Archive for July, 2020

This is what life looks like for a family with serious chronic illnesses.  Each basket is filled with daily meds for one person.  All medicine is bagged individually with detailed information on each bag.  Each month, I dose medicine into weekly pill containers.  This usually takes me between 3-5 hours.  I dose the meds, rewrite everyone’s med lists on notecards for their backpacks, and then I put a med shopping list together.  Since I usually do this on the weekend or in the evening, I do the med shopping the next day.  This usually requires phone calls to various pharmacies.  Then follow up phone calls to doctor’s offices.   #notallillnessesarevisible #chdwarrior #dysautonomiaawareness


By the way, these are just the daily meds.  This doesn’t include the occasional medication.  And these baskets have been a lot leaner and also a lot fuller.  Medication is an everchanging subject.


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And not to leave our youngest medical mystery out, we have Miss A. our youngest child.  Several years ago, Ainsley had a seizure and quit breathing after receiving a flu shot (the whole episode was NOT due to the vaccine itself). Luckily we were at our local pharmacy and the pharmacist (also owner)’s  wife, a nurse, and Chris were there. Chris gave Ainsley mouth to mouth, and after a few minutes they were able to bring Ainsley back to consciousness. While we waited for the paramedics to arrive, they were able to do their medical magic to check blood pressure, sugar levels, etc.  Checking to see if there was any obvious reason for Ainsley’s seizure.  Nothing was obvious.  Because of Ben’s heart condition, we took this seriously and had her checked out.  Nothing.  Probably just a vasovagal event and she actually didn’t have a seizure.  After all, I had never seen one before, maybe I didn’t know what I was seeing.

Fast forward another six months. Ainsley has another one while at school.  No one saw her.  She didn’t tell anyone.  She just went to the nurse and called me.  She was crying because she knew she had one and didn’t know what to do.  (Details are being left out to protect the innocent). We took her in to the ER to have an EKG run as close to the actual event as possible. Nothing. Normal.

Two months later. We are at the pediatricians office.  Ainsley has to have a routine blood draw.  She is laying down, but when the nurse first missed the vein and started to fish, Ainsley looked at me, said, “Bye, mom”, and went into a tonic-clonic seizure for over two minutes.  Luckily, it was at the doctor’s office, and our pediatrician saw it.  She was able to verify that it was a seizure.  She had timed it and said that we needed to up our game to take her into a neurologist.

So, in two years, Ainsley had a total of four seizures that we know of.

Saw the neurologist, had a ton of testing done, and the doctor diagnosed A with epilepsy.

The neurologist started Ainsley on seizure medication.   When it didn’t agree with her, we went back and happened to see a different doctor in the same clinic.  He felt that it wasn’t epilepsy, but dysautonomia (Ben had seen this same doctor before, so the doctor knew his health history).  They had just hired a dysautonomia specialist and we went to go see him.

This doctor did great things for Ainsley.  Diagnosed her with POTS, Ehler-Danhloe Syndrome, Mast Cell Activation, and others issues.  Regardless of the diagnosis, the treatments helped Ainsley tremendously.  She didn’t have any more seizures.

But . . . .

this doctor left the clinic.  The other doctors didn’t want to take our cases.  And after years of trying, I was able to get all three kids into Dr. Butler and the dysautonomia clinic at UT-Medical Center.  It was a miracle.

The first things that Dr. Butler ordered was a tilt-table test.  None of the kids had had one.  This test is a standard in the dysautonomia world.

Ainsley was the first kid up for the test (out of all three).  We didn’t think anything of it.  It is not invasive (which is why we said yes).  The cardiologist who partners with Dr. Butler is in the room and monitors everything.

And then of course it happened . . . 7 minutes into a 45 minute test and Ainsley passes out and has a seizure.  Again. I expected her to have one, after all, she had been diagnosed with dysautonomia already.

What I wasn’t expecting was the doctor’s report to us.

His diagnosis for Ainsley is severe dysautonomia.  Ainsley actually flatlined on the table for 13 seconds while the doctors moved the table back to a horizontal position after she started her seizure.  Her heart stopped beating.  Seriously.  I was in shock.  My actual question to the doctor was, “Is that bad?”  That was my question.  (Oh, the answer was yes.)

He also said that she didn’t have an epileptic seizure.  She had seizure like activity.  Meaning . . . instead of her brain misfiring causing a seizure to occur. It was different.  Her blood dropped to her feet.  She lost 60% of her blood volume in her brain and chest.  It went straight to her legs and feet.  So her heart stopped beating because there was not enough blood to pump (no gas in the tank).  Her brain had a seizure because there was not enough oxygen in her brain, so the brain just turned the light switch off (causing a seizure).  All because she went from laying down to standing.

Her body’s autonomic nervous system, which tells your body to do things like breathing without you having to actually think about it, does not work properly.  The doctor said that out of all of his dysautonomia patients with POTS (and he said he over 1500) only about 80 have as serious of a case as Ainsley.

So long story short (and we have certainly skipped over a ton), Ainsley has been added to the list of medical anomalies in the Lake house.  She is rather proud of this one.  (When her brother had his tilt-table test and he made it through the whole test without passing out. She said, “I won!”  Really, kid. This isn’t a contest.)

We are dealing with this diagnosis.  I’m glad we figured it out before she started driving.  That could have been dangerous. She won’t be eligible to drive until she is closer to 17.  She is the master of her own health, and has is really working on being healthier.  She has many other health issues that we have also been dealing with, but none are as dramatic as this one.  She is a trooper and I’m very proud of her!







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As of today, July 4, 2020, Ben is NOT currently in the hospital.  Thank you, God! I posted a picture of Ben and since I rarely post updates on Ben in the last several years, I created a lot of confusion.  Sorry about that.

