Monday, October 15, 2018

Hypertension In a Child


In previous blog posts I have written about Ellie's high blood pressure.  I have an update er, well updates.

According to the new pediatric guidelines, for Ellie's age, height %tile, and gender, her systolic blood pressure [top # of BP] should fall around 111 mmHg.  Ellie's average without blood pressure medication is 124 in the LEFT arm only and normal in the right arm.

High blood pressure in a child is usually due to cardiac issues such as narrowing of the aorta (a large artery that branches off the heart) or renal [kidney] issues.  However, with the increasing rates of childhood obesity, doctors are seeing more and more hypertension related to weight, similar to adults.

Flashback: October is Down Syndrome Awareness Month


In August, we were able to rule out all cardiac issues so that is not the cause of her hypertension.  We are currently evaluating her kidney function.  Many doctors start off with a basic metabolic panel that looks at things such as glucose, sodium, potassium, etc, but the main values that a physician looks at from that lab study is BUN and Creatinine.  I won't get into what those things are for, but high levels of BUN and Creatine can be a sign of impaired kidney function.  Ellie's were normal.  Other, more in-depth testing include assessing Renin, Aldosterone, and Angiotensin - again, I will spare you the details of their function.  Ellie's Renin levels are high.  Renin is an enzyme that is secreted by the kidney that helps regulate blood pressure. One of the causes of high renin levels is renal artery stenosis, which is is the narrowing of the artery that supplies blood flow to one or both of the kidneys.  This is something that is very rare with systolic BP usually above 170 mmHG and it is highly unlikely that Ellie has this . . . BUT, as the nephrologist said "this is Ellie".  Plus, she has been on clonidine for years and she is also on a calcium channel blocker BP med so we don't really know how high it would be without these medications. At this point, we are working on scheduling a CT with contrast (angiogram) or her kidneys, but it is not considered urgent.

Flashback: October is Down Syndrome Awareness Month


Hypertension in a child is typically diagnosed when the systolic reading is 10 mmHG above the 95% tile for age, height, and gender.  First line treatment for high blood pressure in a child is a calcium channel blocker - i.e. amlodipine. (Calcium Channel blockers tend to end in "ipine.)  A few months ago we started Ellie on Norvasc.  We some mild improvement in her blood pressure.  The nephrologist says that kids typically end up on higher doses than adults due to their higher metabolic rates so he bumped up her dose.

I don't know what happened and I don't understand it.  I measure Ellie's blood pressure with an automated cuff. This is the same cuff that I have been using for months.  After we increased her Norvasc, I noticed that her systolic readings were back into the mid-120s.  A few weekends ago, I took her BP and it was in the 130s.  I waited a few hours and she was 140 mmHg.  I didn't believe it.  I switched out the cuff to an adult one and took mine.  The I compared it to my Qardio cuff that I use with an app.  Both of mine were the same.  So . . . Ellie's reading was accurate.  I waited an hour and she was 147 mm HG.  Note that she had both clonidine and the higher dosage of Norvasc in her system.  A BP that high in a child is dangerous.  I had to call nephrologist on-call who told me to immediately give her clonidine (it is pretty fast acting) and that if it was still in the 140s in a few hours, she'd have to receive IV anti-hypertensives in the hospital.  It was odd.  I don't know why this happened, but the clonidine did work.

Uh huh . . . how long do you think she'll keep that on?  The eye patch is over her good eye.  The thought is the eye that turns in will correct itself.  We've tried glasses and then keeping her good eye dilated with no change (okay, she refused the glasses).  So this is our last ditch effort.

A few days later, the nephrologist upped her dosage of Norvasc again and it seems to be working.  Her BP has been averaging 110/60 so we are pretty happy with that.  Her renal CT scan with contrast still isn't scheduled as we are trying to do it the same day as her ABR and cecostomy tube replacement.  I hate waiting, but I only want to put her under sedation once.

