Say What?! - Pediatric Hirsutism and Stress!

Hi y'all!  Can you all feel the stress radiating from me and hear my screams from Texas?  Just when my stress and anxiety levels start to taper down, something happens to send them skyrocketing again.  Seriously, can this kiddo have anything else?  It is like Ellie is the poster child for random, non major (thank goodness!) medical conditions that always leave health care providers stumped.  Why?!

*Please note that aside from Ellie's low thyroid, these medical conditions discussed in this blog are not related to Down syndrome.*

Hirsutism:

Yesterday was Ellie's neuropsychology appointment (more on that in a later post).  I brought to the nurse practitioner's attention abnormal hair growth - something that started a few months ago.  My thought was that it may be her Trileptal (mood stabilizer/antiseizure medication).  Yes, it isn't listed as a side effect, but. . . "it's Ellie".  This led to the other neuropsychologist evaluating her and saying it's odd followed by the Comprehensive Care doctor also saying it's odd.  All said - "I am not sure what the cause is.  This is atypical.  Perhaps it is the Trileptal, but that makes no sense.  However, this is Ellie".

Abnormal hair growth or hirsutism is not often seen in kids.  For Ellie, she has increased and darkening hair along her arms and legs as well as fine hair, also darkening along her spine. Some people refer to it as lanugo.  The Princess refused to cooperate for a picture so check out the one from a Dermatology journal below.  Often times, the hirsutism also occurs on the face along upper lip and occasionally the chin and side burn area.  Thankfully, we are not seeing that . . . yet. . . hopefully never.  The anxiety over facial hair makes me want to puke.  I don't want to shave my 9 year-old's face!  I don't know if it is transient and will eventually fall out or if it will be permanent.  Cue: Mama Bear crying, screaming, and having a heart rate of 180.

Photo Credit: Our Dermatology Online Journal

Hirsutism is most common among woman who have polycystic ovarian disease.  Ellie is too young for that and there is no family history.  Four, yes, I said FOUR, healthcare providers (including endocrine from a few months ago) and myself have concluded she is not in puberty.  Plus, the distribution of hair would be more along the lines of the usual puberty patterns - think legs, underarms, and genital region.

Other causes include long-term use of steroids such as prednisone and Cushing's Syndrome.  All of these increase one's cortisol levels.  Cushing's Disease, which can develop suddenly, is rare in children and is often the result of an adrenal gland, which sits above the kidneys, tumor which also causes increased cortisol levels.  

Testing for Cushing's Disease is a pain in the butt and not all that accurate.  One test involves analyzing cortisol levels via blood draw in the morning and at midnight.  Oh yes, midnight.  What lab is open at midnight?  A 24 hour urine collection.  Yep, you read that right - you are basically going to deliver a gallon of pee to the lab.  Also a dexamethasone-suppression test, which I don't completely understand.

Symptoms include:

-moon face/round face

-truncal obesity: excess fat along the back, abdomen

-high blood pressure

-high blood sugar

-striae/purple lines 

-hirsutism 

-absent menstrual cycle

-males: increased breast tissue

-poor wound healing

All of us have concluded that Cushing's Disease is very unlikely since her only signs are the hair growth and the high blood pressure.  The high blood pressure is also atypical in that it is in her left arm only and she has high renin levels (produced in kidney).  Ellie has her follow up with the nephrologist this afternoon so I plan to talk to him about it.

As it stands, the Comprehensive Care doctor is going to do an extensive literature review as well as consult with endocrine.  I for one would Like to have for harmonium levels drawn especially testosterone. 

Not-So-Fun Fact: hair along the spine is is slightly more common in Down syndrome than in the general population, BUT not even close to people Ellie's age.

I hope to get in a blog post soon on Ellie's strabismus surgery from the end of January as well as a psych update.  In other news, I managed to re-pinch a nerve in my neck which is causing left-side neck, head, should, and the upper back of the arm pain.  It isn't intolerable, but the headaches and shoulder get pretty bad and I can barely turn my neck to the left.  So, hello PT twice a week because I totally have all the time in the world for that.  

Thank you everyone who offered up prayers, good vibes, positive thoughts, etc after my last blog post.  I would very much appreciate if you would continue to think of Ellie Bear.

Ellie Bear Fashion Through the Years

I think it is time for us to do a fun post!  I was going through some photos the other day and came across several where Ellie was wearing various outfits as a way of expressing herself.  For people who have been following the blog for a while, do you remember the multi-headband stage?  

