PWS Awareness Month- Day 15

Hey Everyone,

Thank you for taking the time to read our blog this month!  Today we will start a series about food and PWS.  Today I will talk about EoE and this week about nutrition and meal planning, and a post for family and friends.

This week is actually Eosinophilic Esophagitis Awareness week!  Kemett was diagnosed with EoE at 8 months old.  So far he has not shown any of the symptoms below but we keep an eye out for choking and food getting stuck.  There has not been a lot of research on EoE but we are happy to report that there is currently more research going on and they are finding out some great information!  If you’d like to research it further, I’ve put some resources below.  We actually know of a couple of PWS kids who have EoE as well.  Tomorrow, I will talk more about Kemett’s diet and what it looks like.

Our last scope showed he has mild EoE.  Our next scope is in June.  Kemett is also on Nexium to control it.  Below is a description about EOE from American Partnership for Eosinophilic Disorders (Apfed).

Eosinophilic esophagitis (EoE) is a chronic, allergic inflammatory disease of the esophagus (the tube connecting the mouth to the stomach). It occurs when a type of white blood cell, the eosinophil, accumulates in the esophagus and persists despite acid-blocking medicine. The elevated number of eosinophils cause injury and inflammation to the esophagus. This damage may make eating difficult or uncomfortable, potentially resulting in poor growth, chronic pain, and/or difficulty swallowing.

What are the symptoms?

Symptoms of EoE may vary from one individual to the next and may differ depending on age. Infants and toddlers often refuse their food or have trouble growing properly. School-age children may have recurring abdominal pain, trouble swallowing, or vomiting. Adolescents and adults most often have difficult or painful swallowing. Their esophagus may narrow and cause food to become stuck (impaction), causing a medical emergency.

Symptoms also may vary given the developmental ability and communication skills of the age group affected.

Common symptoms include:

Reflux that does not respond to medication (acid suppressors) – infant, child, adult

Difficulty swallowing – child, adult

Food impactions (food gets stuck in the esophagus) – older children, adult

Nausea and Vomiting – infant, child, adult

Failure to thrive (poor growth, malnutrition, or weight loss) and poor appetite – infant, child, rarely adult

Abdominal or chest pain – child, adult

Feeding refusal/intolerance or poor appetite – infant, child

Difficulty sleeping due to chest or abdominal pain, reflux, and/or nausea – infant, child, adult

What causes eosinophilic esophagitis?

While the exact cause of EoE is not yet known, the general belief is that it’s typically caused by an immune response to specific foods. Many patients with EoE have food or environmental allergies. Researchers have identified a number of genes that play a role in EoE, including a recently discovered gene, calpain14 (CAPN14), that is expressed primarily in the esophagus. These pathways may provide new direction to diagnose, monitor and treat EoE in the future.

Who is affected?

EoE is a newly recognized disease that is now increasingly diagnosed in children and adults. Eosinophilic esophagitis is a rare disease, but increasing in prevalence with an estimated 1 out of 2,000 people affected. EoE affects people of all ages and ethnic backgrounds. While both males and females may be affected, a higher incidence is seen in males. People with EoE commonly have other allergic diseases such as rhinitis, asthma, and/or eczema. Certain families may have an inherited tendency to develop EoE.

How is eosinophilic esophagitis diagnosed?

A gastroenterologist must evaluate a patient for the symptoms consistent with eosinophilic esophagitis, taking a careful history. Since EoE can mimic other conditions, more common diseases such as gastroesophageal reflux disease (GERD) must first be ruled out.

If acid blockers do not relieve symptoms (typically after 2 months of treatment), a doctor will perform an upper endoscopy. During this procedure, the patient is sedated or put under anesthesia, and a small tube called an endoscope is inserted through the mouth. The esophagus, stomach, and the first part of the small intestine are examined for tissue injury, inflammation and thickening of the esophageal wall. Small tissue samples are taken (biopsy). This procedure is typically not uncomfortable and may be done on an outpatient basis.

A pathologist will analyze the tissue samples under a high-powered microscope to see the small cell structures. If eosinophils are present in the sample, the pathologist will count how many are visible. A count of 15 or more eosinophils per high-powered microscopic field warrants a diagnosis of EoE.