The blood pressure machine that I use for Ellie is by Contec.  It is no longer sold on Amazon, but eBay has it and a few online medical supply sell it.  I have tried several automatic and manual cuffs, but this machine is the ONLY one that has a TRUE pediatric BP cuff.  Most the ones marketed as pedi are actually infant cuffs.  A blood pressure cuff isn't just about length to wrap around the arm.  It is about width.  It is supposed to cover a specific portion on the upper arm.  If the cuff isn't wide enough, the pressure will read higher.  If the cuff is too wide, the pressure may read lower.



photo: uptodate.com



Some cuffs are labeled with #s.  i.e. a child cuff may be labeled as size 9 so it is good to read the measurements.

While the Norvasc is working, it comes with an unfortunate side effect.  For Ellie, it is significantly making her constipation worse (it may cause the opposite problem in some people).  She used to get 250 mL of saline enemas through her cecostomy every other day.  She is now getting 250 mL SMOG enemas [saline, mineral oil, and glycerin] through her tube 2-3 x week - the max we can do and then 2 rectal ones a week - also the max.  It is frustrating because she is still only having a bowel movement every 5-7 days.  Seriously?!?!  She has a freaking tube in her colon for this stuff!  Unfortunately, it is something that Ellie will have to suffer through because I really don't want to her to be at risk for a stroke from a really really really high blood pressure.  If it gets to where is going more than a week, then we will have to figure something out because colitis and bowel perforation would suck.

Anyway, that is the big blood pressure update.  My next post on The Chronicles of Ellie Bellie Bear will be fun!  Horse therapy with Jack?  Yes!  Seeing the dolphins at Sea World?  Yes!  Reuniting with friends from The Wish Connection and seeing a Tim St. John, the magician?  Absolutely!  What about Morgan's Wonderland, an amusement park designed for children and adults of ALL abilities?  What fun!

Fun Photo Flashback:




Sunday, September 16, 2018

Happy 9th Birthday, Ellie!

Can you believe it?  Nine years ago, our world changed forever. . . for the better.  Yes, we were off to a rocky beginning.  Andrew and I nervous about being new parents.  (Andrew was scared to hold her and feed her) What if we mess up?!  Yes, we will mess up and then what?!  We were trying to get over the shock that our tiny, perfect baby surprised us with her extra 21st chromosome.  We were elated.  She's perfect!  We were scared.  We had this vision on how things were going to be and then the sassy Ellie Bellie Bear (yes, she was sassy even as a newborn) threw us through a bunch of loops.

I still cannot believe that I have a 9 year-old (read: that I am old enough to have a 9 year-old even though I have the white hairs prove it).  I feel like she was just a little toddler yesterday.  This year has brought many changes.

Ellie Bear is making a ton of friends at schools and when she is on her best behavior - with kids on playscapes.  She loves to dance in her 2dance2dream class and at home to YouTube videos.  Turkey Bear enjoys her horse therapy sessions and always asks to dance with her respite care provider, Kyla.  Over the summer, there was lots of father-daughter bonding time at the swimming pool.  The Bear stills favors Mickey Mouse over all other shows, but she does like The Wiggles (who she got to meet on her Wish Connection trip), Frozen, Nina Has to Go short stories, and Daniel Tiger's Neighborhood.  Her favorite bands to dance to include: Like a Storm, Skid Row, Guns n Roses, Queen, and Aerosmith (yes, I am raising a metal head). . . and just to add in some country (a true Texan), Cole Swindell.  Her favorite playtime is pretend play with her stuffed princess dolls.

She is trying to talk!  Ellie is imitating everything we say. . . as in after I said a choice cuss word at a guy who cut me off and she repeated the second half of the word. Hint: it rhymes with "sass".  Right now, her two favorite phrases are "there is (she/he) is!" and "bye y'all!"

Ellie Bear finally gained weight after being diagnosed with Failure to Thrive for 3 years.  She also grew a lot.  Who's taking bets that she will eventually be taller than my 5'2"?  Any takers?  