Her latest, which I do not have a picture of, is wearing a medical mask.  I had to explain that one to school.  Her bus aide has been wearing a mask as means to prevent catching the flu.  Of course, the Bear thinks it is a fashion statement.

I wish I had a picture of the time where she wore a nightgown, leg warmers on her arms, and fireman rain boots. . . to Top Notch.

The year of the Chewy Tubes

How sassy does she look with her hand on her hip?

Not pictured - when she pulled leg warmers over my socks

She went through a hat phase

Self Harm and Emotional Rollercoaster: Pediatric Psychiatric Illness

Things have been quite stressful and chaotic over here to the point where I feel exhausted and really need break.  To see my child struggling and not being able to help her is devastating.  I am begging you readers for any advice that you can give me - you can respond in the comments section.

I know that mental illness is something that is not often discussed and that there is a major stigma, especially when it comes to mood disorders.  I write about these issues so that people can become more aware and so they may feel like they are not the only parents out there caring for a child with mental illness.  We all need to support other as it is so easy to feel alone.

She has 8 barrettes and bows in her hair.  She is fashionable like that!

Out of all the random medical issues that Ellie has, the psychiatric stuff is the worst and most stressful.  Being developmentally delayed and mostly nonverbal makes it difficult to know what is going on in that head of hers.  A while back she was diagnosed with a "mood disorder related to general medical condition", but let's just call it what it really is - bipolar disorder.  After an extensive google search for Down syndrome and bipolar, all I found was that there is a significant decreased incidence of mood disorders within the Down syndrome population.  Yippee! There is a strong history of bipolar in my family so there was always the chance that she would have an Axis 1 disorder (depression, anxiety, mood d/o).  To be truthful, I feel like the significant adverse reaction to Vyvanse turned on the gene and triggered this brain change in her.  Add in the fact that both the  neurologist and psychiatrist have determined Ellie's case to be "complex" as she has atypical responses to the typical medications used to treat ADHD and bipolar disorder and an odd way of metabolizing medications.  I mean, of course she is complex.  That is her thing.

I think we wore her out

For the past two weeks, Ellie has been extremely emotional in the mornings.  She may wake up crying and trying to lock herself in her room (a bit of humor: the lock on her door is on the outside so that she can't lock herself in. However, she thinks by pushing in the button before shutting the door that we can't get in) or in the bathroom.  There doesn't appear to be any trigger.

After her morning medications of Adderall (ADHD stimulant) and Trileptal (mood stabilizer), her mood is more stable and her ADHD is well under control.  She takes a lower dose of Adderall late morning, but I do not believe it is a high enough dose as her hyperactivity and impulse behaviors comes back full force.  

Immediately upon getting off the bus in the afternoon, it is like a switch has flipped.  She is extremely hyper, impulsive, oppositional and agitated.  For the past few weeks, if I tell her "no" she screams, cries, and kicks me.  Occasionally, she shoves my face away and has caused me neck pain from the force.  Afternoons and evenings are the worst for her aggression, defiant behaviors, and anger, but then after getting her bedtime dose of Trileptal, she seems to do okay for the bedtime routine.

On Wednesday evening, I saw something that I have not seen before - self harming behaviors.  We were at horse therapy and we told her that she couldn't use a certain horse saddle.  She started to scream and cry while using her fists to hit her head and face and then bit her hand.  Afterwards, she wanted a strong hug from me while crying.  This is not normal behavior for her, even with her psychiatric issues.  This scared me because she could have really hurt herself. 

I have been in near daily contact with the neuropsychologist this week and we determined that she is having trouble regulating her moods and emotions (think a mixed state of both mania and depression) and then she is having rebound ADHD after the stimulants wear off.  Med changes are tricky.  We can only change one medication at a time and I have to do them on a weekend so that I can observe for side effects.  We had to change her blood pressure medication so the psych med adjustments had to be delayed.  We have discussed a few options and we now have a plan in place.  I am hopeful that we will eventually find something that will help her, but I am worried about how long it may take.

My heart breaks for her and I am asking for prayers, positive vibes, and good thoughts so that we may get through this.  I can have my Ellie Bear back.

We are hoping that this mini trampoline will help Ellie.