A patient may have EoE even if the esophagus looks normal during the endoscopy. The biopsies will help in making an accurate diagnosis. Endoscopy with the biopsies is the only reliable method of diagnosing EoE at this time, although promising research for less invasive diagnostic and monitoring is currently underway.

How is eosinophilic esophagitis treated?

The two main treatments recommended for EoE are diet management and medication.

Diet management may include:

  • Targeted Elimination Diets- Foods that test positive on allergy testing or history are removed from the diet.
  • Empiric Six-food Elimination Diet- This type of diet has shown success in some patients. Instead of basing dietary elimination on allergy testing results, patients eliminate common allergy-causing foods (milk, eggs, wheat, soy, peanuts/tree nuts, fish/shellfish).
  • Elemental diet – All sources of protein are removed from the diet and the patient drinks only an amino acid formula. Sometimes, a feeding tube may be required.
  • Food trial – Specific foods are removed from the diet, and then added back, one at a time, to determine which food(s) cause a reaction.

Diet management involves repeat endoscopies with biopsies as foods are reintroduced to determine which foods are tolerated.

Medications may include:

  • Topical steroids – There are currently no FDA approved medicines available for the treatment of EoE. However, doctors have found that topical steroids are often successful in putting EoE into remission. Topical steroids (fluticasone or budesonide) are swallowed from an asthma inhaler or mixture to control inflammation and suppress the eosinophils. Systemic corticosteroids such as Prednisone are not used for chronic management of the disorder, but may be prescribed for acute situations and short periods of time.
  • Acid suppressors – May also help relieve reflux symptoms in some patients in combination with dietary therapy or medications.

In some situations, both medications and dietary therapy may be used together.

What is the prognosis?

Eosinophilic esophagitis is a chronic disease that requires ongoing monitoring and management. There is no cure. EoE does not appear to limit life expectancy and there is currently no strong data suggesting EoE causes cancer of the esophagus. In some patients, EoE is complicated by the development of esophageal narrowing (strictures) which may cause food to lodge in the esophagus (impaction). It can also make eating very difficult and uncomfortable for children and adults. It is not clear how long EoE has to exist before strictures form. However, since the natural history of EoE is only emerging, careful monitoring and long-term follow-up is advised.

The initial diagnosis of EoE can be overwhelming and often affects the entire family. A positive attitude and a focus on non-food activities go a long way in learning to live with EoE. With proper treatment, individuals with EoE can lead a normal life.

http://apfed.org

http://www.cincinnatichildrens.org/research/divisions/a/allergy-immunology/labs/rothenberg/default/

Please let us know if you have questions!  Thank you for your support!

Love,

The Demands

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PWS Awareness Month- Day 5

Hi Everyone,

First, we want to say happy birthday to my mom!  We love you!

Today we will be talking about GI and Allergy.

GI: We see Dr. Scheimann every 6 months at Texas Children’s Hospital in Houston.  We see her mainly for Kemett’s eosinophilic esophagitis.  So far Kemett has not had any GI issues associated with PWS.  For that we have been lucky.  Here are some updates on his EOE.  Also, EOE Awareness week is later this month- so if you have questions, please  feel free to ask!  We will do a blog post specifically about EoE in two weeks.  It is a rare condition, and many people have not heard of it before.

  • Last August, Kemett had his third scope with biopsies in his esophagus as well as an impedance probe study done.  The scope biopsies showed that his EOE is still mild and gotten a little better, which is wonderful! When they examine the biopsies they do not know if they are allergy cells or caused by reflux.
  • Impedance Probe: This was a probe that went into Kemett through his  nasal cavity, and went down into his stomach.  It had a devise that tracked reflux levels as he ate, laid down, drank liquids, and slept.  We had good news from this test, that showed Kemett did not have reflux issues.
  • Nexium: Kemett is on nexium 2 times a day.  A protein pump inhibitor is the only treatment for EoE at the moment.  Dr. Scheimann feels that if we can will be able to start weaning him off of it starting in May!  Then we would move to another med that won’t interfere with his absorption of vitamins and minerals.
  • December Visit- we saw Dr. Scheimann in December and she thought he looked good, except that he had not grown or gained weight since August or before.  This could be because he was not getting enough protein in him.  We decided to increase protein volume and come up with ways to add vitamin C when he ate protein to help absorb iron.  Then we did more blood work a few months later.  Dr. Scheimann said that she was not as concerned about his new levels.  She did further blood work and we sent that on to our allergist.  We did add in an iron supplement.
  • We see her again in June.
  • So far Kemett isn’t exhibiting any signs of EoE like food getting stuck or choking but we are on the look out at all times!