Wednesday, August 29, 2018

Surviving the Storm and a Speech Explosion

I want to thank everyone for the support that I received with my last post.  It is hard to be so open and honest, but I have learned that many people have felt the same way and many people have reached out to me offering support.  When I write these posts, it is for 2 purposes - to help me process my thoughts and to also let other people know that they are not alone.  Since the post, I have had actual adult conversations that were not with my husband, mom, or doctors.  So wooohooo!

We are surviving over here.  School has started and Turkey Bear managed to miss the first day.  Because of her psych issues, she lost it at cardiology on August 1st.  The EKG was a catastrophe so we had to give up.  The echo.  Well she was really excited about the echo - just hopped right up on the table and yanked her gown open.  I kept showing her pictures of her doing the renal ultrasound and said it is like that, but on her heart.  She would say “yeah yeah” and point to her heart.  Yeah . . . so when the tech put the wand on her chest, she got angry.  Kicking.  Screaming.  Yanking the wand to her abdomen because the tech was “doing it wrong”.  My little juvenile delinquent’s behavior led to ordering the echo and a CT scan angiogram (a fancy way of saying looking at blood vessels via CT scan) under sedation.  Of course the only day they could do it was the first day of school.  She’s totally starting off the school year on the right foot.





Ellie "helping" with her renal ultrasound in May.


I believe it was her 18th or 19th time under sedation/anesthesia and sadly they remember us.  Although, mainly because I stand there and say things like “she’ll need and I&O cath due to urinary retention” and  “could you give her that propofol again before you pull her out of anesthesia because she has emergence delirum?” as I hand them a word document detailing medications, dosages, why on them, and who prescribes them.  Apparently, most parents don’t do that? Just in case, y’all are wondering, emergence delirium is when the patient comes out confused after anesthesia.  Bear is a “tad” okay, very combative afterwards.  She has no qualms about ripping her IV out with her teeth (yes, through the tape) while kicking me from her bed with nearly enough form to knock me over.  Procedure and surgery days are so much fun!

I feel like in the top 2 pics that it looks like she just walked in her room drunk and collapsed on the bed.


Happy news!  Ellie has officially been discharged from cardiology!  Happy dance.  Happy dance.  Yay!  We dropped a specialist!  

Ellie’s heart is structurally normal.  We were looking at her aorta to see if it was narrowed and that was the cause of her high blood pressure [BP] in her left arm.  It isn’t because her aorta is normal!  Her left ventricle (the left lower chamber of her heart) has normal thickness.  With high BP, the heart has to work very hard to pump and the left ventricular wall gets thick.  She does not have this! Ellie does have a right aberrant subclavian artery that is looping around the back of her esophagus causing some narrowing.  The cardiologist says she will not move the artery unless she has lots of choking on solid food episodes and that has only happened a few times where she had to make herself throw up.  Anyway, we still don’t know what is causing her high BP.


Normal position of arteries branching off the aorta of the heart.



Ellie's arteries


Of course this means we get to go to nephrology every 2 weeks until we get her BP normal.  She is one Norvasc since the clonidine caused aggression and we have since gone up on the dose.  It seems to be working.  At her well check, we will do blood work that looks at kidney function.  I am pretty sure The Bear is fine from a kidney stand point.  Who wants to bet that we will never find the reason for her high BP?  Those who say we will get to watch Ellie for an entire week while I run off to a deserted island.  

Now in other, fun exciting news, Ellie Bear is really trying to talk.  





When I first heard her say “bye y’all”, we were leaving the drop in day care and I had no idea what she was saying.  It wasn’t until I put her in the booster seat that I figured it out.  The little turkey is a true Texan.






Someone loves swimming!







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Sunday, August 12, 2018

Runaway Train: Emotionally Raw

Emotional Mess:

I like to think that sometimes it’s ok to have a pity party. To have it out and then take those deep breathes and dust yourself off. Sometimes the pity party lasts an hour, other times a week.  Sometime things suck for a while and you appear to handle it with grace and suddenly, it all comes crashing down at the most inopportune time. I mean, hey, it is totally normal to start crying in the workout section in the middle of Kohls (I’m thinking it would have gone better in the purse section, but oh well).