The Busted Knee, Pediatric Mood Disorder, and a Boyfriend

Goodness!  Last week's blog post was getting w a y  too long as that is what happens when I fail to update the blog regularly. . . that and my kiddo seems to have a ton of random stuff that keep popping up.  Anyway, I left off where I typed:

"BUSTED KNEE WHILE AT THE DOCTOR "

On the first day of winter break, Bear ended up with a busted knee.  She was having a follow up neuropsychiatrist appointment when I mentioned that she was peeing copious amounts of urine and frequently.  To the extent we put her back into pull-ups and it was soaking through them and her clothes about every hour.

The question was: UTI vs. Increased Urination secondary to medication?

Because I am a medical nerd, I researched Adderall XR and urine production.  I discovered that up to 5% of people on stimulants get urinary tract infections [UTI], but no mention of increased urination.  Consequentially, we tested Ellie for a UTI.  This kiddo usually needs to be catheterized for a urine sample. Sadly, she is relatively used to cathing due to her history of urinary retention.  Turkey Bear is strong.  Very strong.  To do a urinary catheterization, I have to hold her upper body while a nurse takes each leg.  The third nurse does the actual catheterization.  Being in a frog-legged position with pressure being pressed down on her leg while Ellie pushes up is a recipe for disaster.

Photo: limoncello.ie

After the cath, I told her to pull up her pants.  She kept falling down and crying.  Being the horrible mom that I am, I thought that she was being defiant.  I figured the crying was because she was still pissed off.  Nope, her left leg was buckling and she couldn't support her weight.  I refused to leave the clinic until the PCP assessed her.  It didn't seemed fractured, but a knee and bilateral hip X-rays were ordered.  She was finally able to stand without too much pain, but still falling.  I had to carry her around until the X-ray.  Of course, I threw out my back. . . again.  After her X-ray, she was going much better in that she was able to walk without pain or falling, but you could see her knee continue to buckled.  It was determined that she strained either the supporting ligaments or tendons.  She wore an ace bandage for a few days after which she decided it was a stylish fashioned statement and wanted one on each leg.  After a week of this, her ace bandage magically disappeared one night.

Ellie head banging to Like A Storm's "LTWYHM" aka
Love The Way You Hate Me
Like A Storm on Spotify: https://spoti.fi/2ATh0Bl

PSYCHIATRIC CARE

In addition to making her pee, the Adderall XR didn't do anything for her ADHD.  She was super hyper, impulsive and not listening to directions.  It was so bad that her teacher asked if we stopped her medication.  Given the increase urination, the doctor hypothesized that she just didn't absorb the extended release Adderall.  We decided to go back to morning Adderall and add in a booster dose.

This seemed to help.  After nearly a year of having poor concentration, Ellie was finally able to sit down and color, paint, and play with play-doh.  However, it triggered her mood disorder.  She was taking 3 hours to fall asleep most nights and that was with an increase in her sleep meds and she would wake up much earlier than usual.  Note: this is not the Adderall.  Her midday does is at 10:30am and is out of her system at 1:30pm.  Some nights, she would only take an hour (this was her norm).

Soon, she was extremely energetic and very impulsive, but she could still attend to tasks.  She has always been a big repetitive with her speech - "mama!  first school. then dance", but it was nonstop.  As in as soon as she would finish the sentence, it would start all over.  It is sort of like the typical pressured speech you would see in an adult mania.  She was also emotional and defiant.  We'd tell her no on something and she would just melt down.  The neuropsychiatrist witnessed all this and just knew the E couldn't get control of herself.

For people with mood disorders, it is very common for other psychiatric medications such as stimulants and antidepressants to trigger a mixed state (both mania and depression at once) or a mania.  This is why people are placed on mood stabilizers.  So earlier this week, we increased her Trileptal dose.  I am really hoping that this works for her.  It is so trying for us and especially for her.

Hanging out with Brandon, the co-owner for Noble Sandwich Co
Formerly Noble Pig featured on Food Network.
 We go there every Saturday morning and she will not leave until she
gets to talk to him!

Best Friend/Boyfriend

Before Winter Break, The Bear's class had a winter party which parents were invited to.  It was during this party that I discovered Ellie had a best friend. Not to mention that she and S were all lovey dovey.  They are the same age and apparently were besties last year.  His mom said that S talks about her all the time.  This warms my heart to know that Ellie is making friends!  Over break, they were both at the same drop-in daycare and apparently they went off to a corner to play. . . um, should I be worried?!   What is this canoodling that is going on?!   Nonetheless, I always worry about Ellie connecting with other kids because she is predominately nonverbal and also has her impulsive behaviors such as shirt pulling and stealing toys (and putting them away).