Allergy:  We see Dr. Varshney every 6 months.  We saw her in February and had blood work completed before the visit.  Unfortunately, Kemett’s levels went up on all the foods he is allergic to, so we cannot add anymore new foods in at the moment.  She said that we will not do another scope until Kemett is completely off of Nexium, so might not be this year.  So he is still allergic to:

  • Eggs
  • Soy
  • Dairy
  • Wheat
  • Peanuts
  • Tree nuts

Dr. Scheimann has given us names of doctors who specialize in EoE across the country, but until Kemett regresses or has symptoms, we will wait to visit them.

We are happy things are going the way the are with Kemett so far.  His diet is always a struggle, and making sure he is getting enough protein.  We have to be creative.  We will do a post this month on meal planning!  This way of eating has become part of our life, and I’m not sure I know any other way of having a child.  We’ve been dealing with his allergies since he was 8 months old.  We are so lucky to have family, friends and a school who are protective of Kemett and his health.  We will have a blog post on this and how others can help too!

Please let us know if you have any questions!  Thank you for you the love and support!

Love,

The Demands

 

Kemett and Mama

Kemett and Mama

 

Kemett Goes to Florida and Houston (Again!)

Hello everyone!

Two weeks ago, we took off for Florida for a family vacation and another trip to see Dr. Miller (PWS Endo).  After that, we went to Houston to see Dr. Scheimann (PWS GI).  Here is a recap of each trip.

FLORIDA:

We spent the first three days of our trip on Sand Key near Clearwater Beach.  We had a great time relaxing, going to the beach and enjoying some good cuban food and fresh seafood.  On our first day we made an afternoon trip to Dunedin, one of the oldest settlements on Florida’s West coast.  It was settled by Scottish immigrants in 19th century.  It is also home to Florida’s oldest brewery.  Saturday morning we went to Ybor City and learned about how the cigar industry grew in Florida.  We enjoyed some great cuban food and learned about all the immigrant groups that settled in this area.  Sunday, we had the pleasure of meeting two more PWS families from Clearwater!

After three days at the beach, we headed to Gainesville to see Dr. Miller.  It is always such a joy to see Dr. Miller.  She is so happy and inspiring!  She had nothing but good things to say about Kemett.  She was impressed by his strength, happy with his growth and igf-1 levels.  We will be putting Kemett on B-12 shots monthly because his levels are low.  We are also going to try him on L-carnitine.  This is a standard supplement our lovebugs take for muscle development and energy.  Kemett has a ton of energy, so we are just going to test it for 2 weeks and see if he does ok on it!  I’m worried that I will have a baby who won’t sleep for 2 weeks.   She felt good about his therapy and speech.  We will also end up working with Melanie Silverman again now that we know Kemett’s allergies (see below).

She only wants to see us once a year since we live far away.  It is always such a positive and motivating trip.  Kemett is doing so well and he works so hard!  Glad it is paying off!

HOUSTON:

Once home, Kemett and I turned around and drove to Houston to see Dr. Scheimann.  She is from Johns Hopkins, but comes to Texas Children’s Hospital every other month.  She was shocked when she saw Kemett and commented on how great he looked!  She thought his skin was perfect and his growth had been great.  I was a little nervous about this visit because through email she has been hard to read.  I let her know that Kemett is doing great with chunks of food, and she said that was a great sign that his esophagus has healed!