I am guessing you have by now figured out that I had a pity party in the middle of store and at home and in the car that lasted intermittently for a few days.  It was all an accumulation of things and then the fact that Ellie got 4 diagnoses and 4 new medications all within the span of the week.  I don’t know why the diagnoses bother me.  It is just a label for all the crap that she has been going through for a while now.  Anyway . . . 



It isn’t all medical.  Sometimes raising a developmentally delayed child is rough.  Sometimes raising a child who is delayed with a psychiatric disorder is rough. Sometimes, it is really hard to be around typical kids because then I know what “it could be like”.  

Ellie is almost 9 years-old.  Her speech is lagging way behind - at that of a 22 month-old according to her most recent speech evaluation. She has come a long way since getting her communication device (more on that in a future post), but at times, I am sad.  I want to have a conversation with her.  Hell, I can have conversations with 2 year-olds.  I want to ask Ellie what she did at school today with her not just sign “school” - essentially repeating the last word I say.  Yes, she is really starting to understand things and I am seeing a cognitive explosion, but I want to be able to do things like have tea parties at restaurants.  To have a conversation.  To have her fully potty trained (she is almost there!) and not pooping in her pull up.  I want to snuggle up on the couch and watch an actual movie with her.  Seriously, I still haven’t seen Brave or Moana or Trolls.

On top of that, Ellie’s behaviors are out of control.  For months, we haven’t found anything that worked.  The morning of her neuropsych appt was bad.  I internally screamed “I CAN’T do this anymore!  I am done!”  This of course led to more tears.




The Unquiet Mind:

Ellie has Down syndrome and that is nothing exciting.  She has multiple medical issues.  Also nothing of great excitement.  What is something.  What makes it most difficult to get by day by day is her behavior, her mood swings, her defiance.  

I always knew that she’d end up with an Axis 1 psychiatric disorder given our family history.  I just thought it was years down the road. She definitely has ADHD.  Her case is text book. She’s had it since she was 2 years-old.  We found the miracle drug of Adderall when she was 4.5 years-old.  A light switch went off.  All good things come to an end though.  She was maxed out of Adderall.  Thus began the search for new meds - for months!  We are still searching.  Medications she used to be on either didn’t work (i.e. after 2 months of stopping Risperdal and then restarting it - we saw no change in hyperactivity anymore) or they caused adverse effects (tics, uncontrolled body movements, mood swings, aggression).

I mean it is totally normal to have a Kleenex box flying by your head while in an exam room waiting for the psychiatrist because I wouldn’t give her a tongue blade.

This summer I thought we would get her regulated.  I had discussed with neuro that I was seeing signs of bipolar in addition to her ADHD and thus transferred her care to a neuropsychiatrist.  We’ve been seeing her every 6 weeks and we discovered a few things. 


The Rapid Cycler:

Ellie was on short-acting guanfacine (Tenex) for 3 years.  It is a blood pressure medication that works in the brain.  It worked great for hyperactivity.  WORKED as in past tense being the operative word.  This time, it caused massive mood swings.  Changing from minutes to minutes. Going from extremely hyperactive and singing to yelling, agitation, and hitting herself because I didn’t make her sandwich fast enough.  This is not a normal reaction.  After 3 days, I stopped it.

The Violent Child:

Ellie takes 0.2 mcg of clonidine at night for sleep.  This is another blood pressure medicine that works in the brain. It is also used for hyperactivity.  Nephrology and Neuropsych wanted to do a low dose (0.05 mcg) of clonidine in the morning for both her high BP and ADHD.  It was disastrous.  For the first 3 hours, she was very aggressive - biting, kicking, shoving my face hard, hitting herself.  Then at the peak, she would be a calm angel.  So yes, the clonidine was working once it reached peak, but we couldn’t release her in the world with her violent behaviors.

