Her pediatrician advised me she was petite and still fell within the growth chart. We decided to supplement her diet with Similac™ as she was not thriving on my breast milk.

This is where it all began. Sure it seems clear to me now, but back then this one chain of events seemed to spiral out of control, leaving me lost and wanting answers.

Two subsequent feedings of Similac™  yielded the same results, passing of blood in her stool. Her pediatrician advised me she might have a milk allergy. We switched her formula to Isomil™.  Being soy based, Ty’s system seemed to process this better, but we still dealt with constant gas and throwing up. It became factual in our family not to handle her too much after a feeding as her breakfast/lunch/dinner might end up on the front of the person holding her.

Still underweight and hungry, we decided to introduce Tylyn to rice. She immediately broke out into hives on her back, arms and face. Later on ice cream would do the same. We would also learn to avoid creams, lotions, perfume or perfumed soaps.

Unhappy with my current pediatrician I took the advice of my summer Chemistry professor at the college I was attending and got a second opinion about Tylyn’s failure to thrive.

Dr. Scagnelli out of Binghamton, NY is a pediatric gastroenterologist, or for those of you without a medical background- a stomach and bowel doctor. Immediately he identified Tylyn with a milk/soy protein allergy. She was placed on Alimentum, a very nasty smelling expensive formula. I am eternally grateful for the WIC program that covered all the costs. It’s hefty price tag was $28 a can and only came in liquid form which would fill four bottles. I am also thankful to CVS pharmacy that was the only place in town other that had a regular supply.

She would continue her formula diet for the first two years of her life. She would hardly ever consume any baby foods and when she did it usually resulted in hives. She was short in stature and small. I rejoiced the day I could turn the car seat forward, by law in NY state one year or twenty pounds. Tylyn was a year when her car seat made the switch; she did not break 20 pounds until 15 months, two months after she was walking.    

 Above: Tylyn at 15 months

The early years were pretty uneventful aside from her very regular doctor’s appointments and medications for ear infections that landed her regularly in the ER because at that time there was no outpatient clinic on the weekend.

She would never cry, fail to sleep, get a fever or pull at her ear. Quite simply when she had an ear infection she got diarrhea. They claimed it was her diet but she only drank the formula. I wonder now if they were right despite the Ceclor making the ear infection and ultimately the loose stools disappear. The doctor’s treated her with the Ceclor instead of penicillin due to me and her father being allergic to penicillin. They thought as a precaution they shouldn’t introduce her to other potential allergens.

I’m not sure any of those precautions helped or would ultimately hide the trouble that would take 15 more years to discover.

Up Next: Years 2-10 The quieter ones

Introduction to Eosinophilic Esophagitis and Gastroenteritis

I’m a little surprised that my very first blog is not about the topic that consumes my life 24/7. Most people, including me, would think it would be about photography, cameras and all things wedding related. It’s actually about a love that started a long time ago, one that started with the birth of my first and only child.

I want to use this blog as a resource to help other families that are going through a similar process as us. It is a place for questions and answers, enlightenment and support and most of all information because there is little to find on the vast internet about these newly discovered diseases- eosinophiliac esophagitis and gastroenteritis.

Before I share a little background about my 17 year old daughter and why we now think her diagnosis began long before this past year of testing, I would like to help those of you that aren’t familiar with these conditions to understand what they are called, their symptoms, statistics and treatment. I say treatment because there is no cure.

The content out there about these diseases is very confusing and conflicting. I know when my daughter was going through the process of being tested and diagnosed in the spring of 2011 it was extremely overwhelming searching for answers until I stumbled across another man’s blog about his daughter. I found helpful and comforting to know the long testing (years) is not uncommon, nor is the frustration of lack of resources. As we continue to one of the few sites in the country to pursue treatment, I will keep you informed of her journey. I will best describe our experience in easy to use terms to avoid further confusion while explaining tests and related procedures.

Now without further ado here is the bread and butter about Eosinophilic Esophagitis and Gasteroenteritis.

Key Medical Terms:


Eosinophils (pronounced: ee-oh-sin-oh-phils):  

Above: A normal peripheral smear of blood containing an Eosinophil  with red blood cells.

These specialized types of white blood cells (WBC) are present in normal healthy individuals.  They are found in the blood at typical concentrations of 1-3%. As part of the immune system they assist with parasites and certain infections. They are found in areas of the brain, the lower intestinial tract, ovary, uterus, spleen, lynph nodes.

They are also associated with hay fever, asthma and other allergic reactions.