Kemett had a skin test done last month at his allergist’s office that shows he is potentially allergic to: Dairy, Eggs, Soy, Wheat, Corn, Beef, Seafood, Shellfish, and Peanuts.  WOW.  I will say that none of this phases me.  We will figure this out!

We will continue to work with GI and the Allergist to figure out when we can introduce allergens that come out negative in blood work.  It will be a process.  He will more than likely have another endoscopy later this year.

Everything was positive from her visit; Dr. Scheimann said that he might end up only being allergic to one or two of these things.  She let us know that little ones with Eosinophilic Esophagitis often heal well, but there is a chance of irritation again when they are little older.  We will just have to be careful with foods and work with these two doctors closely.

Another positive note is that she only wants him on formula for 6 more months!  We moved to Elecare Jr and found an unflavored kind.  We did not want him to go on vanilla flavored formula that would be full of sugar.  After that, we will make sure he has enough protein and will phase him off of it.  So excited about this!  Another reason to work with Melanie, so we can make this a reality!

OVERVIEW:

These visits were great.  They help us to continue to stay positive!  We know there will be roadblocks, there have been a ton so far, but we know we can make it through.  I mean, Kemett does it all and with a smile!  Next post- Kemett will be 1 year!

Enjoy some vacation pics below!

Love,

The Demands

 


View from room.

View from room.

 

First morning at the beach.

First morning at the beach.

 

Kemett not liking the sand.

Kemett not liking the sand.

Dunedin Brewery.

Sunrise.

Sunrise.

 

Kemett loves Museums!

Kemett loves Museums!

 

Ybor City

Ybor City

 

Mama and Kemett

Mama and Kemett

 

Frenchy's

Frenchy’s

 

We had to visit Hulk Hogans store...

We had to visit Hulk Hogan’s store…

 

Beautiful day!

Beautiful day!

 

Loving the beach!

Loving the beach!

 

Playing in the sand

Playing in the sand

 

Sand Key Beach

Sand Key Beach

 

Sand Key Beach

Sand Key Beach

 

Fun in Hotel

Fun in Hotel

 

Dr. Miller

Dr. Miller

 

Flight home.

Flight home.












Kemett is 9 Months (on May 25th)

Hi Everyone,

As you can see I’m almost ONE whole month late for this post!  Kemett turns 10 months this week, so I hope to get another post out soon!

This month has been a big one for Kemett.  He is getting so big and looking so grown up!  Here are some highlights:

  • He is rolling all over the place to get what he needs and wants!
  • He can sit supported on his own.
  • We are working on building strength for crawling.
  • We removed eggs from my diet and changed his formula again.
  • He had an endoscopy and flex-sigmoidoscopy with biopsies- he did great!
  • He got teeth 5 and 6.
  • He got his first professional haircut!  (it was haircut number 2 if you are counting!)
  • He is eating chunks/finger foods and can feed himself.  He holds spoon and can put in his mouth.
  • I stopped pumping breast milk because of the diet restrictions and his allergies, so he had his last bottle of breast milk.  It was bitter sweet.
  • Kemett celebrated Grandma’s birthday
  • He swam for the first time!
  • Kemett is 28 inches long and is 16 lbs 10 oz

We are working with a GI doctor and allergist to figure out what is going on.  The biopsies revealed that he has eosinophilic cells in his lower esophagus, as well as reflux.  Now we have to figure out with an allergist what is causing these allergy cells.  He has been on a restricted diet for almost 2 months now and Kemett has had a hard time gaining weight.  I look forward to him getting more food added in and gaining weight.

One good thing is that we saw the Cranio Facial plastic surgeon and he thinks Kemett looks good.  He is holding his head up on his own and not compromising his airway.   He does not want to see us for another year and does not feel that he will need a jaw distraction surgery!  It was such good news to be able to take one more thing and doctor off our list!

Below are some pictures from this past month!

 

Love,

The Demands

 

9 Months!

9 Months!

9 Months!

9 Months!

Look at those eyes!

Look at those eyes!

My first professional haircut!

My first professional haircut!

Mama Fred Fest

Mama Fred Fest

Before endoscopy

Before endoscopy

Exersaucer

Exersaucer

I love GInger!

I love Ginger!