PWS Awareness Month- Day 7

Hey Everyone,

Today, we will be giving you an update on medications that Kemett takes!

Morning:

  • L-Carnitine– This medication is used to give our kids energy and to build lean muscle mass. This year we took him off of it for a month to see if he really needed it because he is pretty energetic, and he was sleeping until 8 am and falling asleep in the afternoons (ok maybe that’s not a bad thing for a kid who doesn’t nap!), so we put him back on it.
  • CoQ10- Also used for energy.
  • Nexium– Reflux (Kemett has Eosinophilic Esophagitis- another rare disease that has nothing to do with PWS).  Has to be giving 20-30 minutes before a meal.  We give this to him 1x a day and are waiting for scope results.
  • MCT Oil– Helps with focus, developing the brain, appetite and building lean muscle mass.
  • Calcium Supplement
  • Culturelle Pro-biotic– Kids with PWS have major GI issues.  Because Kemett is on Nexium twice a day, he needs the probiotic to help keep his gut healthy.
  • Nasonex– This is keeping fluid out of his ears, prescribed by ENT.
  • Qvar Inhaler-Kemett has allergy induced asthma.  This has been amazing in stopping a lot of illness- we are off from now until right before school starts.
  • Iron Supplement
  • Multivitamin
  • Antibiotic cream– in nose to prevent nosebleeds.  He’s has a few this winter. 2X a day.

Evening:

  • Qvar Inhaler- Again we are off of this right now.
  • Singulair- Prescribed by our Pulmonologist.  This has helped with any inflammation in Kemett’s nasal cavity and ear canals.
  • Zyrtec- Allergies

Before Bed: 

  • Growth Hormone: His dosage to .6 mg.  He gets an injection each night.

As Needed:

  • Albuterol- Nebulizer/Inhaler
  • B-12- 2 X a Month Shots- Kemett is B-12 deficient.  This also helps with energy levels.
  • EpiPen
  • Pulse Oximeter- Just spot check one because insurance denied coverage on his pulse ox.
  • Desonide Cream– For his eczema

Everything he takes has been approved by one or multiple doctors.  We make sure everyone is aware of what is going on and why he is getting them.  I also keep a sheet with all the meds and doses listed out.  Things change quite frequently, so I want to make sure everyone is on board.

Let us know if you have any questions!  Thanks for your support!

Love,

The Demands

 

IMG_4960

Kemett’s Medications and Supplements

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PWS Awareness Month- Day 1

Hi Everyone,

Welcome back to Prader-Willi Awareness Month!  We want to thank everyone ahead of time for taking the time to read our blog, ask questions and just learn about Kemett.

It has been a great year so far with a lot of progress being made.  I cannot wait to share it all with you.

First I wanted to give you all a quick refresher on PWS.  Here is a great picture that describes the genetics.

PWS Genetics

Handout from PWSA

________________________________________________________________________________

What are the symptoms of Prader-Willi syndrome?

The symptoms of Prader-Willi syndrome are likely due to dysfunction of a portion of the brain called the hypothalamus. The hypothalamus is a small endocrine organ at the base of the brain that plays a crucial role in many bodily functions, including regulating hunger and satiety, body temperature, pain, sleep-wake balance, fluid balance, emotions, and fertility. Although hypothalamic dysfunction is believed to lead to the symptoms of PWS, it is not yet clear how the genetic abnormality causes hypothalamic dysfunction.

The symptoms of PWS change over time in individuals with PWS, and a detailed understanding of the nutritional stages of PWS has been published.  Overall, there are two general stages of the symptoms associated with PWS:

Early Life

Infants with PWS are hypotonic or “floppy”, with very low muscle tone. A weak cry and a poor suck reflex are typical. Babies with PWS usually are unable to breastfeed and frequently require tube feeding. These infants may suffer from “failure to thrive” if feeding difficulties are not carefully monitored and treated. As these babies grow older, strength and muscle tone generally improve.  Motor milestones are achieved, but are usually delayed.  Toddlers typically enter a period where they may begin to gain weight easily, prior to having a heightened interest in food.

Childhood and Beyond

An unregulated appetite and easy weight gain characterize the later stages of PWS. These features most commonly begin between ages 3 and 8 years old, but are variable in onset and intensity.  Individuals with PWS lack normal hunger and satiety cues. They usually are not able to control their food intake and will overeat if not closely monitored. Food seeking behaviors are very common. In addition, the metabolic rate of persons with PWS is lower than normal. Left untreated, this combination of problems leads to morbid obesity and its many complications.