Pajama Thief!

Next Friday will be a fun filled day - cecostomy tube replacement, an ABR (fancy hearing screen), and an angiogram of her abdomen (to look at kidneys).  Currently, we are attempting to add on eye surgery, but it is complicated as the ophthalmologist doesn't usually have OR time on Fridays.  Fingers crossed we can get it all worked out!  I believe that this is her 17th or 18th time under anesthesia and I really want to avoid more procedures.

Updates: Happy New Year, insurance companies, psych/ADHD

I know. I know.  I said back in October that I would write about Morgan's Wonderland (an all abilities theme park) in San Antonio and Sea World.  It is partially written and then I forgot because life was busy happening. I promise though, I will get the post up hopefully soon.

I hope that you all had a great holiday and New Year!  Earlier in December, my parents came to visit and we had a nice time dining out for family dinners (Jack Allen's Kitchen and the historical Threadgills), visiting Santa, and decorating the tree.  On Christmas Eve, Bear's best friend, Jack, and his siblings came over to bake and decorate cookies.  I think that there were cookies buried under the mound of icing and entire bottle of sprinkles.

My life seems to be full of nothing but doctor's appointments and phone calls to either insurance companies or the CDS company that manages our respite care provider's paycheck.  Last week, it was hours of taking politely, but forcefully on the phone with both.  In some ways, I am happy to have a degree in health care because I know how to approach things or when something isn't right.  On the other hand, none of this is my responsibility.  

MEDICAL PROCEDURES AND INSURANCE ISSUES

Ellie was scheduled to have her cecostomy tube replacement, CT angiogram of her abdomen, and an ABR (a fancy hearing test) under sedation on January 4th.  I was so proud of myself for getting all 3 coordinated during winter break.  Nope!  Due to Ellie's hospitalization last month for RSV, adenovirus, pneumonia, asthma, and a pleural effusion, her procedures all had to be rescheduled. I also had to reschedule her pre-op surgical exam. I am so thankful that the interventional radiology nurse was able to get them all together on the same day.  That meant I had to contact GI, ENT, and nephrology to get new orders/authorizations.  Again, this should not be my responsibility.  

Of course, things could not go smoothly.  Two Fridays ago, I got the letter from her disability Medicaid that the CT scan of her abdomen was denied .  It is always on a late Friday afternoon after the offices close and on a holiday weekend. Apparently, she needed to have seen the doctor in the last 60 days and have submitted the reason for test and recent lab results (this sort of seems like common sense to me when ordering something).  I wasn't worried about seeing the doctor as we were seeing the nephrologist this past Monday.  As for the labs, it was unclear if those too needed to be within 60 days.  My concern was having to get them re-drawn.  Back in September, the Dell's lab neglected to tell me that the renal bloodwork needed to be sent to an outside lab an oh, they decided to send it to an out-of-network lab!  I didn't know this until Cigna sent me a letter about it.  The 20% out-of-network coinsurance that I would have been required to pay would have been $1,200!!!!

Ellie's particular disability Medicaid contracts with EviCare for radiology.  The number for questions and appeals on the letter was for EviCare.  That person was incompetent and told me that I am supposed to go through the Medicaid.  The Medicaid rep who was really nice, said that because it is through EviCare, they have no record of the order or denial.  She offered to call them for me.  Gah!  Fortunately, one phone call from the nephrologist (who, by the way, called the same number as me) spoke with the medical director who turned over the decision.

Ellie and Jack

WHY ISN'T MY CARE PROVIDER GETTING PAID?

I actually have no answer for that.  Ellie is approved for respite and PCS services through MDCP.  The consumer directed services company that we went with is paid $202.17 through her MDCP budget to manage K's paychecks.  After years of things going relatively well, K started getting paid for only some of her hours - 3 missing paychecks in the last 4 pay periods.  Sometimes the PCS check was missing while other times it was the respite paycheck. The team members assigned to Bear's case would not return phone calls so I had to speak with a supervisor each time. I would be told that they didn't receive the email for x timesheet.  I will tell you that is BS because they were sent in the same email.  Then their excuse of must be an attachment issue or a computer glitch. Well, I started to send them together and separately.  This last one took the cake because I resent the timesheet 4 times, spoke with a supervisor and emailed him the timesheet who then forwarded it to her two team members.  Nothing.  K still wasn't paid.  I call the team members and left another message followed by calling their direct supervisors.  So I left 4 unanswered messages.  It finally got worked out, after  I threatened to contact Health and Human Services for mismanagement of Medicaid funds.  Clearly this was unacceptable so now I am spending time on the phone this week trying to switch over companies.