Places Eosinophils are not typically found are the lung, skin, esophagus and some other internal organs. The presence of eosinophils in these organs is associated with disease.

Eosinophilic Esophagitis (pronounced: ee-oh-sin-oh-phil-lic  ee-sapho-gi-tis)   

This is the presence of Eosinophils in the esophagus mucosal lining. A minimum of 15 eosinophils per high power field are required to make the diagnosis. The sample that is analyzed to determine the concentration is collected during an Endoscopy via a biopsy.

Eosinophilic Esophagitis, or more commonly called EE, is newly discovered, only in the past four years. Complications include food getting stuck in the esophagus due to restrictions caused by rings or furrows because of  changes in the esophagus. Symptoms besides food impactation are heartburn and difficulty swallowing.

Treatment of EE may include cortisteroids, esophagus stretching, food restrictions due to allergies or the implementation of a feeding tube in severe cases. However, there are a small percentage of patients that do not have any food allergies. Most treatments are tailored specifically to the patient.

Although mostly in children, it can be found in adults and is being diagnosed more commonly and can be mistaken for GERD (Gastroesophageal reflux disease) in some cases. Conflicting data states that it is predominantly white males. Occurance is 1 in 10,000.

Current studies are being done at specialty hospitals in Ohio, Pennsylvania and Colorado looking into whether this has a genetic correlation.

Eosinophilic Gastroenteritis (pronounced: ee-oh-sin-oh-phil-lic  gas-tro-enter-i-tis): This is the increased presence of Eosinophils in the gastrointestinal (GI) tract. The stomach is most commonly affected followed by the small intestine and the colon. The sample that is analyzed to determine the concentration is collected during an Endoscopy via a biopsy for the stomach  and upper small intestine. A colonoscopy is used for the lower small intestine and colon.

This disease is also newly discovered, only 300 documented medical cases since 1937. Symptoms include nausea, vomiting, diarreha, weight loss and abdominal distention..

Treatment may include corticosteroids. Typical persons affected are 30-50 year olds with slightly higher incidence in men.

So you or your child need to undergo some tests for diagnosis? These will be done and may be required as often as quarterly or yearly for some individuals to track disease progress and treatment.  

Endoscopy (also called an Upper GI study)- Typically a twilight anesthesia is given so the patient is sedated but not unconscious. This type of anesthesia allows for easy wakening and a “sleepy” state by the patient to be responsive to the doctors simple requests. Most often they use a IV to administer the drug. During the endoscopy they may do biopsies of the esophagus, stomach, and small intestine (upper). Procedure is done in an outpatient facility, normally by a GI doctor but will require someone to drive you home afterwards. No eating or drinking 8 hours before the procedure. Total test time is 15-20 minutes but leave enough time for the required thirty minutes of observation afterwards and the preparation before.

Colonoscopy- Typically general anesthesia is given so the patient is sedated. During the colonoscopy they may do biopsies of the small intestine (lower), large intestine, colon and rectum. .  Procedure is done in an outpatient facility, normally by a GI doctor but will require someone to drive you home afterwards. Laxatives are given to clear out the system the day before the test. No eating for 24 hours. Drinking is minimal 8 hours before the procedure. Total test time is approximately 30 minutes but observation afterwards and the preparation before totaling about 2-3 hours.

Blood Tests- Complete Blood Count (CBC) which is a panel of the blood that includes but is not limited to the counts of red blood cells, white blood cells and platelets. They give an exact count of the number of cells and may reveal an increase of the peripheral blood count of Eosinophils.

They may also undergo the following tests:  (my daughter did)

Barium Enema – To test for Chron’s

HIDA Scan (cholescintigraphy)- To test gallbladder

CT Scan of Abdomen- To see organs and thickening of abdomen wall

Sonogram Abdomen - To see organs and look at gall bladder

Barium Swallow Study- To watch barium pass from esophagus to intestines (Scheduled next week)

IBS Blood Panel-A new blood test from Prometheus that helps differentiate between Irritable Bowel Syndrome and other GI disorders (To be scheduled in future- needs Board approval)

Some helpful links:

NJ/PA/DE Support group for Eosinophilic Diseases


Pictures of Rings, Furrows & Biopsies


Andrew's Blog About Ruthie

The International Gastrointesinal Eosinophil Researchers (TIGERS)

Campaign Urging Research for Eosinophilic Disease (CURED)

American Partnership for Eosinophilic Disorders

Quick Facts about Eosinophil Gastrointestinal Diseases (EGID's)

Coming up Next: The first 2yrs- What it meant then and what it means now.