In addition to obesity, a variety of other symptoms can be associated with Prader-Willi syndrome. Individuals usually exhibit cognitive challenges, with measured IQs ranging from low normal to moderate intellectual disability. Those with normal IQs usually exhibit learning disabilities. Other issues may include growth hormone deficiency/short stature, small hands and feet, scoliosis, sleep disturbances with excessive daytime sleepiness, high pain threshold, speech apraxia/dyspraxia, and infertility. Behavioral difficulties may include obsessive-compulsive symptoms, skin picking, and difficulty controlling emotions. Adults with PWS are at increased risk for mental illness. PWS is a spectrum disorder and symptoms vary in severity and occurrence among individuals.

Information taken from FPWR.org.

Let us know if you have any questions during this month.  We are very open and honest about our lives with Kemett!

See you tomorrow!

Love,

The Demands

PWS Awareness Month- Day 7

Hey Everyone,

Today, we will be giving you an update on medications that Kemett takes!

Morning:

  • L-Carnitine– This medication is used to give our kids energy and to build lean muscle mass.
  • CoQ10- Also used for energy.
  • Nexium– Reflux (Kemett has Eosinophilic Esophagitis- another rare disease that has nothing to do with PWS).  Has to be giving 20-30 minutes before a meal.  Hope to go down to 1X a day later this month!
  • MCT Oil– Helps with focus, developing the brain, appetite and building lean muscle mass.
  • Calcium Supplement
  • Culturelle Pro-biotic– Kids with PWS have major GI issues.  Because Kemett is on Nexium twice a day, he needs the probiotic to help keep his gut healthy.
  • Nasonex– This is keeping fluid out of his ears, prescribed by ENT.
  • Qvar Inhaler-Kemett has allergy induced asthma.  This has prevented us from heading to the ER for major resporitory issues.  Kemett started Qvar last August after having retractions while breathing and having to go to the ER.  We get to stop Qvar for the summer as long as Kemett stays healthy.  Fingers crossed!
  • Iron Supplement

Mid-Afternoon

  • More MCT Oil
  • Fish Oil as needed

Evening:

  • Qvar Inhaler
  • Singulair- Prescribed by our Pulmonologist.  This has helped with any inflammation in Kemett’s nasal cavity and ear canals.
  • Nexium
  • Zyrtec- Allergies

Before Bed: 

  • Growth Hormone- In January we increased his dosage to .6 mg.  He gets an injection each night.

As Needed:

  • Albuterol- Nebulizer/Inhaler
  • B-12- 2 X a Month Shots- Kemett is B-12 deficient.  This also helps with energy levels.
  • EpiPen
  • Pulse Oximeter
  • Desonide Cream– For his eczema

Over winter, we had to do a skin test for our allergist and went off of all allergy meds for 7 days.  Kemett developed a runny nose, cold and low grade fever pretty quickly.  As soon as we got him back on the meds it all went away.  I’m sure to some this may seem excessive, but we must be preventative and cannot let him get so sick that he ends up in the ER.
Everything he takes has been approved by one or multiple doctors.  We make sure everyone is aware of what is going on and why he is getting them.  I also keep a sheet with all the meds and doses listed out.  Things change quite frequently, so I want to make sure everyone is on board.

Let us know if you have any questions!  Thanks for your support!

Love,

The Demands

 

IMG_4960

Kemett’s Medications and Supplements

PWS Awareness Month- Day 6

Hey Everyone,

Today we will be talking about PWS and dental care.  Kemett see’s an oral surgeon every 6 months since he was 12 months old.  We see Dr. Jacobs because Kemett has micrognathia (small jaw).  

Kids with PWS need to have consistent dental care because:

Most dental problems with PWS are related to decrease salivary flow and mouth breathing. These two concerns combined promote tooth decay, periodontal disease and crowded arches.  Persons with PWS also have a tendency towards dry mouth because of their mouth breathing which leads to thicker saliva, which promotes both decay and periodontal disease. Normal saliva is thin and washes the teeth clean, while thick saliva sticks to the teeth and harbors bacteria that cause tooth decay and periodontal disease. Finally, as a result of mouth breathing during their early years, individuals with PWS have a greater chance of developing narrow arches which causes crowded teeth. Getting orthodontic care early (age 6-9) to help develop the arches to a more normal shape can make it easier to keep the teeth clean, which helps prevent tooth decay and periodontal disease. It can also help reduce their tendency to mouth breathe, which would decrease tooth decay and periodontal disease. Professional dental cleanings every 3 to 6 months is also a big help in keeping the teeth and gums clean and healthy.