The Moody Child:
We started Ellie on Strattera for ADHD in May.  We noticed it helped with her focus.  We bumped up the dose in June, less focus but still better than without it.  I finally realized that her moodiness was caused by Strattera.  Interesting thing about this medication.  It works on norepinephrine.  Most antidepressants work with serotonin or norepinephrine - both neurotransmitters in the brain.  In people with bipolar or other mood disorders, a medication like Strattera or Zoloft without a mood stabilizer trigger the mood swings. Finally, Ellie now has the diagnoses of Neuro-developmental disorder and mood disorder with general medical condition (basically a mood disorder, but not a specified on like bipolar or borderline, etc).  We have started her on Trileptal which an anti-seizure medication that also doubles as a mood stabilizer.  Fingers crossed that this medication helps her without many adverse effects.

Coffee Therapy: Happiness in a cup


Superwoman Is A Fictional Character:

There are days where I say “I am done.  I can’t do this anymore.” and those days have lately seems to occur more frequently.  The thing is, we all know that I am not done.  It is what it is.  Ellie and I will face this together.  I am her mother.  Her caregiver and I will never give up on my child.  Ellie is my everything and I would do anything for her.  So no, I am not done and I will move forward as I always have for the past ~9 years.  This does not make me make me superwoman.  This makes me a mother who loves her child and you would be doing the same too.




 photo IMG_2291_zpsudykfi0h.jpg

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Thursday, July 12, 2018

Summer Fun and Medical Issues (secondary hypertension, coarctation of aorta, perioral dermatitis)


First, let's talk about the fun stuff!

Summer has been underway since the last week of May.  Ellie had her Wish Connection Trip at the beginning of June (see blog post to learn more about her trip and The Wish Connection) and had a blast!  It was such a wonderful experience for her and the rest of the family.

She is back to doing swim lessons.  I wanted to sign her up for 2 sessions, but the Bear has been busy with camps.  Ellie's swim instructor is incredible.  She teaches kids with special needs in the pool in her back yard.  A few years ago, the instructor and Ellie accomplished more in 1 lesson compared to the 6 lessons we had with the YMCA!



This year we signed Ellie up for horse camp in both June and and in July.  Her June camp went so well.  She had a blast and was on her best behavior. She managed to win the award for The Most Energetic Camper surprise, surprise.  However, since then, we increased her Strattera and noticed that she is less cooperative - not following directions as well and is more oppositional (the low dose helped so much with the shirt pulling, tantrums, and cooperation).  Her days are about 50/50 good/bad.  She has horse camp this week.  The first day she acted out the entire time with shirt pulling.  Tuesday, her behaviors were better, but she was crying for Daddy and fell asleep for 10 minutes. I thought that she may have been sick.  Yesterday was a great day.  On top of that, girlfriend is refusing to ride.  They put her on the horse and she screams and kicks.  She has been doing great the past 5 years so I am not sure what is going on.  She has a horse show tomorrow so we'll see if she is actually in it. Still, she is loving it and asks for the "horses and donkeys" daily.





My daughter is a Metal Head.  Ellie keeps asking to dance and grabs my phone to select songs.  She has her favorites.  She has favorites to dance to and songs that she claps when they come on the radio.  She loves Like A Storm (a modern metal band from New Zealand that uses the didgeridoo in some of their songs), Skid Row, Guns N Roses "You Could Be Mine", Aerosmith, Queen (yes, I know, not metal), and to throw a bit of country in there - Cole Swindell's "Flatliner" and The Wiggles, of course.  Hey, a girl needs well rounded musical tastes.