Here is the current update:

  1.  Ben’s heart is stable.  Not better. Not worse.  Stable.  That is important.  After his last procedure in the Cath lab in 2016 where a Melody Valve was placed in Ben to help with a pulmonary stenosis had been getting worse.  The pulmonary stenosis started after the Ross-Kono procedure in 2015.  Inflammation was getting worse in Ben’s pulmonary valve where Ben’s body met the donated cadaver valve.   So the Melody Valve was placed.  The inflammation has returned, but . . . it is yet at a point that requires surgical intervention.  He is on the bubble.  So no better. no worse. Stable.
  2. Ben’s kidneys are currently in Stage 2 renal failure.  This sounds worse that it is.  His kidneys were damaged during the last two open heart surgeries, which can happen when you are placed on a bypass machine.  His kidney are currently working between 70-80%.  This will NOT get better.  Over time and has he ages, it will get worse and to the point of dialysis.  Hopefully not for a long, long, long time.
  3. Because of the kidney damage, Ben has chronic high blood pressure.  He has had this since he was six years old.  We all know what high blood pressure does to a person’s body over time.  He is medicated.  The medicine serves two purposes.  It helps control his blood pressure and it helps take some of the pressure off of his heart to work as hard.
  4. Ben has dysautonomia.  Specifically, he has POTS,  Postural orthostatic tachycardia syndrome.  After Ben’s last open heart surgery, he began to experience VERY unusual symptoms and was having a ton of difficulty recovering.  Ben has always had unusual health issues and complications.  But after his last difficult surgery, the doctors began to pay more attention.  After seeing a ton of different specialists over the last five years, he has finally been able to get into the renowned Dysautonomia Clinic in Houston with Dr. Ian Butler.  This diagnosis has been a life saver.  Ben has missed so much school because of unusual health symptoms that were debilitating causing him to go on homebound for several years.  Last year, he first was able to get into one Dysautonomia specialist, Dr. Burkholter. This doctor did miracles.  He started Ben on a variety of new supplements, exercises, and IV infusions that kept Ben in school for a full year! But this doctor left his clinic less than a year after arriving, the rest of the doctors at that clinic refused to see Ben as a patient because he was “too complex”.  And then after years of trying to get into Dr. Butler, we succeeded.
  5. Ben has also been diagnosed with motility problems in his GI tract. He has had heart burn since he was born.  It become increasingly problematic after Ben started kindergarten.  He would just get up and throw up in the trash can after he would eat anything, and then go back to his smiling self.  The school didn’t like that.  I wonder why? ;).  The end result of this ten year saga of trying to figure out why he would go through periods in his life of throwing up constantly was a diagnosis of motility issues due to Dysautonomia.  He would periodically get gastroparesis after suffering from a stomach virus.  Ben does have damage to his esophageal lining due to the chronic heart burn.  Ben has been able to wean off of one of his stomach medications in the last year.  Mostly due to changes in his diet.
  6. Ben has also been diagnosed with dyslexia, ADD,  and other learning disabilities (mostly short term memory loss).  So he has difficultly in school.  He is unmedicated for this because of his heart.  Some of the diagnoses, like ADD, could be caused by the dysatuonomia.  Dysautonomia and specifically POTS, can cause a major decrease of blood flow to the brain.  In particular, Ben’s body is more sensitive to this issue.  It is very complicated and I’m glad we have a doctor to help.

Those are the main issues. The main ones.  Not the only ones.

Ben currently sees 10 different specialist through the year. Ten.  10.  That is every finger on both hands.  Think about the impact that makes on our family.

These are the following phrases that we OFTEN hear about Ben from doctors:

  • Interesting.
  • Conundrum.
  • I have no idea.
  • I once read a study about this.
  • He is a puzzle.
  • He is a bit of mystery.
  • I don’t know.
  • There is nothing I can do.
  • Let’s run more tests.
  • He’s too complicated.  I can’t help him.
  • Rarer but still have heard:
    • Come back to me when you are ready to do surgery.  Until then, I’ll just keep refilling his medicine.  You don’t have to see me any more.
    • You have reached the end of western medicine. Have you tried some Eastern therapies?

Having said that . . . we have had other doctors who have said:

  • We are going to get to the bottom of this.  No one should have to go through life feeling this way.
  • I have a 100 tools in my tool box.  If this doesn’t work, we will try something else. We will keep at it until we find one that works.
  • I was thinking about Ben last night when I was going to bed and I had an idea.  So I got up and I’ve been reading a ton of medical literature about ____.  I think this might be Ben.  Let’s get you into a doctor that specializes in this and see what she says.  I’ve already called her and talked to her about Ben’s case.

These doctor’s are rare gems.  I love each and every one of them.  They give Chris and I hope when we had none.  (By the way, the last comment is from our pediatrician.  She is the absolute best doctor in the entire world.  She has been seeing the kids since we moved here, and she is the best team leader for Ben that I could have ever asked for. She has all of his doctor’s cell phone numbers and isn’t afraid to call them.)

As soon as we have been able to cobble together a great team of specialist who have managed to work together, Ben starts to age out of the pediatric world.  Scary.  Terrifying.

In the last two years, Ben has started seeing a transition team at Texas Children’s Hospital.  This includes seeing a nurse and a social worker.  This team helps Ben to transition to adult doctors and also helps Ben to learn how to manage his own health.  Like learning about his medications (names, dosages, and why he takes them).  Being able to explain to someone else what his medical issues are.  Making his own appointments. etc. etc.

Ben’s health is in a constant state flux.  Things change all of the time.  So until the next change this is the current state of Ben’s health in a shortened version!

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