What can be done? First, make sure the child or adult has either a fluoridated water supply or fluoride tablets (1 mg /day to help fight tooth decay.) Have them brush with a fluoride toothpaste after each meal and then before bedtime. Parents will need to check their teeth after brushing, especially at night so that they don’t leave food along their gum lines. This leads to decay and periodontal disease.

So far, Kemett’s teeth have looked great.  He does not have overly thick saliva and we brush and floss his teeth twice a day.  He got all of his teeth in early and already  has his 3 year molars.  We have talked about sealants, but will not do anything about it until he is going under for another procedure.    I would say he does not drink enough water though!

If you have any questions, please let us know!  Thank you for your support!

Love,

The Demands

Kemett

Kemett

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

 

PWS Awareness Month- Day 4

Hi Everyone,

Today we will talk about ophthalmology.  We see Dr. Busse every 6 months to check on Kemett’s eyes.  We starting going when Kemett was 12 months old.

Kids with PWS often have Strabismus:

Perhaps 3% of the general population have strabismus, or squint, but it occurs much more frequently in congenital conditions that involve chromosome abnormality. The strabismus might be constant or, less commonly, intermittent. One eye may consistently be the straight one, or the eye which is the straight one may swap from one to the other (alternating). About 60% of children with Prader-Willi syndrome have strabismus, which is present at birth or occurs soon after. Almost all squints in PWS are of the type called esotropia (crossed eyes). When one eye looks straight ahead, the other eye turns inward towards the nose. Exotropia (wall eyes), in which the eye not looking straight ahead turns outward away from the nose, is much less common as an original fault in PWS. However, it is a feature in some teenagers and adults as a later result of the surgical correction of esotropia due to ‘post-operative drift’. The development of strabismus at an early age in PWS could be due to the hypotonia (weak muscle tone) present at birth. Another factor could be a restriction of eye muscle movement caused by the reduced space available in the eye socket, which is associated with the narrow face and almond shaped eyes seen in many people with PWS. – PWSA-UK

We saw Dr. Busse in August 2015 for our second annual check up.  We had noticed that Kemett seemed to go crosseyed but didn’t track how often it happened or what eye.  So Dr. Busse said we should come back in 6 months.  We went back in March for a follow up.  We had tracked his eye movement and noticed his right eye goes inward but only when he is tired, sick or looking at something up close.  It goes back to normal pretty quickly.  At the moment, the only treatment would be to get Kemett glasses.  The doctor says that once we do that, we cannot go back.  So we will see Dr. Busse in 6 more months unless there is change.  Our hope is that as he gets stronger, so will his eyes.  Dr. Busse also feels that if we see his eye going like this daily and at least 30% of the time, then we will need to talk more about glasses.

Right now it’s a wait and see game.  We will go back in October.

Please feel free to ask any questions!  Thank you for your support!

Love,

The Demands

 

Kemett!

Kemett!

PWS Awareness Month- Day 3

Hey Everyone,

Today we are going to continue our updates on Kemett’s doctors.  Today we will talk about Pulmonology, ENT and Cranial Facial Plastic Surgery.   (To catch up from last year, here is the link from our blog last year https://lovethedemands.wordpress.com/2015/05/08/pws-awareness-month-day-8/).

Pulmonology: We still see Dr. Kang every 4 months for Kemett’s apnea and asthma.  We absolutely love he and his team.  Kemett has his 4th sleep study in March and it came back with mild sleep apnea.  There was not much change from last year’s sleep study.  At least it did not get worse but wish it would continue to get better.  Kemett is still on the same meds for his asthma as before and we are keeping them under control.  We actually had a good winter with no sickness, just some allergies.

ENT:  We see Dr. Zapata every 6 months to check on his tonsils and adenoids.  We saw her two weeks after our sleep study, and his tonsils and adenoids are still small.  So that is good news.  We just keep monitoring them for now.  At any point they could get big and he would need them removed.  So we listen for snoring at night, look for daytime sleepiness, etc.  If we see signs of this, we get him back to ENT and pulmonology before our appointment times.

Cranial Facial Plastic Surgery: We don’t see Dr. Kelly until May 9th, and will be able to give you all a small update at that point.

This team is so important to us because if we didn’t have Kemett monitored for his breathing, respiratory function and tonsils/ adenoids Kemett could die being on growth hormones at the same time.

If you have any questions, please feel free to ask us anything!  Thank you for your support!

Love,

The Demands

 

Strong and Smart

Strong and Smart