Dancing to "18 and Life" by Skid Row

Hypertension and Possible Heart Problem:

About a year ago, we saw nephrology due to high blood pressure, but her BPs were only high some of the time.  She got referred back this winter by cardiology as she had tachycardia (high heart rate) and her BPs were more consistency high - especially the diastolic, but not enough for a diagnosis of hypertension.  We were supposed to have a follow up in August, but we had to move up her appointment because:

1. her systolic (upper #) was climbing into the mid-120s (her diastolic normalize)
2. the blood pressure in her left arm is significantly higher than her right arm - by 9-13 mmHg

Typically, the most common causes of high blood pressure in kids are related to kidney disease and congenital heart defects.  This is called secondary hypertension.  As the childhood obesity rates rises, us health care providers are seeing more and more incidences of primary hypertension (adult hypertension).

Normal blood pressures for kids are based off age, gender, and height %tile.  For Ellie, her systolic should be closer to 112 mmHg instead of 125 mmHg.

Having one arm measure consistently more than 10 mmHg higher than the other arm suggests cardiac defects such as coarctation of the aorta or an aberrant subclavian or carotid artery (an artery that branches abnormally off the aorta).  In adults, this is usually a sign of a clogged artery.  We are wondering how long this has been going on because at some of doc visits over the past 3 years, her BP was performed on the right arm and other times on the left.  I discovered it when I was checking her a few times a week at home.
Ellie likes to do her own blood pressure after I do it.
This is the most accurate blood pressure machine that I have found  and that has the true pediatric cuff.
It is $64 on Amazon Prime and includes infant, pedi, and adult cuffs.


Coarctation of the Aorta is a cardiac defect often discovered at birth.  It is a narrowing of the aortic (that big vessel you see at the top of the heart).  Symptoms include a murmur heard on the child's back, breathing problems, easily fatigued, and blood pressure differences between the arms and the legs.  In mild cases, the child may experience no symptoms.  The problem with coarctation is that the left ventricle of the heart has too work a lot harder.  This can cause the high blood pressure and thickening of the ventricular wall.

Photo Credit: Health Jade


Ellie's nephrologist looked at the report of her January basic echo and it said normal aorta.  He contacted the cardiologist to see if we needed to do a doppler radar of the arteries or do anything.  Cardiology would like to repeat her echo.  Interestingly, typically, the abnormal artery is the right side, meaning the right blood pressure would be higher.  However, in Ellie's case, hers is the left arm.  That would be rare and I haven't been able to find any medical articles or case studies where patients had abnormal left artery. So, I am pretty sure her heart is fine and this is just another random health issue that The Bear has.

In the meantime, she has to take blood pressure medication as she officially has hypertension.  The nephrologist and Neuropsychologist  discussed and opted to start her on a morning dose of clonidine (she takes it at night for insomnia) as it helps with blood pressure and hyperactivity.  We start that this weekend - fingers crossed it helps her ADHD!


Perioral Dermatitis:

We are still dealing with Ellie's perioral dermatitis. Perioral dermatitis is a relatively uncommon dermatologic condition (~0.5-2% of the population) that occurs on the face - typically around the nose and mouth.  Women ages 25-35 are most commonly affected, especially those who wear makeup.  Steroids make it worse.  Unfortunately, Ellie takes an inhaled steroid for her asthma using a spacer and a mask.  Ellie started getting these round bumps around her chin and later her nose - all areas covered by her inhaler mask.  We tried treating is as though it was impetigo (a bacterial skin infection) with Bactroban (a prescription antibiotic ointment).  With no improvement, we switched to metronidazole (flagyl ointment), which is the primary treatment for perioral dermatitis.  Then we did hydrocortisone in case it was contact dermatitis.  I then decided to use lotramin (an anti fungal).  With the metronidazole, hydrocortisone, and lotramin, it would look like it was clearing up by day 3, but then get worse by days 5-6.  Eczema cream and her sunscreen make it worse. We tried switching her inhaled steroid from Flovent back to Qvar and that seems to have helped a bit. In May, she got referred to dermatology who is "working her in" on August 1st.  While it is not contagious and it doesn't bother her, it looks horrible.  I am thinking that perhaps she needs the rash scraped and maybe an oral antibiotic.


Perioral Dermatitis