MOOD DISORDER AND ADHD

Did you know that stimulants can cause UTIs (urinary tract infections) in about 5% of those who take it?  Yeah, me neither.  Ellie had been on morning Adderall for about a month and it was working great aside from the whole wearing off after 2.5 hour part!  I was so worried about the tics given the debacle 10 months ago, but it never happened.  We ended up trying Adderall XR and well, let's just say the school asked me if I stopped her ADHD medications.  At this point, she went from being almost completely potty trained to urinating every 45 minutes with complete saturation through her pull-up and clothes.  

While at neuropsych, we tested for an UTI and determined that the increased urination is the result of Adderall XR (of course, increased urination is not a known side effect of Adderall XR) and that it wasn't working because she was peeing it out.  She is now on 2 doses of short-acting Adderall a day.  It is completely out of her system by 1:00pm so it should not affect her ability to fall asleep . . . yeah, you see where this is going.  

However, she started to have her weird sleep issues of taking either 1 hour (this is her norm) to 3 hours to fall asleep.  She'll go 3 or 4 nights of not sleeping to a few nights of good rest. This is even after an increase in her sleep medications.  She is now super hyperactive and impulsive.  Now we need to determine if it is ADHD for the hyperactivity or has she has she gone into a mixed state over as a result of making stimulant medications.

You can read more about E's history of ADHD management and her Mood Disorder:

Abnormal reactions to common ADHD medications 

DNA testing for psychotropic medications

Uncontrolled movements due to Vyvanse / Adderall XR

Thinks she's awesome because she is wearing my PJs

BUST KNEE WHILE AT THE DOCTOR - sorry, y'all this post is getting long and I am getting finger cramps so I will write about this and some other fun stuff in my next post!

We are home!

Ellie was finally released from the hospital yesterday afternoon after a long 6 night stay. It wasn't until late Wednesday evening that she was able to tolerate being off the oxygen for more than 20 minutes. Well, she must have turned a corner because she didn't need to go back on oxygen since 4:30 pm that afternoon. We busted out yesterday and have been taking it easy since then.

Her lungs sound clear and her saturations are nearly normal. She has not needed an albuterol treatment at all today.  She is in great spirits with lots of playing. Our kitchen is trashed because her entire book library from her room apparently needed to be located next to the kitchen island.  So the Bear is definitely feeling better! Her pediatrician cleared her to return to school on Monday and not a moment too since since we have family arriving this week and I still have to finish shopping, wrapping presents, cleaning, etc.  

Two viruses, one bacterial infection, asthma, and a pleural effusion

So. . . we are still here. At this point, I think our resort fees should be waived as we continue to enjoy these lovely accommodations for an extended stay. I want to thank all of you for your prayers, positive thoughts, checking in on us, and offers to help out.  It is very much appreciated.

Ellie Bear was admitted to Dell Children's Medical Center back on Friday night for low oxygen saturations related to an asthma exacerbation caused by RSV and adenovirus (very fancy word for common cold).  She continues to improve, but we are having trouble getting her off the oxygen.

On Monday, Ellie spiked a 102.5 fever.  They did not do a chest x-ray, but rather wanted to see if she would repeatedly spike. I was not exactly thrilled by this since it was a new onset high fever.  The reason that they wanted to wait was to 1. not unnecessarily exposure her to radiation and 2. her lungs sounded much better and she needed less oxygen.   

Yesterday, her temperature was 101.4 so the doctors did decide to go ahead with a chest x-ray, which revealed left-sided pneumonia and a small pleural effusion (fluid between lining of the lungs).  She has been started on antibiotics for the pneumonia, but we will not drain her effusion since it is so small.  The effusion should resolve on its own.

Yesterday and so far today she has been requiring minimal oxygen support, but she continues to fail trials off it.  We did have to increase her oxygen last night while asleep, but we were able to wean down twice this morning.  However, the entire 5 minute trial off oxygen she dropped from 95% oxygen saturation to 83-85%.  Needless to say she is back on oxygen.  

Even with the new pneumonia and the pleural effusion, her lungs continue to sound good.  She is on only a little bit of oxygen and she is perkier.  Yesterday she sat in the chair for 12 hours and she didn't want to get back into bed at night.  That means she is headed in the right direction.